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Volume 7 / Issue 5 May 2012 in the Neonate By P. Syamasundar Rao, MD and Srilatha defects is also similar at 1.5%.17 With the IN THIS ISSUE Alapati, MD known prevalence of congenital heart defects of 0.8% of live births, it is estimated that tricuspid Tricuspid Atresia in the Neonate atresia occurs approximately 1 in 10,000 live by P. Syamasundar Rao, MD and births.16 There is not a gender preponderance for Srilatha Alapati, MD Introduction Page 1 tricuspid atresia, but male preponderance appears In the previous issues of Today, we to be present in tricuspid atresia patients with as- DEPARTMENTS discussed general topics of congenital heart dis- sociated transposition of the great arteries: male to ease in the neonate,1-5 but began addressing indi- female ratio was 2:1.16,18 Medical News, Products & vidual cardiac lesions6-8 recently. In this issue, Information tricuspid atresia will be discussed. In this paper, we will discuss: classification, anat- Page 13 omic, physiologic and clinical features, non- Tricuspid Atresia invasive evaluation, differential diagnosis, man- Global Neonatology Today agement and prognosis of tricuspid atresia in the Monthly Column Page 14 Tricuspid atresia is a cyanotic, congenital cardiac neonate. anomaly and is defined as congenital absence or agenesis of the morphologic .9,10 It is Classification Upcoming Medical Meetings the third most common cyanotic congenital heart (See website for additional meetings) defect and is the most common cause of Tricuspid atresia may be classified on the basis 19 Annual International Neonatal with left ventricular hypertrophy. Whereas there is of valve morphology, appearance of pulmonary Conference a controversy with regard to terminology Jun. 14-16, 2012; (tricuspid atresia, univentricular heart or Billingham, Teesside Valley, UK univentricular connection), the authors are www.neonatalconference.co.uk/home.html of the opinion that the term “tricuspid atresia” is the correct and logical term to 11th International Fetal Heart describe this well-characterized pathologic and clinical entity; the reasons are detailed Symposium 10-12 May 31-Jun. 2, 2012; Monte Carlo elsewhere. www.gyn2012.com/ A thorough review by Rashkind13 indicates that the first documented case of tricuspid neoFORUM 2012 14 June 7-8, 2012; Morristwon, NJ USA atresia was that of Kreysig in 1817, al- www.mananewborn.com though the 1812 report by the editors of London Medical Review15 appears to fit the Annual International Neonatal description of tricuspid atresia. Conference Figure 1. Diagrammatic portrayal of anatomic types of The true prevalence of tricuspid atresia is June 14-16, 2012; Billingham, Teesside tricuspid atresia based on the morphology of the atretic not known. Extensive review of the litera- Valley, UK tricuspid valve. a. muscular type, b. membranous type, ture revealed an autopsy prevalence rate www.neonatalconference.co.uk/home.html c. valvular type, d. Ebstein’s type, e. atrioventricular canal of 2.9% and a clinical prevalence rate of type, and f. unguarded valve with muscular shelf. For the 1.4% among subjects with congenital heart sake of simplicity, great vessels are not shown. Also note NEONATOLOGY TODAY disease.16 The clinical prevalence of tricus- © 2012 by Neonatology Today that no ventricular septal defects are shown. LA, left ; ISSN: 1932-7129 (print); 1932-7137 (online). pid atresia in neonates with congenital Published monthly. All rights reserved. LV, left ; RA, right atrium; RV right ventricle. Corporate Offices: 8100 Leaward Way, Nehalem, OR 97131 USA Mailing Address: PO Box 444 Manzanita, OR 97131 USA NEONATOLOGY TODAY

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Neonatology Today (NT) is a monthly newsletter for Neonatologists and Perina- tologists that provides timely news and information regarding the care of newborns Do you have interesting research results, observations, human and the diagnosis and treatment of prema- interest stories, reports of meetings, etc. to share? ture and/or sick infants.

Statements or opinions expressed in Neo- Submit your manuscript to: [email protected] natology Today reflect the views of the authors and sponsors, and are not neces- sarily the views of Neonatology Today. vascular markings on chest X-ray,20 and asso- tum, i.e., intact, small or large ventricular septal ciated cardiac defects.9,21-24 defect (VSD), or multiple VSD’s and other asso- ciated malformations should be described. If Looking at the morphology of the atretic tricus- one wants to follow the terminology of congeni- pid valve (Figure 1), it may be classified into tal heart disease proposed and re-emphasized muscular, membranous, valvular, Ebstein’s, by Van Praagh,30 one could include the remain- unguarded with muscular shelf and atrioven- ing segmental subsets, namely visceroatrial tricular canal types.19,24-26 The muscular type, situs and ventricular loop. Each case could be constituting 89% of cases,24,26 is the most described by notations {S,D,S}, {S,D,D}, {S,D,L} common type; the remaining types account for and so on as the case may be.30,31 11% of cases. Pathologic Anatomy A classification based solely on the x-ray ap- pearance of pulmonary vascular markings was The most common type of tricuspid atresia, put forward by Astley:20 Group A. Decreased muscular variety, is characterized by a dimple pulmonary vascular markings, and Group B. or a localized fibrous thickening in the floor of Increased pulmonary vascular markings. Dick the right atrium (Figure 2) at the expected site of and his associates18 added another group to the tricuspid valve23 and constitutes 89% of the Astley’s classification: Group C. Transition from cases.24,26 No valvar material can be identified increased to decreased pulmonary vascular either by gross or microscopic examination.23 markings in serial chest films. The above three Other anatomic types, namely, membranous classifications have clinical value but a classifi- type (6.6%) with the atrioventricular portion of Figure 2. Heart Specimen of a patient with cation based on associated cardiac defects the membranous septum forming the floor of the muscular type of tricuspid atresia; the right appears to be more useful clinically.9,24 right atrium,32,33 valvular type (1%) with minute atrium is opened by cutting through the right valve cusps which are fused,23,34,35 Ebstein’s atrial appendage (RAA). Note dimple (arrow) in A classification based on great artery inter- type (2.6%) with Ebstein’s deformity of the tri- the floor of the right atrium with muscle fibers relationship was first proposed by Kühne in cuspid valve leaflets with fusion of the valve radiating around it. An (ASD) 1906.21 The classification was later refined by leaflets,18,32,36 common atrioventricular canal is also shown. The impression that one gets Edwards and Burchell22 and by Keith, Rowe, type (0.2%) in which a leaflet of the common from the literature is that this dimple is present and Vlad.23,27,28 Because of some apparent atrioventricular canal completely seals off the in most cases of tricuspid atresia. Careful inconsistencies in sub grouping and the need only entry into the right ventricle,25,37 and un- inspection of the heart specimen by several for inclusion of all variations in great artery guarded type (0.6%) with muscular shelf38 have investigators suggests that this dimple is seen anatomy, we proposed a new, unified classifi- also been described and are diagrammatically in only 29 to 83% of muscular type of tricuspid cation,9,29 and this is listed in Table I. First, the portrayed in Figure 1. For further details of valve atresia cases. tricuspid atresia is classified into four major morphology types of tricuspid atresia the reader types based on the great artery relationship. is referred to our previous reviews.24,26 is clearly a morphologic left ventricle with only Each type is identified with a Roman numeral: occasional abnormalities;32 however, it is en- Type I – Normally related great arteries, Type II With tricuspid atresia, the right atrium is usually larged and hypertrophied. – D-transposition of the great arteries, Type III enlarged and its wall thickened and hypertro- – Malpositions of the great arteries other than phied. The interatrial communication, which is The VSD may be large, small or non-existent D-transposition, and Type IV – Truncus arterio- necessary for survival, is usually a stretched (intact ventricular septum), or multiple VSDs sus. The type III is again subdivided into sev- patent ; sometimes an ostium may be present. When present, it may be: eral subtypes (see Table I) and is identified secundum atrial septal defect and rarely an a) conoventricular or perimembranous with an Arabic number (1 thru 5). Each type ostium primum atrial septal defect may be pre- (located inferior to the septal band), and subtype are further divided into subgroups sent. Occasionally the interatrial communication b) conal septal malalignment VSD (located on the basis of pulmonary arteries; each sub- is obstructive and may form an aneurysm of the in between the anterosuperior and group is indicated by a lower case letter: Sub- fossa ovalis causing obstruction to the mitral posteroinferior limbs of septal band), group a – , b – Pulmonary flow. The left atrium is enlarged and may be c) muscular (located inferiorly when stenosis or hypoplasia, and c – Normal pulmo- more so if the pulmonary blood flow is in- compared to a and b), and nary arteries (no pulmonary stenosis).9,24,26,29 creased. The is morphologically a d) atrioventricular canal type.31 Once a patient with tricuspid atresia is thus mitral valve, usually bicuspid, but its orifice is classified, the status of the interventricular sep- large and rarely incompetent. The left ventricle In the author’s experience, muscular VSDs are most common.39-42 Table 1. A Unified Classification of Tricuspid AtAtresia Also, most of these VSDs are restrictive and Type I Normally related great arteries Each Type and Subtype are divided produce sub-pulmonary stenosis in the Type I Type II D-transposition of the great arteries Subgroup a. Pulmonary atresia patients and subaortic stenosis in the Type II patients.39-47 Type III Malpositions of the great arteries other than Subgroup b. Pulmonary stenosis or D-transposition hypoplasia The right ventricle is small and hypoplastic; • Subtype 1. L-transposition of the great arteries Subgroup c. Normal pulmonary even the largest of the right ventricles that are Subtype 2. Double outlet right ventricle arteries (no pulmonary stenosis) • present in patients with large VSDs and/or • Subtype 3. Double outlet left ventricle • Subtype 4. D-malposition of the great arteries (anatomically transposition of the great arteries are smaller corrected malposition) than normal. It may be extremely small so that • Subtype 5. L-malposition of the great arteries (anatomically it may escape detection on gross examination corrected malposition) of the specimen as in Type Ia cases. It can be Type IV Persistent truncus arteriosus identified at the right upper aspect of the ven- Reproduced from Rao9 with permission n tricular mass. On occasion, it can be identified

NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 3 only on microscopic examination.22,23 However, In Type I (normally related great arteries) pa- In Type II (D-transposition of the great arteries) in most cases the ventricle is a true right ven- tients with intact ventricular septum and/or pul- patients, the pulmonary blood flow is directly tricle32,48 consisting of: monary atresia (Type Ia) and Type II (transposi- derived from the left ventricle. The systemic a) a sharply demarcated infundibulum with tion of the great arteries) patients with pulmo- blood flow is via the VSD and the right ventri- septal and parietal bands and nary atresia (Type IIa), the pulmonary blood flow cle. In Type III, Subtype 1 with L-transposition b) a sinus with trabeculae which communi- must be derived entirely through the ductus. cates with the left ventricle via a VSD. Since the ductus is carrying only the pulmonary blood flow, representing 8 to 10% of the com- The inflow region of the right ventricle, by defi- bined ventricular output in contradistinction to nition, is absent; although papillary muscles 66% in the normal fetus,1,51 the ductus arterio- may be present occasionally.28 sus is likely to be smaller than normal. This and the acute angulation of the ductus at its aortic The relative position of the great vessels is origin because of reversal of direction of ductal quite variable and has been the basis for clas- flow may render the ductus less responsive to sification of this anomaly, which has been de- the usual post-natal stimuli.51 scribed above. The ascending aorta may be normal in size or large. Pulmonary outflow In Type I patients with VSD, the amount of obstruction may be either subvalvar or valvar anterograde blood flow from the left ventricle in patients with transposition of the great arter- through the VSD into the right ventricle, the ies, while in patients with normally related great , and ductus arteriosus versus arteries the pulmonary obstruction is often at the amount of blood flow retrograde from the the VSD level In a few cases, subvalvar pul- aorta to the ductus arteriosus varies with size monary stenosis, narrow tract of the hypoplas- of the VSD. The larger the VSD, the greater is tic right ventricle and, rarely, valvar pulmonary the quantity of anterograde ductal flow. stenosis may also be responsible for pulmo- nary outflow tract obstruction. With pulmonary In Type I patients with a small or no VSD, most atresia, either a or of the left ventricular blood is ejected into the aortopulmonary collateral vessels may be pre- aorta which is then carried to the entire body sent. including the placenta and lower part of the body. Thus, the aortic isthmus carries a larger Figure 3. Box diagram of tricuspid atresia. The A large number of additional abnormalities may proportion of ventricular output than normal; systemic venous return cannot exit into the right be present in 30% of tricuspid atresia presumably, this is the reason for the rarity of ventricle and its egress has to be into the left patients.49,50 Significant among these are per- aortic coarctation in these subgroups of tricuspid atrium (LA) via a patent foramen ovale or an sistent left superior vena cava and coarctation atresia patients. In Type II (transposition) pa- atrial septal defect (ASD). Then, the blood of the aorta; the latter is much more common tients without significant pulmonary stenosis, traverses into the left ventricle (LV) and aorta in Type II (transposition) patients. The possible because the VSD is usually smaller than the (Ao) and body. See Figure 4 for further de- physiologic reason for the latter is discussed in ring,52 a larger proportion of scription of blood flow in tricuspid atresia. RA, the next section. blood traverses the pulmonary artery and duc- right atrium, PA, pulmonary artery. tus arteriosus, and therefore, the aortic isthmus Pathophysiology flow decreases, thus accounting for higher inci- dence of aortic coarctation and aortic arch Prenatal Circulation anomalies seen with these types of tricuspid atresia. Tricuspid atresia is not detrimental to normal fetal development. In a normally formed fetus, Postnatal Circulation the highly saturated inferior vena caval blood is preferentially shunted into the left atrium via An obligatory right-to-left shunt occurs at the the patent foramen ovale, and from there into atrial level in all types and subtypes of tricuspid the left ventricle and aorta. The superior vena atresia (Figure 3). Usually, this shunting is caval blood containing desaturated blood is through a patent foramen ovale, but on occa- directed towards the tricuspid valve and right sion, secundum or primum atrial septal defects ventricle, and from there into the pulmonary may be present. Thus, the systemic and coro- arteries, ductus arteriosus, and descending nary venous blood mixes with pulmonary ve- aorta. Thus, in a normal fetus, the head, nous return in the left atrium. These mixed pul- heart and upper extremities are supplied with monary, coronary and systemic venous returns Figure 4. Box diagrams of tricuspid atresia: left blood at higher PO2 while the lungs, the lower enter the left ventricle. panel with ventricular septal defect (VSD) and part of the body, and placenta are perfused by the right panel with patent ductus arteriosus blood with lower PO2. In tricuspid atresia, In Type I (normally-related great arteries) pa- (PDA). As seen in Figure 3, the right atrial (RA) both vena caval streams have to be shunted tients with a VSD, left-to-right ventricular shunt blood exits into the left atrium (LA), mixes with across the foramen ovale into the left atrium occurs, thus perfusing the lungs (Figure 4, left pulmonary venous return then into the left and left ventricle. Therefore, the PO2 differen- panel). In the absence ofa VSD, the pulmonary ventricle (LV). In the presence of a VSD (left tial to various parts of the body that is nor- circulation is derived either via a patent ductus panel), shunting from the left to right (RV) mally present does not exist. Whether this arteriosus (Figure 4, right panel) or through ventricle takes place, thus perfusing the higher PO2 to the lungs influences the pulmo- broncho-pulmonary or persistent embryonic pulmonary artery (PA) and lungs. In the ab- nary arteriolar smooth muscle development or aortopulmonary collateral vessels. The pres- sence of VSD, but with PDA (right panel) left to not, is not known.51 The lower than normal ence of either a VSD or other means of blood right shunting occurs at the ductal level perfus- PO2 to the brain and upper part of the body supply to the lungs is crucial for the patient’s ing the PA and lungs. The presence of VSD, does not seem to impair their development, at survival. The aortic blood flow is derived directly PDA or aorto-pulmonary collateral vessels is least as observed clinically. from the left ventricle. critical for patient survival.

