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Hiromi Matsubara - One of the best experts on this subject based on the ideXlab platform.
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use of vasodilators for the treatment of Pulmonary veno occlusive disease and Pulmonary Capillary Hemangiomatosis a systematic review
Respiratory investigation, 2019Co-Authors: Aiko Ogawa, Hiromi Matsubara, Seiichiro Sakao, Nobuhiro Tanabe, Koichiro TatsumiAbstract:Abstract Background There are several medications available to treat Pulmonary arterial hypertension (PAH): PAH-targeted drugs. However, in patients with Pulmonary veno-occlusive disease and Pulmonary Capillary Hemangiomatosis (PVOD/PCH), rare diseases that cause Pulmonary hypertension, the effectiveness and safety of vasodilators, including PAH-targeted drugs, are unclear. Methods We searched English-language publications listed in three electronic databases (PubMed, Cochrane Library, and the Japan Medical Abstracts Society). Reports with efficacy outcomes (survival, improvement in 6-minute walk distance, and Pulmonary vascular resistance) and data on development of Pulmonary edema after administration of vasodilators to patients with PVOD/PCH were selected (1966 to August 2015). Results We identified 20 reports that met our criteria. No randomized controlled or prospective controlled studies were reported. The survival time ranged from 71 minutes to 4 years or more after initiation of vasodilators. Most of the reported cases showed an improvement in the 6-minute walk distance and Pulmonary vascular resistance. Pulmonary edema was reported in 15 articles, some cases of which were lethal. Conclusions The present study demonstrates the potential efficacy and difficulties in the use of vasodilators in patients with PVOD/PCH; however, drawing a firm conclusion was difficult because of the lack of randomized controlled trials. Further research is needed to ascertain if vasodilator use is beneficial and safe in patients with PVOD/PCH.
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clinical prediction score for identifying patients with Pulmonary veno occlusive disease Pulmonary Capillary Hemangiomatosis
Journal of Cardiology, 2018Co-Authors: Aiko Ogawa, Yukari Takahashi, Hiromi MatsubaraAbstract:Abstract Background Pulmonary veno-occlusive disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) are rare causes of Pulmonary hypertension. Although diagnosis is based on pathological findings, an early diagnosis is crucial because of poor prognosis compared to other types of Pulmonary hypertension. Furthermore, vasodilators may cause fatal Pulmonary edema in patients with PVOD/PCH. This study aimed to identify specific characteristics for patients with PVOD/PCH to clinically diagnose PVOD/PCH. Methods Clinical data were obtained at baseline and were compared between 19 patients with PVOD/PCH and 55 patients with idiopathic/heritable Pulmonary arterial hypertension. Receiver operating characteristic analyses were used to determine characteristics specific for patients with PVOD/PCH and a scoring system to diagnose PVOD/PCH was developed. Results Patients with PVOD/PCH had a smoking history and were predominantly male. Six-minute walk distance was significantly lower and oxygen desaturation was severe during the walk. Diffusion capacity of carbon monoxide was significantly low. Radiological findings included ground glass opacity on chest high-resolution computed tomography (CT) in all patients with PVOD/PCH, and thickened septal lines in 90% of the patients. Lung perfusion scintigraphy showed defect in >70% of the patients. Pulmonary edema after initiation of vasodilation therapy was frequently observed in PVOD/PCH patients. Based on these results, we identified nine important clinical characteristics and a novel scoring system was designed to clinically diagnose PVOD/PCH: male sex, smoking history, 6-minute walk distance Conclusions Our novel prediction rule for diagnosing PVOD/PCH may offer an early clinical diagnosis of these diseases.
