Single-stage repair of tetralogy of Fallot with partial anomalous pulmonary venous connection in an adult: a case report

Abstract

Abstract

Tetralogy of Fallot (TOF) with partial anomalous pulmonary venous connection (PAPVC) is rare and may be missed preoperatively, which may produce persistent right heart volume overload and contribute to right ventricular dilatation and dysfunction after TOF repair. Reports of this combination in adults with unrepaired TOF remain limited. A 24-year-old woman with congenital heart disease diagnosed in childhood presented with exertional fatigue and dyspnea relieved by squatting. Transthoracic echocardiography demonstrated TOF, a superior sinus venosus atrial septal defect, and incomplete visualization of the pulmonary veins. Computed tomography angiography (CTA) with three-dimensional reconstruction confirmed anomalous drainage of the right upper pulmonary vein into the superior vena cava (SVC). Single-stage repair was successfully performed, comprising TOF repair, a modified Warden procedure, and tricuspid valvuloplasty. Central venous pressure was 9 mmHg postoperatively, with no rhythm disturbance. Short-term follow-up indicated unobstructed venous pathways, with no residual intracardiac shunting, normal left ventricular systolic function, and no rhythm abnormalities. This case highlights the importance of careful preoperative assessment of pulmonary venous anatomy in TOF. Single-stage repair was feasible and can achieve a favorable short-term outcome.