Sinus venosus atrial septal defect with partial anomalous pulmonary and hepatic venous drainage: a case report

BACKGROUND: Sinus venosus atrial septal defect is an uncommon congenital cardiac anomaly frequently associated with partial anomalous pulmonary venous drainage. The coexistence of this defect with anomalous hepatic venous drainage, cor triatriatum, and two arterial ductal connections is extremely rare and may create diagnostic and surgical challenges. CASE SUMMARY: An eleven-month-old female infant with tachypnoea and growth retardation was found to have sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage and anomalous hepatic venous drainage to the coronary sinus on CT angiography. Cardiac catheterisation demonstrated a moderate left-to-right shunt and mildly elevated pulmonary artery pressure. Intraoperative assessment unexpectedly revealed two arterial ductal connections and cor triatriatum. Surgical management consisted of closure of the atrial septal defect, rerouting of pulmonary veins, and ligation of both arterial ductal connections. Hepatic venous drainage was left unaltered due to low flow and lack of hemodynamic effect. Postoperative recovery was uneventful. CONCLUSION: This rare constellation of anomalies highlights the importance of advanced imaging, careful preoperative planning, and intraoperative vigilance for unanticipated findings in CHD.