Total Anomalous Pulmonary Venous Connection

Abstract

on behalf of the British Congenital Cardiac AssociationBackground-Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results-We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Key Words: follow-up studies Ⅲ pulmonary veins Ⅲ pulmonary vein stenosis Ⅲ heart defects, congenital T otal anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease in which all pulmonary veins connect to the systemic veins, right atrium, or coronary sinus. TAPVC can occur in conjunction with a wide variety of cardiac malformations, especially atrial isomerism. Before the advent of cardiac surgery, almost all of these children died in the first few months of life. With treatment, there has been continued improvement in the mortality and morbidity of isolated TAPVC. [1][2][3][4] This can be attributed to progress in surgical expertise and to developments in intensive care such as use of nitric oxide and extracorporeal membrane oxygenation, which have led to salvage of the sickest neonates. Conclusions-Preoperative Clinical Perspective on p 2726Nevertheless, there is an ongoing late mortality in patients with TAPVC. It is frequently associated with postoperative pulmonary venous obstruction (PVO), with an incidence of 5% to 18%. [1][2][3][5][6][7][8][9] Postoperative PVO may be a consequence of an inadequate ana...

Total anomalous pulmonary venous return repair in adult patients

“…[2,3] по трем группам: 1-я груп-па -(2+2), 2-я -(3+1), 3-я -(комбинации вариантов ТАДЛВ). 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].…”

“…Отличительной чертой взрослой популяции ТАДЛВ является отсутствие сопутствующих ВПС и обструкции легочных вен, что является ведущим предиктором выживаемости больных без коррекции по-рока. Больные с III типом ТАДЛВ не доживают до под-росткового возраста, что обусловлено 80-100% частотой обструкции легочных вен, присущей этому варианту ТАДЛВ, и ранним рестриктивным поражением легочно-сосудистого русла [6,14,24].…”

“…), тотальный аномальный дренаж легоч-ных вен (ТАДЛВ) отмечен у 7,1 из 100 000 новорожденных [24], составляя 1,5-3% в общей структуре врожденных пороков сердца (ВПС) [14].…”

“…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].…”

Pulmonary Hypertension Caused by Pulmonary Venous Hypertension

“…This occurs in several settings: idiopathic, in prematurely born infants with bronchopulmonary dysplasia, after surgical repair of anomalously connected pulmonary veins (including those not obstructed at time of repair), in patients with congenital heart defects without anomalous pulmonary vein connection, and under other circumstances. 19,20,[23][24][25] It seems unlikely that a single stenotic pulmonary vein can cause significant PH, 26 although manifest obstruction of one or more pulmonary veins in one lung can exist with occult obstruction of small veins in the contralateral lung, and additional obstructed veins can develop over time. 27 ii) Pulmonary veno-occlusive disease.…”