Cases reported "Hypertension, Pulmonary"

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1/203. 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.
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2/203. 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.
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3/203. 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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4/203. Primary pulmonary hypertension with fatal outcome in a young woman and review of the literature.

    A 32-year-old female is described, who was admitted with symptoms of severe right heart failure. The most likely diagnosis of pulmonary embolism was excluded. Echocardiography and left-right catheterisation confirmed the diagnosis of primary pulmonary hypertension. A possible mediator in the process of PPH could be the appetite suppressants she had taken for some months after her second pregnancy. Before further pharmacologic tests could be performed the patient died in circulatory collapse. Postmortem pathological examination confirmed the diagnosis of PPH by the presence of narrowed pulmonary arterioles, media hypertrophy, thrombotic lesions and normal surrounding pulmonary parenchyma. The literature on primary pulmonary hypertension is revised with special emphasis on diagnosis and treatment algorithms.
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5/203. 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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6/203. 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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7/203. Combined lung and liver transplantation in a girl with cystic fibrosis.

    PURPOSE: To describe the anesthetic considerations of a combined lung and liver transplant in a 14-yr-old girl with cystic fibrosis. CLINICAL FEATURES: A 14 yr-old girl with cystic fibrosis presented for combined liver and lung transplantation. Anesthetic management was complex in that the pulmonary, hemodynamic, and hematological changes after cardiopulmonary bypass and lung transplantation made the management of the subsequent liver transplant unique. We used a moderate dose fentanyl and isoflurane anesthetic with invasive monitoring including a pulmonary artery catheter. Upon reperfusion of the new liver our patient exhibited severe pulmonary hypertension that was associated with a decrease in cardiac output and systemic hypotension. Utilizing a pulmonary artery catheter, this episode was treated with an increase of prostaglandin E1 (PGE1) infusion to 0.025 microg x kg(-1) x min(-1) and the initiation of 3 microg x kg(-1) x min(-1) dobutamine. The pulmonary hypertension resolved and the cardiac output and blood pressure returned to baseline levels. CONCLUSION: The anesthetic considerations for a combined lung and liver transplant are complex because of the interactions and alterations in cardiovascular, pulmonary and hemostatic systems. The use of a pulmonary artery catheter was critical to the management of our patient because it allowed us to accurately treat an episode of hypotension occurring during liver transplantation. This episode was secondary to acute pulmonary hypertension which is common after pulmonary transplantation but unusual during liver transplantation. It is also critical that a team approach is used to consider all of the concerns of the multiple services managing these complex patients.
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8/203. Anastomotic pulmonary hypertension after lung transplantation for primary pulmonary hypertension: report of surgical correction.

    This report describes a patient who developed pulmonary hypertension 6 years after lung transplantation for primary pulmonary hypertension (PPH). Evaluation with right heart catheterization followed by pulmonary angiography, however, demonstrated that the pulmonary hypertension was secondary to an anastomotic narrowing of the pulmonary artery, rather than a recurrence of her PPH. Vascular complications of lung transplantation should be considered in patients who experience exertional dyspnea after lung transplantation. The suggestion of pulmonary hypertension on echocardiography should prompt further evaluation, including meticulous hemodynamic measurements.
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9/203. Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis.

    We report the case of a patient with liver cirrhosis who successively developed hepatopulmonary syndrome and portopulmonary hypertension. Initially, the patient presented with severe dyspnea and hypoxemia at rest. technetium-99 macroaggregated albumin lung perfusion scan demonstrated right-to-left shunt, and hemodynamic study revealed a hyperdynamic state with low pulmonary vascular resistance, thus confirming the diagnosis of hepatopulmonary syndrome. More than 2 years after the onset of pulmonary symptoms, a marked improvement in dyspnea and gas exchange was observed. Lung perfusion scan did not disclose any right-to-left shunt and right-sided heart catheterization showed evidence of severe pulmonary hypertension. We conclude that hepatopulmonary syndrome and portopulmonary hypertension are not mutually exclusive. We hypothesize that, by reversing the phenomenon of intrapulmonary vasodilatation, the development of portopulmonary hypertension interfered with each of the potential causes of hypoxemia in hepatopulmonary syndrome (ventilation-perfusion inequalities, intrapulmonary shunting, oxygen diffusion limitation) and, as a result, led to a correction of hypoxemia.
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10/203. Pulmonary hypertension in a child with juvenile-type autosomal recessive polycystic kidney disease.

    An 11 year-old girl, whose condition was diagnosed as juvenile-type autosomal recessive polycystic kidney disease (ARPKD) at five years of age, presented with chest pain and dyspnea that had developed suddenly two months previously. Two-dimensional echocardiography, doppler study and cardiac catheterization confirmed pulmonary hypertension. The underlying mechanism of the diagnosis was not defined. Two and a half months after the onset of symptoms, the patient died of pulmonary hypertensive crisis. Careful regular checks of cardiopulmonary status using two-dimensional echocardiography and Doppler should be considered for the early detection of pulmonary hypertension even in an asymptomatic patient with juvenile-type ARPKD.
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