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...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2020-03-01
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| Series: | Pulmonary Circulation |
| Online Access: | https://doi.org/10.1177/2045894019885352 |
| _version_ | 1828311522133147648 |
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| author | Alex Pizzini Thomas Sonnweber Renate Frank Markus Theurl Günter Weiss Ivan Tancevski Judith Löffler-Ragg |
| author_facet | Alex Pizzini Thomas Sonnweber Renate Frank Markus Theurl Günter Weiss Ivan Tancevski Judith Löffler-Ragg |
| author_sort | Alex Pizzini |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-13T16:00:24Z |
| format | Article |
| id | doaj.art-1caab4064cfe41a5a9d0d177bc6ae366 |
| institution | Directory Open Access Journal |
| issn | 2045-8940 |
| language | English |
| last_indexed | 2024-04-13T16:00:24Z |
| publishDate | 2020-03-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj.art-1caab4064cfe41a5a9d0d177bc6ae3662022-12-22T02:40:32ZengWileyPulmonary Circulation2045-89402020-03-011010.1177/2045894019885352Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertensionAlex Pizzini0Thomas Sonnweber1Renate Frank2Markus Theurl3Günter Weiss4Ivan Tancevski5Judith Löffler-Ragg6Department of Internal Medicine II, Infectious Diseases, Pneumology, Rheumatology, Medical University of Innsbruck, Innsbruck, AustriaDepartment of Internal Medicine II, Infectious Diseases, Pneumology, Rheumatology, Medical University of Innsbruck, Innsbruck, AustriaDepartment of Radiology, Medical University of Innsbruck, Innsbruck, AustriaDepartment of Internal Medicine III (Cardiology, Angiology), Medical University of Innsbruck, Innsbruck, AustriaDepartment of Internal Medicine II, Infectious Diseases, Pneumology, Rheumatology, Medical University of Innsbruck, Innsbruck, AustriaDepartment of Internal Medicine II, Infectious Diseases, Pneumology, Rheumatology, Medical University of Innsbruck, Innsbruck, AustriaDepartment of Internal Medicine II, Infectious Diseases, Pneumology, Rheumatology, Medical University of Innsbruck, Innsbruck, AustriaIsolated 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.https://doi.org/10.1177/2045894019885352 |
| spellingShingle | Alex Pizzini Thomas Sonnweber Renate Frank Markus Theurl Günter Weiss Ivan Tancevski Judith Löffler-Ragg Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension Pulmonary Circulation |
| title | Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension |
| title_full | Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension |
| title_fullStr | Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension |
| title_full_unstemmed | Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension |
| title_short | Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension |
| title_sort | clinical implications of partial anomalous pulmonary venous connection a rare cause of severe pulmonary arterial hypertension |
| url | https://doi.org/10.1177/2045894019885352 |
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