4 NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 of the great arteries, the atretic morphologic VSD level.39-42,45,46 When the VSD is large and nosis, hypoxemia, and acidosis. An infant tricuspid valve is a left-sided atrioventricular non-restrictive and the pulmonary valve non- with markedly increased pulmonary flow does valve and, therefore, in a physiological sense, stenotic, the pulmonary flow is proportional to not have significant cyanosis, but usually pre- it behaves as mitral (left or pulmonary venous the pulmonary-to-systemic vascular resistance sents with signs of heart failure. Although atrial) obstruction. In other Type III and Type ratio. When a systemic-to-pulmonary artery there is some overlap, patients with de- IV53 patients, the systemic and pulmonary shunt has been performed, the pulmonary blood creased pulmonary flow usually belong to blood flows are determined by the size of the flow is proportional to the size of the shunt. Type I (normally related great arteries) and VSD and other associated defects. those with increased pulmonary blood flow Left Ventricular Volume Overloading. Be- are usually Type II (transposition of the great Other Physiologic Principles cause the entire systemic, coronary, and pul- arteries) and occasionally Type Ic. Approxi- monary venous blood returns are pumped by mately one-half of the patients with tricuspid Arterial Desaturation. Because of complete the left ventricle, the left ventricle has a greater atresia manifest symptoms on the first day of admixture of the systemic, coronary, and pul- volume overload than that in the normal heart. life and 80% would be symptomatic by the monary venous blood returns in the left atrium This volume overloading is further increased if end of the first month of life.18,59 Two modes and left ventricle, systemic arterial desaturation the Qp:Qs is high either because of mild or no of presentation are recognized: is always present. The oxygen saturation is obstruction to pulmonary blood flow or because 1. Decreased pulmonary blood flow, and proportional to the magnitude of the pulmonary of large surgical shunts, either of which may 2. Increased pulmonary blood flow. blood flow.51,54 The data from our collection of result in heart failure. Normal left ventricular patients are plotted in Figure 5; the pulmonary- function is critical for successful Fontan-type of Decreased Pulmonary Blood Flow to-systemic blood flow ratio (Qp:Qs) which procedure and should be maintained within represents the pulmonary blood flow has a normal range. Several studies have shown that Infants with pulmonary oligemia present with curvilinear relationship with the arterial oxygen the left ventricular function tends to decrease symptoms of cyanosis within the first few days saturation. A Qp:Qs of 1.5 to 2.5 appears to with increasing age, Qp:Qs, and arterial of life; more severe the pulmonary oligemia, result in an adequate oxygen saturation.54 desaturation.55-57 the earlier is the clinical presentation. These Further increase in Qp:Qs does not result in hypoxemic infants may have hyperpnea and better oxygen saturation, but may subject the Size of the Interatrial Communication. The acidosis if the pulmonary blood flow is mark- left ventricle to larger volume overloading and, interatrial communication is usually a patent edly decreased. The majority of these infants therefore is not advisable.54 foramen ovale. Because of the obligatory shunt- belong to Type Ib. Patients with pulmonary ing, this fetal pathway persists in the postnatal atresia (Subgroup a) irrespective of the type Pulmonary Blood Flow. The magnitude of period; this is in part related to low left atrial will also present with early cyanosis, especially pulmonary blood flow in an unoperated patient pressure. However, the entire systemic venous when the ductus begins to close. Hypoxic is dependent upon the degree of obstruction of return must pass through the patent foramen spells are not common in the neonate although the pulmonary outflow tract and patency of the ovale and consequently, interatrial obstruction is the spells can occur later in infancy. ductus arteriosus. The pulmonary outflow ob- expected, but very few patients with tricuspid struction is valvar or sub-valvar in Type II pa- atresia have clinically significant obstruction.18 Physical examination reveals central cyano- tients, and valvar, subvalvar, or at VSD level in The right-to-left shunt occurs in late atrial dias- sis, or hyperpnea, normal pulses, Type I patients. In our own experience with tole with augmentation during atrial systole ('a' prominent 'a' wave in the jugular venous pulse several series of tricuspid atresia, we found the wave).54,58 A mean interatrial pressure differ- (if there is significant interatrial obstruction), obstruction to be located most commonly at the ence greater than 5 mmHg is usually indicative and no hepatic enlargement (presystolic he- of interatrial obstruction. A tall 'a' wave in the patic pulsations may be felt if there is severe right atrium is also suggestive of interatrial ob- interatrial obstruction). Quiet precordium and struction. absence of thrills is usual. The second heart sound is usually single. A holosystolic type of Changing Hemodynamics. As the infant with murmur, suggestive of VSD may be heard at tricuspid atresia grows and develops, several the left lower or mid sternal border. No diastolic changes may take place. Closure of the ductus murmurs are heard. In patients with associated arteriosus occurring in the early neonatal period pulmonary atresia, no murmurs are usually may result in severe hypoxemia. The size of the heard, although in an occasional patient, a interatrial communication may diminish either in continuous murmur of patent ductus arteriosus absolute terms or relative to the volume of the may be heard. Clinical signs of congestive systemic venous return and cause systemic heart failure are notably absent. venous congestion and may require atrial sep- tostomy. The ventricular septal defect may close Increased Pulmonary Flow spontaneously,39-47 causing pulmonary oligemia Figure 5. The systemic arterial saturations (left and hypoxemia in Type I patients and subaortic Infants with pulmonary plethora usually present ventricular [LV] or aortic [Ao]) in patients with obstruction in Type II patients. Such VSD clo- with signs of heart failure within the first few tricuspid atresia are plotted against the sures occur over a period of months and years weeks of life, although, an occasional infant respective pulmonary to systemic blood flow and are not germane to our discussion of tricus- may present within the first week of life.17 ratios (Qp:Qs). Both Type I and Type II pid atresia in neonates. The reader is referred to They are only minimally cyanotic, but mani- anatomy are included. Note curvilinear other publications26,41,42,46 for further discussion fest symptoms of dyspnea, fatigue, difficulty to relationship between two parameters. At low of this subject. feed, and marked perspiration. Recurrent Qp:Qs levels, a slight increase in Qp:Qs respiratory tract infection and failure to thrive produces large increases in systemic O2 Clinical Features is another mode of presentation. The majority saturation, whereas at higher Qp:Qs further of these patients belong to Type IIc, although increase does not produce significant increase The magnitude of pulmonary blood flow is the a small number of patients may be of Type Ic. in O2 saturation. Ideal Qp:Qs appears to be major determinant of clinical features in tri- The association of aortic coarctation with between 1.5 to 2.5, giving O2 saturations in low cuspid atresia. An infant with markedly de- Type II patients has already been mentioned 80’s. Aortic saturations are marked as solid creased pulmonary blood flow will present and coarctation, when present, makes them circles and LV saturations as open circles. early in the neonatal period with severe cya- vulnerable to early cardiac failure.

NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 5 Figure 7. Twelve lead electrocardiogram showing abnormal, superiorly-oriented mean QRS vector in frontal plane (-45°, ), left ventricular hypertrophy, diminished anterior (R waves in leads V1 Figure 6. Chest roentgenograms (Posteroanterior views) of babies with tricuspid atresia showing and V2) and rightward (S waves in leads enlarged cardiac size and increased pulmonary vascular markings (A); during follow-up, the size of the V5 and V6) forces. Prominent P waves, heart diminished and the pulmonary vascular markings decreased (B & C). Echocardiographic and indicative of biatrial enlargement are also angiographic studies demonstrated progressive decrease in the size of the ventricular septal defect. seen in several leads. This electrocardiogram is highly suggestive of tricuspid atresia. Examination reveals tachypnea, tachycardia, L-transposition may be seen in association decreased femoral pulses (when associated with or confused with tricuspid atresia.63 related great arteries) while only less than 50% with aortic coarctation but without significant- of patients with Type II and Type III anatomy sized patent ductus arteriosus), minimal cya- The greatest use of the chest roentgenogram show such a typical electrocardiographic nosis, prominent neck vein pulsations and is its ability to categorize babies into those with pattern.66 Normal (0 to +90°) or right axis de- hepatomegaly. Prominent 'a' waves in jugular decreased pulmonary vascular markings and viation is present in a minority of patients and veins and/or presystolic hepatic pulsations into those with increased pulmonary vascular most of these patients belong to Type II or III may be observed with associated interatrial markings. Often, this is all that is necessary to anatomy. It has been suggested that the ASV obstruction. The precordial impulses are in- make a correct diagnosis once a history, may be related to destructive lesions in the left creased and hyperdynamic. The second heart physical examination, and electrocardiogram anterior bundle,64 fibrosis of left bundle branch,67 sound may be single or split. A holosystolic have been obtained.63 abnormal distribution of the conduction system murmur of VSD is usually heard at the left (unusually long right bundle branch and origin of lower sternal border. An apical mid-diastolic Electrocardiogram left bundle branch very close to the nodal-His murmur may be heard. Clear-cut signs of bundle junction),68-70 a small right ventricle or a congestive cardiac failure are usually present. The electrocardiogram can be virtually diagnos- large left ventricle.65 Ventricular activation data tic of tricuspid atresia in the neonate suspected from our group66,71 suggested that this charac- Noninvasive Evaluation to have a cyanotic . teristic QRS pattern in tricuspid atresia is pro- Right atrial hypertrophy, an abnormal, superiorly duced by interaction of several factors, the most Chest Roentgenogram oriented major QRS vector (so called left axis important being the right-to-left ventricular dis- deviation) in the frontal plane, left ventricular proportion and asymmetric distribution of the left Roentegenographic appearance is, by and hypertrophy, and diminished right ventricular ventricular mass favoring the superior wall. large, dependent upon the total pulmonary forces (Figure 7) are characteristic findings. blood flow (Figure 6C). In patients with dimin- Regardless of the frontal plane mean QRS vec- ished pulmonary flow, the heart size is either Right atrial hypertrophy, manifested by tall, tor orientation, electrocardiographic criteria for normal or minimally enlarged, whereas, in those peaked P waves in excess of 2.5 mm, is pre- left ventricular hypertrophy are present in the with increased pulmonary blood flow, the heart sent in the majority of the patients with tricuspid vast majority of patients. This may be mani- size is moderately to severely enlarged. Sev- atresia.64 Although it has been suggested that fested by increased (above 95th percentile) S eral patterns of cardiac configuration, namely the amplitude of the P wave in lead II is directly waves in right chest leads and R waves in left “characteristic” tricuspid atresia appearance,60 proportional to the interatrial pressure difference chest leads or by “adult progression” of the QRS coeur en sabot configuration,61 “egg-shaped,”28 and inversely proportional to the size of the in- in the chest leads in the neonates and infants. “bell-shaped”62 and square20 heart have been teratrial communication, detailed analysis of ST-T wave changes suggestive of left ventricu- described, but in the authors’ experience and these parameters did not suggest a consistent lar strain is present in 50% of patients.66 The that of others,28 there is no consistent pattern relationship.18,65 A double peak, spike and dome reason for left ventricular hypertrophy is the that would be diagnostic of tricuspid atresia. configuration of the P wave, referred to as anatomic nature of the lesion, left ventricular There may be concavity in the region of pulmo- “P-tricuspidale”64 may be present. The first taller volume overload and lack of opposition of the nary artery segment in patients with pulmonary peak is caused by the right atrial depolarization forces of left ventricular activation by the hy- oligemia and small pulmonary artery. The right and the second smaller peak is presumed to be poplastic right ventricle. Rarely, biventricular atrial shadow may be prominent. due to left atrial depoloarization.64,66 hypertrophy may be present and the majority of these patients belong to Type II or III anatomy Right aortic arch may be present in approxi- Abnormal, superiorly-oriented major QRS vec- with good-sized right ventricle. Diminished R mately 8% of patients with tricuspid atresia28 tor (ASV), more popularly called left axis devia- waves in right chest leads and S waves in left and is less common than that observed in pa- tion, between 0 to -90° in the frontal plane is chest are related to right ventricular hypoplasia. tients with (25%) and truncus present in the majority of the patients with tri- Vectorcardiographic features closely resemble arteriosus (40%). An unusual contour of the cuspid atresia. ASV is present in excess of the scalar electrocardiogram, but vectorcar- left border of the heart suggestive of 80% of patients with Type I anatomy (normally diography is no longer available for routine use.

