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Thursday, 28 November 2013

Pulmonary vascular disease – Pulmonary veno-occlusive disease – The Symptoms

Pulmonary vascular disease is defined as a condition of blood flow to the lung’s artery is blocked suddenly due to a blood clot somewhere in the body, including pulmonary embolism, chronic thromboembolic disease, pulmonary arterial hypertension, pulmonary veno-occlusive disease, pulmonary arteriovenous malformations, pulmonary edema, etc.
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.
I. Symptoms
1. progressive dyspnea, hypoxemia, and pulmonary hypertension
Some patients may experience symptoms of progressive dyspnea. There is a report of a case of a patient with a long history of progressive dyspnea of over 8 years, who with a diagnosis of chronic cor pulmonale confirmed elsewhere, was ultimately diagnosed as PVOD via histological analysis of a lung biopsy. After treatment with combined bosentan, diuretics and digoxin, his symptoms and function improved, according to the scase reposted by the the Second Affiliated Hospital of Nanchang University(1). Other study also report a case of a case of neuroblastoma with progressive dyspnea, hypoxemia, and pulmonary hypertension and patient was diagnosed as PVOD and successfully treated with low-molecular-weight heparin (LMWH) and corticosteroid(1a)
2. Shortness opf breath
According to the she study by the Hôpital Antoine Béclère, Assistance Publique – Hôpitaux de Paris, Université Paris-Sud, Patients with the diseases are experience to symptoms of Lower arterial oxygen tension and lower diffusing capacityand and lower oxygen saturation nadir during the 6-min walk test(2)
3. Dyspnoea, cough and fatigue
According to the study by the Madigan Army Medical Center, pulmonary veno-occlusive disease (PVOD) is a rare and challenging cause of pulmonary hypertension. Clinical presentation is non-specific, including dyspnoea, cough and fatigue(3).
4. Hemoptysis
There is a report of an extremely unusual case presenting as massive and intractable hemoptysis, in which pulmonary venous occlusion was attributed to granulomatous venulitis in the absence of other pulmonary or systemic inflammatory abnormalities(4).
5. Symptoms of right-sided heart failure
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension that mainly affects children and young adults. Its cause is unknown, although viral infections and drugs have been implicated. Patients with PVOD present with symptoms of right-sided heart failure, according ot the study by the University of Southern California School of Medicine(5).
6. Similar to PAH clinical presentation
PVOD has similar clinical presentation, hemodynamic characteristics and genetic background with PAH although main pathology is in venules, According to the study by the Department of Pneumology and Intensive Care, Hôpital Antoine Béclère, Assistance Publique(6).
7. Other symptoms
According to the study by ESS MANDEL, EUGENE J. MARK, and CHARLES A. HALES “Pulmonary Veno-occlusive Disease”, American Journal of Respiratory and Critical Care Medicine, Vol. 162, No. 5 (2000), pp. 1964-1973., most patients with PVOD present with nonspecific complaints such as dyspnea on exertion and lethargy, presumed secondary to an inability to adequately increase cardiac output with exercise (23). Many cases present after a respiratory infection and progress despite treatment with antibiotics (4). Chronic cough (either productive or nonproductive) is present in some individuals (57). As pulmonary hypertension becomes more severe, cyanosis, chest pain, right upper quadrant pain secondary to hepatic congestion, and exertional syncope may be noted. Hemoptysis may occur but is rarely massive and life-threatening (58). Orthopnea is reported by patients with PVOD, but is unusual among those with primary pulmonary hypertension (13). Rarer presentations of PVOD include diffuse alveolar hemorrhage and sudden death (58-60)(6a)
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Sources
(1) http://www.ncbi.nlm.nih.gov/pubmed/21673470
(1a) http://www.ncbi.nlm.nih.gov/pubmed/21707224
(2) http://www.ncbi.nlm.nih.gov/pubmed/19118230
(3) http://casereports.bmj.com/content/2013/bcr-2012-007752.abstract
(4) http://www.ncbi.nlm.nih.gov/pubmed/23050515
(5) http://www.ncbi.nlm.nih.gov/pubmed/10461536
(6) http://www.ncbi.nlm.nih.gov/pubmed/19118230
(6a) http://www.atsjournals.org/doi/full/10.1164/ajrccm.162.5.9912045#h15