Early- and intermediate-term results of surgical correction in 122 patients with total anomalous pulmonary venous connection and biventricular physiology

Abstract

BackgroundWe retrospectively reported our 26-year experience with operative repair of total anomalous pulmonary venous connection (TAPVC) with biventricular physiology.MethodsBetween December 1982 and December 2008, 122 TAPVC patients with biventricular heart underwent surgical repair in our department. Moderate or deep hypothermia was induced at the time of cardiopulmonary bypass (CPB). Follow-up was conducted for 5 postoperative years. Surgical outcomes of early and intermediate deaths after TAPVC repair were retrospectively analyzed.ResultsSix deaths occurred operatively; and three deaths, during follow-up. The 5-year survival rates after TAPVC repair was 92.6 %, without gradient across the anastomosis. The survival rate of the patients who were younger was 78.8 %, significantly lower than those older than 1 year. It was also lower in those who were less than 6 kg in weight. Three patients died during follow-up. Three patients died of ventricular arrhythmia, right heart failure, and pneumonia, respectively, during follow-up. If the left atrium pressure was higher than 15 mm Hg, the snare of the vertical vein was loosened after CPB ceased in the patients with supracardiac connection. It decreased from 21 ± 5 to 13 ± 3 mm Hg. The vertical vein was ligated in 57 cases and left open in 20 cases. A patient with an intact vertical vein had a large shunt and was cured by intervention afterward. Supraventricular arrhythmia occurred in 19 patients with the supercardiac type repaired through a biatrial incision. One patient died of ventricular arrhythmia, and none of the remaining patients had arrhythmias.ConclusionSurgical treatment of TAPVC carried a low operative risk and had satisfactory immediate and intermediate results. Age younger than 1 year and weight less than 6 kg were risk factors. It was a good choice to leave the vertical vein open in the patients with a left atrial pressure higher than 15 mm Hg.

Long-Term Transplant-Free Survival After Repair of Total Anomalous Pulmonary Venous Connection

“…Long-term survival, causes of death and risk of organ transplantation remain largely undefined. Most contemporary reports addressing these issues are from small, individual centers [3, 4] or national registries from countries with state-directed health care systems [5, 6]. The use of linkage strategies with current government-sponsored registries to ascertain outcomes of specific patient groups has prompted increases in financial support to address critical clinical gaps in the understanding of their long-term outcomes [7, 8].…”

Perioperative and Anesthetic Considerations in Total Anomalous Pulmonary Venous Connection

“…43,44 Low weight, young age, elevated pulmonary vascular resistance, mixed-type TAPVC, single-ventricle physiology, and aortic crossclamp time >60 minutes are risk factors for mortality. 4,23,42 In this series, late mortality at a median follow up of 3 years was 24%. 4 Common postoperative complications include pulmonary hypertensive crisis, low cardiac output, renal failure, and arrhythmia, as well as other standard sequelae of cardiac surgery.…”

“…Ventricular arrhythmias are uncommon. 42 Particularly in TAPVC with PVO, the lungs are edematous with dilated lymphatic channels and thick alveolar walls. 13 Thus, several days of postoperative mechanical ventilation should be anticipated.…”

Diagnosis and Management of the Unligated Vertical Vein in Repaired Total Anomalous Pulmonary Venous Connection

“…Percutaneous device closure of the VV after confirmation of unobstructed pulmonary venous return to the left atrium is a safe and effective approach in patients with right-sided chamber enlargement. We know that in patients with repaired TAPVC, the VV may be patent in up to 60% of them 7 and ongoing left-to-right shunt may increase the risk of pulmonary vascular disease. Hence, it is essential for clinicians to know whether the vertical vein (VV) was ligated or left patent at the time of surgery.…”