DOCSLIB.ORG

Corrected Transposition of the Great Arteries

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Corrected Transposition of the Great Arteries C. Alva-Espinosa: Corrected transposition of the great arteries Contents available at PubMed www.anmm.org.mx PERMANYER Gac Med Mex. 2016;152:357-65 www.permanyer.com GACETA MÉDICA DE MÉXICO REVIEW ARTICLE Corrected transposition of the great arteries Carlos Alva-Espinosa* Planning, Teaching and Research, Hospital Regional de Alta Especialidad Ixtapaluca, Ixtapaluca, Méx., Mexico Abstract Corrected transposition of the great arteries is one of the most fascinating entities in congenital heart disease. The apparent corrected condition is only temporal. Over time, most patients develop systemic heart failure, even in the absence of associated lesions. With current imaging studies, precise visualization is achieved in each case though the treatment strategy remains unresolved. In asymptomatic patients or cases without associated lesions, focalized follow-up to assess systemic ventricular function and the degree of tricuspid valve regurgitation is important. In cases with normal ventricular function and mild tricuspid failure, it seems unreasonable to intervene surgically. In patients with significant associated lesions, surgery is indicated. In the long term, the traditional approach may not help tricuspid regurgitation and systemic ventricular failure. Anatomical correction is the proposed alternative to ease the right ventricle overload and to restore the systemic left ventricular function. However, this is a prolonged operation and not without risks and long-term complications. In this review the clinical, diagnostic, and therapeutic aspects are overviewed in the light of the most significant and recent literature. (Gac Med Mex. 2016;152:357-65) Corresponding author: Carlos Alva-Espinosa, [email protected] KEY WORDS: Corrected transposition of the great arteries. Double discordance. Anatomic correction. Double switch. that characterizes this condition: an atrioventricular ntroduction I discordance combined with ventriculoarterial discor- dance, and second, because caval venous return flow Corrected transposition of the great arteries is one is correctly carried to the lungs, whereas pulmonary of the most fascinating entities in congenital heart dis- veins-originating oxygenated blood reaches the aorta ease. When a doctor sees for the first time a patient or and systemic circulation without any problem (Fig. 1). a heart where the morphological right atrium is con- This situation of apparently “corrected” transposition nected with the left ventricle (LV) and this in turn with goes on with no problem at least for the first decades the pulmonary artery, while the left atrium is communi- of life, as long as there are no associated lesions. Things cated with the right ventricle (RV) and this in turn with are, of course, more complex. Most cases have associ- the aorta, he/she cannot be less than surprised. First, ated lesions, which will determine the clinical presenta- due to the magnitude of alterations in the connections tion, and having the RV supporting systemic circulation of cardiac segments: we are facing the discordances carries, over time, ventricular failure in most cases. Correspondence: *Carlos Alva-Espinosa Dirección de Enseñanza e Investigación Hospital Regional de Alta Especialidad Ixtapaluca Carretera Federal México – Puebla Km. 34.5 Pueblo de Zoquiapen, C.P. 56530, Ixtapaluca, Méx., México Date of reception: 04-03-2015 E-mail: [email protected] Date of acceptance: 06-04-2015 357 Gaceta Médica de México. 2016;152 l d k g h l c f a b e Figure 1. Drawing where the double discordance in situs solitus is visualized. The atria are recognized by the morphology of their au- ricles: the right one, broad-based, and the left one, narrow and with an irregular, scallop shape. The left ventricle with smooth endocar- dium and the right with thick trabecula. Observe that the great arte- Figure 2. Original drawing of a corrected transposition in situs soli- ries do not cross but emerge in parallel, where the aorta appears tus made by Rokitansky in 1875. It is a cross-sectional cut at the left and straight. By virtue of the double discordance, circulations level of the atrioventricular valves. The right ventricle (A) is observed have a normal course. RA: right atrium; LV: left ventricle; LA: left to be dilated, (B) Tricuspid valve, supporting the aorta (C), (D) atrium; P: pulmonary artery; A: aorta. Aorta, (E) Left ventricle and left with normal size (F), and in relation to the pulmonary artery (G). The view is very much alike to that obtained with bi-dimensional echocardiography. In this review, we will analyze all these aspects with more detail, with particular emphasis on clinical mani- aorta in “L position”, such as the RV double outflow festations, diagnosis, prognosis and treatment. way. Therefore, the accurate form is to describe it as atrioventricular (AV) and ventriculoarterial discordance History in situs solitus or inversus and then the associated lesions, without forgetting the position of the heart with- The first