Total anomalous pulmonary venous connection with dysmorphic pulmonary vein: a risk for postoperative pulmonary venous obstruction

Abstract

Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection remains potential and understanding of its mechanisms is warranted. Morphology of the pulmonary vein was qualitatively analyzed in 48 consecutive patients undergoing repair of non-isomeric total anomalous pulmonary venous connection. Pulmonary venous drainage was supracardiac in 26, cardiac in 7, infracardiac in 13, and mixed in 2. Nine had dysmorphic pulmonary venous confluence or tributary veins (Group A). Four had excessive (>or=5) tributary veins with a hypoplastic confluence (Type 1 abnormality). In the other four cases, the vertical vein was atretic (Type 2 abnormality). In a case with cardiac type, pulmonary veins had stenosis at orifices (Type 3 abnormality). The rest (n=39; Group B) had normal pulmonary vein. Eight patients (7 in Group A and 1 in Group B) developed postoperative pulmonary venous obstruction. Overall actuarial survival was 90.0% after 2.3 months up to 10 years. Actuarial freedom from pulmonary venous obstruction was 79.5% after 5.0 months up to 10 years. It was 22.2% at 1 year among Group A compared with 96.7% at 10 years among Group B (P<0.001). Morphological analysis of the pulmonary vein best predicted the incidence of postoperative pulmonary venous obstruction.

Total Anomalous Pulmonary Venous Connection

“…Haworth 13 showed that in infracardiac TAPVC, prenatal structural changes in the extrapulmonary veins may predispose to the later development of PVO despite a successful surgical repair. Other groups have suggested that a small left atrium 14 or confluence morphology 1,15 may predispose to subsequent PVO.…”

“…Preoperative morphological features of the pulmonary veins and confluence were the most important risk factors for the presence of such postoperative obstruction, which confirms the results of other studies. 1,12,14,15 Hancock Friesen et al 7 demonstrated that none of the current perioperative or operative strategies have had an impact on the incidence of postrepair PVO, which implies that the process may reflect an underlying predisposition.The present findings concur with those of Bando et al, 1 who found that a small venous confluence and diffuse pulmonary venous narrowing were risk factors for adverse outcome. We found that patients who did not have a single confluence were at greater risk of postoperative PVO.…”

Surgical Outcome of Total Anomalous Pulmonary Venous Return; Single Center Experience

“…[14] yaptıkları çalışmada hastaneye başvurdukları anda şiddetli asidozu bulunan infrakardiak tip TPVDA tanılı hastalarda asidozun erken hastane mortalitesinde en önemli risk faktörü olduğunu göstermişlerdir. Yapılan çalışmalarda TPVDA cerrahisi sonrası mortalitenin nedenleri olarak, ameliyat öncesi entübasyona ihtiyaç duyulması, hastanın yaşı, atriyumlar arası ilşkinin büyüklüğü, sol kalp boşluklarının boyutu, hastanın arteriyel oksijen saturasyon değerleri ve pulmoner venöz obstrüksiyon ile pulmoner hipertansiyon olarak gösterilmiştir [15][16]. Kirklin ve ark.…”

“…Teorik olarak ventrikülotomi yapılmayan hastalarda erken hastane mortalitesi sıfıra yaklaşabilir, ancak hastaların tedavi için hastaneye baş-vurduklarında genel durumlarının kötü olması ve ameliyat öncesi genel durumu düzeltmedeki başarısızlık ve düşük vücut ağırlığı-na sahip olmaları bu durumu engellemektedir [23]. TPVDA cerrahi düzeltme sonrası mortaliteyi etkileyen en önemli faktörlerden biri de pulmoner venöz obstrüksiyondur [13][14][15]. Sinzobahamvya ve ark.…”

Tomografia computadorizada na avaliação tardia do tratamento cirúrgico da conexão anômala total de veias pulmonares

“…Among these mechanisms are inadequate alignment of the incisions between the CPA and LA, causing shrinkage in the anastomosis, which leads to distortion of PV [16]. Also, some authors attribute the need for reoperation stenosis and intimal hyperplasia with proliferation of thickened inflammatory tissue [17].…”