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Pulmonary arterial hypertension is a life-threatening disorder that refers to a group of diseases characterized by an abnormal elevation of the blood pressure within the pulmonary circulation due to a vasculopathy of the pulmonary microcirculation (1). If left untreated, the overall prognosis of pulmonary arterial hypertension is poor, with a 5-year survival rate of 34%. The most common cause of death is progressive right-sided heart failure (2). There is no pharmacologic cure for pulmonary arterial hypertension, and a team approach is required for the proper care of these patients (3). Treatment is directed at improving clinical symptoms, increasing exercise tolerance and extending survival. Until just a few years ago, standard treatment options for the treatment of pulmonary arterial hypertension were limited and included coumarin derivatives, calcium channel blockers, diuretics, digoxin and oxygen supplementation (4). In the last decade, the therapeutic options for pulmonary arterial hypertension have made considerable advances. In at least three major randomized controlled trials, the continuous intravenous infusion of epoprostenol, a synthetic salt of prostacyclin with potent vasodilatory ability, has been shown to improve exercise tolerance, hemodynamics, survival and quality of life in patients with New York Heart Association (NYHA) functional classes III and IV with either primary pulmonary hypertension or pulmonary arterial hypertension associated with scleroderma (5-9). Epoprostenol has now become a mainstay therapy in the long-term treatment of primary pulmonary hypertension or pulmonary arterial hypertension associated with scleroderma. Nevertheless, epoprostenol is far from an ideal form of therapy. Issues such as the short half-life of the drug, need for continuous central intravenous access and significant side effects have led to the development of more stable prostacyclin analogues as alternative therapies for primary pulmonary hypertension. These alternative therapies include iloprost (inhaled delivery) and treprostinil (subcutaneous delivery). As a result, the Food and Drug Administration (FDA) recently approved treprostinil for the treatment of pulmonary arterial hypertension in patients with NYHA classes II-IV. This review will offer a discussion of the basic pharmacology of treprostinil, its similarities to and differences from epoprostenol, the animal studies as well as the initial investigational studies leading to its FDA approval, and clinical uses of the drug alone or in combination with newer therapies directed at pulmonary arterial hypertension developed over the last decade.
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PMID:Overview of treprostinil sodium for the treatment of pulmonary arterial hypertension. 1514 31

A 6-year-old boy was diagnosed as having atrial septal defect with oversystemic pulmonary hypertension, and gradually developed hypoxia and heart failure. At the age of 11, living-related bilateral lobar lung transplantation from his parents was indicated, because of his critical condition. The estimated forced vital capacity calculated by the donors' lower lobes was 104% of his age. The operation was carried out with simultaneous closure of the atrial septal defect under full cardiopulmonary bypass. The postoperative course was complicated with pulmonary edema and phrenic nerve palsy, which were eventually resolved. Six months after surgery he was free from heart failure and rehabilitating at home without oxygen. The final diagnosis was primary pulmonary hypertension with atrial septal defect. Living-related bilateral lobar lung transplantation with simultaneous intracardiac repair may be an optional strategy for children with Eisenmenger syndrome or primary pulmonary hypertension with intracardiac defect when cadaveric transplantation is not possible.
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PMID:Living-related lobar transplantation and simultaneous atrial septal defect closure in a young patient with irreversible pulmonary hypertension: a case report. 1527 96

Primary pulmonary hypertension (PPH) is a progressive disease for which there is no cure. Continuous intravenous infusion of epoprostenol in selected patients with advanced PPH improves symptoms and survival, but the long-term impact has not been reported in Taiwanese patients. Four patients with advanced PPH treated with epoprostenol therapy were enrolled in this study from July 2000 to September 2001. The basic hemodynamic status, cardiothoracic ratio, 6-minute-walk test results, maximum oxygen consumption, New York Heart Association functional class and survival were re-evaluated before and after 12 months of epoprostenol treatment. One of the patients died after 10 months of epoprostenol therapy due to heart failure, and the other 3 were still alive after a mean of 28 months. The surviving patients had pronounced symptomatic, hemodynamic, functional and survival benefit from epoprostenol therapy. Our results suggest that chronic infusion of epoprostenol can improve functional status and survival of Taiwanese patients with advanced PPH disease.
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PMID:Effects of continuous intravenous epoprostenol therapy on advanced primary pulmonary hypertension in Taiwanese patients. 1566 Jan 81