known description of corrected transposition of in the thorax, as we will address later. the great arteries (CTGA) was made by baron von Roki- tansky in 1875 with a very beautiful illustration (Fig. 2), Etiology where both discordances can be appreciated in situs solitus. Since then, an elevated number of CTGA cas- The etiology of this malformation is unknown; howev- es and all type of associated lesions have been de- er, the L-loop during embryogenesis is known to be scribed and documented. determinant in ventricular inversion and it is responsi- ble for the presence of double discordance. There is Nomenclature predominance in the male gender in a 1.6 to 1 ratio. In this article, when it reads LV, we always refer to Frequency the morphological left ventricle, the same way than when it reads RV, reference is being made to the mor- CTGA affects from 0.4 to 1% of all children with phological right ventricle, regardless of their spatial congenital heart disease1-4 but it is likely to be under- situation within the thorax. estimated, since those with no associated lesions usu- On the other hand, although CTGA is referred to ally have clinical manifestations at older ages and in rather frequently as “L” transposition due to its location some cases they may not show manifestations of this left to the aorta in the frontal projection, this is inaccu- heart condition and die of other cause. In approximate- rate, since situs inversus CTGA itself has the aorta at ly 80 to 95% of the cases it occurs in situs solitus and the right and other complex pathologies can have the in 5 to 20% in situs inversus5-7. 358 C. Alva-Espinosa: Corrected transposition of the great arteries whereas the His bundle is longer, with a trajectory anterior to the infundibular septum that predisposes to atrioventricular block, as we will see in the clinical section10,11. The aortic annulus, supported by the morphological right ventricle infundibulum is located up and to the right, giving rise to a straight, ascending and left aorta. Practically any known associated lesion in patients with concordant hearts can occur in CTGA (Fig. 3); however, there are some lesions and combinations thereof that are characteristic: the combination of ven- tricular septal defect with pulmonary stenosis (Fig. 4) and tricuspid lesion with regurgitation is a characteris- tic triad of CTGA12-14. Figure 3. Specimen showing atrioventricular discordance and, as If we analyze the lesions separately, the most com- associated lesion, a complete atrioventricular septal defect can be mon lesion is interventricular communication (IVC), observed. A common atrioventricular valve riding on the interventri- which is present in at least 80% of cases15; usually, it cular septum is appreciated. LA: left atrium, LV: left ventricle, RA: right atrium, RV: right ventricle. is perimembranous, but it can be found at any site or it can be multiple. Pulmonary obstruction is observed in 40 to 70%16; characteristically, it is a subpulmonary Anatomical characteristics obstruction by accessory tissue (Fig. 4), but it may be and associated lesions combined with stenosis at the valvular level. Pulmonary atresia has been found in 8% of cases17. Tricuspid Notably, in most cases, the interventicular septum anomalies are universal in autopsy cases and, clinical- has a sagittal orientation with the ventricles at both ly, at least one third of patients have significant tricus- sides in hearts that tend to mesocardia within the thorax pid insufficiency18. Aortic insufficiency has been re- (Fig. 3), but the cardiac shape can appear in levo- or ported in approximately 30% of cases19. Of course, dextrocardia8,9. CTGA is also accompanied by interatrial communica- The pulmonary ring is wedged onto the center of the tion (IAC) or patent ductus arteriosus (PDA), either in heart between the mitral and tricuspid annulus and isolated form or associated with important lesions such therefore the membranous septum has a larger area, as IVC, pulmonary stenosis and tricuspid insufficiency. Figure 4. A: specimen with CTGA. This view corresponds to the morphological left ventricle. Smooth septum and fine trabecula are appre- ciated. Observe a restrictive subpulmonary interventricular communication (asterisk). B: closer view of the same specimen. Subpulmonary obstruction pointed by vertical arrows and, in dotted line, the route of the conduction tissue across the anterior and superior margin of the septal defect. RA: right atrium; LV: left ventricle (adapted from Alva C. et al.)20 359 Gaceta Médica de México. 2016;152 Coronary circulation originates in the sinuses of Val- A large number of patients without associated le- salva posterior to the aorta. In situs solitus, the left sions initiate their deterioration with the presence of coronary artery arises from the right one and irrigates tricuspid insufficiency, which determines volume over- the LV with its usual pattern, whereas the right coro- load, annular dilatation and further insufficiency. This nary artery arises from the left one and is normally vicious circle precipitates ventricular failure of the sys- distributed across the RV.
