ortopulmonary hypertension (PPHTN) was previously known as idiopathic pulmonary arterial hypertension (IPAH) with liver injury, but is now established as a clinical entity that is defined as pre-capillary pulmonary hypertension accompanied by hepatic dysfunction and/or portal hypertension. 1 The appearance of PPHTN does not correlate with the severity of the liver dysfunction, 2 and although the cardiac index is relatively preserved, 1,3 there are no reports on the cardiac configuration, pulmonary vascular changes assessed by pulmonary angiography, and plasma levels of brain natriuretic peptide (BNP) and atrial natriuretic peptide (ANP), which are indices of loading to the heart. We hypothesized that the changes described would be less prominent in patients with PPHTN, compared with those in IPAH.
MethodsOur subjects were 10 patients with PPHTN (2 males, 8 females; mean age 38.5 years, range 22-57) ( Table 1) and 18 patients with IPAH (3 males, 15 females, p=1.00; mean age 38.9 years, range 19-76, p=0.87). The severity of liver failure was classified as A, B or C by Child-Pugh's score. 4 In all subjects, we measured the pulmonary arterial wedge pressure, pulmonary artery pressure, right atrial pressure, aortic pressure, oxygen content in the aorta and pulmonary artery, and cardiac output by routine catheter examination. Fractional pulse pressure was computed as the pulse pressure by the mean pulmonary artery pressure. 5 Additionally, in 8 PPHTN patients and 14 IPAH patients, pulmonary angiography was carried out to examine the configuration of the pulmonary artery tree. Within 1 week after cardiac catheter examination, enhanced electron beam tomography (EBT) (Imatron C-150) was carried out to assess both the ventricular volume and myocardial mass in 5 PPHNT patients and 12 IPAH patients. Electrocardiographic electrodes were attached to each patient's thorax to provide both continuous monitoring of the heart and a trigger signal to the scanner. Patients were placed in a supine position with a 17°axial (feet down) tilt and a 13°slew (to the patient's right) to approximate the short-axis view of the heart. 6-8 A total of 50-60 ml of iopamidol 370 contrast medium was injected into an antecubital vein at a rate of 0.8-1.2 ml/s. The gathering of data was started 40 s after the onset of injection. Ventricular and myocardial volumes were calculated using Simpson's rule from 1-cm slice images (7 mm width and 3 mm gap) in the cine mode (Fig 1). End-diastole was defined according to the timing of the R wave on electrocardiography, and end-systole at the smallest ventricular size. Ventricular volumes were calculated at both end-diastole and end-systole. The left ventricular myocardial volume, including that of the interventricular Background The goal of the present study was to examine the cardiac configuration and pulmonary vascular changes in patients with portopulmonary hypertension (PPHTN) and compare them with those of idiopathic pulmonary arterial hypertension (IPAH).
Methods and ResultsThe subjects were 10 patients w...