Supracardiac total anomalous pulmonary venous connection with cor triatriatum sinister: A rare diagnosis confirmed by saline contrast echocardiography

Abstract

In cor triatriatum sinister, the left atrium is divided by a membrane into a proximal and a distal chamber. Usually proximal chamber receives all the pulmonary veins and drains through an opening in the dividing membrane into distal chamber, which empties into left ventricle through the mitral valve. Rarely, the two chambers lack a communication and there is associated total anomalous pulmonary venous connection (TAPVC). We report a 1-month-old infant with cyanosis and heart failure, who had cor triatriatum sinister associated with supracardiac TAPVC. The case is reported for rarity of the association with a focus on contrast echocardiographic imaging.

Pulmonary Vein: Embryology, Anatomy, Function and Disease

“…In cases that the echocardiographic result alone is controversial or is suspicious of having multiple cardiovascular developmental diseases, additional imaging tools should be considered. A special case that was diagnosed as CTS with TAPVC by echocardiography combined with saline contrast technique was report in 2020 [35]. In some conditions especially when our target area is located near the heart base, the transesophageal echocardiography can provide better image resolution and details than the transthoracic echocardiography.…”

“…Three veterinary cases were found to have concurrent ASD (secundum type in 1 dog [41] and 1 chicken [44]; sinus venous type in another dog [42]) and the case of foal [45] had concurrent PFO. A special child case had been recognized recently that all of his pulmonary veins were anatomically connected to the left atrium but the blood inside actually was drained into superior vena cava via an innominate vein, therefore corresponded to the definition of supra-cardiac type of anomaly [35].…”

A case of total anomalous pulmonary venous return in combination with concealed left atrium membrane presence

“…Частота встречаемости данного порока невелика и составляет 0,1-0,4% в структуре всех ВПС (по данным североамериканского регистра) [1]. Сочетание же трехпредсердного сердца и супракардиальной формы тотального аномального дренажа легочных вен (ТАДЛВ) встречается крайне редко, найдены единичные описания [2,3]. В ряде случаев у пациентов с ТАДЛВ отмечается выбухание межпредсердной перегородки в левом предсердии (ЛП) ввиду высокого давления в правых отделах, что нередко требует дифференциальной диагностики с мембраной ЛП [2,4].…”