Cases reported "Hypertension, Pulmonary"

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1/130. 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.
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2/130. Transient left ventricular failure following bilateral lung transplantation for pulmonary hypertension.

    BACKGROUND: Bilateral lung transplantation is an established therapy for end-stage pulmonary hypertension. Its early postoperative outcome may be biased by various complications resulting in unexpected deterioration of the patient in terms of hemodynamics and blood gases. methods: We have reviewed the early postoperative course of patients who underwent bilateral lung transplantation for pulmonary hypertension at our institution and analyzed all available data, especially hemodynamic measurements, echocardiographic documentation and therapeutical strategies, in those cases where cardiac dysfunction was found to be responsible for clinical deterioration. RESULTS: Three out of 20 lung transplant recipients operated for pulmonary hypertension experienced severe respiratory insufficiency accompanied by hemodynamic decompensation during the first days after surgery. Clinical and laboratory findings together with results of echocardiography and pulmonary artery catheterism helped establish the diagnosis of left ventricular failure. This proved to be transitory, but the response to therapy (inotropic drugs, afterload reduction and eventually prostaglandins) was very variable. Adequately treated, this complication did not preclude the outcome of transplantation by itself. CONCLUSION: Left ventricular failure is a possible complication after lung transplantation for pulmonary hypertension. echocardiography and pulmonary artery catheterism may be useful adjuvant diagnostic tools, beside routine physical examination, chest X-ray, and laboratory analysis. Therapy of this complication must be adapted individually and may be complex.
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3/130. Complex vascular lesions at autopsy in a patient with phentermine-fenfluramine use and rapidly progressing pulmonary hypertension.

    Anorectic agents, such as aminorex and fenfluramine derivatives, have been associated previously with the development of primary pulmonary hypertension. The combination diet drug phentermine-fenfluramine (or "phen-fen") was recently marketed in the united states. We describe a case of a 39-year-old woman with rapidly progressing, fatal pulmonary hypertension following administration of phentermine-fenfluramine. autopsy was remarkable for complex pulmonary vascular lesions most consistent with thrombotic arteriopathy.
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4/130. Progressive pulmonary vascular disease after pulmonary artery banding and total correction in a case of ventricular septal defect and pulmonary hypertension.

    A 7-month-old infant with ventricular septal defect and pulmonary hypertension underwent pulmonary artery banding, which resulted in a decrease in the pulmonary arterial peak pressure from 102 to 54 mmHg. Lung biopsy findings showed at most an early grade 3 Heath-Edwards classification, and an index of pulmonary vascular disease of 1.4, both of which indicated operability for total correction. Small pulmonary arteries less than 100 microns in diameter, however, showed marked hydropic changes in the medial smooth muscle cells. Total correction was performed at the age of 2 years, but the pulmonary arterial pressure failed to decrease. A lung biopsy taken just after the closure of the ventricular septal defect contraindicated operability due to progressive pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.4. The patient died at 8 months after the operation, and an autopsy revealed still more advanced pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.8. The pathogenesis of arterial changes is discussed.
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5/130. Combination of inhaled nitric oxide and intravenous prostacyclin for successful treatment of severe pulmonary hypertension in a patient with acute respiratory distress syndrome.

    OBJECTIVE: To investigate the combination of inhaled nitric oxide (iNO) and intravenously administered prostacyclin (i.v. PGI2) in a patient with severe pulmonary hypertension and acute respiratory distress syndrome (ARDS). DESIGN: Single case study. SETTING: intensive care unit of a university hospital. methods: In an ARDS patient with severe pulmonary hypertension, gas exchange and hemodynamics were measured during combined treatment with iNO and i.v. PGI2. On two subsequent days, a protocol consisting of four 20-min periods was performed: baseline, 10 ppm iNO, 10 ppm iNO plus 4 ng kg-1 min-1, and 4 ng kg-1 min-1 PGI2 alone. At the end of each period hemodynamic and gas exchange data were obtained. RESULTS: The combination of iNO and i.v. PGI2 resulted in a marked decrease in pulmonary artery pressure and a concomitant increase in cardiac output which was more pronounced than the effect of either drug alone. During iNO, as well as during the combination of iNO and i.v. PGI2, oxygenation was improved, whereas during i.v. PGI2 alone oxygenation was worse than baseline. CONCLUSION: We conclude that the combination of iNO and i.v. PGI2 might be more useful than either drug alone when severe pulmonary hypertension leading to impaired right ventricular function is present in ARDS. A systematic study of this observation is warranted.
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6/130. nifedipine-induced gingival hyperplasia: non-surgical management of a patient.

