Tricuspid atresia. Clinical course in 62 cases (1967--1974).

Abstract

Tricuspid atresia constitutes 1 to 3 per cent of all congenital cardiac lesions (Nadas and Fyler, 1972). Diagnosis is usually obvious from electrocardiographic, radiological, and echocardiographic features. Long-term survival without palliative procedure is unlikely (Campbell, 1961). Recent advances in surgical treatment (Fontan and Baudet, 1971;Kreutzer et al., 1973) have prompted us to review the outcome in a group of patients with tricupsid atresia in order to identify those suitable for a Fontan procedure.In all the cases there was atresia of the right ventricular orifice (Anderson et al., 1977 Received for publication 23 January 1978 included in the study. The ventricular morphology of the larger chamber was that of the left ventricle and will be referred to as the left ventricle. The outlet chamber (Anderson et al., 1977) had the morphology of a right ventricle and will be referred to as the right ventricle. Burchell, 1949).

The Ventricular Septal Defect in Tricuspid Atresia: Its Natural History and Surgical Importance

“…51 The scope of these papers is such that the authors could not provide further details on these patients. [49][50][51] Partial closure of VSD in tricuspid atresia has been reported previously. 25,30,41,52,53 Gallaher and Fyler reported three patients with tricuspid atresia who had cardiac failure and pulmonary plethora during infancy; over varying periods of time they became more cyanotic; cardiomegaly and pulmonary blood flow decreased; finally they required palliative shunt procedures to relieve symptoms of hypoxia.…”

“…[49][50][51] Dick et al found one patient with complete closure (documented by autopsy) among 101 patients with tricuspid atresia reviewed. 49 Patel and associates, in a review of 62 patients with tricuspid atresia, found two cases with complete spontaneous closure of VSD; both closures were documented by angiography.…”

“…49 Patel and associates, in a review of 62 patients with tricuspid atresia, found two cases with complete spontaneous closure of VSD; both closures were documented by angiography. 50 Somerville, in a review paper on changing form and function in congenital heart disease, observed that complete closure of VSD occurred in 7 out of 74 patients with tricuspid atresia followed at her institution. 51 The scope of these papers is such that the authors could not provide further details on these patients.…”

Designing therapeutic strategies for patients with a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs

“…Only 5 of these 14 lived beyond the 2nd year of age, with 6 dying within the first year of life, 1 at the time of banding and the other 5 without having any form of palliation and dying in severe congestive heart failure. Patel et al 92 also reported the clinical course of patients with tricuspid atresia seen between 1967 and 1974. Of 6 patients requiring banding, 2 died.…”

“…Any number of surgical manoeuvres have been used to palliate the patient with a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood into the lungs. [6][7][8][9][10][11]27,31,33,[35][36][37][38][39]42,43,[45][46][47][48][49][50]62,66,[85][86][87][88][89][90][91][92][93][94][95][96]115,116,122 Some of these are now of historical interest only, and were performed either prior to or at the beginning of the era of Fontan surgery and thus before we became aware of the significance of myocardial hypertrophy. With increasing recognition and concern about the impact of obstruction to the systemic ventricular outflow tract and the development of myocardial hypertrophy, a number of surgical "solutions" were suggested to treat the subaortic stenosis, or indeed to prevent its occurrence ( Table 2).…”