Highresolution ct revealed the triad of diffuse ground glass opacities, interlobular septal thickening figure 1a, arrows, and mediastinal lymphadenopathy, leading to a presumed diagnosis of pulmonary venoocclusive disease and pulmonary capillary hemangiomatosis pvodpch. It leads to high blood pressure in the lung arteries pulmonary hypertension. Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension which occurs in 0. Pulmonary venoocclusive disease pvod is a rare form of pah that shares many features with other types of pah but retains a unique histopathological pattern. Some reports suggest that pvod accounts for 520% of cases classified as idiopathic pulmonary arterial hypertension pah.
The authors present the case of a 22yearold female patient, white, referred to the cardiovascular outpatient clinic in november 2006 for pulmonary arterial hypertension. The occlusion is caused by a buildup of abnormal fibrous tissue in the small veins in the lungs, which narrows the vessels and impairs blood flow. Understanding the similarities and differences between hepatic. Abstract pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension ph characterised by preferential. Pulmonary venoocclusive disease pubmed central pmc. Pulmonary venoocclusive disease in a patient with history of major depressive disorder volume 3 issue 4 2016 ahmad amin, 1 mahdieh khabazian,2 mitra chitsazan,3 hamidreza pouraliakbar4 1assistant professor of cardiology, department of heart failure and transplantation, iran 2cardiologist, department of heart failure and transplantation, iran. Pulmonary venoocclusive disease pvod is a very rare disorder of the small veins in the lungs. Full text get a printable copy pdf file of the complete article 4. Imaging description pulmonary venoocclusive disease pvod is considered a cause of pulmonary hypertension that preferentially affects the postcapillary pulmonary vasculature. Third, the dilated pulmonary artery might have contributed to slower disease progression, as reported previously. Pulmonary venoocclusive disease as a primary cause of pulmonary hypertension in a patient with mixed connective tissue disease.
Ali ataya md, eloise harman md, in rare and interesting cases in pulmonary medicine, 2017. When caring for our patients with pulmonary arterial hypertension pah, how many times have we wondered, could this patient really have pulmonary venoocclusive diseasepulmonary capillary hemangiomiatosis pvodpch. Described as a form of pulmonary arterial hypertension pah, pulmonary venoocclusive disease pvod is a pathological process involving small veins in the pulmonary circulation, and is estimated to encompass about 510% of cases previously diagnosed as idiopathic pah 1 2 3 4. Pulmonary venoocclusive disease pvod is a rare entity that is usually mistaken with pulmonary arterial hypertension pah but is considered class i.
The incidence and prevalence of pvod is not well characterized. Pulmonary venoocclusive disease pulmonary venoocclusive. Pulmonary venoocclusive disease pvod is a very rare disease. Rare, usually children and adolescents, especially females also after cancer chemotherapy causes pulmonary hypertension secondary to widespread thromboemboli of large and medium size branches of pulmonary vein. Pulmonary venoocclusive disease pvod is considered an uncommon variant of primary pulmonary hypertension pph that preferentially affects the postcapillary pulmonary vasculature. Clinical phenotypes and outcomes of heritable and sporadic. Qin luo, qi jin, zhihui zhao, qing zhao, xue yu, lu yan, yi zhang, changming xiong and zhihong liu abstract background. Pvod and idiopathic pah share a similar clinical presentation, genetic background and hemodynamic profile.
We describe a series of 4 patients with systemic sclerosis ssc, concomitant ph, and biopsyprovenpresumed pvod. Pulmonary venoocclusive disease circulation aha journals. Pulmonary venoocclusive disease pvod is currently classified as a subgroup of pulmonary arterial hypertension pah and accounts for 510% of cases initially considered to be idiopathic pah. Pulmonary venoocclusive disease in a patient with history.
Pulmonary arterial hypertension pah is a severe condition characterised by elevated pulmonary artery pressure leading to right heart failure and death 1, 2. In humans, pulmonary venoocclusive disease pvod is a rare cause of severe pulmonary hypertension with a mean survival time of 2 years without lung transplantation. In general, pvod progresses rapidly such that early recognition and treatment of this entity is critical. Pulmonary venoocclusive disease european respiratory journal. Impressive clinical signs and symptoms often obscure the true underlying capillary disorder, thus severely compromising timely and appropriately directed therapy. It is difficult to distinguish pvod from idiopathic primary artery hypertension, chronic thromboembolic disease, and other pulmonary diseases, because the clinical features, laboratory data, and radiological. Pulmonary venoocclusive disease pvod is a rare disorder and can be misdiagnosed as idiopathic pulmonary arterial hypertension ipah. Pulmonary hypertension is a wellknown though poorly characterized disease in veterinary medicine.
Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension ph characterised by preferential remodelling of the pulmonary venules. Research article open access targeted therapy in pulmonary venoocclusive disease. Pulmonary veno occlusive disease pvod is currently classified as a subgroup of pulmonary arterial hypertension pah and accounts for 510% of cases initially considered to be idiopathic pah. The patient was referred for lung transplantation, and. The diagnosis of pulmonary venoocclusive disease and pulmonary capillary hemangiomatosis disease should be considered when ct findings of pulmonary hypertension are combined with centrilobular groundglass opacities, mediastinal lymph node enlargement, and smooth thickening of interlobular septa. There are rare families that have been described where multiple family members have been affected by the disease. Pulmonary venoocclusive disease pvod is characterized by the blockage occlusion of the blood vessels that carry oxygenrich oxygenated blood from the lungs to the heart the pulmonary veins.
