Sub-pulmonary ventricular failure resulting from pulmonary hypertension in transposition of the great arteries palliated by atrial switch.

摘要

We would like to highlight our concerns about sub-pulmonary failure in transposition of great arteries based on two cases seen at our institution. The first case (DS) was born in 1969 with TGA and VSD. Initially treated with septostomy/PA banding, he had an atrial (Mustard) repair and debanding in 1972. He was asymptomatic until presentation in 2002 with atrial flutter, successfully treated with amiodarone. In 2003 he presented with ascites and systemic venous congestion. Transthoracic echocardiography (TTE) showed a massively dilated sub-pulmonary ventricle with PaP of 70 mm Hg on the MR jet. His RV function was mildly impaired. He did not have atrial pathway obstruction or recent thromboembolism on CT angiography. He had sudden death whilst waiting transplant assessment. Post-mortem histology confirmed pulmonary hypertension without evidence of thromboembolism. The second case (TG), born in 1968 with simple TGA, had an atrial repair (Mustard) in 1970. PA pressure was 18/6 mm Hg. Apart from two episodes of atrial flutter he remained well until 1999 when he became increasingly breathless with systemic venous congestion. TTE demonstrated marked sub-pulmonary ventricular dilatation. His systemic ventricle was mildly impaired. Iliac and femoral veins were known to be blocked following childhood surgery and transhepatic catherisation demonstrated patent atrial pathways-direct PaP was not possible. A ventilation perfusion scan confirmed multiple thromboemboli which were treatedwith warfarin. His heart failure was treated with diuretics and fluid restriction for 3 years before deterioration in 2003. Accepted for transplantation, he had a sudden cardiac death. Post-mortem examination confirmed histological evidence of long standing pulmonary hypertension secondary to thromboembolic disease.