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efficacy and safety of long term imatinib therapy for patients with Pulmonary veno occlusive disease and Pulmonary Capillary Hemangiomatosis
Respiratory Medicine, 2017Co-Authors: Aiko Ogawa, Katsumasa Miyaji, Hiromi MatsubaraAbstract:Abstract Background Pulmonary veno-occlusive disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) are categorized as Group 1′ in the clinical classification of Pulmonary hypertension. No medical therapy has been proven to be effective in patients with PVOD/PCH. Imatinib is a molecular targeted drug and was expected to be effective in patients with Pulmonary arterial hypertension. We evaluated its efficacy and safety in patients with PVOD/PCH. Methods In the present observational study, 9 patients with PVOD/PCH received imatinib. Clinical data including exercise capacity and hemodynamics at baseline and at follow-up were compared. Survival rate of patients treated with imatinib was compared to those of 7 patients who did not treated with imatinib. Results Imatinib was prescribed at doses of 100–400 mg/day and was well-tolerated. At follow-up, World Health Organization functional class and brain natriuretic peptide levels significantly improved. Mean Pulmonary arterial pressure was significantly reduced (from 56.8 ± 8.3 to 43.7 ± 9.0 mmHg) with preserved cardiac index. Patients were treated with imatinib for 797.2 ± 487.0 days. Seven patients (77.8%) died and 2 patients (22.2%) underwent lung transplantation. Mean survival time in patients treated with imatinib therapy was 1493.7 ± 196.3 days (95% confidence interval, 1108.9–1878.5 days), significantly longer than those without imatinib treatment (713.0 ± 258.1 days, log-rank test, P = 0.04). Conclusions Imatinib improved exercise capacity, hemodynamics and survival in patients with PVOD/PCH. In patients with PVOD/PCH, who have no effective medical therapy available, imatinib might function as a bridge to lung transplantation, and may become a potential therapeutic option to improve their survival.
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imatinib is partially effective for the treatment of Pulmonary Capillary Hemangiomatosis
Internal Medicine, 2014Co-Authors: Shiro Adachi, Toyoharu Yokoi, Akihiro Hirashiki, Takahisa Kondo, Masato Nakaguro, Aiko Ogawa, Katsumasa Miyaji, Hiromi Matsubara, Toyoaki MuroharaAbstract:A 43-year-old man presented with dyspnea on exertion. Right heart catheterization demonstrated Pulmonary arterial hypertension (PAH). He was treated with bosentan, sildenafil and intravenous epoprostenol. Despite the administration of such intensive therapy, the patient's condition deteriorated to a World Health Organization functional class (WHO-FC) of IV. He participated in a clinical trial of imatinib for PAH. After three months of treatment with imatinib, the chest X-ray and echocardiography findings improved, and the WHO-FC class was III. One year after, however, the PAH worsened again, and the patient died 2.6 years after the first diagnosis. At autopsy, patchy Capillary proliferation was observed in the lungs. The definitive diagnosis was Pulmonary Capillary Hemangiomatosis.
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different sizes of centrilobular ground glass opacities in chest high resolution computed tomography of patients with Pulmonary veno occlusive disease and patients with Pulmonary Capillary Hemangiomatosis
Cardiovascular Pathology, 2013Co-Authors: Aya Miura, Keiko Ohtaogo, Aiko Ogawa, Kengo Kusano, Satoshi Akagi, Kazufumi Nakamura, Katsushi Hashimoto, Satoshi Nagase, Kunihisa Kohno, Hiromi MatsubaraAbstract:Abstract Background Centrilobular ground-glass opacity (GGO) is one of the characteristic findings in chest high-resolution computed tomography (HRCT) of patients with Pulmonary veno-occlusive disease (PVOD) and patients with Pulmonary Capillary Hemangiomatosis (PCH). However, clinical differential diagnosis of these two diseases is difficult and has not been established. In order to clarify their differences, we compared the sizes of GGOs in chest HRCT and the sizes of Capillary assemblies in Pulmonary vascular casts between patients diagnosed pathologically with PVOD and PCH. Methods We evaluated chest HRCT images for four patients with idiopathic Pulmonary arterial hypertension (IPAH), three patients with PVOD and three patients with PCH, and we evaluated Pulmonary vascular casts of lung tissues obtained from those patients at lung transplantation or autopsy. Results Centrilobular GGOs in chest HRCT were observed in patients with PVOD and patients with PCH but not in patients with IPAH. We measured the longest diameter of the GGOs. The size of centrilobular GGOs was significantly larger in patients with PCH than in patients with PVOD (5.60±1.43 mm versus 2.51±0.79 mm, P P Conclusion Measurement of the sizes of centrilobular GGOs in HRCT is a simple and useful method for clinical differential diagnosis of PVOD and PCH.
Aiko Ogawa - One of the best experts on this subject based on the ideXlab platform.