6 NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 Echocardiogram Interrogation of right ventricular outflow tract in Type I patients and pulmonary artery region in M-mode echocardiography, while not diagnos- Type II patients may reveal pulmonary or sub- tic, is useful in evaluating the size of the left pulmonary stenosis; higher the velocity, more atrium and left ventricle and left ventricular severe is the obstruction. Doppler evaluation of function. Two-dimensional echocardiography, descending aortic flow is helpful in demonstrat- apart from showing enlarged right atrium, left ing aortic coarctation. atrium and left ventricle and a small right ven- tricle demonstrates the atretic tricuspid valve In summary, delineation of the majority of directly. In the most common muscular type, a anatomic and physiologic issues related to dense band of echoes is seen at the site tricuspid atresia is feasible by M-mode, 2- where tricuspid valve should be55,72 and the dimensional and Doppler (pulsed, continuous anterior leaflet of the detectable atrioventricular wave and color) echocardiography, and when valve is attached to the left side of interatrial indicated, contrast echocardiography. septum (Figure 8). Apical and subcostal four- chambered views are best to demonstrate the Figure 9. Selected video frame from an infant Other Laboratory Studies anatomy. Atrial and ventricular septal defects with tricuspid atresia demonstrating right-to-left can also be demonstrated by 2D echocardi- (blue flow) shunt (thick arrow) across the Pulse oxymetry and blood gas values are useful ography. Semilunar valves can be identified as interatrial communication in a subcostal in quantitating the degree of hypoxemia, pulmonary or aortic by following the great vessel four-chamber view. Atretic tricuspid valve (thin thereby indicating the severity of pulmonary until the bifurcation of the pulmonary artery or arrow) is seen. LA, Left atrium; RA, Right oligemia. Hemoglobin and hematocrit values arch of the aorta is seen; this will help decide atrium. are not particularly useful in the neonate, but whether there is associated transposition of the the degree of polycythemia is useful in estimat- great arteries. Suprasternal notch imaging will ing the severity of hypoxemia at a later age.73 be of use in demonstrating coarctation of the aorta which is often seen in Type II patients. Cardiac Catheretization

Contrast echocardiography with two- The diagnosis of tricuspid atresia based on dimensional imaging will clearly demonstrate clinical, electrocardiographic and echocardio- sequential opacification of the right atrium, left graphic features is relatively simple, and cardiac atrium, left ventricle and then the right ventricle. catheterization and selective cineangiography, However, contrast study is not essential for rarely, if ever, are essential for establishing the making the diagnosis. diagnosis.54 Even neonates with significant arte- rial desaturation need not undergo cardiac Doppler examination is useful in the evaluation catheterization and selective cineangiography; of tricuspid atresia patients. The obligatory the diagnosis of tricuspid atresia is usually made right-to-left shunt across the atrial septal defect on the basis of clinical and non-invasive evalua- Figure 10. Selected video frames from an can be demonstrated by placing pulsed Doppler tion, particularly echo-Doppler studies. Cathe- infant with tricuspid atresia of modified apical sample volume on either side of the atrial sep- terization may be indicated: four-chamber 2-dimensional echocardiographic tum and by color flow imaging (Figure 9). Left- 1) prior to bidirectional and views without (A) and with color-flow Doppler to-right shunting across the VSD may also be Fontan correction, particularly to define (B) illustrating enlarged left ventricle (LV), small the pulmonary artery anatomy and right ventricle (not labeled), atretic tricuspid 2) if catheter-based atrial septostomy is valve (not labeled), open mitral valve (MV) and required. a ventricular septal defect (VSD) with shunting across it. Since the neonates do not usually require car- diac catheterization and selective cineangi- demonstrated by Doppler (Figure 10). In Type ography, these will not be discussed; the inter- I (normally related great arteries) patients, the ested reader is referred to elsewhere.54,74,75 VSD peak Doppler velocity is helpful in esti- mating the size of the VSD; the higher the Differential Diagnosis velocity, the smaller is the VSD. Right ven- tricular and pulmonary arterial pressure may Differential diagnostic considerations differ with also be estimated using modified Bernoulli the mode of presentation: a) moderate to se- equation: verely cyanotic infants with decreased pulmo- nary vascular markings on chest roentgeno- 2 RV/PA systolic pressure = systolic BP – 4V gram, and b) mildly cyanotic infants with in- creased pulmonary vascular markings and with Figure 8. Apical four-chamber 2-dimensional Where, RV is right ventricle, PA is pulmonary or without signs of congestive heart failure. echocardiographic view of an infant with artery, BP is arm blood pressure and V is VSD tricuspid atresia showing enlarged left ventricle peak Doppler velocity. Decreased Pulmonary Blood Flow (LV), a very small right ventricle (RV), and a dense band of echoes at the site where the In the presence of pulmonary hypertension or Once decreased pulmonary blood flow is rec- tricuspid valve echo should be (TV). Partially severe infundibular or valvar pulmonary steno- ognized on the chest film, several possibilities, open mitral valve (MV) is also seen. Note the sis, the VSD Doppler velocities are not indica- as listed in Table II, should be considered. attachment of the anterior leaflet of the tive of the size of the VSD. In Type II (D- Most often, they can be differentiated with the detectable atrio-ventricular valve to the left side transposition) patients, high VSD velocity is help of an electrocardiogram (Figure 11).3 of the interatrial septum. LA, Left atrium; RA, suggestive of subaortic obstruction. Right atrium.

NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 7 Echocardiography and/or cineangiography are (double-inlet left) ventricle, double outlet right 3) medical management following confirmatory. ventricle, transposition of the great arteries with palliative surgery, VSD, and others, all as- 4) physiologically “corrective” surgery, and Tetralogy of Fallot (including VSD with sociated with severe pulmonary stenosis or 5) follow-up after corrective operation. Pulmonary Atresia). The frontal plane mean atresia. The electrocardiographic mean frontal QRS vector is between 90° and 180° in this plane vector and ventricular hypertrophy pat- Items 3 to 5 are not relevant to a chapter on group and right ventricular hypertrophy is pre- terns vary markedly. Echocardiography and/or tricuspid atresia in neonates and, therefore, will sent. Echocardiograms demonstrate a large angiography are often necessary for accurate not be detailed. right ventricle, a large aorta that overrides the diagnosis. , a large subaortic VSD Medical Management at the Time of Initial and increased Doppler flow velocity across the Increased Pulmonary Blood Flow Presentation pulmonary outflow tract. Angiographic features are characteristic for this anomaly. The differential diagnostic considerations are The need for prompt identification and rapid also listed in Table II. Although the characteristic transfer of a cyanotic/distressed neonate with Pulmonary Atresia (or Stenosis) with Intact electrocardiographic pattern (abnormal, superior suspected serious heart disease to a regional Ventricular Septum and Hypoplastic Right vector or “left axis deviation”) is helpful, it is not pediatric cardiology center is well recognized. Ventricle. The mean frontal plane vector is present in all cases of tricuspid atresia with During the process of identification, transfer to a between 0° to 90° without right ventricular hy- transposition. Furthermore, some of the condi- pediatric cardiology center, initial work-up, and pertrophy; left ventricular hypertrophy may be tions listed in Table IIB also have similar dis- palliative surgery, as well as following surgery, present and right ventricular forces may be placement of mean frontal plane vector. Often, neutral thermal environment, normal acid-base decreased. Echocardiogram shows a large left echocardiograms and occasionally, angiocar- status, normoglycemia, and normocalcemia ventricle, a hypoplastic right ventricle and small diograms are necessary for final diagnosis. should be maintained by appropriate monitoring but demonstrable tricuspid valve leaflets. An- and correction if needed.4 No more than 0.4 giocardiography will confirm the diagnosis. Management F1O2 is necessary unless pulmonary paren- chymal disease is present. Tricuspid Atresia. These patients will not only Physiologically “corrective” surgery for tricuspid 77,78 79-81 have an abnormal, superiorly-oriented QRS atresia and its modifications have im- Infants with low arterial PO2 and decreased vector (0° to -90°) on the frontal plane, but will proved the prognosis of patients with tricuspid oxygen saturation may be ductal-dependent; also have left ventricular hypertrophy and de- atresia. Such physiologic correction is usually therefore, the ductus should be kept open by creased right ventricular forces. performed in patients older than 2 years, at an intravenous administration of prostaglandin E1 4,82 Complex Pulmonary Stenosis. This group approximate weight of 15 Kg. As stated previ- (PGE1). The ductal dilating effect of this drug includes several defects, namely, single ously, most tricuspid atresia patients manifest results in an increase in pulmonary blood flow, symptoms in the neonatal period and should be thereby improving oxygenation and reversing effectively palliated to enable them to reach the the metabolic acidosis so that further diagnostic age at which surgical correction could be under- studies and surgical intervention can be per- taken. The objective of any management plan, formed with relative safety. Current suggestions apart from providing symptomatic relief and are for intravenous infusion of PGE1 at a dose increased survival rate, should be to preserve, of 0.05 to 0.1 µg per kilogram of body weight protect, and restore anatomy (good-sized and per minute. We usually begin with a dose of undistorted pulmonary arteries) and physiology 0.05 µg/kg/min and reduce the rate of infusion, (normal pulmonary artery pressure and pre- provided the desired oxygen saturation levels served left ventricular function) to normal such are achieved; this has been most helpful in re- that a “corrective” procedure could be per- ducing the incidence and severity of some of the formed at an appropriate age. Keeping the drug’s bothersome side effects, namely, apnea above objective in mind, the management plan and hyperpyrexia. PGE1 infusion rate may be may be discussed under the following head- increased if there is no increase in PO2. ings: 1) medical management at the time of initial The occasional infant that presents with signs of presentation, congestive heart failure (more common in Type 2) palliative treatment of specific II patients) should be treated with routine anti- physiologic abnormalities, congestive measures.83 Patients with associ- ated severe coarctation of the aorta may also be

Table II. Differential Diagnosis of Tricuspid Atresia in the Neonate Figure 11. The figure illustrates the usefulness of frontal plane mean QRS vector (axis) in the A. Decrease ed Pulmonary Blood Flow differential diagnosis of cyanotic heart defects 1. Tetralogy of Fallot including pulmonary atresia with ventricular septal defect with decreased pulmonary flow. An axis 2. Pulmonary atresia or severe stenosis with intact ventricular septum 0 3. Tricuspid atresia between 0 and –90 is suggestive of tricuspid 4. Complex cardiac defects with severe pulmonary stenosis or atresia atresia, an axis between 0 and +900 is indicative of pulmonary atresia with intact B. Increased d Pulmonary Blood Flow ventricular septum and an axis between +900 1. D-Transposition of the great arteries with large ventricular septal defect and ±1800 is associated with tetralogy of Fallot. 2. Coarctation of the aorta with ventricular septal defect The associated ECG abnormalities are shown 3. Multiple left-to-right shunts (ventricular septal defect, common atrioventricular canal and patent ductus arteriosus) in the respective quadrant. Cyanotic infants 4. Single ventricle, double outlet right ventricle and other complex cardiac defects without with more complex heart defects with severe pulmonary stenosis pulmonary stenosis/atresia may fall in any 5. Total anomalous pulmonary venous connection without obstruction quadrant. 6. Hypoplastic Left Heart Syndrome

8 NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 helped with PGE1 infusion; this time the ductal be considered in Type I patients; perhaps a dilatation improves systemic perfusion. This serious consideration for using absorbable band should be followed by surgical relief of coarcta- material93-95 should be given. Bonnet et al. used tion. Alternatively, balloon angioplasty may be absorbable pulmonary artery band for palliation utilized to relieve the aortic obstruction.84-86 in such infants.94 By restricting the pulmonary blood flow, the absorbable polydioxanone band Palliative Treatment of Specific Physiologic decreases pulmonary artery pressure initially Abnormalities and helps abate symptoms of heart failure. As the VSD spontaneously closes, the pulmonary The palliation of patients with tricuspid atresia artery band gets resorbed and does not pro- would largely depend upon the hemodynamic duce the severe pulmonary oligemia that might abnormality produced by the basic lesion and have been associated with a conventional non- associated cardiac defects. These abnormalities absorbable band. This is an innovative ap- may be broadly grouped4,50 into: proach, although it is likely to be helpful in a 1) decreased pulmonary blood flow limited number of patients.95 In those that did 2) increased pulmonary blood flow, and not have pulmonary artery banding performed, 3) intracardiac obstruction. Figure 12. Diagrammatic portrayal of classic careful follow-up studies with measurement of Blalock-Taussig shunt with subclavian pulmonary artery pressure and appropriate Decreased Pulmonary Blood Flow. Since the artery-to-ipsilateral pulmonary artery treatment are necessary to prevent pulmonary description of subclavian artery-to-ipsilateral anastomosis. The disconnected right (RSA) vascular obstructive disease. pulmonary artery anastomosis (Figure 12) in and left (LSA) subclavian arteries are shown. 1945 by Blalock and Taussig,87 several other Ao, Aorta; PA, Pulmonary artery. In Type II patients, banding of the pulmonary types of operations/procedures have been de- artery (Figure 13B) should be performed once vised to improve the pulmonary blood flow. nary blood flow, most of them are either not the infant is stabilized with anticongestive ther- These include other types of systemic- effective or, if effective, may produce serious apy. If there is associated coarctation of the pulmonary artery shunts, namely: the Potts an- complications to deter from performing a suc- aorta, or aortic arch interruption or hypoplasia, astomosis (descending aorta-to-left pulmonary cessful Fontan-Kreutzer procedure subse- adequate relief of the aortic obstruction should artery shunt), ascending aorta-to-main pulmo- quently. Blalock-Taussig anastomosis or one of be provided concurrent with pulmonary artery nary artery anastomosis (central shunt), its modified versions is the preferred procedure banding, and the patent ductus arteriosus Waterston-Cooley shunt (ascending aorta-to- and has the least number of long-term compli- should be ligated, if present. The importance right pulmonary artery anastomosis), aorta-to- cations, but at the same time, preserves suit- of PGE1 administration in temporarily relieving pulmonary artery Gore-Tex shunt, and Gore-Tex able anatomy for subsequent corrective proce- aortic obstruction and thereby control conges- interposition graft between the subclavian artery dures. Therefore, it is recommended as the pro- tive heart failure has already been alluded to. and the ipsilateral pulmonary artery, superior cedure of choice for palliation of tricuspid atresia The role of balloon dilatation angioplasty of the vena cava-to-right pulmonary artery anastomo- patients with decreased pulmonary blood flow. coarctation84-86 in these complicated lesions sis (Glenn Procedure), formalin infiltration or has not yet been completely delineated. Be- stenting the ductus arteriosus, and enlarging the Increased Pulmonary Blood Flow. Infants cause of higher risk for poor outcome in pa- VSD. Systemic-pulmonary artery shunts are with a modest increase in pulmonary blood flow tients with transposition and those requiring most commonly used in the palliation of pulmo- do not have any significant symptomatology pulmonary artery banding and/or aortic arch nary oligemia. Because of the problems asso- and, indeed, are less cyanotic than the pulmo- repair,96 early, adequate and appropriate inter- ciated with central shunts, the Blalock-Taussig nary oligemic patients. Markedly increased pul- ventions are desirable. type of shunt is preferred. At present, a modified monary blood flow, however, can produce con- Blalock-Taussig shunt with a Gore-Tex graft gestive heart failure. Only Type Ic and Type IIc Intracardiac Obstruction. Intracardiac obstruc- (Figure 13A) interposed between the subclavian patients, i.e., without associated pulmonary tion can occur at two different levels, namely, artery and the ipsilateral pulmonary artery88 stenosis, will fall into the category of pulmonary patent foramen ovale and VSD. appears to be the first choice in most institutions plethora. A majority of these patients will have for palliation of the neonate and young infant Type II anatomy and will usually manifest during Interatrial Obstruction. Since the entire sys- with pulmonary oligemia. early infancy. temic venous return must egress through the patent foramen ovale, it should be of adequate Enlargement of the VSD and/or resection of the In Type I patients, aggressive anticongestive size to accommodate it. A mean atrial pressure right ventricular outflow tract stenosis has been measures should be promptly instituted. The difference of 5 mmHg or more with very promi- performed and recommended by Annecchino natural history of the VSD has been well docu- nent 'a' waves (15 to 20 mmHg) in the right and his colleagues89 as a palliative procedure to mented in this group;39-46 the VSD becomes atrium is generally considered to represent ob- augment the pulmonary blood flow. This is an smaller and patients with pulmonary plethora structed interatrial septum.50 Balloon atrial sep- ingenious approach and attacks the site of ob- will, in due course, develop pulmonary oligemia tostomy;97 if unsuccessful, blade atrial sep- struction rather than bypassing it. However, it is (Figure 6), requiring palliative surgical shunts. tostomy;98,99 and; rarely surgical atrial sep- an open heart procedure and may not be feasi- These patients can also develop right ventricu- tostomy may become necessary to relieve the ble or necessary in the neonatal period.50 lar outflow tract obstruction with resultant de- obstruction. Significant interatrial obstruction Placement of a stent in the ductus, to keep it crease in pulmonary blood flow. Therefore, it is requiring atrial septostomy in the neonate is rare open to provide pulmonary flow is an attractive generally recommended that pulmonary artery and unusual, although this can be a significant option,90,91 but because of limited experience banding not be performed in this group of pa- problem later in infancy.41 and the technically demanding nature of the tients. Among our 40 consecutive patients with procedure, it currently is not a therapeutic pro- tricuspid atresia,46,50 only two with Type I anat- Interventricular Obstruction. Spontaneous cedure of choice. If the predominant obstruction omy required pulmonary artery banding and closure of the VSD causing severe pulmonary is at the pulmonary valve level, balloon pulmo- there are only a few cases reported in the litera- oligemia in Type I patients and subaortic ob- nary valvuloplasty may be considered.92 ture that required pulmonary artery banding. If struction in Type II patients can occur;39-47 this optimal anticongestive therapy with some time usually takes months to years to develop. For In conclusion, despite the availability of many delay does not produce adequate relief of further discussion of this subject, the reader is types of palliative procedures to increase pulmo- symptoms,50 pulmonary artery banding should referred elsewhere.41,42,46

NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 9 Prognosis

Untreated, the prognosis of live born infants with tricuspid atresia is poor; only 10 to 20% may survive their first birthday.17,18 Palliative surgery to normalize the pulmonary blood flow has markedly improved the survival rate. But, as one can see from survival data from several large studi- es,26,59,96 there is still considerable early mortality. Because of recent improvement in surgical mortality for the palliative surgery and advances in neonatal care, the initial mortality should decrease. Introduction of physiologically “corrective” surgery in the early 1970s has, to some de- gree, improved the second bout of mortality seen in children beyond 15- years-of-age. Because of this improved prognosis, each neonate with tricuspid atresia should be offered aggressive medical and surgical ther- apy.

Summary/Conclusion

Tricuspid atresia is the third most common cyanotic congenital heart defect. There are significant variations in the morphology of the atretic tricuspid valve, associated cardiac defects and physiology, resulting in different clinical presentations. The diagnosis is relatively simple and can often be made by careful review of clinical features and simple labora- Figure 13. Palliation of tricuspid atresia patients. Patients with pulmonary tory studies (chest roentgenogram and electrocardiogram) which can be confirmed by echocardiography. Aggressive management to normalize oligemia with markedly decreased O2 saturation may be palliated by a modified Blalock-Taussig shunt, connecting the subclavian artery to the the pulmonary blood flow and correct physiologically important associ- ipsilateral pulmonary artery with a Gore-Tex graft (GG); the left panel (A) ated defects (for example coarctation of the aorta) should be undertaken demonstrates GG opacified on left subclavian artery (LSA) cineangiogram at the time of presentation. Follow-up and treatment plans should strive with excellent opacification of the pulmonary artery (PA). In patients with to maintain or normalize cardiac structures and function (pulmonary ar- tery anatomy and pressure, and left ventricular function). Finally, per- pulmonary oligemia, but with O2 saturations in low 80s may be followed clinically without any intervention. Patients with markedly increased forming modified, staged Fontan-Kreutzer surgery prior to deterioration pulmonary blood flow and severe congestive heart failure may re- of the left ventricular function should markedly improve the prognosis for quire banding of the pulmonary artery, as shown in right panel (B). tricuspid atresia patients. References Management Beyond Neonatal Period 1. Rao PS. Perinatal circulatory physiology: It’s influence on clinical Detailed discussion of this subject is beyond the scope of this review and manifestations of neonatal heart disease – Part I. Neonatology Today the reader is referred to other publications50,76,100,101 for further treatment of 2008; 3(2):6-12. this subject. Since the description by Fontan77 and Kreutzer,78 the proce- 2. Rao PS. Perinatal circulatory physiology: It’s influence on clinical dure has evolved79-81 and continues to evolve.76,100,101 At the time of this manifestations of neonatal heart disease – Part II. Neonatology To- writing, most authorities seem to prefer total cavopulmonary connecc- day 2008; 3(3):1-10. tion,79 performed as a two-staged procedure; bidirectional Glenn102 ini- 3. Rao PS. An approach to the diagnosis of cyanotic neonate for the tially followed by Fontan conversion either by an intra-atrial lateral tunnel primary care provider. Neonatology Today 2007; 2 (6):1-7. or an extracardiac nonvalved conduit diversion103 of the inferior vena 4. Rao PS. Principles of management of the neonate with congenital caval and hepatic blood into the pulmonary circuit a year or so later. In heart disease Neonatology Today 2007; 2(8):1-10. the presence of risk factors,104 fenestration105,106 may be performed 5. Rao PS. Neonatal cardiac emergencies: Management strategies, which could be closed either in the immediate post-operative period106 or Neonatology Today 2008; 3(12):1-5. at a later time by transcatheter methodology.105,107 6. Rao PS. Transposition of the great arteries in the neonate. Neonatol- ogy Today 2010; 5(8):1-5. Summary of Surgical Management 7. Alapati S, Rao PS. Tetralogy of Fallot in the neonate. Neonatology Today 2011; 6(5):1-10. Physiologically corrective surgery is feasible in most patients with 8. Alapati S, Rao PS. Hypoplastic left heart syndrome in the neonate, tricuspid atresia. The age and size of the patient at presentation and Neonatology Today 2011; 6(12):1-9. anatomic and physiologic substrate dictate the type of palliative/ 9. Rao PS. A unified classification for tricuspid atresia. Am Heart J 1980; corrective procedures. In the neonate and young infant (! 3 months) 99:799-804. with pulmonary oligemia, a modified Blalock-Taussig shunt is the 10. Rao PS. Terminology: tricuspid atresia or univentricular heart? In Rao procedure of choice. Some infants with O2 saturations in low 80s may PS (ed): Tricuspid Atresia, Mount Kisco, NY: Futura Publishing Co, not require any intervention. Between 3 to 12 months, a modified 1982, pp. 3-6. Blalock-Taussig shunt and a bidirectional Glenn procedure are the 11. Rao PS. Is the term "tricuspid atresia" appropriate? (editorial). Am J available options; we would prefer bidirectional Glenn. Between 12 Cardiol 1990; 6:1251-1254. to 24 months, bidirectional Glenn is preferred. Beyond 2-years-of- 12. Rao PS. Terminology: Is tricuspid atresia the correct term to use? In age, total cavopulmonary connection with or without fenestration, Rao PS (ed): Tricuspid Atresia, ed 2. Mount Kisco, NY: Futura Pub- depending upon the presence of risk factors, appears to be the lishing Co, 1992, pp. 3-15. choice, although some authorities stage the Fontan even at this age. 13. Rashkind WJ. Tricuspid atresia: a historical review. Pediat Cardiol Early pulmonary artery banding, relief of aortic arch obstruction and 1982; 2:85-88. relieving or bypassing subaortic obstruction should be incorporated 14. Kreyseg FL. Die Krankheiten des Herzens. Dritte Thies 1817, pp. into the treatment plan in patients with tricuspid atresia and transposi- 104-106. tion of the great arteries. 15. Editors. London Medical Review 1812; 5:262-263.

