Cases reported "Pulmonary Heart Disease"

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1/5. 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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ranking = 1
keywords = vascular disease
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2/5. Severe pulmonary vascular disease in systemic lupus erythematosus.

    A young woman with systemic lupus erythematosus (SLE) had clinical evidence of acute cor pulmonale. autopsy disclosed vascular lesions in the lungs resembling those seen in advanced pulmonary hypertension. This case illustrates that severe pulmonary vascular disease may complicate SLE and mimic pulmonary thromboembolic disease.
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ranking = 5
keywords = vascular disease
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3/5. Mustard operation and creation of ventricular septal defect in two patients with transposition of the great arteries, intact ventricular septum and pulmonary vascular disease.

    The condition of patients with transposition of the great arteries, intact ventricular septum and severe pulmonary vascular disease is inoperable with present techniques. In a series of 260 surgically treated patients with transposition of the great arteries and intact ventricular spetum, 5 had severely increased pulmonary vascular resistance, and all 5 died; postmortem examination confirmed the presence of severe pulmonary vascular disease. The concept of the "palliative Mustard" procedure was applied in two children with transposition of the great arteries, intact ventricular spetum and pulmonary vascular disease who underwent the Mustard operation with creation of a ventricular septal defect. The postoperative course was uneventful in both patients. Thirteen and 5 months, respectively, after operation, both are physically active and have respective arterial oxygen saturation levels of 94 and 92 percent.
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ranking = 7
keywords = vascular disease
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4/5. Pulmonary hypertension and cor pulmonale in the sickle hemoglobinopathies.

    Although pulmonary hypertension is frequently mentioned as a complication of the sicklemic state, careful review of the medical literature revealed only a single subject in whom cardiac catheterization data substantiated this diagnosis. In two additional patients, both clinical and autopsy findings of pulmonary vascular disease and cor pulmonale were described, although no hemodynamic studies had been performed. We have therefore detailed the clinical history, cardiac catheterization results, and autopsy findings in three previously undescribed patients. These three patients, along with the three case reports culled from the medical literature, from the substance of this review. Pulmonary hypertension should be suspected in patients with sickle hemoglobinopathy in whom either fixed dyspnea or unexplained syncope develops. Early in the course of the disease, right heart catheterization remains the only way to establish the diagnosis with certainty. Noninvasive studies such as chest x-ray, electrocardiography, and echocardiography tend to be nondiagnostic until late in the course of right ventricular failure. Although specific therapy has yet to be defined, the ominous prognosis of this complication of sickle hemoglobinopathy supports the application of experimental modalities such as continuous oxygen therapy, partial exchange transfusion, or even limited phlebotomy.
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ranking = 1
keywords = vascular disease
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5/5. Oral hydralazine in patients with pulmonary vascular disease secondary to congenital heart disease.

    Two patients with pulmonary vascular obstructive disease secondary to congenital heart disease were evaluated hemodynamically before and during oral hydralazine therapy. Both patients were assessed postoperatively and had no significant residual shunts. Pulmonary vascular resistance failed to decrease, and an increase in pulmonary arterial pressure occurred because of increased cardiac output secondary to systemic arteriolar dilatation. These responses were sufficiently consistent to warrant a warning against the use of oral hydralazine except under controlled conditions in patients with secondary pulmonary vascular obstructive disease.
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ranking = 4
keywords = vascular disease
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