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Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension

Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with...

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Detalles Bibliográficos
Autores principales: Pizzini, Alex, Sonnweber, Thomas, Frank, Renate, Theurl, Markus, Weiss, Günter, Tancevski, Ivan, Löffler-Ragg, Judith
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7103593/
https://www.ncbi.nlm.nih.gov/pubmed/32257114
http://dx.doi.org/10.1177/2045894019885352
Descripción
Sumario:Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension.