Pulmonary Capillary Hemangiomatosis: A Rare Cause of Pulmonary Hypertension

O'Keefe, Mary C.; Post, Miriam D.

doi:10.5858/arpa.2013-0500-rs

Cited by 42 publications

(57 citation statements)

References 17 publications

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“…Typically, PVOD and PCH have histological characteristics that are indicative of pulmonary venous and capillary proliferation with a diffuse presentation 53,54 . Patients with PVOD or PCH respond poorly to available vasodilatating agents, and these drugs might provoke lifethreatening pulmonary oedema 54,55 . Occasionally, pulmonary arterial involvement is also observed 53.56 .…”

Section: [H2] Autosomal Recessive Pah Subtypesmentioning

confidence: 99%

Molecular genetic framework underlying pulmonary arterial hypertension

et al. 2019

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| Pulmonary arterial hypertension (PAH) is a rare, progressive disorder typified by occlusion of the pulmonary arterioles owing to endothelial dysfunction and uncontrolled proliferation of pulmonary artery smooth muscle cells and fibroblasts. Vascular occlusion can lead to increased pressure in the pulmonary arteries, often resulting in right ventricular failure with shortness of breath and syncope. Since the identification of BMPR2, which encodes a receptor in the transforming growth factor-β superfamily, the development of highthroughput sequencing approaches to identify novel causal genes has substantially advanced our understanding of the molecular genetics of PAH. In the past 6 years, additional pathways involved in PAH susceptibility have been described through the identification of deleterious genetic variants in potassium channels (KCNK3 and ABCC8) and transcription factors (TBX4 and SOX17), among others. Although familial PAH most often has an autosomal dominant pattern of inheritance, cases of incomplete penetrance and evidence of genetic heterogeneity support a model of PAH as a Mendelian disorder with complex disease features. In this Review, we outline the latest advances in the detection of rare and common genetic variants underlying PAH susceptibility and disease progression. These findings have clinical implications on lung vascular function and can help to identify mechanistic pathways amenable to pharmacological intervention. P ag e | 2

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Section: [H2] Autosomal Recessive Pah Subtypesmentioning

confidence: 99%

Molecular genetic framework underlying pulmonary arterial hypertension

et al. 2019

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“…In 2 other dogs, hemosiderosis was a notable finding. Chronic alveolar hemorrhage from engorgement of fragile pulmonary capillaries leads to hemosiderin‐laden macrophages in airway lavage and sputum of humans with PVOD and PCH . Congestive heart failure is another differential for hemosiderin‐containing pulmonary macrophages potentially adding confusion to the diagnostic evaluation of a dog with acute onset of respiratory distress, PAH, and radiographic changes supportive of pulmonary edema.…”

Section: Discussionmentioning

confidence: 99%

“…The value of thoracic CT in dogs with suspect PVOD or PCH is not only to rule out other diseases with similar clinical presentations but also, as is critical in humans, to help guide the treatment. Although treatment with pulmonary vasodilators can improve the survival times of humans with idiopathic PAH and dogs with other forms of PH, fatal or life‐threatening pulmonary edema may result in cases of PVOD or PCH when there is relative vasodilatation of the precapillary resistance vessels with fixed resistance at the level of the capillaries or venules . Oral administration of sildenafil to a cat with PCH‐like disease was speculated to cause acute massive pulmonary edema that ultimately contributed to death .…”

Section: Discussionmentioning

confidence: 99%

Clinical features of canine pulmonary veno‐occlusive disease and pulmonary capillary hemangiomatosis

Reinero

Jutkowitz

Nelson

et al. 2018

Veterinary Internal Medicne

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BackgroundHistologic features of pulmonary veno‐occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) have been described in dogs but without a thorough clinical description.ObjectivesTo report the clinical features, diagnostics, treatment, and outcome of dogs with histologic evidence of PVOD and PCH.AnimalsFifteen pet dogs meeting histopathologic criteria of PVOD (occlusive remodeling of small‐sized to medium‐sized pulmonary veins) or PCH (alveolar capillary proliferation and congestion), or both.MethodsMedical records of dogs with PVOD and PCH identified based on histopathologic features between 2003 and 2017 were retrospectively reviewed.ResultsFifteen dogs met inclusion criteria of a histologic diagnosis of PVOD or PCH or both. Dogs were older (median 11 years) with no apparent breed or sex predisposition. Dogs presented with acute clinical signs (median 3 days), usually respiratory distress. Thoracic radiography (available in 10 dogs) revealed right cardiomegaly and patchy or diffuse interstitial to alveolar patterns, with 9 dogs having a normal left cardiac silhouette. In 5 dogs tested, pulmonary arterial hypertension (PAH) was documented. In all 3 dogs, thoracic computed tomography scans showed pulmonary arterial enlargement and perivascular diffuse nodular ground‐glass opacities. Ten of 15 dogs died within 1 day; median survival was 3 days.Conclusions and Clinical ImportanceIn dogs with PAH, the inability to document left‐sided congestive heart failure and failure to identify another cause of signs of respiratory disease should increase suspicion for PVOD and PCH. With increased awareness of PVOD and PCH by clinicians and pathologists, dogs with compatible clinicopathologic features should be evaluated for these pulmonary vascular disorders.

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“…These nodules are often diffuse in nature, involve all lobes, and spare the lung periphery. 16,21 Although the distinction between PCH and PVOD on imaging can be difficult, septal thickening and pleural effusions are less common in PCH. 21 In PCH, they represent the capillary proliferation within the alveolar walls; however, in PVOD these nodules represent the looplike capillary engorgement seen secondary to pulmonary venous narrowing and stenosis.…”

Section: Nodulesmentioning

confidence: 99%

“…16,21 Although the distinction between PCH and PVOD on imaging can be difficult, septal thickening and pleural effusions are less common in PCH. 21 In PCH, they represent the capillary proliferation within the alveolar walls; however, in PVOD these nodules represent the looplike capillary engorgement seen secondary to pulmonary venous narrowing and stenosis. 16 Given that PCH/PVOD share similar hemodynamic, clinical, and radiographic findings, revised guidelines recommend that PAH with PVOD/ PCH belong to a subgroup of Group 1 PH (subgroup 1.6) for the spectrum of pulmonary vascular disease known as "PAH with overt features of venous/ capillaries (PVOD/PCH) involvement," as opposed to two distinct entities.…”

Section: Nodulesmentioning

confidence: 99%