Clinical analysis of 7 cases of portopulmonary hypertension

ABSTRACT

Objective To investigate the clinical features with to highlight the main criteria for diagnosis of portopulmonary hypertension(POPH).Methods The clinical and laboratory data of 7 patients with POPH were retrospectively analysed.Results The patients included 2 males and 5 females with median age at diagnosis of 51 years(ranging from 26 to 73 years).The underlying diseases of portal hypertension were post-type B hepatitis cirrhosis(3 cases),autoimmune hepatitis(2 cases),immunologic deficiency disease(1 case)and portal vein thrombosis(1 case).The initial symptom of pulmonary hypertension(PH)in 5 patients was dyspnea on exertion,but 2 patients remain asymptomatic.All patients was assessed by transthoracic echocardiogram(TTE)with the pulmonary arterial systolic pressure(PASP)above 55 mmHg,1 patient accepted right heart catheterization(RHC)with mean pulmonary arterial pressure of 62 mmHg and pulmonary vascular resistance of 8.64 mN/(s?cm5).Immunologic abnormality was found in 6 patients,and the severity of liver function does not appear to correlate with the severity of the PH.Follow-up study demonstrated 3 patients died,2 of whom were due to the cardiac involvement(right heart failure and sudden death).Conclusion PH is a rare but severe complication of portal hypertension.It is the portal hypertension,not the underlying liver disease causing POPH.TTE is an important screening procedure,but the gold standard for diagnosis of POPH is hemodynamic data from RHC.