Efficacy and Safety of Bosentan Treatment for Portopulmonary Hypertension Associated With Syncope

Abstract

SummaryPulmonary arterial hypertension (PAH) in patients with portal hypertension is also referred to as portopulmonary hypertension (PPHTN). Here, we report a case of PPHTN caused by alcoholic liver cirrhosis in a 43-year-old male who experienced repetitive syncope on exertion. The continuous monitoring of pulmonary artery pressure and radial artery pressure revealed that his PAH was aggravated with a drop in systemic arterial pressure during an exercise test. Bosentan, an endothelin A/B receptor antagonist, improved the patient's hemodynamic parameters and abolished his syncope without adverse effects. This is the first report that bosentan may be effective and safe for PPHTN associated with syncope. (Int Heart J 2011; 52: 243-245) Key words: Portopulmonary hypertension, Syncope, Bosentan, Liver cirrhosis P ortopulmonary hypertension (PPHTN) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension.1) The diagnostic criteria for PPHTN are abnormal increases in the mean pulmonary arterial pressure (PAP) (> 25 mmHg) and pulmonary vascular resistance (> 240 dynes/second/cm 5 ), and a normal pulmonary capillary wedge pressure (< 15 mmHg) in patients with liver disease causing clinical portal hypertension.1) The prognosis of PPHTN is very poor, 2) and the pathogenesis and management of PPHTN are not well understood.We present a patient with PPHTN associated with syncope who was treated successfully with the oral endothelin A/B receptor antagonist bosentan. Case ReportA 43-year-old male was admitted to another hospital for deterioration of alcohol-related liver damage in September 2009. He recovered after rest and abstinence from alcohol. However, he developed repetitive faintness and syncope during walking or light exertion after leaving the hospital. During an exercise treadmill test, which was performed according to the Bruce protocol, electrocardiography revealed a shift from sinus tachycardia (120 bpm) to an atrioventricular junctional rhythm (60 bpm) with sinus pauses, followed by syncope at 4 minutes in that protocol ( Figure 1A). He was admitted to our hospital for syncope in November 2009.On physical examination, his second heart sound was increased and a pansystolic murmur attributable to a tricuspid regurgitation was detected. No leg edema or cranial or peripheral neuropathy was observed. Resting electrocardiography showed a normal sinus rhythm with negative T waves in the right precordial leads. Echocardiography revealed a tricuspid regurgitation with a high pressure gradient (55 mmHg), a dilated right ventricle and a leftward bulging of the interventricular septum, especially in systole ( Figure 1B and 1C). Cardiac catheterization showed that the coronary arteries and left ventricular wall motion were normal. His pulmonary capillary wedge pressure and systemic vascular resistance were within the normal range (14 mmHg and 1493 dynes/second/cm 5 , respectively). However, his pulmonary arterial pressure (PAP) and pulmonary vascular resistance were high (68/30 [43] mmHg and ...

Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature

“…According to the ammonia-poisoning theory ( 17 ), we speculated that there might be a disturbance of consciousness caused by elevated blood ammonia in our case. As far as we know, syncope is not uncommon in patients with liver cirrhosis ( 11 , 18 ), even in the absence of HE. Blood ammonia can be checked to assist in diagnosis.…”

ISHLT consensus statement: Perioperative management of patients with pulmonary hypertension and right heart failure undergoing surgery

“…Phosphodiesterase-5 inhibitors (PDE-5I) are generally well tolerated, and small studies demonstrate improved functional capacity and hemodynamics (322,(330)(331)(332)(333)(334). Use of endothelin receptor antagonists (ERA) are also effective (335)(336)(337)(338)(339)(340)(341)(342)(343). Although, caution should also be taken given the increased risk of fluid retention and hepatotoxicity (348)(349)(350).…”