Early and Intermediate Term Results for Surgical Correction of Total Anomalous Pulmonary Venous Connection

Abstract

Background: This retrospective study evaluated factors associated with mortality and morbidity in patients having functionally biventricular heart (FBH) and functionally univentricular heart (FUH) undergoing total anomalous pulmonary venous connection (TAPVC) repair. Methods: We reviewed medical records retrospectively and identified 72 patients undergoing TAPVC repair between July 2002 and December 2010; and 62 patients had FBH and 10 patients had FUH. The median age and weight at repair was 9.1 months (range, 8 days–16 years) and 6.7 kg (range, 2.5–57 kg). The TAPVC anatomy was supracardiac in 41 (56.9%), cardiac in 18 (25%), infracardiac in eight (11.1%), and mixed in five patients (6.9%). Results: There were seven early deaths (2 FBH [25%] and 5 FUH [62.5%]) and one late death (FUH). Presence of FUH (p < 0.01), heterotaxy syndrome (p = 0.03), young age (p < 0.01), low weight (p < 0.01) and long CPB time (p = 0.04) at the time of surgery were statistically significant risk factors for mortality. Morbidity was related to long duration of postoperative mechanical ventilation, intensive care unit and hospital stay. Presence of FUH was the only significant risk factor for morbidity (p < 0.01). After a mean follow‐up of 52.1 months (range, 3–74 months), 59 (92%) of 64 survivors were asymptomatic without any medication, and five have mild symptoms with regular physical activity. Conclusion: Operative results of TAPVC have dramatically improved in recent years. However, patients having FUH (especially with concomitant palliative surgery), heterotaxy syndrome, low weight, early age, and long CPB time still have an increased operative mortality. (J Card Surg 2012;27:376‐380)

Stenting of the obstructed ductus venosus as emergency and bridging strategy in a very low birth weight infant with infradiaphragmatic total anomalous pulmonary venous connection

“…In this neonate with obstructive infracardiac TAPVC, primary surgical correction was not the treatment of choice due to very small patient size. Outcomes of TAPVC repair are worse in patients of young age, low weight, and pulmonary venous obstruction . Therefore, palliation was needed and was carried out by transjugular catheter intervention with stenting of the venous duct to relieve intrahepatic blood flow obstruction.…”

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

“…[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.…”

“…Three large-scale studies were conducted in Turkey on this topic. [7][8][9] Ozkara et al [7] performed surgery on 61 patients and found that 44.2% (n=27) had the supracardiac drainage of TAVPR while 39.3% (n=24) had the cardiac drainage, 6.5% (n=4) had the infracardiac drainage, and 9.8% (n=6) had the mixed drainage. In our study, the most common pathology was supracardiac TAPVR, which was identified in 58% (n=18) of the patients.…”

Total Anomalous Pulmonary Venous Connection in Children

“…8,9 Complex forms of TAPVC, encountered in 4 patients, were associated with heterotaxy syndrome and single ventricle physiology, in concordance with studies concerning surgical approach and postoperative course. 14,15 The diagnosis was established at a mean age of 125 days, later than in similar studies. 2,3,10,16…”