Scientific evidence is mounting that cardiopulmonary exercise testing (CPET) offers different potential clinical applications in cardiology. In comparison with traditional ECG stress testing, CPET provides more accurate diagnostic information and prognostic insights as well, in particular in patients with chronic heart failure and primary pulmonary hypertension. The barriers that actually limit the diffusion of CPET among cardiologists are primarily "cultural", because modern ergospirometric tools are easy to use, and the time required for a CPET is not dissimilar from that of a standard ECG stress test. At present, time seems to be ripe for considering CPET as the new exercise stress test in cardiology.
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PMID:[Cardiopulmonary exercise testing: the exercise stress test of the future?]. 1582 31

Endothelins are powerful vasoconstrictor peptides that also play numerous other roles. The endothelin (ET) family consists of three peptides produced by a variety of tissues. Endothelin-1 (ET-1) is the principal isoform produced by the endothelium in the human cardiovascular system, and it exerts its actions through binding to specific receptors, the so-called type A (ET(A)) and type B (ET(B)) receptors. ET-1 is primarily a locally acting paracrine substance that appears to contribute to the maintenance of basal vascular tone. It is also activated in several diseases, including congestive heart failure, arterial hypertension, atherosclerosis, endothelial dysfunction, coronary artery diseases, renal failure, cerebrovascular disease, pulmonary arterial hypertension, and sepsis. Thus, ET-1 antagonists are promising new agents. They have been shown to be effective in the management of primary pulmonary hypertension, but disappointing in heart failure. Clinical trials are needed to determine whether manipulation of the ET system will be beneficial in other diseases.
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PMID:Endothelins in health and disease. 1757

Whereas the right ventricle tolerates volume loads without any substantial increase of the pressure in the pulmonary circulation by recruiting capacitance vessels and capillaries, it possesses only small contractile reserves and reacts unadapted with right ventricular dysfunction. Its size and pressure load are relevant factors for prognosis of all forms of pulmonary hypertension, in particular if linked to left-sided heart failure. Differentiation of pulmonary hypertension according to the Venice classification is highly important. Right-sided ventricular heart failure worsens left ventricular hemodynamics due to reduced ejection fraction and in addition due to direct diastolic ventricular interaction in which left ventricular diastolic dysfunction increases even though the left ventricular systolic function is still intact. Right ventricular ejection fraction <40% is an important predictor of prognosis after myocardial infarction or chronic stages of left ventricular heart failure. The most important noninvasive diagnostic method is transthoracic echocardiography with determination of the Tei index and Doppler echocardiographic estimation of pulmonary artery pressure. Chronic obstructive pulmonary disease is the most frequent cause of cor pulmonale. While long-term oxygen therapy in patients with COPD and cor pulmonale and for example the administration of endothelin receptor antagonists in patients with idiopathic pulmonary hypertension is beneficial, the therapeutic use of drugs effective for left-sided heart failure is very limited in patients with right ventricular dysfunction.
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PMID:[Right heart failure and cor pulmonale]. 1762 62

Over a 7-year period, the chart records of six pregnant women with cardiac diseases at potential risk of thromboembolism were reviewed. All six patients survived and recovered well eventually. LMWH and beta-adrenergic blocker were effective to deal with atrial fibrillation. Digitalis and dobutamine were beneficial to prevent heart failure resulted from degenerated porcine valve and dilated cardiomyopathy. In a patient with mechanical mitral valve, low-dose warfarin did not cause fetal malformation, and was effective to prevent thrombus formation. Protamine sulfate was safely administered to neutralize intravenous heparin effect before vaginal delivery. Life-threatening postpartum pulmonary hemorrhage occurred as a result of pulmonary hypertension with an aberrant right pulmonary artery, absolutely necessitating a long-term cardiopulmonary bypass resuscitation. Patient with primary pulmonary hypertension gave birth safely with forceps assistance under epidural anesthesia. From literatures reviewed and successful experiences presented here, prenatal correction of the underlying cardiac malformation, precise switch of anticoagulant administration, optimizing cardiac function, early delivery prior to heart failure, postpartum fluid restriction, minimized peripartum blood loss, and meticulously intensive cares are essential to achieve satisfactory outcomes.
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PMID:Management of pregnant women with cardiac diseases at potential risk of thromboembolism--experience and review. 1863 71

