n.s Paediatrica Indonesiana 18 128 --'- 140. May --.... June 1978.

ORIGINAL ARTICLE

Some Aspects of Tricuspid Atresia

by

THAHEr?. D]ALIL *, PALUPI SVNARNO *, OTTE]. RACHMAN• .and BAMBANG MADIYONO ~"''.

(Dep(lrtments of Cardiology* and Child Health**, Medical School, Universvty of Indonesia/ Dr. Cipto Mangunlcusumo General Hospital, f,akarta)

Abstract

D!lliring the period of 5 years, frc,m the begin:17!~ng of 1911 up to the end of 1975, ou.t of 2054 sick children seen in the Pediatric Outpatient DiJvision of the Dt?partment pf Cardiology Medical School, University of lndonesia!Dr. Cipto Mcmi!§Uinkusumo General HospitaE f,akfwrtra, 1073 patients were found to have congenital heant dit>ease. Ten out of these 1073 CHD patients have been diagnosed as Tricuspid Atresia Cln the clinioal basis, givmg .the incidence of 0,93% of aU CHD, .of whom 5 were boys ,and 5 were girls, mll.an age bei:ng 36,5% months (range 2 months to 12 years). Hemodynamic ,st.udies have been .done .to 3 'of the 10 patients, all of which confirmed the dnagnosis. Two childrdf.\ went to s.urgery. Anatomic and radiologic classification 'and types of the disease in those 3 children and some cUniaa:l 1aspects .and hennody;namtlc ,s~udies are di;wussed. It is stressed that early recogliition !Nld surgical intetrvetntz'on are indioated, and still should be vmproved.

Received 19th. February 1977.

.. TRICUSPID ATRESIA 129

Introduction and 2 types in the 3rd group with 1~ trarrsposftion, (a) with· Pulmonary/Sub~ Tricuspid Atresia.· as an anatomical pulmonary stenosis, (b) with Sub-aortic entity was recognized as early as 1823 stenosis. Recently Dick et al. (1975) iri by Holmes, as cited by Dick et al. the review of their 101 cases added 3 (1975). Since then many publications of groups of radiological classification to this disease as a clinical entity, have each of the 3 types of anatomical classi­ been described in textbooks as well as fication described by many authors befo­ in various medical jour.nals. re so that they had 9 1types iri all. Radio­ logical groups they mentioned are: It may be defined as a congenital group-A with decreased lung vascularic complete absence of the right sided atri­ ty, group-B with .normal or increased oventricular valve, with hypoplasia of lung vascularity, and group"C with cha­ the right , usually associated nging lung vascularity. . . with patency of the interatrial septum and hyperplasia of the and The prevalence of this disease ac­ counts· for approximately 1 ·to 3% of all left ventricle. (Kjellberg et al. 1955; congenital defects in ·various cen­ Keith and Vlad 1967; Nadas and Fyler ters (Friedberg, 1972; Hanafiah et al., 1972). Variations of clinical features 1965; Maemunah S.A. et al., 1974; have made many authors to differentiate Kaplan, 1969; Nadas et al., 1972). The various anatomical classifications of this most frequent type reported in liter­ disease. ature is of the type I an11tomy (70% ), Edward and Burchell (1949) intro­ while group-A was said to be the :most duced 2 groups, with and without common radiological group (62%). transposition of the great vessels, and Prognosis of Tricuspid Atresia was they . described 2 types in each group. said to be very poor. It was generally Keirth and Vlad (1967) amplified this considered as an anatomically uncorrec­ anatomical classification to include 8 table lesion. types in all: 3 types in the 1st group without transposition, (a) with Pul­ In a review of the age of death in 111 monary Atresia and intact ventricu­ cases Keith and Vlad (1967) disclosed lar septum, (b) with Pulmonary hy­ that 49.5% died before the age of six poplasia and small ventricular septal months; 66% at o.ne year of age, while defect, (c) with normal Pulmonary ar­ only 10% survived over the age of 10 tery and large ventricular septal defect; years. A later report by Dick et al. 3 types in the 2nd group with d- trans­ (1975) mentioned that their patients of position, (a) with , the most frequent category, type I group (b) with Pulmonary I Subpulmonary ste­ A, without surgery had only 10% chan­ nosis, (c) with large , ce of survival to 15 years while sur- 130 THAHER DJALIL ET AL.