Load more

Recommended publications
  • Congenitally Corrected Transposition Gonzalo a Wallis1*, Diane Debich-Spicer2,3 and Robert H Anderson4
    Wallis et al. Orphanet Journal of Rare Diseases 2011, 6:22 http://www.ojrd.com/content/6/1/22 REVIEW Open Access Congenitally corrected transposition Gonzalo A Wallis1*, Diane Debich-Spicer2,3 and Robert H Anderson4 Abstract Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has been reported to be around 1/33,000 live births, accounting for approximately 0.05% of congenital heart malformations. Associated malformations may include interventricular communications, obstructions of the outlet from the morphologically left ventricle, and anomalies of the tricuspid valve. The clinical picture and age of onset depend on the associated malformations, with bradycardia, a single loud second heart sound and a heart murmur being the most common manifestations. In the rare cases where there are no associated malformations, congenitally corrected transposition can lead to progressive atrioventricular valvar regurgitation and failure of the systemic ventricle. The diagnosis can also be made late in life when the patient presents with complete heart block or cardiac failure. The etiology of congenitally corrected transposition is currently unknown, and with an increase in incidence among families with previous cases of congenitally corrected transposition reported. Diagnosis can be made by fetal echocardiography, but is more commonly made postnatally with a combination of clinical signs and echocardiography. The anatomical delineation can be further assessed by magnetic resonance imaging and catheterization. The differential diagnosis is centred on the assessing if the patient is presenting with isolated malformations, or as part of a spectrum.
    [Show full text]
  • Subpulmonic Obstruction by Membranous Ventricular Septal Aneurysm in Congenitally Corrected Transposition of Great Arteries
    © 2013, Wiley Periodicals, Inc. DOI: 10.1111/echo.12279 Echocardiography The Windsock Syndrome: Subpulmonic Obstruction by Membranous Ventricular Septal Aneurysm in Congenitally Corrected Transposition of Great Arteries Louai Razzouk, M.D., M.P.H., Robert M. Applebaum, M.D., Charles Okamura, M.D., and Muhamed Saric, M.D., Ph.D. Leon H. Charney Division of Cardiology, New York University Langone Medical Center, New York, New York Anomalies of the membranous portion of the interventricular septum include perimembranous ventric- ular septal defect and/or membranous septal aneurysm (MSA). In congenitally corrected transposition of the great arteries (L-TGA in sinus solitus), the combination of ventricular inversion and arterial trans- position creates a unique anatomic substrate that fosters subpulmonic left ventricular outflow tract obstruction by an MSA. The combination of an L-TGA with subpulmonic obstruction by an MSA is referred to as the windsock syndrome. We report a case of windsock syndrome in a 25-year-old man which is to our knowledge the first three-dimensional echocardiographic description of this congenital entity. (Echocardiography 2013;30:E243-E248) Key words: congenitally corrected transposition of great arteries, L-TGA, obstruction, aneurysm, windsock Anomalies of the membranous portion of the (LV) into the lower pressure right ventricle (RV) interventricular septum include perimembranous rarely causes significant right ventricular outflow ventricular septal defect and/or membranous tract (RVOT) obstruction. This is because the septal aneurysm (MSA). Anatomically, MSA fre- MSA is located infracristal and distant from the quently resembles a windsock, a conical cloth pulmonic valve (PV).1 tube used to show wind direction. In congenitally corrected transposition of the In otherwise normal hearts, the protrusion of great arteries (TGA) (also referred to as L-TGA the MSA from the higher pressure left ventricle in situs solitus) the combination of ventricular Figure 1.
