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Anomalies of pulmonary venous return

  • Progress Symposium—Progress in the Surgical Management of Congenital Heart Surgery
  • Published:
World Journal of Surgery Aims and scope Submit manuscript

Abstract

Total anomalous pulmonary venous return is a rare but life-threatening congenital heart lesion. We present our experience with 48 consecutive infants operated on from 1977 to 1983. Improved results are due to earlier diagnosis, improved surgical technique, and meticulous postoperative care. Since 1980 some patients with this lesion were submitted to surgery on the basis of two-dimensional echocardiography, thus avoiding cardiac catheterization and angiocardiography. The operative procedure should achieve a large unobstructed anastomosis between the pulmonary veins and the left atrium. Details of operative techniques currently used in our unit are discussed for each anatomic type of total anomalous pulmonary venous drainage. Although the results are improving, the overall mortality rate (hospital and late) remains around 30% in most series. Very young age (neonates), poor preoperative condition, and presence of associated cardiac and noncardiac congenital anomalies increase the risk. There were 12 hospital and 2 late deaths in our series of 48 infants (29%). Detailed analysis of the causes of death is presented.

Résumé

Le retour veineux pulmonaire anormal total met en jeu le pronostic vital. Les auteurs rapportent leur expérience qui concerne 48 nourrissons opérés de 1977 à 1983. Les résultats du traitement se sont améliorés grâce à un diagnostic précoce, une technique opératoire affinée, et des soins post-opératoires méticuleux. Depuis 1980 l'indication opératoire fut portée sur les seules données de l'échographie bi-dimensionnelle qui permet d'éviter le cathétérisme et l'angiocardiographie. L'opération doit réaliser une large anastomose perméable entre les veines pulmonaires et l'oreillette gauche. Les détails de la technique opératoire employée en fonction de chaque type anatomique de cette anomalie congénitale sont analysés. Bien que les résultats soient en voie d'amélioration la mortalité globale (mortalité hospitalière et mortalité posthospitalière) reste aux environs de 30% dans la majorité des séries publiées. Les risques de décès augmentent en fonction du jeune âge (nouveaux nés), du mauvais état générale, de la présence d'anomalies congénitales cardiaques et extracardiaques. Dans leur série les auteurs déplorent 12 décès chez 48 nourrissons opérés (29%). L'analyse des causes du décès postopératoire est présentée.

Resumen

La malformación de retorno venoso anómalo total es una lesión congénita del corazón rara pero que pone en peligro la vida del paciente. Presentamos nuestra experiencia con 48 infantes consecutivos operados entre 1977 y 1983. La mejoría en los resultados se debe a un diagnóstico más temprano, a superior técnica quirúrgica y a un meticuloso cuidado postoperatorio. A partir de 1980 algunos pacientes con esta lesión fueron sometidos a cirugía sobre la base del estudio con ecocardiografía bidimensional, evitándose así el cateterismo cardiaco y la angiocardiografía. La operación debe lograr una amplia anastomosis libre de toda obstructión, entre las venas pulmonares y la aurícula izquierda. Se discuten los detalles relativos a las técnicas operatorias actualmente en boga en nuestra unidad para cada tipo anatómico de drenaje venoso anómalo total. Aun cuando los resultados están mejorando, la mortalidad global (hospitalaria y tardía) se mantiene alrededor de 30% en la mayoría de las series. La edad muy temprana (neonatos) una pobre condición preoperatoria y la presencia de anomalías congénitas cardiacas y no cardiacas asociadas aumentan el riesgo. Doce muertes hospitalarias y dos muertes tardías ocurrieron en nuestra serie de 48 infantes (29%). Se presenta un detallado análisis de las causas de muerte.

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Stark, J. Anomalies of pulmonary venous return. World J. Surg. 9, 532–542 (1985). https://doi.org/10.1007/BF01656055

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