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Table of Contents   
Year : 2020  |  Volume : 13  |  Issue : 4  |  Page : 377-379
Pulmonary hypertension secondary to pulmonary veno occlusive disease: Catastrophe in the catheterization laboratory

Department of Pediatric Cardiology, Childnrens Heart Center, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India

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Date of Submission27-Jun-2020
Date of Decision04-Aug-2020
Date of Acceptance04-Aug-2020
Date of Web Publication23-Sep-2020

How to cite this article:
Sharma A, Bobhate PR, Karande T, Pawar R, Kulkarni S. Pulmonary hypertension secondary to pulmonary veno occlusive disease: Catastrophe in the catheterization laboratory. Ann Pediatr Card 2020;13:377-9

How to cite this URL:
Sharma A, Bobhate PR, Karande T, Pawar R, Kulkarni S. Pulmonary hypertension secondary to pulmonary veno occlusive disease: Catastrophe in the catheterization laboratory. Ann Pediatr Card [serial online] 2020 [cited 2021 Dec 4];13:377-9. Available from:


Pulmonary veno occlusive disease (PVOD) is rare form of pulmonary hypertension (PH) and classified in group 1.6. PVOD carries worse prognosis due to limited response to pulmonary vasodilators. We present a case of PVOD, who was misdiagnosed as idiopathic pulmonary artery hypertension (PAH) and had a catastrophic episode of pulmonary oedema during acute vasodilator testing (AVT).

A 5-year-old child presented with easy fatigability and had one episode of syncope. Clinically the child had severe PAH and the baseline oxygen saturation was 95%. Echocardiogram confirmed the diagnosis of PAH with moderate right ventricular systolic dysfunction. Initial high resolution computerized tomography (HRCT) was reported to be unremarkable. The child was on diuretics and sildenafil, and was referred in view of unsatisfactory clinical response. Cardiac catheterization with AVT was performed at our centre under conscious sedation and local anaesthesia. Baseline catheterization data is mentioned in [Table 1]. On initiation of inhaled nitric oxide (NO) @ 20 ppm for AVT, pulmonary artery pressure increased, and oxygen saturations decreased requiring intubation. Bilateral extensive crepitations with frothy sputum was noted, suggesting flash pulmonary oedema. Inhaled NO was stopped, and the child was stabilized with intravenous diuretics and inotropes. Chest X-ray was suggestive of pulmonary oedema which cleared on follow up X-ray [Figure 1]a, [Figure 1]b, [Figure 1]c. Repeat HRCT chest revealed multifocal ground glass opacities, thickening of interlobar fissures, prominent pulmonary arteries and normal caliber pulmonary veins, highly suggestive of PVOD [Figure 2] and [Figure 3]. Patient was discharged on diuretics and referred for lung/heart lung transplant.
Table 1: Baseline cardiac catheterization data

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Figure 1: (a) Baseline chest X-ray done prior to cardiac catheterisation suggestive of mild cardiomegaly with dilated right atrium, (b) chest X-ray done immediately after cardiac catheterisation suggestive of pulmonary oedema, (c) chest X-ray done prior to extubating shows pulmonary oedema has resolved

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Figure 2: (a-c) Computerized tomography images showing multifocal ground glass opacities with thickening of interlobar fissures (Arrow: Interlobar fissure thickening, Asterisk: Ground glass opacity)

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Figure 3: Coronal image obtained from computerized tomography showing ground glass appearance with prominent interlobar fissures

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PVOD accounts for 3%–12% of patients labelled as “idiopathic PAH.”[1],[2],[3] It could be inherited as autosomal recessive or acquired secondary to exposure to inhaled toxins and chemotherapeutic agents.[1] No history suggestive of either hereditary PAH or toxin exposure was noted in our patient. Apart from mild baseline desaturation which increases on exercise, clinical features and baseline catheterization data of PVOD are indistinguishable from other causes of PAH. Although the anatomical obstruction in PVOD is in the postcapillary vascular bed, the pulmonary artery wedge pressure is often normal.[4] AVT must be avoided in cases of suspected PVOD due to high incidence of pulmonary oedema.[5] On HRCT, a triad of mediastinal lymphadenopathy, centrilobular ground-glass opacities, and thickening of the interlobular septa is the hallmark of PVOD.[1] Although reduction in diffusion capacity of the lung for carbon monoxide and pulmonary haemorrhage in broncho-alveolar lavage have been described, they are nonspecific and difficult to perform in children.[4] Lung biopsy is the gold standard for diagnosis of PVOD, which is limited by high risk involved.[5] Data on specific PAH therapies in PVOD are weak and conflicting, pulmonary oedema has been reported with use of pulmonary vasodilators.[4] Although, Lung or heart–lung transplantation is the only definitive therapy it has guarded long term prognosis.[5]

Our patient's clinical symptoms, echocardiography, and baseline cardiac catheterization finding were consistent with that of PAH. Development of flash pulmonary oedema on AVT and HRCT was characteristic of PVOD. This case emphasises the importance of HRCT in an experienced centre prior to AVT and use of short acting agents like inhaled NO.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Montani D, Girerd B, Jaïs X, Levy M, Amar D, Savale L, et al. Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: A population-based study. Lancet Respir Med 2017;5:125-34.  Back to cited text no. 1
Hajouli S, Moustafa MA, Memoli JS. Pulmonary veno-occlusive disease: A rare cause of pulmonary hypertension. J Investig Med High Impact Case Rep 2019;7:2324709619840375.  Back to cited text no. 2
Baconnier PF, Eberhard A, Grimbert FA. Theoretical analysis of occlusion techniques for measuring pulmonary capillary pressure. J Appl Physiol (1985) 1992;73:1351-9.  Back to cited text no. 3
Montani D, Achouh L, Dorfmüller P, Le Pavec J, Sztrymf B, Tchérakian C, et al. Pulmonary veno-occlusive disease: Clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore) 2008;87:220-33.  Back to cited text no. 4
Montani D, Lau EM, Dorfmüller P, Girerd B, Jaïs X, Savale L, et al. Pulmonary veno-occlusive disease. Eur Respir J 2016;47:1518-34.  Back to cited text no. 5

Correspondence Address:
Prashant Raviprakash Bobhate
Department of Pediatric Cardiology, Childnrens Heart Center, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/apc.APC_142_20

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  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]