Total Anomalous Pulmonary Venous Connection Treatment & Management

Updated: Dec 29, 2020
  • Author: Allen D Wilson, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Medical Care

No catheter-corrective treatment is possible for total anomalous pulmonary venous connection (TAPVC), although atrial septostomy is used in some patients when the foramen ovale is restricted and corrective surgery is delayed for some reason. Catheter placement of a stent has been reported for pretreatment of obstructed vertical vein prior to surgery. [6, 7, 8] If a vertical vein is left patent postoperatively and significant shunt persists it may be possible to close this vessel with an Amplatzer PDA device. [9, 10]

Surgical repair is used as treatment for total anomalous pulmonary venous connection whenever it best serves the individual patient. Stabilizing the patient prior to surgery as much as possible from a cardiovascular and metabolic standpoint is important. In a newborn with obstructive total anomalous pulmonary venous connection, stabilization often involves mechanical ventilation, correction of acidosis, inotropic support, and administration of prostaglandin E1 for patency of patent ductus arteriosus and, in patients with total anomalous pulmonary venous connection type III, for patency of the ductus venous.

Nitric oxide may be useful as a pulmonary dilator postoperatively in patients experiencing episodic pulmonary hypertension that is affecting cardiac output. Reports indicate that magnesium sulfate is a useful pulmonary vasodilator in these patients. Extracorporeal membrane oxygenation (ECMO) may be life saving in some patients. If transesophageal echocardiography is used intraoperatively in infants with pulmonary vein obstruction, waiting for probe insertion until after chest is opened may be safer. [11]


Surgical Care

The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. In patients with a supracardiac or infracardiac connection, the common pulmonary vein is opened wide and connected side to side to the left atrium. The foramen ovale is closed, and the ascending or descending vein is usually ligated. In a cardiac connection (to right atrium or coronary sinus), the atrial septum is resected partially and a new septum is surgically created, directing pulmonary veins to the left atrium. A coronary sinus may be separately tunneled to the right atrium or left to drain with the pulmonary veins to the left atrium.

In a retrospective study, Shi et al investigated the outcomes of repair in a pediatric cohort of 768 patients with total anomalous venous connection (TAPVC) who underwent conventional repair (n = 690) or repair with a sutureless technique (n = 78). The investigators found that a younger age at the time of repair (P = 0.001), mixed (P = 0.004) and infracardiac (P = 0.035) TAPVC, prolonged cardiopulmonary bypass time (P <  0.001), and longer duration of ventilation (P = 0.028) were associated with mortality. Risk factors for recurrent pulmonary venous obstruction included preoperative pulmonary venous obstruction (P <  0.001), infracardiac TAPVC (P <  0.001), mixed TAPVC (P = 0.013), and prolonged cardiopulmonary bypass time (P <  0.001). [12]