Perioperative Anesthetic Management for Surgical Repair of an Adult with Supracardiac Total Anomalous Pulmonary Venous Communication and Pulmonary Hypertension: A case report and Mini Review

The total anomalous pulmonary venous communication [TAPVC] is a rare cyanotic congenital cardiac defect accounting for 1.5-3% of the congenital heart disease, in which pulmonary venous [PV] blood drains directly into the right side of the heart or into the systemic veins. Neonates with obstructive TAPVC may present with cyanosis, metabolic acidosis, respiratory failure, and shock. A subset of patients with unobstructed TAPVC may remain symptoms free and attain adulthood, or present with pulmonary congestion, pulmonary arterial hypertension [PAH]. The anesthetic management of either obstructed TAPVC or unobstructed with PAH can be quite challenging. The described patient is a 23-year male who presented with self – limiting single episode of chest pain, palpitations and dyspnea, diagnosed as supracardiac unobstructed TAPVC with ostium secundum atrial septal defect [OS – ASD] and PAH, who underwent successful intracardiac repair under cardiopulmonary bypass [CPB]. The protocol for the cardiac surgery during the COVID-19 pandemic for perioperative considerations and triage recommendations was strictly followed to reduce the risk of exposure to patients and healthcare workers. The objective of this case report and review is to recognize the spectrum of various clinical presentations in TAPVC, and to describe the diagnosis and perioperative management of TAPVC. Key Words: Adult Supra cardiac TAPVC, unobstructed, PAH, cardiopulmonary bypass, corrective surgery, balanced general anesthesia