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Objective: To determine the occurrence of hepatopulmonary syndrome (HPS) in patients with cirrhosis who are candidates for liver transplantation; to compare demographic, clinical, laboratory, and spirometric characteristics, as well as echocardiography results, arterial blood gas analysis, and severity of liver disease between the groups of patients with and without HPS; and to describe the occurrence of HPS in the subgroup of patients with cirrhosis and schistosomiasis mansoni (mixed liver disease). Methods: Between January and November of 2007, we evaluated 44 patients under treatment at the Liver Transplant Outpatient Clinic of the Federal University of Pernambuco Hospital das Clínicas, in the city of Recife, Brazil. The diagnostic criteria for HPS were intrapulmonary vascular dilatation, identified by transthoracic echocardiography, and an alveolar-arterial oxygen tension difference ≥ 15 mmHg or a PaO 2 < 80 mmHg. Results: The mean age of the patients was 52 years, and 31 patients (70%) were males. The most common cause of cirrhosis was alcohol use. Schistosomiasis was present in 28 patients (64%). Of the 44 patients, 20 (45.5%) were diagnosed with HPS. No significant differences were found between those patients and the patients without HPS in terms of any of the characteristics studied. Of the 28 patients with cirrhosis and schistosomiasis, 10 (35.7%) were diagnosed with HPS. Conclusions: In the population studied, HPS was highly prevalent and did not correlate with any of the variables analyzed.Keywords: Hepatopulmonary syndrome; Liver transplantation; Liver cirrhosis; Hypertension, portal; Schistosomiasis mansoni; Echocardiography. ResumoObjetivo: Verificar a ocorrência da síndrome hepatopulmonar (SHP) em pacientes cirróticos candidatos a transplante de fígado; comparar as características demográficas, clínicas, laboratoriais e espirométricas, resultados de ecocardiografia, análise de gases arteriais e da gravidade da doença hepática nos pacientes com e sem SHP; e descrever a ocorrência de SHP no subgrupo de pacientes com cirrose associada à esquistossomose mansônica (doença hepática mista). Métodos: Entre janeiro e novembro de 2007, foram avaliados 44 pacientes inscritos no Ambulatório de Transplante Hepático do Hospital das Clínicas da Universidade Federal de Pernambuco, em Recife (PE). Os critérios diagnósticos para SHP foram a presença de dilatações vasculares intrapulmonares, identificadas por ecocardiografia transtorácica, assim como diferença alveoloarterial de oxigênio ≥ 15 mmHg ou PaO 2 < 80 mmHg. Resultados: A idade média foi 52 anos, e 31 pacientes (70%) eram do sexo masculino. A causa mais frequente de cirrose foi uso de etanol. A esquistossomose esteve presente em 28 pacientes (64%). Dos 44 pacientes, 20 (45,5%) foram diagnosticados com SHP. Não foram observadas diferenças significativas em relação às características estudadas. No subgrupo de pacientes com cirrose associada à esquistossomose, 10/28 (35,7%) receberam o diagnóstico de SHP. Conclusões: A SHP apresentou elevada prevalênc...
