An Unusual Cause of an Uncommon Condition: Pulmonary Vein Stenosis.

Pulmonary vein stenosis in adults is historically a rare condition, but is becoming a recognised complication, albeit an uncommon one, of radiofrequency ablation around the pulmonary veins for treatment of atrial fibrillation. It may also be due to infiltrating mediastinal processes such as neoplasm or sarcoidosis. In this case report, a 55-year old man underwent resection of a mediastinal phaeochromocytoma involving the left atrial wall and the right inferior pulmonary vein. One year later he subsequently presented with increasing dyspnoea and atypical chest pain. Transthoracic echocardiogram showed severe pulmonary hypertension, right ventricular dilatation and dysfunction. Transesophageal echocardiogram demonstrated severe bilateral pulmonary vein stenosis with peak/mean gradients across the left pulmonary veins of about 25/20mmHg. The diagnosis was also confirmed on CT pulmonary angiography with 3D reconstruction. Open pulmonary vein stenting was planned but unfortunately the patient died suddenly before the procedure. Pulmonary vein stenosis is an uncommon but serious condition and may present with signs and symptoms indistinguishable from other conditions and may easily be missed. Clinicians should have a high index of suspicion when patients present with unexplained respiratory symptoms, especially in the context of catheter ablation or mediastinal processes such as neoplasm. Transesophageal echocardiography played an indispensible part in the correct diagnosis in our patient.

Drenagem anômala total das veias pulmonares: 29 anos de experiência em uma única instituição / Total anomalous pulmonary venous drainage: 29 years of experience in a single institution

FUNDAMENTO: A drenagem anômala total das veias pulmonares (DATVP) é uma doença rara, com incidência aproximada de 0,058 a 0,083:1.000 nascidos vivos. Apresenta mortalidade em torno de 80 por cento a 90 por cento no primeiro ano de vida, se não tratada precocemente. OBJETIVO: Utilizando a experiência de 29 anos de um único serviço, foram testadas possíveis variáveis relacionadas ao óbito hospitalar. MÉTODOS: Análise retrospectiva de 123 pacientes consecutivos operados com o diagnóstico de DATVP entre janeiro de 1979 e março de 2008. Foram incluídos apenas pacientes com DATVP isolada, excluídas as associações complexas. As variáveis foram testadas para a identificação da influência sobre os óbitos e da interferência da evolução temporal. RESULTADOS: As medianas de peso e idade foram de 4,1 kg e 120 dias, respectivamente. O subtipo anatômico supracardíaco foi o mais prevalente em todo o período analisado. Obstrução à drenagem esteve presente em 30 por cento dos casos, sendo o subtipo infracardíaco o mais associado. A taxa de mortalidade geral foi de 27 por cento, chegando a zero nos últimos cinco anos. Tanto na análise univariada quanto na multivariada, a presença de obstrução ao fluxo venoso pulmonar foi a única variável positivamente associada aos óbitos (p=0,005), independentemente da evolução temporal. A mortalidade dentre estes casos foi de 44 por cento. CONCLUSÃO: Após três décadas de evolução, pode-se observar melhora na mortalidade precoce da drenagem anômala total das veias pulmonares em nosso serviço, que atualmente apresenta níveis consistentes com a literatura. A obstrução à drenagem pulmonar continua sendo fator de mau prognóstico em nosso meio.

BACKGROUND: Total anomalous pulmonary venous drainage (TAPVD) is a rare disease, with an approximate incidence of 0.058 to 0.083:1,000 live births. It has a mortality rate of around 80 percent to 90 percent in the first year, if not treated early. OBJECTIVE: Using the 29-year experience of a single center, possible variables related to hospital death were tested. METHODS: Retrospective analysis of 123 consecutive patients with a diagnosis of TAPVD that underwent surgical treatment between January 1979 and March 2008. Only patients with isolated TAPVD were included and the complex associations were excluded. The variables were tested for the identification of their influence on death and of the interference of temporal evolution. RESULTS: The medians of weight and age were 4.1 kg and 120 days, respectively. The supracardiac anatomic subtype was the most prevalent throughout the analyzed period. Obstruction to drainage was present in 30 percent of the cases and the infracardiac subtype was the most frequently associated form. The general mortality rate was 27 percent, being equal to zero in the last five years. At both the univariate and multivariate analysis, the presence of obstruction to pulmonary venous flow was the only variable positively associated to the deaths (p = 0,005), regardless of the temporal evolution. The mortality among these cases was 44 percent. CONCLUSION: After three decades of evolution, an improvement in the early mortality rate of TAPVD can be observed in our service, which currently presents levels consistent with the literature. The obstruction to pulmonary drainage is still a bad prognostic factor in our country.