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use of vasodilators for the treatment of Pulmonary veno occlusive disease and Pulmonary Capillary Hemangiomatosis a systematic review
Respiratory investigation, 2019Co-Authors: Aiko Ogawa, Hiromi Matsubara, Seiichiro Sakao, Nobuhiro Tanabe, Koichiro TatsumiAbstract:Abstract Background There are several medications available to treat Pulmonary arterial hypertension (PAH): PAH-targeted drugs. However, in patients with Pulmonary veno-occlusive disease and Pulmonary Capillary Hemangiomatosis (PVOD/PCH), rare diseases that cause Pulmonary hypertension, the effectiveness and safety of vasodilators, including PAH-targeted drugs, are unclear. Methods We searched English-language publications listed in three electronic databases (PubMed, Cochrane Library, and the Japan Medical Abstracts Society). Reports with efficacy outcomes (survival, improvement in 6-minute walk distance, and Pulmonary vascular resistance) and data on development of Pulmonary edema after administration of vasodilators to patients with PVOD/PCH were selected (1966 to August 2015). Results We identified 20 reports that met our criteria. No randomized controlled or prospective controlled studies were reported. The survival time ranged from 71 minutes to 4 years or more after initiation of vasodilators. Most of the reported cases showed an improvement in the 6-minute walk distance and Pulmonary vascular resistance. Pulmonary edema was reported in 15 articles, some cases of which were lethal. Conclusions The present study demonstrates the potential efficacy and difficulties in the use of vasodilators in patients with PVOD/PCH; however, drawing a firm conclusion was difficult because of the lack of randomized controlled trials. Further research is needed to ascertain if vasodilator use is beneficial and safe in patients with PVOD/PCH.
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clinical prediction score for identifying patients with Pulmonary veno occlusive disease Pulmonary Capillary Hemangiomatosis
Journal of Cardiology, 2018Co-Authors: Aiko Ogawa, Yukari Takahashi, Hiromi MatsubaraAbstract:Abstract Background Pulmonary veno-occlusive disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) are rare causes of Pulmonary hypertension. Although diagnosis is based on pathological findings, an early diagnosis is crucial because of poor prognosis compared to other types of Pulmonary hypertension. Furthermore, vasodilators may cause fatal Pulmonary edema in patients with PVOD/PCH. This study aimed to identify specific characteristics for patients with PVOD/PCH to clinically diagnose PVOD/PCH. Methods Clinical data were obtained at baseline and were compared between 19 patients with PVOD/PCH and 55 patients with idiopathic/heritable Pulmonary arterial hypertension. Receiver operating characteristic analyses were used to determine characteristics specific for patients with PVOD/PCH and a scoring system to diagnose PVOD/PCH was developed. Results Patients with PVOD/PCH had a smoking history and were predominantly male. Six-minute walk distance was significantly lower and oxygen desaturation was severe during the walk. Diffusion capacity of carbon monoxide was significantly low. Radiological findings included ground glass opacity on chest high-resolution computed tomography (CT) in all patients with PVOD/PCH, and thickened septal lines in 90% of the patients. Lung perfusion scintigraphy showed defect in >70% of the patients. Pulmonary edema after initiation of vasodilation therapy was frequently observed in PVOD/PCH patients. Based on these results, we identified nine important clinical characteristics and a novel scoring system was designed to clinically diagnose PVOD/PCH: male sex, smoking history, 6-minute walk distance Conclusions Our novel prediction rule for diagnosing PVOD/PCH may offer an early clinical diagnosis of these diseases.
