ABSTRACT
The classic definition of pulmonary arterial hypertension (PAH) is a mean pulmonary artery pressure (mPAP) of 20 mmHg. The gold standard for assessing pulmonary hemodynamics is right heart catheterization (RHC), which is necessary to confirm the diagnosis of PH. In some instances, RHC evaluates the degree of hemodynamic dysfunction and performs vasoreactivity tests. Measurement of the hepatic venous pressure gradient remains the gold standard diagnostic for identifying portal hypertension. This review aims to describe the procedure of RHC and the hemodynamic measurement in patients with PAH and Portopulmonary hypertension (PoPH). The RHC remains the gold standard for diagnosing PAH and PoPH.
Subject(s)
Hypertension, Portal , Hypertension, Pulmonary , Pulmonary Arterial Hypertension , Humans , Hypertension, Pulmonary/diagnosis , Cardiac Catheterization/methods , Hemodynamics , Hypertension, Portal/diagnosisABSTRACT
PURPOSE OF REVIEW: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are both pulmonary vascular complications of advanced liver disease; however, these syndromes have distinct pathophysiology, clinical implications, and management. RECENT FINDINGS: While both conditions are associated with portal hypertension, HPS results from diffuse pulmonary capillary vasodilation and PoPH results from vasoconstriction and vascular remodeling of pulmonary arteries. In HPS, no medical therapies clearly improve outcomes; however, patients have excellent post-LT outcomes with near uniform reversal of hypoxemia. In PoPH, several medical therapies used in idiopathic pulmonary hypertension have been shown improve pulmonary hemodynamics, symptoms, and potentially LT outcomes; however, further study is needed to determine best treatment regimens, long-term outcomes on medical therapy, and role of LT. SUMMARY: While HPS results in severe hypoxemia that is usually reversible by LT, PoPH patients develop progressive pulmonary hypertension that may improve with medical therapy.
ABSTRACT
Las complicaciones pulmonares de las enfermedades crónicas del hígado han sido descritas desde hace más de un siglo. Aunque originadas por una lesión común y vías fisiopatológicas similares, sus manifestaciones clínicas y el diagnóstico son divergentes. Su impacto clínico es importante ya que afectan de forma deletérea el pronóstico de los pacientes. Hasta el momento no existe un tratamiento efectivo para el manejo de estas enfermedades y el trasplante hepático debe ser evaluado de forma muy cuidadosa. En esta revisión se analizan los aspectos más importantes de la hipertensión portopulmonar y del síndrome hepatopulmonar, entidades que bajo el entorno de la hipertensión portal se caracterizan respectivamente por procesos de vasoconstricción y vasodilatación pulmonar.
Cardiopulmonar complications in chronic liver diseases were described 100 years ago. Altough both hepatopulmonary sindrome and portopulmonary hypertension originates from liver damage, clinical findings and diagnosis are very different. These complications are important due to the highly deleterous impact on disease evolution and prognosis. Currently, there is not an ideal treatment for these diseases and liver transplantation should be adequately evaluated. In this review we analyze the most important issues on hepatopulmonary sindrome and portopulmonary hypertension. These complications,under the cornerstone of portal hypertension are characterized by pulmonary constriction and dilatation, respectively.