Factors Associated With Mortality and Reoperation in 377 Children With Total Anomalous Pulmonary Venous Connection

Abstract

Background-We sought to determine era-specific changes in the incidence of mortality and reoperation in children with total anomalous pulmonary venous connection. Methods and Results-We reviewed the records of 377 children presenting from 1946 to 2005 with total anomalous pulmonary venous connection. Multivariable parametric regression models determined the incidence and risk factors for death and reoperation after repair. Pulmonary venous connection was supracardiac in 44%, infracardiac in 26%, cardiac in 21%, and mixed in 9%. Pulmonary venous obstruction was present in 48% at presentation, most frequently with infracardiac connection type (PϽ0.001). In total, 327 patients were repaired (median age, 1.7 months). Overall survival from repair was 65Ϯ6% at 14 years, with a current survival of 97%. Significant (PϽ0.01) incremental risk factors for postrepair death were cardiac connection type, earlier operation year, younger age at repair, use of epinephrine postoperatively, and postoperative pulmonary venous obstruction. More recent operation year was associated with younger age at repair (PϽ0.001), decreased use of deep hypothermic circulatory arrest (PϽ0.001), and use of specific drugs postoperatively (PϽ0.001). Risk-adjusted estimated 1-year survival for a patient repaired at birth with unfavorable morphology in 2005 is 37% (95% CI, 8 to 80) compared with 96% (95% CI, 91 to 99) for a patient with favorable morphology repaired at 1 year of age. Freedom from reoperation was 82Ϯ6% at 11 years after repair, with increased risk associated with mixed connection type (Pϭ0.04) and postoperative pulmonary venous obstruction (PϽ0.001). Conclusions-Mortality after total anomalous pulmonary venous connection repair has decreased but remains highest in young patients and in those with cardiac connection type or pulmonary venous obstruction. Unfavorable anatomic characteristics remain important determinants of postrepair survival despite improved perioperative care. (Circulation.

Prenatal Diagnosis of TAPVC on Monday, Delivery of Tuesday and Cardiac Surgery at Wednesday - A Model of Perinatal Care in 3rd Trimester in Case of Fetal/Neonatal Critical Heart Defect in Tertiary Center.

“…In case of TAPVC, the pulmonary venous obstruction may occur. It is present in 48%-55% of the cases and is more frequent in supracardiac and infracardiac types, due to the longer and more complicated way that blood needs to pass in order to reach the heart 9,10 . In order to prevent the increase of pulmonary blood pressure and consequent decrease of blood saturation leading to neonatal death, neonates with TAPVC and pulmonary venous obstruction should be delivered in a tertiary center and need to undergo a surgical intervention during the first hours after birth.…”

Total anomalous pulmonary venous return repair in adult patients

“…В структуре ТАДЛВ частота I, II, III и IV типов составляет 46-48,6; 15,9-26; 24-26,1 и 5-10% соответ-ственно [4,14,24]. Сопутствующая обструкция легочных вен при I, II, III и IV типах ТАДЛВ наблюдается у 45, 17, 78 и 36% больных [13].…”

“…Непосредственные и отдаленные результаты коррек-ции ТАДЛВ у подростков и взрослых больных сопрово-ждаются, как правило, более гладким п/о периодом, го-спитальной летальностью 0% и высокой сохранностью хороших отдаленных результатов, нежели у педиатриче-ского контингента [1,10,11,13,19,[26][27][28][29][30][31]. Сопровожда-ется средняя госпитальная летальность в последней груп-пе коррекции -10%, составляя от 3,2% (у больных без обструкции легочных вен) до 14,3% (у оперированных с обструкцией легочных вен) [6,12,24].…”

“…Взрослая популяция ТАДЛВ отличается от педиатри-ческого контингента больных [6,9,12,13,15,24] изолиро-ванными формами порока без обструкции легочных вен, сопутствующих ВПС и гетеротаксии [1,5,7,9,[16][17][18][19][20][21][22][23][26][27][28][29][30][31]. Исключение составляют 4 взрослых больных с ТАДЛВ и сопутствующим клапанным стенозом легочной артерии/ сужением выходного отдела правого желудочка (ВОПЖ), оперированных в возрасте 26, 47, 52 и 56 лет [9,19,22].…”

Total Anomalous Pulmonary Venous Connection in Children: Preoperative Evaluation with Low-Dose Multidetector Computed Tomographic Angiography

“…1 Embryologically, TAPVC results from early atresia or failure of the common PV to develop, with persistence of at least one connection to the cardinal or the umbilical vitelline venous system. 2 Total anomalous pulmonary venous connection is a cause of neonatal cyanosis and can result in rapid death when blood is not shunted from the right side of the heart to the left side.…”