gical intervention improved chance of studies were performed by two of the survival to 15 years of age to 50%. authors. They also concluded that the continued need of early surgical palliation for this Criteri~a for diagnosis disease are indicated. , Many authors have described in va­ Anastomosis of the right auricular rious textbooks and other publications appendage to the pulmonary artery as that Tricuspid Atresia should be highly described by Hurwitt et al. (1955), has suspected on the basis of clinical symp­ been developed to right -pulmo­ toms and signs, radiological and electro­ nary artery allograft by Fontan and cardiographic abnormalities. Hemodyna­ Baudet (1971). Stanford et al. (1973) mic studies with cardiac catheterization followed the later procedure and also and angiocardiography may confirm the described it as a corrective surgical repa­ diagnosis and show various anatomical ir for Tricuspid Atresia. Many experts types. Direct sight during surgery will are of the opinion that this new surgical certainly also give more information. approach may result in a more prolon­ At last, autopsy will verify the. precise ged survival. diagnosis, including the anatomical types of the lesion, through macroscopic It is the purpose of this study to learn and microscopic examination. and to present some aspects of Tricuspid Atresia in ·our clinic in order to ask Clinical more attention and to increase awareness Symptoms and signs of early , to this clinical entity, in connection with central and progressive, with or without the hopeful reports that early recogniti­ murmurs, clubbing, frequent episodes- of on and surgical intervention may greatly hypoxic spell, growth retardation, fati­ improve its prognosis. gue, squatting, and other symptoms and si~ns of cyanotic Congenital Heart Dise­ Materials and method ase or sometimes symptoms and signs of congestive heart failure. This study has been done retrospec­ tively. Medical records of patients in · Radi10logical abnormalities, The Pediatric Outpatient Division of The cardiac contour shows right heart the Department of Cardiology in the border or prominent right atrial, bor· period of 1971 - 197 5; and those of der, slight to moderate cardiomegaly, patients in the Inpatient Division, in blunt apex with a concavity or lack of the Hemodynamic Division and in the prominence of Pulmonary Artery area. Surgical Division of The Department of The pulmonary vasculature is usually Cardiology in the same period were se­ decreased (75%), except when associated l~cted and reviewed. Hemodynamic .with Transposition :oJ .the 'Great Arteries TRICUSPID ATRESIA 131

or large Ventricular Septal Defect. (Elli­ c) radioluscent triangular Right Ventri­ ot and Schieber 1971; Franch. 1969 and cular window between the medial Nadas and Fyler 1972). edge of the shadow cast by the pro­ minent Inferior Caval Vein and the Electrocardiographic abnormalities. shadow of the Left Ventricle on the Many authors (Taussig, 1960; Perloff, other. Baron (1971) mentioned this 1970; Sodi Pallares, 1970; Elliot, 1971; sign ,as pathognomonic for Tricuspid Friedman, 1971 and Marriot, 1975) Atresia. Selective angiocardiography described that some ECG abnormalities from the Left Ventricle will show are very specific for Tricuspid Atresia, anatomical classification. Keith and those are : ' , hori­ Vlad (1967) in their series of 143 zontal electrical position, Left Ventricu­ cases mentioned the figures as fol­ lar Hypertrophy, Right Atrial Hyper­ lows: trophy (P-congenitale), or Biatrial Hy­ pertrophy with the right side predomi­ a. 13 1 II 28% nant (P~tricuspidale). b. 73 J69% ~·. 1~ lj c. 13 c. 26 Hemodynamic studies. III a. 1 / 3 Kjellberg (1955), Keith et al., (1967), b. 3 ) % Diehl et al. (1968) and Nadas and Fyler Management ( 1972) ,described that on catherization, catheter cannot go directly from the Right A. Medical : especially for symp­ Ventricle. The route of catheter is from tomatic purposes. the Right Atrium to the Left Atrium B. Surgical : and then to the Left Venricle. The Right Atrial Pressure, either a wave or mean 1. Palliative surgery : pressure, exceeds those of Left Atrium a. To increase Pulmonary blood by varying degrees. Systemic 02 satura­ flow when reduced : with arte­ tion from Left Atrium downward is rial shunting (W aterston/Potts/ lower than normal. Blalock Taussig procedme), or Angiocardiography from Right Atrium with venous shunting (Glenn will show some special features: procedure). b. To remove intracardiac barrier a) typical sequence of opacification which interfere with free flow of from the large Right Atrium to the blood (Rashkind/Blalock Hanlon Left Atrium, to the Left Ventricle, procedure). and then to the Great Arteries; c. To diminish Pulmonary blood b) specific non-opacification of the flow, when excessive (Main Pul­ inflow tract of the Right Ventricle; monary Artery Bandjng) . THAHER DJALIL ET AL. 132

2. Corrective (?) surgery : 1073 patients were found to have con­ genital heart disease (CHD), Fontan Baudet procedure which consists of : (a) Aortic allograft con­ Ten out of these 1073 CHD patients duit is inserted between Right Atrium have been diagnosed as Tricuspid Atresia and Left Pulmonary Artery, (b) Pul­ on the clinical basis (0.93%), of whom monary valve allograft is sutured at were 5 boys and 5 girls, mean age 36.5 the Right Atrium - Inferior Vena months (range 2 months - 12 years). Caval orifice, (c) Superior Vena Ca­ Only 3 of the 10 patients underwent val - Right Pulmonary Artery shunt further hemodynamic studies, all of (Glenn) is also carried out, (d) clo­ \vhich confirmed the diagnosis, and 2 of sure of if these 3 had surgical intervention. In present. thiE connection for further detailed description we prefer to limit the discus­ Results sion to only 3 catheterized children : ( 1) From 2054 sick children seen in the Sa, female, 5 years; (2) Su, :nale, 6 Pediatric Out-Patient Division of the months; (3) J.K., male, 12 years. See Department of Cardiology in that period, tables 1-2 and figures 1-3 below. TABLE 1 : Clinical findings