    [Show full text]
  • Isolated Ventricular Inversion with Situs Solitus
    Br Heart J: first published as 10.1136/hrt.37.3.293 on 1 March 1975. Downloaded from British HeartJournal, I975$ 37, 293-304. Isolated ventricular inversion with situs solitus M. Quero-Jimenez and I. Raposo-Sonnenfeld From Servicio de Cardiologia Pediatrica, Clinica Infantil La Paz, Madrid, Spain The clinical and anatomicalfindings in two patients with isolated ventricular inversion and situs solitus are described. The other 4 previously published cases are reviewed. The 6 patients with this malformation, all without pulmonary stenosis, presented a clinical picture of cyanotic congenital heart disease, associated with increased pulmonary blood flow (hypoxaemia and cardiac failure). The importance of different diagnostic tests is discussed and it is concluded that angiocardiography is the only definitive means of establishing the diagnosis. Because the physiopathological disturbance is the same as in transposition of the great arteries, both malformations should be similarly considered vith respect to diagnosis and treatment. Never- theless, the high incidence ofcertain associated malformations in cases ofisolated ventricular inversion adds to difficulty in diagnosis, and makes a good result from the Mustard procedure less likely than in transposition of the great arteries. In I966 Van Praagh and Van Praagh described a conus, conal septum, and conal free wall, as used in malformation characterized by ventricular inversion this paper. with situs solitus of the viscera and atria and nor- When present, the subaortic and subpulmonary mally
    [Show full text]
  • Juxtaposition of the Atrial Appendages* a Sign of Severe Cyanotic Congenital Heart Disease BARBARA P
    Brit. Heart 7., 1968, 30, 269. Juxtaposition of the Atrial Appendages* A Sign of Severe Cyanotic Congenital Heart Disease BARBARA P. P. MELHUISH AND RICHARD VAN PRAAGHt From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research, and the Department ofPediatrics, Northwestern University School of Medicine, Chicago, Illinois, U.S.A. Juxtaposition of the atrial appendages is an The previously published 21 post-mortem cases of apparently rare congenital cardiac anomaly in which juxtaposition of the atrial appendages were carefully the atrial appendages lie side by side, both to the reassessed (Table III). left or to the right of the great arteries, known as In view of the high incidence of transposition of the great arteries (92%) in these 42 cases ofjuxtaposition left or right juxtaposition of the atrial appendages, of the atrial appendages (Tables I-IV), they were com- respectively (Dixon, 1954). pared with a control group of 100 post-mortem cases of This abnormality now may readily be diagnosed transposition of the great arteries that were randomly by angiocardiography (Ellis and Jameson, 1963), selected, exceptthat juxtaposition ofthe atrial appendages and it is widely regarded as an ominous sign of was not present (Table V) (from Paul, Van Praagh, and severe cyanotic congenital heart disease. Beyond Van Praagh, 1968). This control study was undertaken this general impression, however, the specific types in an effort to discover the difference between trans- of cardiac malformation likely to be associated with position with and without juxtaposed atrial appendages. juxtaposition of the atrial appendages, and the rela- Sections of human embryos from Horizons 2 to 23 (Streeter, 1942, 1945, 1948) were studied histologically tive frequencies of each, remain far from clear.
    [Show full text]
  • Dextroversion, Mirror-Image Dextrocardia, and Laevoversion
    British Heart Journal, 1972, 34, Io85-Io98. Systematization and clinical study of 'dextroversion, mirror-image dextrocardia, and laevoversion Guillermo Anselmi, Simon Mufnoz, Pablo Blanco, Ivan Machado, and Maria V. de la Cruz From the Department of Paediatric Cardiology, Hospital Universitario, Caracas, Venezuela; and the Laboratory of Embryology, Instituto Nacional de Cardiologia, Mexico ; Current embryological and anatomical knowledge makes it possible to systematize, classify, and diagnose the malpositions of the heart by following a logical sequence. (i) The visceral situs is easily established by means of data provided by physical, radiological, and electrocardiographic studies. There is a constant relation between the venous atrium, greater lobe of the liver with suprahepatic segment of the inferior vena cava, and the visceral situs. (2) The direction of the apex: this indicates the heart position in a specific situs, determined by x-rays. Dextroversion is the malposition in situs solitus, as laevoversion is in situs inversus; dextrocardia is the normal position for the latter. (3) Truncoconal morphologies: there are 3 possibilities (a) the vessels derived from the truncus conus are not transposed, (b) the vessels are transposed, or (c) there is a common trunk. Truncoconal morphology is determined in each case by means of a biplane angiocardiogram. (4) The position of the ventricles is deduced from the truncoconal morph- ology, by applying the rule which establishes whether the anterior vessel or single trunk energes from the ventral ventricle; correlating the position of this ventricle with the situs, we find out whether or not there is ventricular inversion. (5) Associated malformations are diagnosed by clinical as well as haemodynamic studies, completing finally the diagnosis in every case.
