Abstract:Pulmonary hypertension is a well-known though poorly characterized disease in veterinary medicine. In humans, pulmonary veno-occlusive disease (PVOD) is a rare cause of severe pulmonary hypertension with a mean survival time of 2 years without lung transplantation. Eleven adult dogs (5 males, 6 females; median age 10.5 years, representing various breeds) were examined following the development of severe respiratory signs. Lungs of affected animals were evaluated morphologically and with immunohistochemistry fo… Show more
“…7 ). These findings were consistent with the findings described by Williams et al [ 11 ] and therefore the human equivalent of PVOD. Fig.…”
Section: Case Presentationsupporting
confidence: 93%
“…The dog in this report was presented with acute onset dyspnea which was also the most common symptom in the 11 dogs with PVOD that were presented in the study of Williams et al [ 11 ]. In humans, respiratory distress is also one of the main clinical symptoms, but the development of it is generally more gradual.…”
Section: Discussionmentioning
confidence: 82%
“…PVOD can affect humans of all ages, with some reports suggesting a higher frequency in children [ 5 , 15 ]. The dog described in this study was 11-year old resembling the reported median age of 10.5 years [ 11 ]. The reasons for the differences in clinical presentation between humans and dogs are currently unclear and further studies are needed.…”
Section: Discussionmentioning
confidence: 99%
“…To the author’s knowledge, no association with drugs or toxins, connective tissue diseases or heritability of PAH has been described in dogs and PVOD was not recognized as a separate subtype of PAH until recently. In 2016, pathologic features resembling human PVOD were retrospectively diagnosed in 11 dogs with severe idiopathic PH and dyspnea by Williams et al [ 11 ]. This group of dogs consisted of various breeds with a median age of 10.5 years and no sex predilection was observed.…”
BackgroundPulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH) in humans and can be classified in idiopathic, heritable, drug and radiation-induced, and associated with connective tissue disease or human immunodeficiency virus infection. Recently, biallelic mutations of the EIF2AK4 gene have been discovered as a cause for an autosomal recessive form of PVOD in humans. In dogs, PAH is poorly characterized and is generally considered to be idiopathic or secondary to (for example) congenital left-to right cardiovascular shunts or heartworm disease. However, recently, the pathologic features resembling human PVOD were retrospectively described in post-mortem lung samples of dogs presenting with respiratory distress and idiopathic pulmonary hypertension (PH), which suggests that PVOD contributes to an unknown percentage of cases with unexplained PH. In dogs, information on the clinical presentation of PVOD is scarce and the cause and pathogenesis of this disease is still unknown.Case presentationAn 11-year-old, intact male German Shepherd dog (GSD) was presented with a 2-day history of acute-onset dyspnea and generalized weakness. Physical examination, laboratory analysis, thoracic radiography, echocardiography, a computed tomography scan and an ante mortem lung biopsy demonstrated severe arterial hypoxemia and severe PH but were not diagnostic for a known disease syndrome. Based on the poor reaction to therapy with oxygen, sildenafil, pimobendan and dexamethasone the dog was euthanized. Histopathology of the lungs showed venous and arterial remodelling, segmental congestion of alveolar capillaries and foci of vascular changes similar to human pulmonary capillary hemangiomatosis, indicating that the dog suffered from PVOD. Whole genome sequencing analysis was performed on the case and a healthy GSD. Validation was performed by Sanger sequencing of five additional GSD's unknown for any form of respiratory stress and aged ≥ 10 years. No causal variants were found in the genes that are known to be involved in human PVOD and PAH.ConclusionsThis case report confirms that PVOD should be a diagnostic consideration in dogs presenting with dyspnea and unexplained PH. In the present case, no casual genetic mutations known to be involved in humans with PVOD and PAH were found.
