TRICUSPID ATRESIA AND ITS PROGNOSIS WITH AND WITHOUT SURGICAL TREATMENT

Abstract

Forty patients with a clinical diagnosis of tricuspid atresia have been reviewed. In 1/5 of them, this diagnosis, based mainly on central cyanosis with left ventricular preponderance in the ecg and on radioscopy, proved wrong. Angiocardiography generally provided the best confirmation. Their clinical features have been reviewed. Surgical treatment was carried out in 12 patients, of whom 7 are still greatly improved after 7 to 10 years, 2 did well for 6 years but then lost ground and died within a few years, and 3 died within a year or so. Only 2 of these 7 have reached the age when they have shown that they can carry on a reasonably normal quiet life at work. The prognosis of the other 28 patients proved extremely bad as 20 of them (71%) are known to have died. All but 5 of these died within 2 years of being first seen and only one lived for more than 6 years. Clearly the bad prognosis that is recognized in infancy continues throughout childhood. Very few patients have a blood flow to the lungs that is adequate yet not too large. This may, however, happen when the ventricular septal defect is big enough to allow an adequate flow to the right ventricle that can receive it and pass it on to the lungs without further obstruction, or when the pulmonary trunk arises from the left ventricle and a suitable degree of pulmonary stenosis protects the lungs from too large a blood flow and yet allows an adequate flow. With the advance in open-heart surgery, these considerations must be borne in mind in planning a more fundamental improvement in the circulation through the heart. In the meantime, the bad prognosis suggests that all patients with tricuspid atresia, except the few with only slight disability and cyanosis, should be given the chance of a subclavian-pulmonary anastomosis this certainly improves their capacity and prognosis, though generally less than in Fallot''s tetralogy.