Pdf pulmonary venoocclusive disease pvod is currently classified as a subgroup of pulmonary arterial hypertension pah and accounts for 510% of. Pulmonary veno occlusive disease is a rare and usually fatal condition in which there is gradual obliteration of the pulmonary veins and venules. Pulmonary venoocclusive disease as an occupational lung. Pulmonary venoocclusive disease pvod is a rare disorder that can be misdiagnosed as idiopathic pulmonary arterial hypertension pah and accounts for 510% of cases initially considered as idiopathic pah. Pvod is a rare and severe form of pulmonary hypertension characterised by occlusion of small pulmonary veins and pulmonary capillary proliferation. Without a lung biopsy the conical diagnosis of this disease is difficult. Pulmonary venoocclusive disease and scleroderma associated pulmonary hypertension. It is still controversial if this condition and pulmonary capillary haemangiomatosis pch are varied expressions of the same condition or different entities. Pulmonary venoocclusive disease pvod is a rare cause of pulmonary hypertension ph. Pulmonary veno occlusive disease pvod is characterized by the blockage occlusion of the blood vessels that carry oxygenrich oxygenated blood from the lungs to the heart the pulmonary veins.
Pulmonary venoocclusive disease radiology reference. Pulmonary venoocclusive disease pvod is a clinico pathologic syndrome that accounts for a small number of cases of pulmonary hypertension. Pulmonary venoocclusive disease pvod, recently reclassified as pulmonary arterial hypertension pah with overt features of venouscapillaries involvement, is a subgroup of group 1 pulmonary hypertension, which has been rarely reported in systemic sclerosis. Pulmonary venoocclusive disease european respiratory. However, some patients with pulmonary venoocclusive disease show, in addition, signs of pulmonary venous and capillary hypertension, which can lead to its clinical recognition when associated with a normal left atrial blood pressure. Pulmonary venoocclusive disease pvod is a clinicopathologic syndrome that accounts for a small number of cases of pulmonary hypertension. Pulmonary venoocclusive diseasepulmonary capillary hemangiomatosis pvodpch is a rare condition that represents a subgroup of patients with pulmonary hypertension. Pulmonary veno occlusive disease pvod is considered an uncommon variant of primary pulmonary hypertension pph that preferentially affects the postcapillary pulmonary vasculature. Psychometric validation of a japanese version of the. Second, rich collateral vessels surrounded the alveoli might have decreased the pulmonary artery pressure, acting like pulmonary veins in a healthy lung. This suggests a possible genetic contribution in some circumstances. As such, it is classified under a distinct subgroup category termed group 1 pah, alongside pulmonary capillary hemangiomatosis pch.
In the current ph classification, pvod and pulmonary capillary haemangiomatosis pch are considered to be a common entity and represent varied expressions of the same disease. Idiopathic pulmonary arterial hypertension and pulmonary veno. Pulmonary hypertension is a serious complication of systemic sclerosis and remains one of the leading causes of mortality. Pulmonary venoocclusive disease pvod is a clinicopathologic syndrome that accounts for a small number of pulmonary hypertension cases. Pulmonary venoocclusive disease pvod is one of the less commonly encountered causes of pulmonary hypertension. Complementary diagnostic exams revealed elevated pulmonary arterial pressure. The high blood pressure occurs in the pulmonary arteries. We strongly recommend that you talk with a trusted. The imaging findings are a result of this fibrotic occlusion. Manifestations and management of venoocclusive disease. Pulmonary venoocclusive disease and pulmonary capillary. Pulmonary venoocclusive disease pvod is classified as a subgroup of pah and accounts for 510% of histological forms of cases initially considered to be idiopathic pah. Pulmonary venoocclusive disease american journal of.
Pulmonary venoocclusive disease pvod is a rare cause of pulmonary hypertension with high morbidity and mortality. Targeted therapy in pulmonary venoocclusive disease. Pdf pulmonary venoocclusive disease pvod is a rare disorder that can be misdiagnosed as idiopathic pulmonary arterial hypertension pah and. Pulmonary vod pvod as a clinical entity is even more poorly understood than hepatic vodsos. The pathologic hallmark of pvod is the extensive and diffuse occlusion of pulmonary veins by fibrous tissue. Pulmonary venoocclusive disease genetics home reference. Pulmonary veno occlusive disease pvod is a rare form of pulmonary hypertension ph characterised by preferential remodelling of the pulmonary venules. It is important to subclassify pvod and distinguish it from pah as treatment with. Pulmonary venoocclusive disease pvod is a rare condition with poor prognosis, and lung. Clinical phenotypes and outcomes of heritable and sporadic pulmonary venoocclusive disease. Longterm survivor with pulmonary venoocclusive disease.
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