    [Show full text]
  • L-Transposition of the Great Arteries
    l-Transposition of the Great Arteries What is it? l-transposition of the great arteries (also known as levo- transposition of the great arteries) is the less common type of transposition. The right and left ventricles are reversed (ventricular inversion). The aorta and pulmonary artery are also connected to the wrong ventricles. Unlike in d-TGA, the aorta receives the oxygen-rich blood from the right ventricle, and oxygen-poor blood is carried back from the body. Likewise, the pulmonary artery receives the oxygen- poor blood from the left ventricle, which pumps it to the lungs. Because the blood flows normally despite the inverted ventricles, this lesion is also called “congenitally corrected TGA.” Some children may also have ventricular septal defects or obstruction to flow into the pulmonary artery. What causes it? The cause is unknown, but genetic factors may contribute to it. How does it affect the heart? In this condition, the blood is normally routed but the right ventricle must pump at higher pressure than is normal. The right ventricular function may decline over time. How does it affect me? Babies born with l-transposition usually aren’t blue. The congenital heart defect may go undetected for a long time. It might not be diagnosed until well into adulthood when congestive heart failure, heart murmurs and abnormal heart rhythms can develop. When there is a ventricular septal defect and pulmonary valve obstruction, the baby may be blue and murmurs are usually heard. Unless these problems are fixed in childhood, an adult patient may still occasionally be blue. If l-transposition was repaired in childhood what can I expect? Most children without a VSD or pulmonary valve obstruction won’t need surgery.
    [Show full text]
  • Electrophysiology of Ventricular Inversion'
    Br Heart J: first published as 10.1136/hrt.36.10.971 on 1 October 1974. Downloaded from British Heart journal, I974, 36, 97I-980. Electrophysiology of ventricular inversion' Paul C. Gillette, Milton J. Reitman, Charles E. Mullins, Robert L. Williams, John T. Dawson, Jr., and Dan G. McNamara From The Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas, U.S.A. Electrophysiological studies were carried out in 7 subjects with angiographically proven ventricular inversion in order to determine if this technique could be used in the study of arrhythmias in such subjects. Three sub- jects with normal PR intervals on the electrocardiogram had normal low right atrium to His (LRA-H) and His to ventricle (HV) intervals at rest. With atrial pacing, I of these 3 with normal PR interval developed Mobitz II second-degree atrioventricular block. Of 2 subjects with first-degree atrioventricular block r was found to have prolonged LRA-H and HV intervals, and the other had only LRA-H prolongation. In both subjects with complete atrioventricular block, the block was below the His bundle recording site. One of these patients with complete A V block wasfound to be able to conduct through his A V conducting system during the supernormal period. This study found that useful information can be obtained by recording the His bundle potential in patients with ventricular inversion. Conduction abnormalities were found from the AV node through the His-Purkinje system. This technique may be useful in making clinical decisions in patients with ventricular inversion and complex arrhythmias.
