Total anomalous pulmonary venous connection: Results of surgical repair of 100 patients at a single institution

Abstract

Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.

Risk Factors of Total Anomalous Pulmonary Venous Connection Surgery

“…Preoperative pulmonary vein obstruction was reported about 20% to 60% of TAPVC patient [1,2]. The preoperative PVO can be diagnosed by echocardiography with fair sensitivity and specificity [9].…”

“…Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease that occurs in only 1% to 3% of children with congenital cardiac anomaly [1,2]. After birth, these patients usually develop progressive pulmonary congestion and heart failure sign due to pulmonary venous return flow obstruction and inadequate cardiac output [3].…”

Surgery-related complications and their management in total anomalous pulmonary venous return during intensive care unit stay

“…[10][11][12] Karaci et al [9] reported that 10% of the patients (n=6) in their study with isolated TAPVR had a pulmonary hypertensive crisis and that in spite of intensive therapy, two of them died. In another study composed of 100 patients, Kelle et al [12] observed a pulmonary hypertensive crisis in 20 patients, and three of these died as a result of the crisis.…”

“…[1][2][3] In the study by Karamlou et al [6] that included 377 TAPVR patients with complex pathologies, they found that the mortality rate had decreased from 50% in the 1960s to 5% in the 1970s. In another single-center study made up of 100 patients, Kelle et al [12] reported a mortality rate of 5% in patients who underwent biventricular repair. Moreover, Song and Yoon [19] reported a mortality rate of 8.3%.…”

“…[10][11][12] Prevention and management strategies for pulmonary hypertensive crises include intraoperative vertical vein opening, deep sedation, muscle paralysis for 24-48 hours, maintaining the pCO 2 between 30 and 35 mmHg, pharmacological therapy (e.g., sildenafil, phenoxybenzamine, ilioprost, and iNO), the use of a selective pulmonary vasodilator, and ECMO life support. [10][11][12] Karaci et al [9] reported that 10% of the patients (n=6) in their study with isolated TAPVR had a pulmonary hypertensive crisis and that in spite of intensive therapy, two of them died. In another study composed of 100 patients, Kelle et al [12] observed a pulmonary hypertensive crisis in 20 patients, and three of these died as a result of the crisis.…”