A Case of Right‐Sided Heart Failure and Severe Pulmonary Hypertension Secondary to Partial Anomalous Pulmonary Venous Connection

Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous circulation. PAPVC is often misdiagnosed as idiopathic pulmonary hypertension in the adult population. We report a case of a 48-year-old woman with long-standing pulmonary hypertension who presented with recurrent pulmonary oedema and right-sided heart failure. Transthoracic echocardiography showed severe pulmonary hypertension with preserved left ventricular function. Computed tomographic pulmonary angiography excluded pulmonary embolism but confirmed an anomalous drainage of the right upper pulmonary vein into the superior vena cava, consistent with isolated PAPVC without an atrial septal defect. Right heart catheterization confirmed severe precapillary pulmonary hypertension with a positive vasoreactivity response to adenosine. She was successfully managed medically with diltiazem and diuretics, showing sustained clinical improvement. This case highlights isolated PAPVC as a critical, under-recognised cause of severe pulmonary hypertension in adults.

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Publication Details

Journal
Respirology Case Reports
Published
2025-12-01
DOI
https://doi.org/10.1002/rcr2.70438
Primary Topic
Congenital Heart Disease Studies
Type
article
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article

A Case of Right‐Sided Heart Failure and Severe Pulmonary Hypertension Secondary to Partial Anomalous Pulmonary Venous Connection

Ahmed T. Shaarawy, Khalifa Abdulrahman Yusuf, A Al-Absi, Shadi Fayez Kanhosh et al.
Respirology Case Reports
Congenital Heart Disease Studies
article

A Case of Right‐Sided Heart Failure and Severe Pulmonary Hypertension Secondary to Partial Anomalous Pulmonary Venous Connection

Ahmed T. Shaarawy, Khalifa Abdulrahman Yusuf, A Al-Absi, Shadi Fayez Kanhosh, Abdulrahman Hasan Al‐Madani, Alya Salman Aldoseri
article en

Abstract

Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous circulation. PAPVC is often misdiagnosed as idiopathic pulmonary hypertension in the adult population. We report a case of a 48-year-old woman with long-standing pulmonary hypertension who presented with recurrent pulmonary oedema and right-sided heart failure. Transthoracic echocardiography showed severe pulmonary hypertension with preserved left ventricular function. Computed tomographic pulmonary angiography excluded pulmonary embolism but confirmed an anomalous drainage of the right upper pulmonary vein into the superior vena cava, consistent with isolated PAPVC without an atrial septal defect. Right heart catheterization confirmed severe precapillary pulmonary hypertension with a positive vasoreactivity response to adenosine. She was successfully managed medically with diltiazem and diuretics, showing sustained clinical improvement. This case highlights isolated PAPVC as a critical, under-recognised cause of severe pulmonary hypertension in adults.

Respirology Case ReportsVol. 13(12)
Royal College of Surgeons in Ireland - Bahrain (BH)
Openalex Percentile: Top 49%
Congenital Heart Disease Studies
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