Bedside cardiopulmonary characteristics distinguishing portopulmonary hypertension from primary pulmonary hypertension

ABSTRACT

The presence of pulmonary hypertension in combination with portal hypertension, otherwise termed porto-pulmonary hypertension [PPHTN], is a well-recognized complication of chronic liver disease. Primary pulmonary hypertension [IryPH] is another type of pulmonary hypertension with obscure aetiology that has similarities to PPHTN. The cardiopulmonary clinical characterestics of both types may be hard to distinguish from each other although they aren't synonymus. To differentiate the bedside cardiopulmonary profile of PPHTN from that of IryPH. A total of 20 patients with PPHTN [Group A] were recruited during the period from August 2005 to September 2007 and compared to a group of 20 patients with IryPH [GroupB]. Clinical assessment, trans-thoracic echocardiography, ECG, chest radiography, room air arterial blood gas measurements and respiratory function tests were done for all patients in addition to the diagnostic liver assessment investigations. Dyspnea on exertion, and cyanosis were main complaints of both groups. Patients with PPHTN [Group A] exhibited less elevated pulmonary pressures than patients with IryPH [mean pulmonary pressure, 51 +/- 2.1 mm Hg versus 62 +/- 2.3 mmHg, [P <0.05]. Chest radiography, Echocardiographic, and ECG findings secondary to pulmonary hypertension were different than group B [p, 0.08]. Echocardiographic signs of diastolic dysfunction were unexpectedly more in group A. Arterial blood gas measurements indicate that PPHTN exhibits a significant accentuation of the chronic respiratory alkalosis [p, 0.04] and has an increased alveolar-arterial gradient [P, 0.000] when compared to patients with IryPH. However, restrictive pulmonary dysfunction and reduced diffusion were more found in IryPH [Group B] [p, 0.05]. PPHTN and IryPH patients possess characteristics common to both groups, but, more importantly, they also have distinctive cardiopulmonary characteristics that allow differentiation. Despite tendency to have less degree of PH, PPHTN patients have more accentuated respiratory alkalosis and more increased alveolar aterial gradient than patients with primary PH also IryPH patients have more restrictive pulmonary functions. Cardiac Diastolic dysfunction is also more prominent in patients with PPHTN than in IryPH patients