Cases reported "Pulmonary Heart Disease"

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1/9. 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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2/9. Reconstruction of bilateral branch pulmonary artery stenosis caused by Takayasu's aortitis.

    A 63 year-old female presented with dyspnea on exertion. Her chest X-ray showed cardiomegaly, and right ventricular overload and tricuspid regurgitation were detected. Her pulmonary ventilation and blood flow scintigraphy findings were suspicious of pulmonary vascular disease; the diagnosis was pulmonary hypertension and bilateral branch pulmonary artery stenosis. After the inflammation settled, the stenotic bilateral branch pulmonary artery was reconstructed with a prosthetic vessel and the pulmonary pressure normalized immediately. A resected specimen revealed that the stenotic changes were from Takayasu's disease. The patient's postoperative course was uneventful, and pulmonary ventilation and blood scintigraphy returned to an almost normal range. At follow-up 5 years and 6 months after the operation, there was no evidence of pulmonary artery disease (eg, stenosis and/or ischemia) or of any change in the central vessels of the retina, the so-called Takayasu's retinopathy.
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3/9. Plexogenic arteriopathy associated with pulmonary vasculitis in systemic lupus erythematosus.

    Pulmonary arterial hypertension and compulmonale were found in a woman with inactive systemic lupus erythematosus (SLE). The patient died of right heart failure 5 years later. Postmortem study showed SLE reparative lesions, plexogenic arteriopathy and vasculitis in the lung vessels. Since no active SLE was found, the pulmonary vasculitis was attributed to plexogenic arteriopathy.
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4/9. Nesiritide for pulmonary arterial hypertension with decompensated cor pulmonale.

    Pulmonary arterial hypertension complicated by decompensated cor pulmonale is a challenging clinical problem with few effective therapeutic options. B-type natriuretic peptide is a pluripotent hormone that promotes diuresis and natriuresis, vasodilates systemic and pulmonary vessels, and reduces circulating levels of endothelin and aldosterone. It may represent a possible therapeutic strategy for decompensated cor pulmonale in the same manner that it is used to treat decompensated left heart failure. The authors report their experience with B-type natriuretic peptide as adjunctive therapy for pulmonary arterial hypertension complicated by decompensated cor pulmonale. A detailed case report is presented followed by the evaluation of a series of 11 cases occurring in eight patients from December 2002 through April 2004.
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5/9. Pulmonary steal syndrome: an unusual case of coronary-bronchial pulmonary artery communication.

    The authors report a patient with angina pectoris in whom selective left coronary angiography demonstrated that the pulmonary artery branch to an apical lung segment was supplied by a bronchial collateral vessel which arose from the left circumflex artery. The anatomic and physiological developmental mechanisms, and the clinical implications, are discussed. Relief of the patient's angina following ligation of the pulmonary artery branch indicated the development of a form of pulmonary steal syndrome.
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6/9. Chronic cor pulmonale in pulmonary sarcoidosis.

    Right-heart overload happens relatively rarely in sarcoidosis, even with fibroemphysematous changes. Of 21 cases that we studied, six (28%) had clinical and/or electrocardiographic features of cor pulmonale. The cause of cor pulmonale often evoked is an invasion of the walls of pulmonary vessels by sarcoid granulomas or their compression by the fibrotic process. Pathological studies in one patient showed compression of large pulmonary arteries associated with specific sarcoid lesions in small small and medium-sized arteries.
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7/9. chondrosarcoma of the pulmonary artery.

    Primary sarcomas of the large vessels are rare. They are observed most commonly in the pulmonary artery. Up to now 28 cases of primary sarcoma of the pulmonary artery have been described in the literature. The clinical features of this disease which presents considerable diagnostic difficulties are dominated by signs of cor pulmonale. In the present paper a primary chondrosarcoma of the pulmonary artery is presented.
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8/9. Occult gastric cancer presenting as cor pulmonale resulting from tumor cell microembolism.

    Cor pulmonale resulting from tumor emboli is a rare presentation of gastric cancer, and only six similar cases have been reported in the English literature. We report the case of a 37-yr-old woman presenting with dyspnea who died of cor pulmonale. autopsy revealed signet cell carcinoma of te stomach with intra-abdominal metastasis and right ventricular hypertrophy. There were no macroscopic pulmonary emboli or parenchymal lesions, but more than 60% of the small pulmonary arteries and arterioles were occluded. In most vessels, fibrocellular intimal proliferation was the major finding with only a few entrapped tumor cells.
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9/9. Lethal pulmonary hypertension in a young woman caused by unrecognized haemangiosis carcinomatosa.

    A 38-year-old female is described, who was admitted with increasing respiratory distress, cough and visible blood stasis in the jugular veins. The most likely diagnosis in this young women taking oral contraceptives was pulmonary embolism, however, a ventilation-perfusion lung scan was normal. echocardiography showed a dilated right ventricle and increased systolic pulmonary pressure. Despite administration of oxygen and intravenous heparin the patient died in circulatory collapse before further investigations could be initiated. Post-mortem examination revealed diffuse adenocarcinoma of the stomach (linitis plastica) with metastasis to local lymphnodes, lymphangiosis carcinomatosa of the liver, pancreas and spleen and tumour cell masses within the lumen of small pulmonary arteries leading to variable occlusions of the vessels. Fibrocellular intimal proliferation was found, leading to further obstruction and increased resistance to flow through the pulmonary vascular bed and to subacute cor pulmonale.
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