Cases reported "Hypertension, Pulmonary"

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1/334. 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.
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2/334. 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.
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3/334. 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.
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4/334. 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.
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5/334. 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.
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6/334. 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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7/334. cesarean section in a mother with uncorrected congenital coronary to pulmonary artery fistula.

    PURPOSE: We report a case of a 33 yr old woman with pulmonary hypertension secondary to uncorrected right coronary artery to pulmonary artery fistula who underwent two successful operative deliveries under general anesthesia. CLINICAL FEATURES: This woman underwent an emergency Caesarean section at 32 wk gestation because she presented in NYHA Class IV, heart failure and premature labour. She did not have antenatal follow-up. For her second pregnancy, she was managed from the first trimester of pregnancy by the cardiologist, obstetrician and anesthesiologist. She received oral furosemide and digoxin from eight weeks gestation. pregnancy was managed to term before she progressed to NYHA Class IV and cardiac failure at 37 wk gestation. She had a Caesarean section under general anesthesia. She received rapid sequence induction of anesthesia and tracheal intubation with 0.1 mg x kg(-1) etomidate, 2 mg x kg(-1) succinylcholine and maintenance with nitrous oxide 50% in oxygen, isoflurane 1% and 0.1 mg x kg(-1) vecuronium. fentanyl, 2 microg x kg(-1) helped to obtund the hypertensive response to intubation. analgesia was provided with 1 mg x kg(-1) morphine. Glyceryl trinitrate infusion, 10-30 microg x min(-1) was used in addition to the anti-heart failure therapy. End-tidal capnography, electrocardiogram, pulse oximetry, continuous arterial blood pressure and pulmonary arterial catheter provided hemodynamic monitoring. The lungs were mechanically ventilated for 24 hr postoperatively. She received anti-heart failure therapy which she continued after discharge. She was NYHA class II upon discharge. She defaulted from further follow-up. CONCLUSION: Although the literature advocates, in this situation, controlled vaginal delivery utilising epidural analgesia, we describe the successful outcome for operative delivery under general anesthesia in a patient with secondary pulmonary hypertension and heart failure.
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8/334. Management of postoperative chylothorax with nitric oxide: a case report.

    OBJECTIVE: To describe the use of inhaled nitric oxide in the management of refractory postoperative chylothorax. DESIGN: Case report. SETTING: A pediatric intensive care unit of a tertiary care children's hospital. PATIENT: A neonate with refractory chylothoraces complicated by moderate pulmonary hypertension after a complicated arterial switch operation. INTERVENTIONS: Administration of inhaled nitric oxide through a ventilator circuit. MEASUREMENTS AND MAIN RESULTS: The institution of inhaled nitric oxide at 20 ppm resulted in a marked reduction in chest tube drainage and a decrease in echocardiographically estimated pulmonary artery pressure from 50%-75% systemic to 30%-50% systemic. Chest tube drainage doubled when the nitric oxide was decreased to 10 ppm and, again, dramatically decreased after raising nitric oxide back to 20 ppm. After 8 days of nitric oxide therapy, the chest tube drainage ceased. nitric oxide therapy was successfully discontinued 19 days after initiation, with no recurrence of chylothorax. There was no effect of nitric oxide on systemic blood pressure. methemoglobin levels while on NO remained <1.7%. CONCLUSION: Consideration may be given to the use of inhaled nitric oxide in the therapy of refractory chylothoraces complicated by central venous hypertension.
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9/334. Post-Mustard procedure pulmonary venous obstruction: An opportunity for anatomic correction with a one-stage arterial switch.

    A 14-year-old boy after a Mustard procedure for transposition of the great arteries developed pulmonary hypertension secondary to baffle obstruction. This occurred over several years without apparent significant symptomatology. Systemic-level pressure prevailed in the left (pulmonary) ventricle and provided an opportunity to perform a successful one-stage arterial switch.
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10/334. Improvement of pulmonary hypertension after liver transplantation.

    Pulmonary hypertension at the end stage of chronic liver disease is not an uncommon situation. This association termed portopulmonary hypertension raises the question of the feasibility of performing orthotopic liver transplantation (OLT). In the case reported herein, there was a favorable outcome after OLT, even though the mean pulmonary artery pressure (MPAP) before transplantation was increased to 45 mm Hg. Before OLT, the cardiac index (CI) was considerably elevated (7.69 L/min/m2), giving evidence of a marked hyperdynamic circulatory state. The CI decreased significantly after OLT (3.38 L/min/m2), and this produced a significant decrease in the MPAP. Our observation suggests that portopulmonary hypertension due to a marked increase in the CI can be managed successfully by OLT.
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