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efficacy and safety of long term imatinib therapy for patients with Pulmonary veno occlusive disease and Pulmonary Capillary Hemangiomatosis
Respiratory Medicine, 2017Co-Authors: Aiko Ogawa, Katsumasa Miyaji, Hiromi MatsubaraAbstract:Abstract Background Pulmonary veno-occlusive disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) are categorized as Group 1′ in the clinical classification of Pulmonary hypertension. No medical therapy has been proven to be effective in patients with PVOD/PCH. Imatinib is a molecular targeted drug and was expected to be effective in patients with Pulmonary arterial hypertension. We evaluated its efficacy and safety in patients with PVOD/PCH. Methods In the present observational study, 9 patients with PVOD/PCH received imatinib. Clinical data including exercise capacity and hemodynamics at baseline and at follow-up were compared. Survival rate of patients treated with imatinib was compared to those of 7 patients who did not treated with imatinib. Results Imatinib was prescribed at doses of 100–400 mg/day and was well-tolerated. At follow-up, World Health Organization functional class and brain natriuretic peptide levels significantly improved. Mean Pulmonary arterial pressure was significantly reduced (from 56.8 ± 8.3 to 43.7 ± 9.0 mmHg) with preserved cardiac index. Patients were treated with imatinib for 797.2 ± 487.0 days. Seven patients (77.8%) died and 2 patients (22.2%) underwent lung transplantation. Mean survival time in patients treated with imatinib therapy was 1493.7 ± 196.3 days (95% confidence interval, 1108.9–1878.5 days), significantly longer than those without imatinib treatment (713.0 ± 258.1 days, log-rank test, P = 0.04). Conclusions Imatinib improved exercise capacity, hemodynamics and survival in patients with PVOD/PCH. In patients with PVOD/PCH, who have no effective medical therapy available, imatinib might function as a bridge to lung transplantation, and may become a potential therapeutic option to improve their survival.
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imatinib is partially effective for the treatment of Pulmonary Capillary Hemangiomatosis
Internal Medicine, 2014Co-Authors: Shiro Adachi, Toyoharu Yokoi, Akihiro Hirashiki, Takahisa Kondo, Masato Nakaguro, Aiko Ogawa, Katsumasa Miyaji, Hiromi Matsubara, Toyoaki MuroharaAbstract:A 43-year-old man presented with dyspnea on exertion. Right heart catheterization demonstrated Pulmonary arterial hypertension (PAH). He was treated with bosentan, sildenafil and intravenous epoprostenol. Despite the administration of such intensive therapy, the patient's condition deteriorated to a World Health Organization functional class (WHO-FC) of IV. He participated in a clinical trial of imatinib for PAH. After three months of treatment with imatinib, the chest X-ray and echocardiography findings improved, and the WHO-FC class was III. One year after, however, the PAH worsened again, and the patient died 2.6 years after the first diagnosis. At autopsy, patchy Capillary proliferation was observed in the lungs. The definitive diagnosis was Pulmonary Capillary Hemangiomatosis.
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different sizes of centrilobular ground glass opacities in chest high resolution computed tomography of patients with Pulmonary veno occlusive disease and patients with Pulmonary Capillary Hemangiomatosis
Cardiovascular Pathology, 2013Co-Authors: Aya Miura, Keiko Ohtaogo, Aiko Ogawa, Kengo Kusano, Satoshi Akagi, Kazufumi Nakamura, Katsushi Hashimoto, Satoshi Nagase, Kunihisa Kohno, Hiromi MatsubaraAbstract:Abstract Background Centrilobular ground-glass opacity (GGO) is one of the characteristic findings in chest high-resolution computed tomography (HRCT) of patients with Pulmonary veno-occlusive disease (PVOD) and patients with Pulmonary Capillary Hemangiomatosis (PCH). However, clinical differential diagnosis of these two diseases is difficult and has not been established. In order to clarify their differences, we compared the sizes of GGOs in chest HRCT and the sizes of Capillary assemblies in Pulmonary vascular casts between patients diagnosed pathologically with PVOD and PCH. Methods We evaluated chest HRCT images for four patients with idiopathic Pulmonary arterial hypertension (IPAH), three patients with PVOD and three patients with PCH, and we evaluated Pulmonary vascular casts of lung tissues obtained from those patients at lung transplantation or autopsy. Results Centrilobular GGOs in chest HRCT were observed in patients with PVOD and patients with PCH but not in patients with IPAH. We measured the longest diameter of the GGOs. The size of centrilobular GGOs was significantly larger in patients with PCH than in patients with PVOD (5.60±1.43 mm versus 2.51±0.79 mm, P P Conclusion Measurement of the sizes of centrilobular GGOs in HRCT is a simple and useful method for clinical differential diagnosis of PVOD and PCH.
Carol R. Reinero - One of the best experts on this subject based on the ideXlab platform.