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Spontaneous closure or WH. Roentgenographic findings of congeni- 1966, pp. 656-685. critical decrease in size of the ventricular tal tricuspid atresia with hypoplasia of the 28. Vlad P. Tricuspid atresia. In Keith JD, Rowe septal defect in tricuspid atresia with nor- right ventricle. Am J Roentgenol 1951; RD, Vlad P (eds): Heart Disease in Infancy mally connected great arteries: surgical 64:712-727. and Childhood, ed 3. New York: Macmillian, implications. Herz 1980; 5:369-384. 62. Elster SK. Congenital atresia of pulmonary 1977, pp. 518-541. 45. Rao PS. Physiologically advantageous ven- and tricuspid valves. Am J Dis Child 1950; 29. Rao PS. Classification of tricuspid atresia. In tricular septal defects (Letter). Pediat Cardio 79:692-697. Rao PS (ed): Tricuspid Atresia, Mount Kisco, 1983; 4:59-61. 63. Covitz W, Rao PS. Noninvasive evaluation NY: Futura Publishing Co, 1982, pp. 41-47. 46. Rao PS. Natural history of ventricular septal of patients with tricuspid atresia (Roentge- 30. Van Praagh R. Terminology of congenital defects in tricuspid atresia. In Rao PS (ed): nography, echocardiography and nuclear heart disease: glossary and commentary. Tricuspid Atresia, ed 2. Mount Kisco, NY: angiography). In Rao PS (ed): Tricuspid Circulation 1977; 56:139-143. Futura Publishing Co, 1992, pp. 261-293. Atresia, ed 2. Mount Kisco, NY: Futura Pub- 31. Weinberg PM. Pathologic anatomy of tri- 47. Rao PS. Subaortic obstruction after pulmo- lishing Co, 1992, pp. 165-182. cuspid atresia. In Rao PS (ed): Tricuspid nary artery banding in patients with tricuspid 64. Gamboa R, Gersony WM, Nadas AS. The Atresia, Mount Kisco, NY: Futura Publishing atresia and double-inlet left ventricle and electrocardiogram in tricuspid atresia and Co, 1982, pp. 49-67. ventriculoarterial discordance (Letter). J Am pulmonary atresia with intact ventricular 32. Bharati S, McAllister HA, Jr, Tatooles CJ, et Coll Cardiol 1991; 18:1585-1586. septum. Circulation 1966; 34:24-37. al. Anatomic variations in underdeveloped 48. Bharati S, Lev M. The concept of tricuspid 65. Patel R, Fox K, Taylor JFN, et al. Tricuspid right ventricle related to tricuspid atresia and atresia complex as distinct from that of the atresia - clinical course in 62 cases (1967- stenosis. J Thorac Cardiovasc Surg 1976; single ventricle complex. Pediat Cardiol 1974). Br Heart J 1978; 40:1408-1414. 72:383-400. 1979; 1:57-62. 66. Rao PS, Kulungara RJ, Boineau JP, et al. 33. Ando M, Santomi G, Takao A. Atresia of 49. Rosenthal A, Dick M, II. Tricuspid atresia. In Electrovectorcardiographic features of tri- tricuspid and mitral orifice: anatomic spec- Adams FH, Emmanouilides GC (eds): cuspid atresia. In Rao PS (ed): Tricuspid

NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 11 Atresia, ed 2.. Mount Kisco, NY: Futura Pub- ease: The US experience. Circulation 100.Schaff HV, Danielson GK. Corrective sur- lishing Co, 1992, pp. 141-164. 1981; 64:899-905. gery for tricuspid atresia. In Rao PS (ed): 67. Puri PS, Neill CA. Vectorcardiographic study 83. Rao PS. Congenital Heart Disease. In Rakel Tricuspid atresia, ed 2. Mount Kisco, NY: in ten cases of tricuspid atresia. In Cassels RE (ed): Conn’s Current Therapy, Philadel- Futura Publishing Co, 1992, pp. 341-360. DE, Ziegler RF (ed): in phia: WB Saunders Co, 1989, pp. 201-213. 101.Rao PS. Tricuspid atresia. In Moller JH, Infants and Children, New York: Grune and 84. Rao PS. Current Status of Balloon Angio- Hoffman JIE (eds): Pediatric Cardiovascular Stratton, 1966, pp. 269-272. plasty for Neonatal and Infant Aortic Coarc- Medicine, New York: Churchill Livingstone, 68. Bharati S, Lev M. Conduction system in tation. Progress Pediat Cardiol 2001; 2000, pp. 421-441. tricuspid atresia with and without regular (d) 14:35-44. 102.Hopkins RA, Armstrong SSE, Serwer GA, et transposition. Circulation 1977; 56:423-429. 85. Rao PS. Role of Interventional Cardiology al. Physiologic rationale for a bidirectional 69. Dickenson DF, Wilkinson JL, Smith A, et al. In Neonates: Part II - Balloon Angioplasty/ cavopulmonary shunt: a versatile comple- Atrioventricular conduction tissues in univen- Valvuloplasty. Congenital Cardiol Today ment to the Fontan principle. J Thorac Car- tricular of left ventricular type with 2008; 6(1): 1-14. diovasc Surg 1985; 90:391-398. absent right atrioventricular connection (“tri- 86. Rao PS, Seib PM. Coarctation of the Aorta. 103.Kumar SP, Rubinstein CS, Simsic JM, et al. cuspid atresia”). Br Heart J 1979; 42:1-8. eMedicine from WebMD. Updated July 20, Lateral tunnel versus extracardiac conduit 70. Guller B, Dushane JW, Titus JL. Atrioven- 2009. 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DeLeval M, McKay R, Jones M, et al. fenestration with subsequent transcatheter dimensional echocardiography in the diag- Modified Blalock-Taussig shunt: Use of closure: modification of the Fontan operation nosis of tricuspid atresia: differentiation from subclavian orifice as a flow regulator in for patients with increased risk. Circulation other hypoplastic right heart syndromes and prosthetic systemic-pulmonary artery 1990; 82:1681-1689. common atrioventricular canal. Br Heart J shunts. J Thorac Cardovasc Surg 1981; 106.Laks H, Pearl JM, Haas GS, et al. Partial 1978; 40:1174-1183. 18:112-119. Fontan advantages of an adjustable in- 73. Rao PS. Pathophysiologic consequences of 89. Annecchino FP, Fontan F, Chauve A, et al. teratrial communication. Ann Thorac Surg cyanotic heart disease. Indian J Pediat An operation for the correction of tricuspid 1991; 52:1084-1095. 1983; 50:479-487. atresia. Ann Thorac Surg 1979; 107.Rao PS, Chandar JS, Sideris EB. Role of 74. Rao PS. 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12 NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 Medical News, Products and Information

