Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension.
Pulm Circ
; 10(1): 2045894019885352, 2020.
Article
in En
| MEDLINE
| ID: mdl-32257114
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Language:
En
Journal:
Pulm Circ
Year:
2020
Document type:
Article
Affiliation country:
Austria
Country of publication:
United States