Role of 2-dimensional Doppler echo-cardiography in screening portopulmonary hypertension in portal hypertension patients

BACKGROUND: Portopulmonary hypertension (PPH) is difficult to recognize in the early and middle stages because it is frequently asymptomatic. As right ventricular function is impaired in patients with moderate and severe PPH, any dramatic hemodynamic changes in liver transplantation or other procedures may result in death from pulmonary and cardiac events. In this study, we investigated the prevalence of PPH in patients with portal hypertension (PHT) mainly caused by hepatitis B virus, and evaluated the effect of 2-dimensional Doppler echocardiography (2D-ECHO) in screening for PPH. METHODS: One hundred and five PHT patients received transthoracic 2D-ECHO preoperatively, systolic pulmonary arterial pressure (SPAP, normal range <30 mmHg) and pulmonary acceleration time (PAT, normal range >= 120 msec) were measured to screen for PPH (positive result: SPAP >= 30 mmHg and/or PAT <100 msec). Subsequently, pulmonary hemodynamic parameters were measured by right heart catheterization (RHC) for definitive diagnosis of PPH. The results of the two methods were compared to assess the screening effect of 2D-ECHO. RESULTS: The prevalence of PPH in this study was 3.8% (4/105). About 90% (95/105) of patients had a detectable tricuspid regurgitation by 2D-ECHO and the mean SPAP was 27.7 +/- 5.9 mmHg. Twenty-two of these 95 patients had an SPAP >30 mmHg. The mean PAT of all patients was 140 23 msec and 5 were <100 msec. Twenty-two patients were screened out by 2D-ECHO and 4 were diagnosed by RHC. A positive significant correlation (r=0.55, P<0.01) was found between SPAP measured by 2D-ECHO and mean pulmonary artery pressure (MPAP) measured by RHC, and a weak but significant negative correlation (r=-0.27, P=0.005) existed between PAT and pulmonary vascular resistance (PVR). The sensitivity, specificity, agreement rate, positive predictive value and negative predictive value of the screening test were 100%, 82%, 83%, 18% and 100%, respectively. CONCLUSIONS: The prevalence of PPH in this study is lower than in Western countries. As a screening test, 2D-ECHO has very high sensitivity and negative predictive value. A negative test result can directly be used to exclude PPH, while a positive result should be confirmed by RHC.

Portopulmonary hypertension and serum endothelin levels in hospitalized patients with cirrhosis

BACKGROUND:Cirrhosis is associated with several extrahepatic manifestations including portopulmonary hypertension (PPHT).Recent data suggest that endothelins (ETs) are related to the pathophysiology of PPHT.The study aimed to measure serum ET levels in hospitalized cirrhotic patients and to determine their association with PPHT and patient outcome.METHODS:Fifty-seven cirrhotic patients [43 males;median age 58 (28-87) years] underwent Doppler echocardiography.Patients with systolic pulmonary arterial pressure ≥40 mmHg and pulmonary acceleration time <100 ms were deemed to have PPHT.ET-1,2,and 3 serum levels were measured with an ELISA assay.All-cause mortality was recorded over a median period of 24 months.RESULTS:Nine out of 57 patients (15.8%) had PPHT.Among various clinical variables,only autoimmune hepatitis was associated with PPHT (OR=11.5;95% CI,1.58-83.4;P=0.01).ET-1 levels [9.1 (1.6-20.7) vs 2.5 (1.4-9.2) pg/mL,P=0.02] and the ET-1/ET-3 ratio [4.73 (0.9-22.4) vs 1.6 (0.3-10.7),P=0.02] were significantly higher in patients with PPHT than in those without.ET-2 and ET-3 levels did not differ between the two groups.There was no difference in survival between the two groups,although ET-1 levels were associated with an adverse outcome in Cox regression analysis (HR=1.11;95% CI,1.02-1.22;P=0.02 per unit increase in ET-1).CONCLUSION:Our data suggest that ET-1 and the ET-1/ET-3 ratio are elevated in patients with PPHT and that ET-1 is associated with a poor outcome irrespective of PPHT.

Clinical management of portopulmonary hypertension

BACKGROUND: Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or without advanced hepatic disease. In spite of the relatively rare prevalence, the clinical implications of PPH are significant. It has high perioperative morbidity and mortality. This review is an update of current pathogenesis, diagnosis and therapy of PPH. DATA SOURCES: An English-language literature search was conducted using PubMed (1980-2006) on portopulmonary hypertension. RESULTS: Echocardiographically identified patients with elevated pulmonary artery systolic pressure (>50 mmHg) receive right heart catheterization. Epoprostenol (prostacyclin), a potent pulmonary and systemic vasodilator with anti-platelet aggregating activity, and bosentan, an endothelin receptor antagonist, have so far proven beneficial to patients with PPH. CONCLUSIONS: After an accurate diagnosis of PPH treatment should (at a minimum) focus on reduction of mean pulmonary arterial pressure to less than 35 mmHg prior to orthotopic liver transplantation. However, orthotopic liver transplantation currently remains the only therapy to resolve PPH.

Portopulmonary hypertension: Current developments and future perspectives

Portopulmonary hypertension(POPH)is a severe pulmonary vascular disease secondary to portal hy-pertension and a subset of Group 1 pulmonary hypertension(PH).The pathological changes of POPH are indistinguishable from other PH phenotypes,including endothelial dysfunction,pulmonary vasocon-striction,and vascular remodeling.These changes cause a progressive increase in pulmonary vascular resistance and afterload of the right ventricle,eventually leading to severe right heart failure.The prognosis of POPH is extremely poor among untreated patients.POPH is associated with a high risk of death after liver transplantation(LT),and severe POPH is considered an absolute contraindication for LT.However,pulmonary arterial hypertension(PAH)-targeted therapies are administered to patients with POPH,and aggressive drug treatment significantly optimizes pulmonary hemodynamics and reduces the risk of death.Therefore,early diagnosis,aggressive PAH-targeted therapies,and proper selection of liver transplant candidates are vital to reduce the risk of surgery and improve clinical outcomes.This article aims to review the results of previous studies and describe biological mechanisms,epidemiology,po-tential risk factors,and diagnostic approaches of POPH.Moreover,we introduce recent therapeutic in-terventions for the early diagnosis of POPH and efficient clinical management decisions.

Cardiac Syndromes in Liver Disease:A Clinical Conundrum

Understanding the interaction between the heart and liver is pivotal for managing patients in whom both organs are affected.Studies have shown that cardio-hepatic interactions are bidirectional and that their identification,assessment,and treatment remain challenging.Congestive hepatopathy is a condition that develops in the setting of long-standing systemic venous congestion.If left untreated,congestive hepatopathy may lead to hepatic fibrosis.Acute cardiogenic liver injury develops as a combination of venous stasis and sudden arterial hypoperfusion due to cardiac,circulatory,or pulmonary failure.The treatment of both conditions should be directed toward optimizing the cardiac substrate.Hyperdynamic syndrome may develop in patients with advanced liver disease and lead to multiorgan failure.Cirrhotic cardiomyopathy or abnormalities in pulmonary vasculature,such as hepatopulmonary syndrome and portopulmonary hypertension may also develop.Each complication has unique treatment challenges and implications for liver transplantation.The presence of atrial fibrillation and atherosclerosis in liver disease brings another layer of complexity,particularly in terms of anticoagulation and statin use.This article provides an overview of cardiac syndromes in liver disease,focusing on current treatment options and future perspectives.