Total anomalous pulmonary venous return (TAPVR) is a rare condition, accounting for 1% of all congenital heart disease, with 50% being of the supracardiac and 20% of the cardiac type. The authors describe the cases of two infants with TAPVR and similar clinical manifestations.
Case 1A three-month-old infant was referred with a diagnosis of lower respiratory tract infection with moderate respiratory distress and cyanosis. Physical examination revealed bilateral pulmonary rales and a low-grade systolic murmur (II/VI) at the upper left sternal (ULS) border. The echocardiogram revealed marked dilation of the right chambers, pulmonary trunk and superior vena cava, and a large atrial septal defect (ASD) with right-to-left shunt. A retroatrial conduit was evident with connection to the innominate vein through a vertical vein. A presumptive diagnosis of supracardiac TAPVR was made.
Case 2A two-month-old infant with poor weight gain and a systolic murmur was referred for cardiac evaluation. On examination he was in moderate respiratory distress and cyanotic, with minimal response to supplementary oxygen. A grade II/VI ULS systolic murmur was present. The echocardiogram revealed a small left atrium, dilated right chambers and pulmonary trunk, and a small ASD with a right-to-left shunt. A retroatrial conduit emptying into the coronary sinus was also evident (Figure 1). These findings were suggestive of a cardiac type TAPVR.
Both diagnoses were confirmed by cardiac computed tomography (Figures 2 and 3).
Successful surgical repair was performed, with subsequent clinical improvement and normalization of right heart chamber dimensions.
Ethical disclosuresProtection of human and animal subjectsThe authors declare that no experiments were performed on humans or animals for this study.
Confidentiality of dataThe authors declare that no patient data appear in this article.
Right to privacy and informed consentThe authors declare that no patient data appear in this article.
Conflicts of interestThe authors have no conflicts of interest to declare.
