Association between primary pulmonary hypertension and portal hypertension: Analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations

Abstract

To determine the clinical, laboratory and hemodynamic profile in patients with primary pulmonary hypertension and associated portal hypertension, 7 new cases and 71 previously reported cases were analyzed. There was no gender predilection and the average age at diagnosis was 41 years. Liver cirrhosis was the most frequent cause of hypertension (82%) and a surgical portosystemic shunt was present in 29%. Almost invariably, portal hypertension either preceded or was diagnosed concurrently with pulmonary hypertension, favoring the hypothesis that in portal hypertension, the pulmonary vasculature may be exposed to vasoactive substances normally metabolized or produced by the diseased liver, possibly inducing vasoconstriction or direct toxic damage to the pulmonary arteries. Clinically, exertional dyspnea was the most frequent presenting symptom (81%); other symptoms, such as syncope, chest pain and fatigue, were present in less than 33%. An accentuated pulmonary component of the second heart sound (82%) and a systolic murmur (69%) were the most common physical findings. At least 75% of these patients had evidence of pulmonary hypertension on electrocardiography (right ventricular hypertrophy) or roentgenography (cardiomegaly or dilated main pulmonary arteries, or both). Hemodynamic findings included severe pulmonary hypertension (mean pulmonary artery pressure 59 +/- 19 mm Hg) with normal pulmonary capillary wedge pressure and cardiac output. Treatment was basically palliative and the mean and median survival times were 15 and 6 months, respectively. In brief, on the basis of clinical presentation and laboratory features, patients with combined primary pulmonary hypertension and portal hypertension seldom represent a diagnostic challenge. Further research is needed on treatment, which remains palliative. The survival rate is poor and worse than that seen in isolated primary pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)

Portopulmonary hypertension: Results from a 10-year screening algorithm

“…A prospective, echocardiography-catheterization algorithm was followed from P ulmonary hypertension associated with advanced liver disease has variable etiologies and prognostic implication. [1][2][3][4] First, the hyperdynamic, high-flow circulatory state (as a consequence of splanchnic vasodilatation caused by portal hypertension) results in high cardiac output (CO) and an increase in mean pulmonary artery pressure (MPAP), but the pulmonary vascular resistance (PVR) remains normal. Second, elevated MPAP with increased central blood volume estimate as measured by the pulmonary arterial occlusion pressure (PAOP) results in variable effect on PVR.…”

Clinical predictors of pulmonary hypertension in patients undergoing liver transplant evaluation

“…Compared with primary pulmonary HTN, there are significant differences with regard to pathophysiological characteristics, 5 pathological characteristics, 16,17 and clinical and hemodynamic variables. 5,16,18 The diagnosis of this condition is often challenging because patients with ESLD have a multitude of clinical symptoms, which may confuse the clinician. The symptoms of ESLD and pulmonary HTN may be indistinguishable.…”

“…This is most important in the light of increasing perioperative morbidity and mortality of the patients with PPHTN, especially those with severe pulmonary HTN and/or RV dysfunction. 5,6 OLT poses a major hemodynamic burden to the RV and pulmonary circulation. After unclamping the inferior vena cava, there is a release of excess volume and vasoactive substances from the transplanted liver and portal and systemic venous circulation that flood the RV and pulmonary vascular bed.…”

“…Nevertheless, 60% are asymptomatic at the time of diagnosis. 5 Many of the symptoms associated with advanced liver disease and portal HTN are identical to those of pulmonary HTN of any cause. 9 Thus, the diagnosis of PPHTN based on clinical presentation may be challenging.…”

High prevalence of pulmonary diffusion abnormalities without interstitial changes in long-term survivors of liver transplantation

“…Also, the prevalence of pulmonary hypertension and the associated deterioration in gas exchange is significantly higher among subjects with portal hypertension. The underlying mechanisms, and the question of whether these changes of HPS or pulmonary hypertension are reversible, are not fully understood [29,42].…”