Case Report of a Gore-Tex TCPC conduit dissection causing severe stenosis

Background The patient is a 15-year-old male with situs inversus, dextrocardia, bilateral superior caval veins, atrioventricular discordance with a single outlet, large perimembranous ventricular septal defect, aortic override, pulmonary atresia and right aortic arch. The complex anatomy with a VSD distant from the aorta (unsuitable for baffling to the aorta) meant he was unsuitable for biventricular repair and proceeded down a univentricular palliation pathway. Case Summary Post TCPC his clinical course was uneventful until the age of five when he developed fatigability with desaturation. An accessory hepatic vein was surgically banded with improved saturations and exercise tolerance. At the age of fifteen CMR was performed to investigate borderline saturations and as work up for transition to adult services. CMR and cardiac CT imaging demonstrated an eccentric thrombus causing stenosis of the extra cardiac conduit and a thrombus outside of the lumen contained by the thin outer membrane of the Gore-Tex conduit. Collateralisation suggested this was longstanding. Cardiac catheterisation demonstrated a 4x6mm stenosis at the junction of the conduit with the pulmonary arteries. The region was successfully balloon dilated and stented with a 34 mm-long Cheatham Platinum (CP) stent, with no complications. Discussion To date this is the first documented case of a dissecting thrombus of a Gore-Tex graft in the literature. This case emphasises the need for anticoagulation and serial cross-sectional imaging (CT or CMR) in Fontan patients with prosthetic grafts throughout a patients’ lifetime.

Isolated ventricular inversion with hypoplastic right ventricle and pulmonary stenosis in an adolescent with situs solitus: a rare combination

Isolated ventricular inversion with situs solitus is a severe and rare congenital cardiac malformation characterised by an atrioventricular discordance but with ventriculo-arterial concordance. Here, we present the rare case of an adolescent with isolated ventricular inversion and hypoplasia of the left-sided morphological right ventricle and pulmonary stenosis, a first of its kind to be reported in the literature.

Cardiac CT in criss-cross heart

A criss-cross heart is an uncommon congenital rotational anomaly. It accounts for less than 0.1% of all congenital heart defects. The anomaly is characterized by crossing of the atrioventricular connections caused by rotation of the heart about its long axis. It is commonly associated with diverse cardiac defects. Cardiac CT imaging of criss-cross heart is sparse. We present a case of 1-year-old child with chief complaints of bluish discoloration of the body and fast breathing. Cardiac CT revealed atrial situs solitus, criss-cross-atrioventricular connections, atrioventricular discordance, double outlet right ventricle and dextro-malposed great arteries (Van Praagh S,D,D).

Staged Surgical Management of Anatomically Corrected Malposition of Great Arteries

A nine-month-old girl diagnosed with anatomically corrected malposition (atrioventricular discordance, ventriculoarterial concordance, and malposed great arteries) complicated by multiple ventricular septal defects (VSD) and multifactorial left ventricular outflow tract obstruction (LVOTO) presented for management after pulmonary artery banding. She underwent interim palliation in the form of bilateral cavopulmonary connections, a modified Damus-Kaye-Stansel-type anastomosis, and subsequent staged one-and-a-half ventricle repair (1.5 repair) at the age of three years in the form of VSD closure, hemi-Mustard, and LVOTO resection.