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clinical features of canine Pulmonary veno occlusive disease and Pulmonary Capillary Hemangiomatosis
Journal of Veterinary Internal Medicine, 2019Co-Authors: Carol R. Reinero, Isabelle Masseau, Ari L Jutkowitz, Nathan O Nelson, Samuel H Jennings, Kurt J. WilliamsAbstract:Background Histologic features of Pulmonary veno-occlusive disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) have been described in dogs but without a thorough clinical description. Objectives To report the clinical features, diagnostics, treatment, and outcome of dogs with histologic evidence of PVOD and PCH. Animals Fifteen 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. Methods Medical records of dogs with PVOD and PCH identified based on histopathologic features between 2003 and 2017 were retrospectively reviewed. Results Fifteen 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 importance In 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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Clinical features of canine Pulmonary veno‐occlusive disease and Pulmonary Capillary Hemangiomatosis
Wiley, 2019Co-Authors: Carol R. Reinero, Isabelle Masseau, Ari L Jutkowitz, Nathan Nelson, Samuel Jennings, Kurt WilliamsAbstract:Background Histologic features of Pulmonary veno‐occlusive disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) have been described in dogs but without a thorough clinical description. Objectives To report the clinical features, diagnostics, treatment, and outcome of dogs with histologic evidence of PVOD and PCH. Animals Fifteen 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. Methods Medical records of dogs with PVOD and PCH identified based on histopathologic features between 2003 and 2017 were retrospectively reviewed. Results Fifteen 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 Importance In 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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Vasoproliferative process resembling Pulmonary Capillary Hemangiomatosis in a cat.
BMC Veterinary Research, 2017Co-Authors: Jared A Jaffey, Kurt J. Williams, Isabelle Masseau, M. Krueger, Carol R. ReineroAbstract:Pulmonary 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. A 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. This 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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Vasoproliferative process resembling Pulmonary Capillary Hemangiomatosis in a cat
BMC Veterinary Research, 2017Co-Authors: Jared A Jaffey, Kurt J. Williams, Isabelle Masseau, M. Krueger, Carol R. ReineroAbstract:Background Pulmonary 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 presentation A 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. Conclusion This 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.
Kurt J. Williams - One of the best experts on this subject based on the ideXlab platform.
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clinical features of canine Pulmonary veno occlusive disease and Pulmonary Capillary Hemangiomatosis
Journal of Veterinary Internal Medicine, 2019Co-Authors: Carol R. Reinero, Isabelle Masseau, Ari L Jutkowitz, Nathan O Nelson, Samuel H Jennings, Kurt J. WilliamsAbstract:Background Histologic features of Pulmonary veno-occlusive disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) have been described in dogs but without a thorough clinical description. Objectives To report the clinical features, diagnostics, treatment, and outcome of dogs with histologic evidence of PVOD and PCH. Animals Fifteen 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. Methods Medical records of dogs with PVOD and PCH identified based on histopathologic features between 2003 and 2017 were retrospectively reviewed. Results Fifteen 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 importance In 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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Vasoproliferative process resembling Pulmonary Capillary Hemangiomatosis in a cat.
BMC Veterinary Research, 2017Co-Authors: Jared A Jaffey, Kurt J. Williams, Isabelle Masseau, M. Krueger, Carol R. ReineroAbstract:Pulmonary 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. A 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. This 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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Vasoproliferative process resembling Pulmonary Capillary Hemangiomatosis in a cat
BMC Veterinary Research, 2017Co-Authors: Jared A Jaffey, Kurt J. Williams, Isabelle Masseau, M. Krueger, Carol R. ReineroAbstract:Background Pulmonary 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 presentation A 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. Conclusion This 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.
Aya Miura - One of the best experts on this subject based on the ideXlab platform.
