Cases reported "Hypertension, Pulmonary"

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1/30. Primary pulmonary hypertension with fatal outcome in a young woman and review of the literature.

    A 32-year-old female is described, who was admitted with symptoms of severe right heart failure. The most likely diagnosis of pulmonary embolism was excluded. echocardiography and left-right catheterisation confirmed the diagnosis of primary pulmonary hypertension. A possible mediator in the process of PPH could be the appetite suppressants she had taken for some months after her second pregnancy. Before further pharmacologic tests could be performed the patient died in circulatory collapse. Postmortem pathological examination confirmed the diagnosis of PPH by the presence of narrowed pulmonary arterioles, media hypertrophy, thrombotic lesions and normal surrounding pulmonary parenchyma. The literature on primary pulmonary hypertension is revised with special emphasis on diagnosis and treatment algorithms.
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keywords = arteriole
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2/30. Congenital misalignment of pulmonary vessels and alveolar capillary dysplasia: how to manage a neonatal irreversible lung disease?

    Congenital misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia is a rare condition consisting of anomalous veins in bronchovascular bundles, a decreased number of alveolar capillaries, and increased muscularization of pulmonary arterioles. In the literature, infants reported as having such a malformation developed respiratory distress with persistent pulmonary hypertension and ultimately died. We report the case of an infant with MPV and alveolar capillary dysplasia who was unresponsive to maximal cardiorespiratory support, including high-frequency oscillatory ventilation and inhaled nitric oxide; the infant died of pulmonary hemorrhage after 19 days, during venoarterial extracorporeal membrane oxygenation bypass. We conclude that the diagnosis of MPV and alveolar capillary dysplasia should be considered during autopsy of infants who have died of irreversible persistent pulmonary hypertension. If a lung biopsy in infants with prolonged refractory hypoxemia confirms such diagnosis before death, expensive and invasive treatments such as extracorporeal membrane oxygenation could be avoided.
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3/30. Congenital alveolar capillary dysplasia with misalignment of pulmonary veins associated with hypoplastic left heart syndrome.

    Three full-term infants died in the first month of life with hypoplastic left heart syndrome (HLH) and persistent pulmonary hypertension (PPH). At postmortem examination, they were found to have alveolar capillary dysplasia with misalignment of pulmonary veins (ACD with MPV). The association of HLH syndrome, and ACD with MPV with intestinal malrotation and/or obstruction, is unique. Decreased blood flow in the ascending aorta in fetuses with left outflow tract obstruction might cause vasoconstriction of pulmonary arterioles to maintain cerebral perfusion. vasoconstriction early during embryogenesis might lead to decreased growth and development of alveolar capillaries and pulmonary veins. This results in pulmonary hypertension, and the arterial blood is forced to bypass the deficient capillary bed and can drain only via the anomalous bronchial veins.
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keywords = arteriole
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4/30. Reduction in recalcitrant pulmonary hypertension after operation for atrial septal defect.

    We present the case of a patient with atrial septal defect and severe pulmonary hypertension with pulmonary artery peak pressure greater than 110 mm Hg. Open lung biopsy was done prior to the corrective operation, and pathological findings in the small pulmonary arteries included "musculoelastosis" and complete occlusion of 70% of these small arteries and arterioles. The atrial septal defect was closed, and long-term oral prostacyclin therapy was initiated. pulmonary artery peak pressure decreased to 65 mm Hg 2 years after the operation. This case demonstrates that in a patient with 70% complete occlusion of small pulmonary arteries and arterioles resulting from "musculoelastosis," not only is surgical intervention possible but also pulmonary artery pressure decreases in the long term after operation.
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keywords = arteriole
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5/30. autopsy findings of heart and lungs in a patient with primary pulmonary hypertension associated with use of fenfluramine and phentermine.

    A 36-year-old woman (height, 157 cm; weight, 117 kg; body mass index, 47.5) received fenfluramine and phentermine (fen-phen) for 7 months, and pulmonary hypertension subsequently developed. Her pulmonary arterial pressure was 56 mm Hg, and echocardiography showed right ventricular dilatation and hypokinesia. Cardiopulmonary arrest developed during right-heart catheterization, and she died 3 days later. At autopsy, right ventricular dilatation with fibroproliferative tricuspid valve was identified. The pulmonary arteries, including the main arteries and elastic arteries to the arterioles, revealed fibroproliferative plaque; the latter was more severe and more prominent in the upper lobes than in the lower lobes. Combined cardiac valvular disease and pulmonary hypertension appear to occur frequently in patients receiving fen-phen, and more autopsy cases of patients with a history of fen-phen usage are warranted to document the frequency of combined cardiac valvular disease and pulmonary hypertension in the united states.
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keywords = arteriole
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6/30. Fatal pulmonary hypertension associated with an atypical case of Hunchinson-Gilford progeria.

    We report the first autopsied case of an incomplete type of Hunchinson-Gilford progeria associated with fatal pulmonary hypertension. Histopathologic findings revealed abnormal deposition of collagen and elastic fibers, as well as cellular proliferation, at intima of the coronary arteries, pulmonary small arteries, and arterioles. These changes could be underlying conditions of the association of the two diseases.
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keywords = arteriole
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7/30. fatal outcome in Eisenmenger syndrome.