“…7 ). These findings were consistent with the findings described by Williams et al [ 11 ] and therefore the human equivalent of PVOD. Fig.…”
Section: Case Presentationsupporting
confidence: 93%
“…The dog in this report was presented with acute onset dyspnea which was also the most common symptom in the 11 dogs with PVOD that were presented in the study of Williams et al [ 11 ]. In humans, respiratory distress is also one of the main clinical symptoms, but the development of it is generally more gradual.…”
Section: Discussionmentioning
confidence: 82%
“…PVOD can affect humans of all ages, with some reports suggesting a higher frequency in children [ 5 , 15 ]. The dog described in this study was 11-year old resembling the reported median age of 10.5 years [ 11 ]. The reasons for the differences in clinical presentation between humans and dogs are currently unclear and further studies are needed.…”
Section: Discussionmentioning
confidence: 99%
“…To the author’s knowledge, no association with drugs or toxins, connective tissue diseases or heritability of PAH has been described in dogs and PVOD was not recognized as a separate subtype of PAH until recently. In 2016, pathologic features resembling human PVOD were retrospectively diagnosed in 11 dogs with severe idiopathic PH and dyspnea by Williams et al [ 11 ]. This group of dogs consisted of various breeds with a median age of 10.5 years and no sex predilection was observed.…”
BackgroundPulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH) in humans and can be classified in idiopathic, heritable, drug and radiation-induced, and associated with connective tissue disease or human immunodeficiency virus infection. Recently, biallelic mutations of the EIF2AK4 gene have been discovered as a cause for an autosomal recessive form of PVOD in humans. In dogs, PAH is poorly characterized and is generally considered to be idiopathic or secondary to (for example) congenital left-to right cardiovascular shunts or heartworm disease. However, recently, the pathologic features resembling human PVOD were retrospectively described in post-mortem lung samples of dogs presenting with respiratory distress and idiopathic pulmonary hypertension (PH), which suggests that PVOD contributes to an unknown percentage of cases with unexplained PH. In dogs, information on the clinical presentation of PVOD is scarce and the cause and pathogenesis of this disease is still unknown.Case presentationAn 11-year-old, intact male German Shepherd dog (GSD) was presented with a 2-day history of acute-onset dyspnea and generalized weakness. Physical examination, laboratory analysis, thoracic radiography, echocardiography, a computed tomography scan and an ante mortem lung biopsy demonstrated severe arterial hypoxemia and severe PH but were not diagnostic for a known disease syndrome. Based on the poor reaction to therapy with oxygen, sildenafil, pimobendan and dexamethasone the dog was euthanized. Histopathology of the lungs showed venous and arterial remodelling, segmental congestion of alveolar capillaries and foci of vascular changes similar to human pulmonary capillary hemangiomatosis, indicating that the dog suffered from PVOD. Whole genome sequencing analysis was performed on the case and a healthy GSD. Validation was performed by Sanger sequencing of five additional GSD's unknown for any form of respiratory stress and aged ≥ 10 years. No causal variants were found in the genes that are known to be involved in human PVOD and PAH.ConclusionsThis case report confirms that PVOD should be a diagnostic consideration in dogs presenting with dyspnea and unexplained PH. In the present case, no casual genetic mutations known to be involved in humans with PVOD and PAH were found.
“…There have been under 100 cases reported in humans in the literature [3]. PCH as a feature of pulmonary venoocclusive disease (PVOD), an obstructive disorder affecting the post-capillary (venous) pulmonary vasculature that has been reported in humans [4] and dogs [5]. To the author’s knowledge PCH has not been described in the cat.…”
BackgroundPulmonary capillary hemangiomatosis is a rare, vascular obstructive disorder that uniformly causes pulmonary arterial hypertension. Clinically, pulmonary capillary hemangiomatosis is indistinguishable from primary pulmonary arterial hypertension and histology is required for definitive diagnosis. The distinctive histologic feature of pulmonary capillary hemangiomatosis is non-malignant extensive proliferation of capillaries in the alveolar septae. Vasodilator treatment of humans with primary arterial hypertension due to pulmonary capillary hemangiomatosis can result in fatal acute pulmonary edema. Computed tomography is thus critical to discern pulmonary capillary hemangiomatosis from other causes of pulmonary arterial hypertension prior to vasodilator therapy. This is the first report of a vasoproliferative process resembling pulmonary capillary hemangiomatosis in the feline species.Case presentationA 15-year-old, male castrated, domestic shorthair cat presented for persistent labored breathing presumptively due to congestive heart failure despite treatment with diuretics for 7 days. Echocardiography showed evidence of hypertrophic cardiomyopathy with severe pulmonary hypertension; however, a normal sized left atrium was not consistent with congestive heart failure. Thoracic computed tomography was performed and showed evidence of diffuse ill-defined nodular ground glass opacities, enlarged pulmonary arteries, and filling defects consistent with pulmonary thromboembolism. The cat acutely decompensated after a single dose of sildenafil and was euthanized. Histopathology of the lungs showed severe multifocal alveolar capillary proliferation with respiratory bronchiolar infiltration, marked type II pneumocyte hyperplasia and multifocal pulmonary arterial thrombosis.ConclusionThis is the first description in a cat of a vasoproliferative disorder resembling pulmonary capillary hemangiomatosis complicated by multifocal pulmonary arterial thrombosis. Inspiratory and expiratory ventilator-driven breath holds with angiography revealed lesions predominantly characterized by ground glass opacification and vascular filling defects with absence of air trapping. The results from this report suggest that, as in humans, the cat can develop a pulmonary capillary hemangiomatosis-like disease in which vasodilator therapy to address pulmonary hypertension may lead to fatal pulmonary edema.
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