Objective: To determine the occurrence of hepatopulmonary syndrome (HPS) in patients with cirrhosis who are candidates for liver transplantation; to compare demographic, clinical, laboratory, and spirometric characteristics, as well as echocardiography results, arterial blood gas analysis, and severity of liver disease between the groups of patients with and without HPS; and to describe the occurrence of HPS in the subgroup of patients with cirrhosis and schistosomiasis mansoni (mixed liver disease). Methods: Between January and November of 2007, we evaluated 44 patients under treatment at the Liver Transplant Outpatient Clinic of the Federal University of Pernambuco Hospital das Clínicas, in the city of Recife, Brazil. The diagnostic criteria for HPS were intrapulmonary vascular dilatation, identified by transthoracic echocardiography, and an alveolar-arterial oxygen tension difference ≥ 15 mmHg or a PaO 2 < 80 mmHg. Results: The mean age of the patients was 52 years, and 31 patients (70%) were males. The most common cause of cirrhosis was alcohol use. Schistosomiasis was present in 28 patients (64%). Of the 44 patients, 20 (45.5%) were diagnosed with HPS. No significant differences were found between those patients and the patients without HPS in terms of any of the characteristics studied. Of the 28 patients with cirrhosis and schistosomiasis, 10 (35.7%) were diagnosed with HPS. Conclusions: In the population studied, HPS was highly prevalent and did not correlate with any of the variables analyzed.Keywords: Hepatopulmonary syndrome; Liver transplantation; Liver cirrhosis; Hypertension, portal; Schistosomiasis mansoni; Echocardiography. ResumoObjetivo: Verificar a ocorrência da síndrome hepatopulmonar (SHP) em pacientes cirróticos candidatos a transplante de fígado; comparar as características demográficas, clínicas, laboratoriais e espirométricas, resultados de ecocardiografia, análise de gases arteriais e da gravidade da doença hepática nos pacientes com e sem SHP; e descrever a ocorrência de SHP no subgrupo de pacientes com cirrose associada à esquistossomose mansônica (doença hepática mista). Métodos: Entre janeiro e novembro de 2007, foram avaliados 44 pacientes inscritos no Ambulatório de Transplante Hepático do Hospital das Clínicas da Universidade Federal de Pernambuco, em Recife (PE). Os critérios diagnósticos para SHP foram a presença de dilatações vasculares intrapulmonares, identificadas por ecocardiografia transtorácica, assim como diferença alveoloarterial de oxigênio ≥ 15 mmHg ou PaO 2 < 80 mmHg. Resultados: A idade média foi 52 anos, e 31 pacientes (70%) eram do sexo masculino. A causa mais frequente de cirrose foi uso de etanol. A esquistossomose esteve presente em 28 pacientes (64%). Dos 44 pacientes, 20 (45,5%) foram diagnosticados com SHP. Não foram observadas diferenças significativas em relação às características estudadas. No subgrupo de pacientes com cirrose associada à esquistossomose, 10/28 (35,7%) receberam o diagnóstico de SHP. Conclusões: A SHP apresentou elevada prevalênc...
TXPortopulmonary hypertension (POPH) is a serious complication of cirrhosis that is associated with mortality beyond that predicted by the Model for End-Stage Liver Disease (MELD) score. Increased pulmonary vascular resistance (PVR) may be initiated by pulmonary vasoconstriction, altered levels of circulating mediators, or shear stress, and can eventually lead to the classic vascular remodeling (plexiform lesion) that characterizes POPH. Portal hypertension is a prerequisite for the diagnosis of POPH, although the severity of pulmonary hypertension is unrelated to the severity of portal hypertension or the nature or severity of liver disease. POPH precludes liver transplantation (LT) unless the mean pulmonary artery pressure (MPAP) can be reduced to a safe level. The concept of an acceptable pressure has changed: we now consider both MPAP and PVR in the diagnosis, and we include the transpulmonary pressure gradient so that we can factor in fluid overload and left ventricular failure. Pulmonary vasodilator therapy includes oral, inhaled, and parenteral agents, and one or more of these agents may significantly lower pulmonary artery pressures to the point that LT becomes possible. The United Network for Organ Sharing recommends MELD exception points for patients with medically controlled POPH, but this varies by region. Patients who undergo LT need specialized intraoperative and postoperative management, which includes the availability of intraoperative transesophageal echocardiography for assessing right ventricular function, and rapidly acting vasodilators (eg, inhaled nitric oxide and/or epoprostenol). Published case series suggest excellent outcomes after LT for patients who respond to medical therapy.
Schistosomiasis is the third most common endemic disease and has been considered the most common cause of pulmonary arterial hypertension (PAH) in the world. Around 6 % of people with chronic schistosomiasis in endemic areas are affected. Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is classifi ed by the World Health Organization as group 1 pulmonary arterial hypertension because it has clinical, pathological, and hemodynamic characteristic that are similar to idiopathic pulmonary arterial hypertension. Sch-PAH is a severe disease that affects mainly middle-aged women. Symptoms and signs of right-heart insuffi ciency may be present, such as dyspnea, syncope, and peripheral edema. It has been demonstrated that Sch-PAH has a more benign clinical course and a better survival than idiopathic pulmonary hypertension. There are few reports of any benefi cial effect from specifi c therapy directed toward group 1 PAH in this population of patients. The mechanisms responsible for the development of pulmonary hypertension in patients with schistosomiasis are unknown. There is growing evidence that vessel obstruction and granulomatous reaction to pulmonary embolization of eggs is not the sole mechanism that causes the disease. Further studies are needed to better elucidate the pathogenesis of Sch-PAH and to develop targeted therapies for this devastating disease.
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