Survival in Portopulmonary Hypertension: Mayo Clinic Experience Categorized by Treatment Subgroups

Swanson, Karen L.; Wiesner, Russell H.; Nyberg, Scott L.; Rosen, Charles B.; Krowka, Michael J.

doi:10.1111/j.1600-6143.2008.02384.x

Cited by 306 publications

(250 citation statements)

References 21 publications

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“…Therefore, a sPAP greater than 50 mmHg at ECHO represents the cut-off point for executing a RHC. At RHC, a mPAP greater than 45 mmHg represents a contraindication to TIPS [24,[70][71][72][73][74]79]. …”

Section: Cardiac Dysfunctionmentioning

confidence: 99%

Consensus conference on TIPS management: Techniques, indications, contraindications

Fagiuoli

Bruno

Venon

et al. 2017

Digestive and Liver Disease

130

120

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a b s t r a c tThe trans jugular intrahepatic Porto systemic shunt (TIPS) is no longer viewed as a salvage therapy or a bridge to liver transplantation and is currently indicated for a number of conditions related to portal hypertension with positive results in survival. Moreover, the availability of self-expandable polytetrafluoroethylene (PTFE)-covered endoprostheses has dramatically improved the long-term patency of TIPS. However, since the last updated International guidelines have been published (year 2009) new evidence have come, which have open the field to new indications and solved areas of uncertainty. On this basis, the Italian Association of the Study of the Liver (AISF), the Italian College of Interventional Radiology-Italian Society of Medical Radiology (ICIR-SIRM), and the Italian Society of Anesthesia, Analgesia and Intensive Care (SIAARTI) promoted a Consensus Conference on TIPS. Under the auspices of the three scientific societies, the consensus process started with the review of the literature by a scientific board of experts and ended with a formal consensus meeting in Bergamo on June 4th and 5th, 2015. The final statements presented here were graded according to quality of evidence and strength of recommendations and were approved by an independent jury. By highlighting strengths and weaknesses of current indications to TIPS, the recommendations of AISF-ICIR-SIRM-SIAARTI may represent the starting point for further studies.

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Section: Cardiac Dysfunctionmentioning

confidence: 99%

Consensus conference on TIPS management: Techniques, indications, contraindications

Fagiuoli

Bruno

Venon

et al. 2017

Digestive and Liver Disease

130

120

View full text Add to dashboard Cite

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“…Swanson and coworkers demonstrated that patients with medically treated PH and OLT had a better 5-year survival (67%) than patients treated only pharmacologically without OLT (45%). 6,11 However, unless patients with preserved RVF demonstrated a good response to vasodilator therapy, OLT was associated with a poor outcome. 6 The pharmacologic therapy of PH usually should be continued for several months after OLT.…”

Section: Discussionmentioning

confidence: 99%

“…39 Historically, patients with POPH have been recognized as having very high risk for OLT with unacceptably increased morbidity and mortality. 11,[40][41] During the past 10 to 15 years, newer medications and aggressive perioperative treatment have enabled centers to successfully transplant these patients. However, there remain specific medical requirements that must be met before these patients are considered candidates for transplant.…”

Section: Discussionmentioning

confidence: 99%

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Untitled

Reid

McQuillan²,

Kadry³

et al. 2017

Exp Clin Transplant

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Good right ventricular function and responsiveness to vasodilator therapy are the most important prerequisites for successful liver transplant in patients with portopulmonary hypertension. A patient with portopulmonary hypertension and good right ventricular function presented for deceased-donor liver transplant. Pulmonary arterial pressure was controlled with epoprostenol and sildenafil preoperatively. After anesthesia induction, pulmonary arterial pressure increased significantly and the procedure was aborted. Additional medical treatment included aggressive vasodilator therapy and the transplant was successfully performed 1 month later. During the procedure, elevations in pulmonary arterial pressure responded to a combination of inhaled nitric oxide, intravenous milrinone and nitroglycerin, and optimization of mechanical ventilation.

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“…1) The prognosis of PPHTN is very poor, 2) and the pathogenesis and management of PPHTN are not well understood.…”

mentioning

confidence: 99%

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

et al. 2011

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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 ...

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Survival in Portopulmonary Hypertension: Mayo Clinic Experience Categorized by Treatment Subgroups

Cited by 306 publications

References 21 publications

Consensus conference on TIPS management: Techniques, indications, contraindications

Consensus conference on TIPS management: Techniques, indications, contraindications

Untitled

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

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