    A clinical report of nifedipine-induced gingivitis in a medically compromised patient is presented. This case history also describes the challenges faced by the oral health practitioner to develop an appropriate regimen of treatment for a patient in whom neither withdrawal of the drug nor substituting for it was feasible, and periodontal surgery was contra-indicated.
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7/130. Diagnosing and treating primary pulmonary hypertension.

    Primary pulmonary hypertension (PPH) is a pulmonary vascular disease characterized by an elevation in mean pulmonary artery pressure and pulmonary vascular resistance. Recently, PPH gained national attention because of its association with appetite suppressants. PPH may also be associated with pregnancy, hypothyroidism, autoimmune disorders, human immunodeficiency virus infection, and the use of drugs such as oral contraceptives and cocaine. patients with PPH may report dyspnea on exertion and fatigue. early diagnosis is crucial. New therapeutic regimens have dramatically reduced mortality rates and improved quality of life by halting the progression of pulmonary vascular remodeling and averting right-sided heart failure. These therapies include high-dose calcium channel antagonists, anticoagulants, and continuous intravenous prostacyclin. Lung or heart-lung transplantation remains a viable therapeutic option for patients who are treated late in the disease process, who are not responsive to medical management, or who remain symptomatic and continue to deteriorate.
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8/130. In utero development of hypertensive necrotizing pulmonary arterial lesions: report of a case associated with premature closure of the ductus arteriosus and pulmonary hypoplasia.

    Premature closure of the ductus arteriosus (PCDA) is an uncommon defect in which pulmonary hypertension (PH) has been documented by echocardiography in patients and by direct measurement after experimental PCDA in animals. The pulmonary vascular histology in human cases has received little attention but in the few recorded observations the vessels were either normal or showed increased muscularity. We report the case of a 31 week hydropic female stillborn monozygotic twin in whom postmortem examination disclosed PCDA and hypoplasia of the lungs. Atypical plexiform lesions with necrotizing pulmonary arteritis were present. These lesions represent vascular consequences of severe pulmonary hypertension produced by greatly enhanced blood flow through a restricted vascular bed resulting from the combined effects of these two abnormalities. The findings in this case of PCDA with presumed severe PH indicate that severe pulmonary vascular changes can develop in utero and that the interval of time needed for development of such chances in secondary PH is relatively short.
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9/130. isosorbide-5-mononitrate in the treatment of pulmonary hypertension associated with portal hypertension.

    Pulmonary hypertension is uncommonly associated with portal hypertension. The current approach for the management of pulmonary hypertension involves the use of vasodilators in patients who show vascular responsiveness during an acute challenge. Since the association of portal hypertension with pulmonary hypertension is very seldomly presented, its optimal therapy has not been defined. Moreover, calcium-channel blockers, which are usually used in pulmonary hypertension treatment, may exert a deleterious effect on portal hypertension. Therefore, the search for drugs that may be active under both conditions has important clinical implications. This report presents the case of a patient with portal hypertension-associated pulmonary artery hypertension that was effectively treated with isosorbide-5-mononitrate (Is-5-Mn). The patient had severe portal hypertension (hepatic venous pressure gradient=14.5 mmHg) and pulmonary hypertension (mean pulmonary artery pressure (PAP)=50 mmHg). Acute administration of prostacyclin and nitric oxide elicited a significant reduction in both PAP and pulmonary vascular resistance (PVR), an effect that was also achieved with Is-5-Mn. The patient was treated with 40 mg Is-5-Mn twice daily and a haemodynamic study performed 6 months later showed that the reduction in both PAP and PVR persisted.
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10/130. Persistent pulmonary hypertension after maternal naproxen ingestion in a term newborn: a case report.

    Constricting effect of indomethacin on the ductus arteriosus of the fetus is well known. The fetal effects of other nonsteroid anti-inflammatory drugs (NSAIDs) like naproxen are not well reported. We report here a case of a 3,790-g term neonate who developed persistent pulmonary hypertension after birth with a closed ductus arteriosus. The mother admitted to taking naproxen sodium immediately prior to the birth of the infant. The course of illness was progressively better on conservative management. Like indomethacin, other NSAIDs can also cause premature closure of fetal ductus arteriosus, pulmonary hypertension, and life-threatening problems to the neonate. Patient education regarding over-the-counter pain medication during pregnancy should be emphasized.
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