6 Sa, ~, 5 Su, ;S, 12 J.K. ;S, 12

HISTORY: antenatal normal normal normal n a t a 1 normal normal normal early progressive central cyanosis ++ ++ + increasing dyspnoea ++ ++ + feeding difficulty ++ ++ + hypoxic spells ++ ++ + squatting ++ ++ + growth retardation ++ ++ mild

EXAMINATION : undernourished ++ ++ mild cyanosis ++ ++ mild clubbing ++ + + bulging of the chest RV impulse single second heart sound (S2) single single harsh harsh pulm. ej. syst. ~urmur harsh heart fail~re TRICUSPID ATRESIA 133

TABLE 1: Clinical findings (continued)

6 Sa, ';>, 5 Su, ~, J.K., iL 12 12

Chest X Ray sl. cardiomegaly sl. cardiomegaly sl. cardiomegaly

(see also left Aortic arch left Aortic arch left Aortic arch fig. 1- 3) widened Aorta widened Aorta widened Aorta

deer. lung vase. straight right heart deer. lung vase. border

ECG LAD -40° LAD .15° LAD -45°

(see also fig. P tricuspidale P tricuspidale P tricuspidale

1, 2, 3). LVI-I, qV5Vo absent LVH, qVGVo absent LVH, qV5Vo absent

Laboratory Hb 18 gm% Hb 17.5 gm% Hb 17,5 gm%

Ery 6.82 mill Ery 6.35 mill Ery 5 .68 mill

Ht 78% Ht 62% Ht 55%

Neurology EEG abnormal EEG dubious re- EEG abnormal (slo-

(irritative) cord wering activity)

Ophthalmology retinopathy· grade I not examined retinopathy grade I

ec. polycythemia ec. polycythemia 134 THAHER DJALIL ET AL.

TABLE 2: Hemodynamic studies

6 Sa, ~. 5 Su, i!>, J.K., i!>, 12 12

CATHETERIZATION :

route of catheter RA ~LA ~LV RA ~LA~ LV RA ~LA~ LV pressure RA>LA RA>LA RA>LA 02 saturation :

RA 41% 56% 70% LA 45% 64% 73%

LPV 100% 95% 96% LV 50% 62% 72%

ANGIOCARDIOGRAPHY:

typical sequence RA ~LA -> LV RA =li> LA~ LV RA ~LA~ LV

~ Aorta ~ Aorta ___, Aorta

RV window present present present Pulmonary Artery poor poor poor filling retrogradely

LAD Left Axis Deviation sl. cardiomegaly = slight cardiomegaly LVH Left Ventricular Hypertrophy Ht Hematocrit RA Right Atrium EEG Electroencepalography LA Left Atrium age 5 = 5 years 6 LV Left Ventricle 6 months 12 LPV Left Pulmonary Vein 12 12 years. TRICUSPID ATRESIA 135

FIG. 1 : ECG and 2 pieces of angiocardiographic pictures of case 1 :Sa, ~, 5 years THAHER DJALIL ET AL. 136

FIG 2: ECG and 2 pieces of angiocardiographic pictures of case 2 : Su. (1), 6 months TRICUSPID ATRESIA 137

~--

FIG 3 : ECG and 2 pieces of angiocardiographic pictures of case 3 f.K. i!!, 12 years

.. 138 THAHER DJALIL ET AL.

On the basis of clinical finoings as this study, found to be 0.93% of Conge­ shown in table 1 the diagnosis of nital Heart Disease, is almost similar to Tricuspid Atresia in the three described the figures reported by various authors, patients, Sa.

notic Congenital Heart Disease may sig­ Tay et al., 1974). But the comparison of nificantly reduce the incidence of this surgical f medical mortality between this complication. clinic and other centers abroad could not be made because of the too small The prognosis of Tricuspid Atr>:!sia is said to be very poor. Without surgery quantity of material here. prospects for long .term survival are Anyhow we have to conclude that early poor, and death is usual within the first recognition and appropriate surgical in­ year of life, especially in the group tervention should be stressed and still with diminished pulmonary blood flow. should be improved in connection with

Shunt operations are reported to incre­ recent 1reports from abroad that the poor ase longevity, but to what extent is un­ prognosis of this clinical entity, in that known. New surgical approaches such way, may be greatly improved .. us right atrial-pulmonary arterial anas­ tomosis a.nd the may Acknowledgement rc.sult i.n a more prolonged survival. The authors wish to thank Dr. Mae­ The death of the 2 patients under­ munah S. Affandi, The Head of The Pe­ going surgical intervention, were pre­ diatric Outpatient Division of the De­ sumably, one due to complication of partment of Cardiology,. and concomit­ Glenn procedure, while the other due tantly Head of The Cardiologic Division to W aterston procedure. This kind of of the Department of Child Health, for surgical death was also encountered in her kindness, and assistance in the. pre­ every center abroad (Nadas et al.,1972; paration of this article.

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