An unexpected and unusual cause of pulmonary hypertension in a patient with hypersensitivity pneumonitis: a partial anomalous pulmonary venous connection causing pulmonary artery hypertension

Submitted: December 1, 2022
Accepted: February 24, 2023
Published: March 3, 2023
Abstract Views: 673
PDF: 205
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.


Partial anomalous pulmonary venous connection (PAPVC) occurs when any pulmonary vein, but not all, drains directly into the right atrium or its venous tributaries. PAPVC can very rarely present as an individual cause of pulmonary artery hypertension. Here we are presenting the case of a 41-year-old farmer with a history of exertional dyspnea for the past 3 years, which increased over 6 months. Chest high-resolution computed tomography was suggestive of non-fibrotic hypersensitivity pneumonitis. Hence, the patient was started on systemic steroids, with which the patient’s oxygen saturation improved. On 2D echocardiography, the right ventricle systolic pressure was 48 + right atrial pressure. Right heart catheterization showed a mean pulmonary artery pressure of 73 mmHg and pulmonary vascular resistance of 8.7. On further evaluation, a computed tomography pulmonary angiogram was done, which surprisingly revealed the left superior pulmonary vein draining into the left brachiocephalic vein.



PlumX Metrics


Download data is not yet available.


Sears EH, Aliotta JM, Klinger JR. Partial anomalous pulmonary venous return presenting with adult‐onset pulmonary hypertension. Pulm Circ 2012;2:250-5.
Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of hypersensitivity pneumonitis in adults. An official ATS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2020;202:e36-69.
Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2022;2200879.
Latson LA, Prieto LR. Congenital and acquired pulmonary vein stenosis. Circulation 2007;115:103-8.
Kiseleva IP, Malsagov GU. Differential diagnosis of anomalous pulmonary venous return. A clinical-roentgenological study. Cor Vasa 1984;26:140-6.
Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007;115:1039-50.
Blyth KG, Groenning BA, Mark PB, et al. NT-proBNP can be used to detect right ventricular systolic dysfunction in pulmonary hypertension. Eur Respir J 2007;29:737-44.
Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev 2009;18:154-61.

How to Cite

Upadhya, Pratap, Shivam Garg, Jeevanandham A., Nesamani Daniel Ponraj, and Ahmed Wayez. 2023. “An Unexpected and Unusual Cause of Pulmonary Hypertension in a Patient With Hypersensitivity Pneumonitis: A Partial Anomalous Pulmonary Venous Connection Causing Pulmonary Artery Hypertension”. Monaldi Archives for Chest Disease 94 (1).