Pneumology - Case Reports Open access

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

Authors

Published
3 March 2023
Section
Pneumology - Case Reports

Abstract

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.

References

  1. Sears EH, Aliotta JM, Klinger JR. Partial anomalous pulmonary venous return presenting with adult‐onset pulmonary hypertension. Pulm Circ 2012;2:250-5.
  2. 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.
  3. 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.
  4. Latson LA, Prieto LR. Congenital and acquired pulmonary vein stenosis. Circulation 2007;115:103-8.
  5. Kiseleva IP, Malsagov GU. Differential diagnosis of anomalous pulmonary venous return. A clinical-roentgenological study. Cor Vasa 1984;26:140-6.
  6. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007;115:1039-50.
  7. 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.
  8. 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.

Metrics

Citations and online attention from Dimensions, Altmetric and PlumX.