    [Show full text]
  • Thromboprophylaxis for Congenital Heart Patients
    Thromboprophylaxis for Congenital Heart Patients Anticoagulation and Thrombophilia Clinic, Minneapolis Heart Institute®, Abbott Northwestern Hospital. Tel: 612-863-6800 | Reviewed August 2016, June 2018, July 2019 61 1. Simple, moderate, or complex congenital heart disease (CHD): follows our current guideline pre-cardioversion 2. Complex CHD with sustained or recurrent intra-atrial reentrant tachycardia (IART) or atrial fibrillation should be on long-term anticoagulation 3. Moderate CHD with sustained or recurrent IART or atrial fibrillation: long-term anticoagulation is reasonable 4. Moderate or complex CHD: vitamin K-dependent anticoagulant of choice (pending safety and efficacy data on newer agents) 5. Simple CHD with nonvalvular IART or atrial fibrillation: vitamin K-dependent anticoagulant, aspirin, or DOAC is reasonable option based on CHA2DS2VASc score and bleeding risk Complexity Type of congenital heart disease in adult patients Native disease Repaired conditions - Isolated congenital aortic valve disease - Previously ligated or occluded ductus arteriosus - Isolated congenital mitral valve disease (except parachute - Repaired secundum or sinus venosus atrial septal defect valve, cleft leaflet) without residua Simple - Small atrial septal defect - Repaired ventricular septal defect without residua - Isolated small ventricular septal defect (no associated lesions) - Mild pulmonary stenosis - Small patent ductus arteriosus - Aorto-left ventricular fistulas - Sinus venosus atrial septal defect - Anomalous pulmonary venous drainage,
    [Show full text]
  • Anatomically Corrected Transposition of the Great Arteries*
    Brit. Heart ., 1967, 29, 112. Anatomically Corrected Transposition of the Great Arteries* RICHARD VAN PRAAGHt AND STELLA VAN PRAAGHt From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research, and the Cardiology Department, Children's Memorial Hospital, and the Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois, U.S.A. It has long been doubted whether or not it is and subpulmonary) (Fig. 2) preventing fibrous contin- possible for a transposed aorta to arise from a uity between the mitral valve and either semilunar valve morphologically left ventricle, and for a transposed (Fig. 1C, D); infundibular and valvar pulmonary steno- sis with a thickened bicuspid pulmonary valve (Fig. ID, pulmonary artery to originate from a morphologi- E); I-transposition of the great arteries (transposed cally right ventricle, this having been designated aortic valve to the left of the transposed pulmonary anatomically corrected transposition by Harris and valve) (Fig. 1E and 2); transposed aorta arising entirely Farber in 1939. Such cases have been regarded as above the morphologically left ventricle (Fig. 1C, E); errors in observation (Lochte, 1898), as inexplicable transposed pulmonary artery originating completely variations of nature (Geipel, 1903), as embryologic- above the morphologically right ventricle (Fig. ID, E); ally impossible and hence non-existent (Van right aortic arch (Fig. lA); and probe-patent ductus Mierop and Wiglesworth, 1963), and as very arteriosus (Fig. ID).
    [Show full text]
  • Evicore Cardiac Imaging Guidelines
    CLINICAL GUIDELINES Cardiac Imaging Policy Version 1.0 Effective March 2, 2020 eviCore healthcare Clinical Decision Support Tool Diagnostic Strategies: This tool addresses common symptoms and symptom complexes. Imaging requests for individuals with atypical symptoms or clinical presentations that are not specifically addressed will require physician review. Consultation with the referring physician, specialist and/or individual’s Primary Care Physician (PCP) may provide additional insight. CPT® (Current Procedural Terminology) is a registered trademark of the American Medical Association (AMA). CPT® five digit codes, nomenclature and other data are copyright 2017 American Medical Association. All Rights Reserved. No fee schedules, basic units, relative values or related listings are included in the CPT® book. AMA does not directly or indirectly practice medicine or dispense medical services. AMA assumes no liability for the data contained herein or not contained herein. © 2019 eviCore healthcare. All rights reserved. Cardiac Imaging Guidelines V1.0 Cardiac Imaging Guidelines Abbreviations for Cardiac Imaging Guidelines 3 Glossary 4 CD-1: General Guidelines 5 CD-2: Echocardiography (ECHO) 15 CD-3: Nuclear Cardiac Imaging 26 CD-4: Cardiac CT, Coronary CTA, and CT for Coronary Calcium (CAC) 33 CD-5: Cardiac MRI 40 CD-6: Cardiac PET 45 CD-7: Diagnostic Heart Catheterization 49 CD-8: Pulmonary Artery and Vein Imaging 56 CD-9: Congestive Heart Failure 59 CD-10: Cardiac Trauma 62 CD-11: Adult Congenital Heart Disease 64 CD-12: Cancer Therapeutics-Related
