Pulmonary veno-occlusive disease
Pulmonary veno-occlusive disease (PVOD) is an extremely rare form of
pulmonary hypertension, affecting mostly in children and young adults as
a result of a progressive obstruction of small pulmonary veins that
leads to elevation in pulmonary vascular resistance and right
ventricular failure.
A. Misdiagnosis
PVOD is an underdiagnosed and commonly misdiagnosed cause of pulmonary
hypertension, which may present with a failure to respond to medical
therapy. Further studies on PVOD may help confirm whether a change in
its classification as a type of PAH is necessary(29). Other study
reported a case of a 27-yr-old female with a 6-month diagnosis of
idiopathic pulmonary arterial hypertension (PAH) confirmed elsewhere was
referred to our centre with worsening dyspnoea. On examination, the
patient had low systemic oxygen saturation despite high oxygen flow and
reduced exercise capacity. Haemodynamics were indicative of severe
pre-capillary PAH. High-resolution computed tomography revealed diffuse
ground-glass opacity with thickening interlobular septa, and
haemosiderin-laden macrophages were identified by bronchoalveolar
lavage. Based on clinical and diagnostic findings, the patient was
re-diagnosed with pulmonary veno-occlusive disease (PVOD)(30). Also,
there is a review of cases of 14 patients with clinically diagnosed PAH
who had failed to respond to medical therapy and had lung tissue
available from autopsy or explant. Control samples (n = 6) were obtained
from lungs explanted for other causes, and a previous transthoracic
echocardiogram excluded pulmonary hypertension(31).
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Sources
(29) http://www.ncbi.nlm.nih.gov/pubmed/19188548
(30) http://www.ncbi.nlm.nih.gov/pubmed/20956140
(31) http://www.ncbi.nlm.nih.gov/pubmed/19188548
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