Dear Sir, We read with interest the article by Pal et al. entitled “Percutaneous transhepatic atrial septal defect closure in a child with total anomalous vena caval connection” published in Annals of Pediatric Cardiology.1 The authors are to be congratulated for successfully performing transhepatic intervention in a child with complex systemic venous anatomy and for highlighting the distinction between systemic venous connection and systemic venous drainage. However, we believe the terminology used requires further clarification, particularly regarding the use of “totally anomalous systemic venous connection.” Although the authors correctly emphasize the conceptual difference between connection and drainage, their discussion subsequently introduces terms such as “venoatrial drainage” and “physiologically concordant connection.” In our view, consistent use of the term drainage, with explicit anatomic description, is both sufficient and clearer in this already uncommon and conceptually challenging group of anomalies. In the reported case, all systemic venous blood ultimately drains into the right atrium via a roofed coronary sinus, resulting in normal systemic venous return to the pulmonary circulation and absence of right-to-left shunt or arterial desaturation. While the systemic venous connections are anomalous, the drainage is normal. Equating this anatomy with a totally anomalous systemic venous connection may therefore be misleading. As emphasized in our earlier publications,2,3 the defining feature of a true totally anomalous systemic venous connection is abnormal systemic venous drainage, typically to the left atrium or pulmonary venous pathway, resulting in a right-to-left shunt and arterial desaturation. Anomalous systemic venous connection with preserved right-sided drainage represents a distinct entity and should be described as such. For a case to be labeled totally anomalous systemic venous connection, the coronary sinus must also connect to and drain into the left atrium. Part of the confusion in this field arises from the inconsistent use of the terms “connection” and “drainage.” To address this conceptual ambiguity, we recently proposed an anatomic–physiological classification of anomalous systemic venous drainage.4 This framework explicitly describes both connection and drainage, separating anomalous systemic venous connections with normal drainage from those with abnormal drainage Table 1. From this perspective, the present case represents an anomalous systemic venous connection with normal drainage rather than a true, totally anomalous systemic venous connection with anomalous drainage.Table 1: Anatomic–physiologic classification of systemic venous anomaliesCareful and consistent terminology grounded in anatomy and physiology is essential for accurate communication, teaching, and reporting. This case underscores that anomalous connections do not always imply abnormal drainage, a distinction fundamental to avoiding confusion in the description of systemic venous anomalies. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Gupta et al. (Thu,) studied this question.