Research on ascites occurring in meat-type chickens reared at moderate and low altitude has shown that the pathogenesis is similar to that of the high altitude disease. Pulmonary hypertension (PH) caused by increased blood flow or increased resistance to flow in the lung results in right ventricular hypertrophy (RVH), valvular insufficiency, increased venous pressure and ascites. The structure of the avian heart, with its thin-walled right ventricle and muscular right atrioventricular valve, allows PH to induce heart failure quickly. The sudden increase in pulmonary hypertension syndrome (PHS) in meat-type chickens in the 1980s was associated with a rapid increase in growth rate and feed conversion. This was a result of a combination of genetic selection for fast-growing, heavy broilers with low feed conversion and a more dense, high caloric, pelleted food that supplied all the nutrients required for rapid growth and encouraged a high nutrient intake. PHS in meat-type chickens is usually primary pulmonary hypertension, that is, PH that occurs without evidence of prior heart or lung disease that could account for the increase in blood flow or resistance to flow that results in the increased pressure in the pulmonary arteries. The lungs of birds are firm and fixed in the thoracic cavity and they do not expand to draw air into the lung. The blood and air capillaries form a rigid network that allows only minimal expansion of the blood capillaries when more blood flow is required. Air is moved through the lung by abdominal movement which draws air in and out of the air sacs. The anatomy and physiology of the avian respiratory system are important in the susceptibility of meat-type chickens to PHS. The small stature of the modern meat-type chicken, the large, heavy breast mass, the pressure from abdominal contents on air sacs, and the small lung volume compared to body weight, may all be involved in the increased incidence of PHS. There is limited space for blood flow in the avian lung. Factors that increase blood flow or increase resistance to flow are additive. Increased blood viscosity caused by the polycythaemia of hypoxia, or increased erythrocyte rigidity of high Na (+), are more likely to produce PH in fast-growing than in slow-growing birds. Increased flow due to cold exposure is also additive. Ascites caused by PH is a production-related disease at low altitude. It can be prevented easily by restricting growth rate. It is possible that some meat-type chickens of the phenotype we have created have reached the limit of blood flow through their lungs and that future improvements in growth rate will only be possible if the lung and abdominal cavity capacities are enlarged.
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PMID:Ascites in poultry. 1867 Oct 31

Tailoring graft size to small paediatric recipients is a challenge. We have developed a reduced left lateral segment as an alternative to monosegment transplantation for small size recipients. Since November 2000, 89 children have been transplanted with 100 deceased donor liver grafts in our unit. Our median patient and graft survival is 89% and 88% respectively. Four of these cases were performed using a new technique of creating a small donor graft by reducing the left lateral segment. The median weight of the reduced liver graft was 264 g (range: 165-390 g). The median blood transfusion requirement was 101 mL/kg body weight (range 69-167 mL/kg). The median values of peak ALT were 1473 IU/L, INR 2.2 and bilirubin 293 micromol/L in the first two wk following surgery. One neonatal recipient died five days after transplantation from a massive intracranial haemorrhage despite satisfactory graft function. Another recipient with excellent graft function died 10 months later from primary pulmonary hypertension and secondary cardiac failure. Hepatic artery thrombosis occurred in one patient with successful revascularization but he was retransplanted three months later for chronic rejection. No biliary or venous outflow complications occurred in this group. This technique of reduced left lateral segment liver transplantation is an alternative to the monosegment graft and allows small recipients to be successfully transplanted with few technical complications related to graft preparation.
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PMID:The reduced left lateral segment in pediatric liver transplantation: an alternative to the monosegment graft. 1878 70

Primary pulmonary hypertension is a fatal disease that frequently becomes evident in pregnancy. The management of pregnant women with primary pulmonary hypertension poses a number of difficult problems, especially where regional anesthesia is considered to be contraindicated. A 30-year-old woman who developed primary pulmonary hypertension at 23 weeks of pregnancy was transferred to our hospital. Systolic pulmonary artery pressure and plasma brain natriuretic peptide levels were markedly elevated. Nitric oxide inhalation and prostacyclin prevented the progression of cardiac failure and reduced both plasma brain natriuretic peptide and pulmonary artery pressure. Cesarean section was performed at 32 weeks under general anesthesia. A combination of nitric oxide, prostacyclin, nitroglycerin, and dobutamine were administered during surgery. Intravenous dexmedetomidine was specifically used during emergence and recovery from anesthesia. This provided effective pain relief and hemodynamic stability. Throughout the clinical course, brain natriuretic peptide levels was monitored and used as an indicator of cardiac failure.
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PMID:Cesarean section and primary pulmonary hypertension: the role of intravenous dexmedetomidine. 1915 50


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