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different sizes of centrilobular ground glass opacities in chest high resolution computed tomography of patients with Pulmonary veno occlusive disease and patients with Pulmonary Capillary Hemangiomatosis
Cardiovascular Pathology, 2013Co-Authors: Aya Miura, Keiko Ohtaogo, Aiko Ogawa, Kengo Kusano, Satoshi Akagi, Kazufumi Nakamura, Katsushi Hashimoto, Satoshi Nagase, Kunihisa Kohno, Hiromi MatsubaraAbstract:Abstract Background Centrilobular ground-glass opacity (GGO) is one of the characteristic findings in chest high-resolution computed tomography (HRCT) of patients with Pulmonary veno-occlusive disease (PVOD) and patients with Pulmonary Capillary Hemangiomatosis (PCH). However, clinical differential diagnosis of these two diseases is difficult and has not been established. In order to clarify their differences, we compared the sizes of GGOs in chest HRCT and the sizes of Capillary assemblies in Pulmonary vascular casts between patients diagnosed pathologically with PVOD and PCH. Methods We evaluated chest HRCT images for four patients with idiopathic Pulmonary arterial hypertension (IPAH), three patients with PVOD and three patients with PCH, and we evaluated Pulmonary vascular casts of lung tissues obtained from those patients at lung transplantation or autopsy. Results Centrilobular GGOs in chest HRCT were observed in patients with PVOD and patients with PCH but not in patients with IPAH. We measured the longest diameter of the GGOs. The size of centrilobular GGOs was significantly larger in patients with PCH than in patients with PVOD (5.60±1.43 mm versus 2.51±0.79 mm, P P Conclusion Measurement of the sizes of centrilobular GGOs in HRCT is a simple and useful method for clinical differential diagnosis of PVOD and PCH.
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safety and efficacy of epoprostenol therapy in Pulmonary veno occlusive disease and Pulmonary Capillary Hemangiomatosis
Circulation, 2012Co-Authors: Aiko Ogawa, Ichiro Yamadori, Katsumasa Miyaji, Yoko Shinno, Aya Miura, Kengo Kusano, Hiroshi Date, Hiromi MatsubaraAbstract:Background: Pulmonary veno-occlusive disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) are rare causes of Pulmonary hypertension. There is no proven medical therapy to treat these diseases, and lung transplantation is thought to be the only cure. Administration of vasodilators including epoprostenol sometimes causes massive Pulmonary edema and could be fatal in these patients. Methods and Results: Eight patients were treated with epoprostenol for 387.3±116.3 days (range, 102-1,063 days), who were finally diagnosed with PVOD or PCH by pathological examination. The maximum dose of epoprostenol given was 55.3±10.7ng·kg-1·min-1 (range, 21.0-110.5ng·kg-1·min-1). With careful management, epoprostenol therapy significantly improved the 6-min walk distance (97.5±39.2 to 329.4±34.6m, P<0.001) and plasma brain natriuretic peptide levels (381.3±136.8 to 55.2±14.4pg/ml, P<0.05). The cardiac index significantly increased from 2.1±0.1 to 2.9±0.3L·min-1·m-2 (P<0.05). However, Pulmonary artery pressure and Pulmonary vascular resistance were not significantly reduced. For 4 patients, epoprostenol therapy acted as a bridge to lung transplantation. For the other patients who had no chance to undergo lung transplantation, epoprostenol therapy was applied for 528.0±216.6 days and the maximum dose was 63.9±19.0ng·kg-1·min-1. Conclusions: This study data suggest that cautious application of epoprostenol can be considered as a therapeutic option in patients with PVOD and PCH. (Circ J 2012; 76: 1729–1736)
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three dimensional structure of Pulmonary Capillary vessels in patients with Pulmonary hypertension
Circulation, 2010Co-Authors: Aya Miura, Aiko Ogawa, Hiromi Matsubara, Kengo Kusano, Satoshi Akagi, Kazufumi Nakamura, Takuro Murakami, Aiji Ohtsuka, Chikao YutaniAbstract:Pulmonary arterial hypertension, Pulmonary veno-occlusive disease, and Pulmonary Capillary Hemangiomatosis are included in the same group (group 1) of clinical classification of Pulmonary hypertension.1 Histological changes in the small Pulmonary arteries (ie, intimal fibrosis and medial hypertrophy) are similar in these 3 diseases, and clinical presentations of the 3 diseases are often indistinguishable.1 However, it is estimated that the hemodynamics of Capillary vessels are quite different in each disease. The hemodynamics of Capillary vessels (ie, Capillary occlusion) play an important role in cardiovascular diseases.2 Thus, clarification of the differences in the hemodynamics is essential to understand the pathophysiology of these 3 diseases. We obtained lung segments from patients with Pulmonary hypertension who underwent living-donor lung transplantation and from patients with …