    [Show full text]
  • Subpulmonary Obstruction Due to Aneurysmal Ventricular Septum in A
    University Heart Journal Vol. 10, No. 1, January 2014 CASE REPORTS Subpulmonary Obstruction Due to Aneurysmal Ventricular Septum in a Patient with Congenitally Corrected Transposition of the Great Arteries and Dextrocardia NAVEEN SHEIKH, SAJAL KRISHNA BANERJEE, MD. ZAHID HOSSAIN, MD. TARIQUL ISLAM, TAHMINA KARIM, ATM IQBAL HASAN, DIPAL KRISHNA ADHIKARY, MD NAZMUL HASAN, MD. HARISUL HOQUE Department of Cardiology, Bangabandhu Sheikh Mujib Medical University,Shahbag, Dhaka. Address for Correspondence: Dr Naveen Sheikh,Assistant Professor, Department of Cardiology, Bangabandhu Sheikh Mujib Medical University,Shahbag, Dhaka. Email: [email protected] Abstract: Congenitally corrected Transposition of Great Arteries is usually associated with multiple cardiac defects. Morphologic left-ventricular outflow (pulmonary) tract obstruction due to aneurysm of the membranous ventricular septum in patients with corrected transposition and ventricular septal defect is rare, but was reported in the past. This is even more uncommon in patients with dextrocardia, prompting us to document this case. Absence of the conus with resultant proximity of the aneurysm to the subpulmonary region and higher pressures in the left-sided morphologic right ventricle lead to obstruction of outflow tract in corrected transposition. Echocardiogram with Doppler interrogation and cardiac catheterization with selective cineangiography are the diagnostic tests of choice. Surgical resection of the aneurysm with patch closure of ventricular septal defect, avoiding injury to the conduction system, is recommended. Key Words: Aneurysm, Dextrocardia,Transposition of the Great Arteries. Introduction: Chest x-ray (Figure 1) revealed dextrocardia, left-sided liver Congenitally Corrected Transposition of the Great Arteries and left-to-right reversal of bronchi, indicating situs is usually associated with multiple cardiac defects.
    [Show full text]
  • Dextrocardia with Situs Inversus, Atrio-Ventricular and Ventricular-Arterial Concordance and a Left Posterior Aorta in a Preterm Twin Neonate
    Avens Publishing Group Inviting Innovations Open Access Case Report J Pediatr Child Care October 2015 Volume:1, Issue:2 © All rights are reserved by Aly et al. AvensJournal Publishing of Group Inviting Innovations Pediatrics & Dextrocardia with Situs Inversus, Child Care Atrio-ventricular and Ventricular- Chelsea Hill, David Lindsay and Ashraf Aly* Department of Pediatrics, Division of Pediatric Cardiology, arterial Concordance and a Left University of Texas Medical Branch, 301 University Blvd, Galveston, TX, 77555, USA Posterior Aorta in a Preterm *Address for Correspondence: Ashraf M Aly, MD, PhD, MSc, FACC, FAAP, Professor of Pediatrics and Maternal Fetal Medicine, Director, Pediatric and Fetal Cardiology, University of Texas, 2.210 Research Building 6, 301 University Twin Neonate Boulevard, Galveston, Texas, 77555-0361, USA, Tel: (409) 772-2507; Fax: (409) 772-5045; E-mail: [email protected] Keywords: Dextrocardia; Situs inversus; Ventricular inversion Submission: 11 August, 2015 Accepted: 12 October, 2015 Abstract Published: 17 October, 2015 In dextrocardia, the main base-apex cardiac axis is directed to the Copyright: © 2015 Hill C, et al. This is an open access article distributed right side of the chest. The majority of dextrocardia is associated with under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided situs solitus and either normally related or L-transposed great vessels. the original work is properly cited. Dextrocardia with situs inversus is rare and presents in different forms depending on the atrio-ventricular (AV) and ventricular-aterial (VA) relationship. We report a rare case of dextrocardia with situs inversus, X-ray (Figure 1) revealed dextrocardia with situs inversus, which was AV and VA concordance, a left posterior aorta and a right aortic arch further confirmed by an abdominal ultrasound.
    [Show full text]