Total Anomalous Pulmonary Vein Connection: Diagnosis, Management, and Outcome

Abstract

Total anomalous pulmonary venous connection (TAPVC) is a congenital cardiac malformation that requires surgical therapy. The majority of patients will present either in the neonatal period or early infancy, with cyanosis and signs of cardiac failure (eg, tachypnea, feeding difficulties, diaphoresis, failure to thrive). Surgical repair is best carried out on diagnosis, as an elective procedure, to avoid potential long-term problems associated with right heart volume and pressure overload, or long-standing cyanosis. Occasionally, in severely sick patients with obstructed forms of pulmonary venous drainage, emergency surgical repair may be required. The operative mortality for isolated TAPVC using current management techniques is less than 10%, and the long-term outlook for the majority of patients is excellent. In contrast, patients with associated complex cardiac malformations have a less favorable prognosis, determined both by the need to palliate the associated defects and the often underdeveloped pulmonary vascular bed. The major long-term complication is pulmonary venous obstruction, which may be recurrent in up to 10% of patients. This requires aggressive management strategies, involving reoperation, and balloon angioplasty or stent implantation. The prognosis for these patients is determined by the number of vessels involved and the degree of stenosis, which can often be recurrent and progressive. Late tachy- and bradyarrhythmias have also been described, requiring antiarrhythmic drug therapy or pacemaker implantation, emphasizing the need for continued long-term follow-up of all patients with TAPVC.