Cases reported "Hypertension, Pulmonary"

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1/1352. A case of truncus arteriosus type II.

    A case of truncus arteriosus type II is reported. Truncus arterious is an uncommon congenital cardiac defect where a single great vessel exits the heart. truncus arteriosus is usually fatal, if untreated. This defect occurs when the conus arteriosus and the truncus divide erroneously in the embryo. Palliative surgery in truncus arteriosus has been unsuccessful. Pulmonary banding has been tried and was ineffective and usually fatal. We operated on a nine-month-old (6200 g) male infant with a type II (Edwards-Collett) defect and a large ventricular septal defect. The pulmonary artery average pressure was 51 mmHg. We performed a cardiopulmonary bypass in the usual manner. Pulmonary arteries were resected from the truncal root, and primary end-to-end anastomosis of the truncal root to the ascending aorta was performed. Right ventricle to pulmonary artery continuity was provided using a valveless Gore-Tex graft. We lost our patient due to intractable pulmonary hypertension on the first postoperative day. ( info)

2/1352. Primary pulmonary hypertension in a patient with hiv infection.

    Several case-reports and small series suggest a causal relationship between human immunodeficiency virus (hiv) infection and pulmonary hypertension. We report on a hiv seropositive man with a high and stable cd4 lymphocyte count ( /- 600/mm3) who developed severe pulmonary hypertension, not attributable to other known causes. This case report underscores the fact that the degree of immunosuppression secondary to the hiv-infection seems to be of little relevance in the pathophysiology of the syndrome. hiv-infected patients with dyspnoea, not related to pulmonary infection, with exercise intolerance, syncope or precordial pain should receive an electrocardiogram and echocardiographic assessment. The exact pathogenetic mechanism of this rapidly progressive disease and whether anti-viral therapy should be promoted is still under investigation. ( info)

3/1352. Reversal of severe pulmonary hypertension with beta blockade in a patient with end stage left ventricular failure.

    A 52 year old man with severe chronic left ventricular failure (new york Heart association class IV) was considered unsuitable for cardiac transplantation because of high and irreversible pulmonary vascular resistance (PVR). In an attempt to produce symptomatic improvement, metoprolol was cautiously introduced, initially at 6.25 mg twice daily. This was slowly increased to 50 mg twice daily over a two month period and continued thereafter. After four months of treatment the patient's symptoms had improved dramatically. His exercise tolerance had increased and diuretic requirements reduced to frusemide 160 mg/day only. Assessment of right heart pressures was repeated and, other than a drop in resting heart rate, there was little change in his pulmonary artery pressure or PVR. His right heart pressures were reassessed showing a pronounced reduction in pulmonary artery pressure and a significant reduction in PVR, which fell further with inhaled oxygen and sublingual nitrates. He was then accepted onto the active waiting list for cardiac transplantation. A possible mechanism of action was investigated by assessing responses to beta agonists during treatment. Not only was there pronounced improvement in PVR but it was also demonstrated that beta receptor subtype cross-regulation may have contributed to the mechanism of benefit. ( info)

4/1352. Thrombolysis with resolution of pulmonary hypertension in a heart transplant candidate.

    We report a patient with idiopathic cardiomyopathy and high pulmonary resistance due to pulmonary emboli of unknown age. Successful thrombolytic therapy returned his pulmonary resistance to normal, allowing orthotopic cardiac transplantation. This case underscores the need to aggressively diagnose and treat pulmonary emboli in potential transplant candidates. ( info)

5/1352. Vanishing pulmonary hypertension in mixed connective tissue disease.

    A 29-year-old woman with mixed connective tissue disease presented with signs of progressive pulmonary hypertension. After admission to the hospital her condition worsened rapidly and she developed a cardiac arrest resistant to cardiopulmonary resuscitation. Therefore, emergency extracorporeal assist was performed. No pulmonary embolism was found. Right heart catheterisation showed severe pulmonary hypertension, which was treated with nitric oxide ventilation. She was weaned from the extracorporeal assist with high doses of inotropic agents. Because of suspicion of exacerbation of her underlying disease, which led to pulmonary hypertension, immunosuppressive treatment was started with high doses of corticosteroids and plasma exchange. This resulted in slow recovery over the next four weeks. Control echocardiography showed complete normalisation of cardiac function without signs of pulmonary hypertension. Two months after admission she was discharged from the hospital in good condition. ( info)

6/1352. Effective immunosuppressive therapy in a patient with primary pulmonary hypertension.

    The case history is described of a young woman who presented with primary pulmonary hypertension and non-specific inflammatory signs. The patient received prolonged immunosuppressive treatment with low dose methotrexate and prednisone without any vasodilator agent. After one year the pulmonary artery pressure fell from a mean value of 47 mm Hg to 30 mm Hg and there was a corresponding clinical response. This case suggests that, in patients with pulmonary hypertension of unknown origin, immunopathogenetic factors should be sought in order to consider the utility of immunosuppressive therapy. ( info)

7/1352. Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension.

    Left main coronary artery compression by the pulmonary artery may be seen in patients with pulmonary hypertension who are undergoing cardiac catheterization. Cardiac magnetic resonance imaging is useful in these patients to document extrinsic compression, which might otherwise be mistaken for intrinsic atherosclerotic disease. ( info)

8/1352. Anaesthesia for caesarean section in the presence of severe primary pulmonary hypertension.

    We describe the case of a pregnant woman, 35 weeks' gestation, with primary pulmonary hypertension and coarctation of the aorta requiring emergency Caesarean section under general anaesthesia. The patient had a pulmonary artery catheter inserted before operation which revealed pulmonary artery pressures in excess of 80/40 mm Hg. These were lowered using an infusion of glyceryl trinitrate. After delivery of the baby and administration of oxytocin, pulmonary artery pressures were more difficult to control. An infusion of prostacyclin was substituted which stabilized pulmonary pressures. After operation, she was transferred to the intensive care unit where prostacyclin was administered by an "aerosolized" route. Her trachea was extubated after 48 h and she made an uneventful recovery. ( info)

9/1352. Inhaled nitric oxide therapy in pregnancy complicated by pulmonary hypertension.

    The use of nitric oxide as an agent to reduce pulmonary artery pressure in a pregnancy complicated by pulmonary hypertension is reported for the first time. This therapy can reduce pulmonary vascular resistance and therefore potentially enable the right ventricle to better compensate for the physiologic changes of pregnancy. ( info)

10/1352. Intravenous injection of talc-containing drugs intended for oral use. A cause of pulmonary granulomatosis and pulmonary hypertension.

    Clinical and morphologic features are described in two patients known to have repeatedly injected intravenously talc-containing drugs intended for oral use. In one patient severe pulmonary hypertension developed; the talc granulomas in him were located predominantly within the pulmonary arteries. The second patient had normal pulmonary arterial pressures, and the talc granulomas in him were located predominantly in the pulmonary interstitium. Of 19 previously described patients with pulmonary talc granulomas, 12 had morphologic evidence of pulmonary hypertension (in three of severe degree); in each, talc granulomas were located predominantly within the pulmonary arteries. In those without signs of pulmonary hypertension, granulomas were located predominantly in the pulmonary interstitium. Why there are differences in the distribution of the talc granulomas is unclear. It is clear, however, as demonstrated by one of our patients, that severe pulmonary hypertension may be a consequence of intravenous injection of drugs intended for oral use. ( info)
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