Women & Infants’ Physician Named 2012 During this highly productive part of his suggesting a potential opportunity for early Legend of Neonatology career, Dr. Oh published virtually non-stop intervention.” in a number of areas of neonatal medi- William Oh, MD, former Chief of Pediatrics cine. The findings were published online ahead at Women & Infants Hospital of Rhode Is- of print publication in the American Tho- land and The Warren Alpert Medical School He continued his efforts at understanding racic Society’s American Journal of Respi- of Brown University, was recently inducted neonatal blood pressure, the role of acid- ratory and Critical Care Medicine. into the Legends of Neonatology Hall of base balance upon abnormal fetal heart Fame which was established in 2007. Dr. rate patterns and neonatal well-being, the The prospective study enrolled a birth co- Oh was one of two physicians inducted this effects of insensible water loss upon neo- hort of 411 at-risk children of asthmatic year at the NEO meeting in February 2012 natal metabolism, nutritional well-being in mothers. Spirometry was performed at one for contributions to the care of the critically neonates, neonatal glucose metabolism, month in 403 (98%) children and again at ill neonate. intrauterine growth retardation, neonatal age seven in 317 (77%). renal function, bilirubin toxicity, and many other issues. In April, Dr. Oh also received another pres- Significant neonatal airflow deficits, as tigious award, the Maureen Andrews Men- measured by forced expiratory flow at 50% torship Award, from the Society for Pediatric Increasingly, Dr. Oh has become interested of vital capacity and forced expiratory vol- Research, during its annual meeting in Bos- in long-term neurodevelopmental outcome ume after 0.5 seconds, were observed ton, in recognition of his mentoring career. following neonatal intensive care unit among the 14% of children who developed In the past four decades, Dr. Oh has trained (NICU) hospitalization and has been a lead- asthma by age seven. Bronchial respon- more than 80 neonatologists, who are now ing figure in the NICHD Neonatal Network. siveness to methacholine, which provokes leaders in their fields all over the world. He has won numerous major awards and narrowing of the airways, was also signifi- honors, including the Apgar Award of the cantly associated with the development of Dr. Oh is one of the founders of the field of American Academy of Pediatrics. Dr. Oh asthma. Neonatal airway reactivity was a neonatal medicine and has been a leader in has long been focused on one of the key stronger predictor of asthma than neonatal teaching about metabolism, minerals, and areas of modern medicine, namely improv- lung function. fluids and electrolytes in the newborn infant. ing outcomes for neonates, and has con- tributed as much as any living neonatologist “We found that approximately 40% of the in that regard. “This is such an honor for Dr. Oh and his airflow deficit that was associated with colleagues at Women & Infants and Brown," asthma in our study was present at birth, said Constance A. Howes, President & CEO In 2009, Women & Infants opened the while 60% developed through early child- of Women & Infants. "The contributions that country’s largest, single-family room neona- hood along with the disease,” noted Dr. Dr. Oh has made to the field of neonatology tal intensive care unit. See a related article Bisgaard. “This indicates that both prenatal are extraordinary. He has impacted the the February 2010 issue of Neonatology and early childhood mechanisms are po- care and caring of some of the tiniest, frail- Today, by James F. Padbury, MD and Barry tential intervention targets for the preven- est babies and has paved the way for in- M. Lester, PhD, entitled, “Millennium Neo- tion of asthma.” credible discoveries and improvements in natology: Building for the Future.” the care that we provide here and globally.” www.neonatologytoday.net/newsletters/ The study used a homogenous study sam- nt-feb10.pdf. ple, which might limit extrapolation of the results to other populations. Originally trained in the Philippines where he received his medical degree, Dr. Oh came to the US in 1958, to do a pediatric Neonatal Lung Function Deficits in “It seems that lung function changes asso- residency at Michael Reese Hospital in Chi- Children Who Develop Asthma ciated with asthma occur very early in life cago, where he later became chief resident and maybe even before birth,” concluded and a research fellow in neonatology. From Children who develop asthma by age seven Dr. Bisgaard. “This may explain the lack of 1964 to 1966, he initiated a series of re- have deficits in lung function and increased effect from early intervention with inhaled search projects at the Karolinska Institute in bronchial responsiveness as neonates, a corticosteroids and should direct research Stockholm that resulted in one of the first new study from researchers in Denmark into the pathogenesis and prevention series of papers to examine neonatal blood suggests. of asthma towards the earliest phases of pressure, neonatal blood volume, neonatal life.” hemodynamics, and neonatal renal func- “Previous research on the relationship be- To read the article in full, please visit: tion. tween neonatal lung function and the devel- www.thoracic.org//media/press-releases/re opment of asthma has been conflicting,” sources/bisgaard.pdf. Dr. Oh became Director of Neonatology at said lead author Hans Bisgaard, MD, Michael Reese Hospital in 1966, and in DMSci, Professor of Pediatrics at the Uni- 1969 joined the faculty as Chief of Neona- versity of Copenhagen and head of the The American Journal of Respiratory and tology at Harbor General Hospital in Cali- Danish Pediatric Asthma Centre. “Our study Critical Care Medicine (AJRRCM) is a fornia until 1974. In 1975 Dr. Oh left Cali- shows that children with asthma by age peer-reviewed journal published by the fornia to become Pediatrician-in-Chief of seven already had significant airflow deficits American Thoracic Society. It aims to pub- Women & Infants Hospital and Professor and increased bronchial responsiveness as lish the most innovative science and the of Pediatrics and Obstetrics at Brown Uni- neonates. Lung function deficits also pro- highest quality reviews, practice guide- versity, where he was appointed Chairman gressed throughout childhood in our study, lines and statements in the pulmonary, of the Department of Pediatrics in 1989. critical care and sleep-related fields.

NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012 13 Global Neonatology Today Monthly Column - Tracking the MGDs: What is the Status of Poverty in 2012

By Dharmapuri Vidyasagar, MD, FAAP, FCCM NEONATOLOGY TODAY Now that the target date of 2015 for reaching “Overall, in 2015, 25% of the United Nation’s MDGs (Millennium De- the world’s population © 2012 by Neonatology Today velopment Goals) is less than 3 years away, ISSN: 1932-7129 (print); 1932-7137 (online). major funding agencies and stakeholders are (1.38 billion) is expected to Published monthly. All rights reserved. continuously monitoring the progress of all MDGs. remain in poverty earning Corporate Offices: 8100 Leaward Way, Nehalem, OR 97131 USA Here are some of the findings of the Interna- less than $1.25/day.” tional Monitory Fund (IMF) and the World Bank Mailing Address: (WB) regarding the MDG #1 - “Eradicate Ex- PO Box 444, Manzanita, OR 97131 USA treme Poverty.” The trends in Sub-Saharan Africa also show a Tel: +1.301.279.2005; Fax: +1.240.465.0692 decrease, but at a much slower rate; it dropped According to the IMF and WB, the world is on from a high of 57% to 51% in 2005, and is www.NeonatologyToday.net track to reduce by half the number of people expected to drop to 36%, but still remain higher living in extreme poverty (i.e living on less than Publishing Management: than in East Asia and South Asia. • Tony Carlson, Founder, President & Senior $1.25/day). According to the report, by 2015 Editor - [email protected] eight-hundred-and-eighty-three million people Overall, in 2015, 25% of the world’s population • Richard Koulbanis, Group Publisher & will live in poverty. This is less than the 918 (1.38 billion) is expected to remain in poverty Editor-in-Chief - [email protected] million that had been projected previously. The earning less than $1.25/day. • John W. Moore, MD, MPH, Medical Editor - observed decline is mainly because of the [email protected] significant changes that occurred in China and Although these dropping numbers of extreme • Virginia Dematatis, Assistant Editor Caryl Cornell, Assistant Editor India. poverty are very encouraging, the poorest • • Loraine Watts, Assistant Editor countries remain poor. At the current rate, by Chris Carlson, Web Manager Overall, there was a large global reduction of • 2015, one billion people will be still extremely • William Flanagan, Strategic Analyst poverty between 1990-2011, and it is projected poor by the standards of middle and high in- • Rob Hudgins, Designer/Special Projects to decrease further by 2015. In 2005, 42% of come countries. The $1.25/day poverty line is the world’s population (1.8 billion) were living the average for the world’s poorest 10-20 Editorial Board: Dilip R. Bhatt, MD; Barry D. on less than $1.25/day. That is expected to countries. A higher line of $2.00/day, the me- Chandler, MD; Anthony C. Chang, MD; K. K. drop to 25% (1.3 billion) by 2015. dian poverty line for developing countries, Diwakar, MD; Willa H. Drummond, MD, MS shows less progress. (Informatics); Philippe S. Friedlich, MD; Lucky Looking at the progress in different regions and Jain, MD; Patrick McNamara, MD; David A. countries, it is clear that most of the progress Munson, MD; Michael A. Posencheg, MD; "Having 22% of people in developing countries DeWayne Pursley, MD, MPH; Joseph has been made in East Asia, particularly in still living on less than $1.25 a day and 43% Schulman, MD, MS; Alan R. Spitzer, MD; China. In 1990, 60% of the population (683 with less than $2.00 a day is intolerable. We Dharmapuri Vidysagar, MD; Leonard E. million) of China continued to earn less than need to increase our efforts..., we need to con- Weisman, MD; Stephen Welty, MD; Robert $1.25/day; it dropped to 16% in 2005, and is tinue attacking poverty on many fronts" par- White, MD; T.F. Yeh, MD expected to drop to 4.8% by the year 2015. By ticularly in low-income countries,” says Jaime then, only 66 million people in China will re- Saavedra, Director of the World Bank’s Pov- FREE Subscription - Qualified Professionals main earning below $1.25/day. This very sig- erty Reduction and Equity Group (Jan. 2012). Neonatology Today is available free to qualified medical professionals worldwide in neonatology nificant achievement is well beyond the set target. and perinatology. International editions avail- The Clock is Ticking !!! able in electronic PDF file only; North American edition available in print. Send an email to: It is interesting to note that in 1990 the poverty NT [email protected]. Include your name, ti- rate in India was lower than in China (52% vs tle(s), organization, address, phone, fax and 60%). Although there is an overall decrease in email. percentage and the number of people living on Dharmapuri Vidyasagar, MD, FAAP, FCCM less than $1.25/day, the decrease in India is at University of Illinois at Chicago Sponsorships and Recruitment Advertising a much slower rate than that is seen in China. Professor Emeritus Pediatrics For information on sponsorships or recruitment In India, it decreased from 52% in 1990 to 42% Division of Neonatology advertising call Tony Carlson at 301.279.2005 in 2005, while during that same period in China Phone: +312.996.4185 or send an email to [email protected] it dropped to 16% in 2005. Fax: 312.413.7901 [email protected]

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14 NEONATOLOGY TODAY ! www.NeonatologyToday.net ! May 2012