    AIM: To assess correlations between fatal outcome and histologic findings of pulmonary vascular disease in different situations of Eisenmenger syndrome, either during the natural course or early-late after surgery. MATERIAL AND methods: The clinical follow-up and fatal outcome of 20 patients affected by Eisenmenger syndrome were investigated. In addition to the pathologic report and gross reexamination of the heart, the lung tissue was studied by histology. patients were divided into three groups: 6 non-operated patients who died during the natural course (Group 1), 11 patients who underwent correction of the congenital defect and died in the perioperative period (Group 2), and 3 patients who died late after surgery (Group 3). RESULTS: In Group 1, five patients (83%) died of cardiac arrest a few days after the onset of hypoxic attacks; in four patients histology showed Grade IV pulmonary vascular disease with diffuse fibrinoid necrosis in the distal pulmonary arterial vasculature. In Group 2, nine patients (82%) died on the first or second postoperative day after a refractory pulmonary hypertensive crisis, with histologic evidence in three patients of fibrinoid necrosis of the distal pulmonary small arteries and arterioles. In Group 3, two patients (67%) died suddenly, 6 and 18 years after cardiac surgery, following onset of dyspnea and cardiogenic shock; autopsy showed aneurysmal dilatation of the pulmonary artery with massive thrombosis in the setting of Grades III-IV pulmonary vascular disease without fibrinoid necrosis. CONCLUSION: fatal outcome in Eisenmenger syndrome, either in the natural course or after refractory hypertensive attacks post surgery, is frequently associated with fibrinoid necrosis of the small pulmonary arteries and arterioles.
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keywords = arteriole
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8/30. Atrial septal defect with borderline pulmonary vascular disease: surgery and long-term oral prostacyclin therapy for recalcitrant pulmonary hypertension.

    The hemodynamic determination of operability in atrial septal defect (ASD) with severe pulmonary hypertension is problematic. Therefore, we perform an open lung biopsy prior to the corrective surgery in cases with pulmonary vascular resistance greater than 8 units x m2 and/or pulmonary arterial peak pressure greater than 70 mmHg. We present 4 cases showing occlusion of more than 70% of the small pulmonary arteries and arterioles by musculoelastosis, thromboembolism and mixed-type (musculoelastosis and plexogenic arteriopathy) which was considered borderline in terms of operability. After complete closure of the ASD and postoperative long-term oral prostacyclin (PGI2) therapy, pulmonary artery peak pressure decreased from 110-72 (mean 84) to 105-45 (mean 74) mmHg immediately after operation and 65-40 (mean 57) mmHg after PGI2 therapy. The New York Heart association functional status of the patients improved from class II-III to class I with oral PGI2 only. Our cases demonstrate that despite more than 70% occlusion of the small pulmonary arteries and arterioles, surgery and long-term PGI2 therapy can reduce pulmonary artery pressure and improve the quality of life.
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ranking = 2
keywords = arteriole
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9/30. Subacute cor pulmonale due to tumor embolism.

    We describe a patient wih subacute cor pulmonale caused by tumor emboli in the lungs. A 64-year-old female suffering from a subacute progressive cough and shortness of breathing died of severe pulmonary hypertension seven days after admission. Neither chest CT scans nor lung perfusion scintigraphy showed any abnormal findings. Microscopic examination after an autopsy revealed diffuse intravascular tumor emboli occluding not only the small pulmonary arteries and arterioles, but also the lymphatic vessels, which were suggested to be metastases of a breast carcinoma resected five years previously. Thus, pulmonary tumor embolism should be considered in the differential diagnosis of primary pulmonary hypertension, particularly in patients with a past history of cancers.
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keywords = arteriole
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10/30. Blue rubber bleb nevus syndrome and pulmonary hypertension: an unusual association.

    INTRODUCTION: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital systemic angiodysplasia with multiple vascular malformations in the skin, gastrointestinal tract and, less often, in other internal organs and the brain. CASE REPORT: A 36-year-old man with past history of BRBNS was admitted to our hospital for progressive dyspnea and fatigue. Primary pulmonary hypertension (PPH) was diagnosed. He then developed acute abdominal pain and dyspnea, dying in a few hours due to sudden cardiac arrest. Postmortem examination demonstrated angiomatous lesions located in the skin, small bowel, heart, lungs, liver and thyroid. The lesions were slightly raised, soft and compressible and microscopically consisted of dilated vascular channels lined by a flattened endothelium. The vascular wall was formed by several layers of smooth muscle cells, intermixed with abundant aggregates of elastic lamellae and thin collagen fibers. Luminal thrombi were a frequent finding. In the small bowel, we identified the presence of an abnormally large artery directly opening into a thin-walled venous channel. The most striking finding in the lungs was the presence of thrombi of varying age in the lumen of segmental and elastic arteries, as well as muscular arteries and arterioles. Severe medial hypertrophy of muscular arteries and muscolarization of arterioles were also present. Intimal proliferative lesions and plexiform lesions were never observed. CONCLUSION: The pulmonary findings are consistent with recurrent thromboembolic events from shunts in the visceral lesions. To our knowledge, this is the first report of BRBNS with visceral arterovenous (AV) fistulae complicated by thromboembolic pulmonary hypertension (PH).
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ranking = 2
keywords = arteriole
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