“Porto-pulmonary hypertension: A comprehensive review”

Liberal, Rodrigo; Grant, Charlotte R.; Baptista, Rui; Macedo, Guilherme

doi:10.1016/j.clinre.2014.12.011

Cited by 32 publications

(42 citation statements)

References 109 publications

(191 reference statements)

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“…11 Portopulmonary hypertension is usually diagnosed in the fourth or fifth decades of life. 3 Female sex and autoimmune hepatitis are independently associated with portopulmonary hypertension. 11 In addition, the prevalence and severity of portopulmonary hypertension are not correlated with the severity of liver disease.…”

Section: Epidemiologymentioning

confidence: 99%

“…Some of them are related to genetic predisposition, pulmonary vascular wall shear stress, and dysregulation of vasoactive, proliferative, angiogenic, and inflammatory mediators, suggesting that portopulmonary hypertension may have a multifactorial pathogenesis. 3 Histologically, it is characterized by obstruction of pulmonary arterial blood flow similar to idiopathic PAH. 13 Intimal proliferation, media hypertrophy, fibrosis, and in situ thrombosis lead to thickened arterial walls and blood vessel occlusion and finally an increased pulmonary vascular resistance.…”

Section: Pathophysiology and Pathogenesismentioning

confidence: 99%

“…Some of these substances are endothelin 1, thromboxane A2, interleukin 1, interleukin 6, angiotensin I, glucagon, serotonin, calcitonin gene-related peptide, and vasoactive intestinal peptide. 3,17 Of these, endothelin 1A is the most studied substance. There are different studies that showed endothelin 1A levels are higher in cirrhosis with portopulmonary hypertension than in cirrhosis without portopulmonary hypertension.…”

Section: Pathophysiology and Pathogenesismentioning

confidence: 99%

“…29 As the disease progresses, a loud pulmonic component of the second heart sound, a holosystolic murmur in the left sternal border resulting from tricuspid regurgitation, a diastolic murmur of pulmonary regurgitation, and a right-sided third sound and then elevated jugular venous pressure, peripheral edema, pulsatile liver, ascites, and cool extremities can appear. 3,29 Screening and Diagnosis Doppler transthoracic echocardiography (TTE) is a noninvasive and useful method for screening, but RHC is the criterion standard for diagnosis of portopulmonary hypertension. 28,30 Electrocardiography may show findings of right ventricular hypertrophy and right atrial dilation, 31 but these are not specific to portopulmonary hypertension.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…They reported a 53-year-old female patient with portal vein thrombosis with spontaneous portocaval shunt who exhibited an enlarged pulmonary artery; the patient's postmortem examination revealed intimal thickening in the medium and large pulmonary arteries and endothelial proliferation of terminal pulmonary arterioles. 2,3 Portopulmonary hypertension is characterized by vascular obstruction and increased resistance to pulmonary arterial flow due to varying degrees of pulmonary endothelial and smooth muscle proliferation, vasoconstriction, and in situ thrombosis. 4 The European Cardiologic Society and the European Respiratory Society Task Force have defined the diagnostic criteria for portopulmonary hypertension as follows: mean pulmonary artery pressure > 25 mm Hg, pulmonary vascular resistance > 240 dyne·s·cm -5, and a pulmonary capillary wedge pressure < 15 mm Hg, measured during right heart catheterization (RHC) and clinical portal hypertension with or without significant chronic liver disease.…”

Section: Introductionmentioning

confidence: 99%

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Coşarderelioğlu

Coşar

Gürakar

et al. 2016

Exp Clin Transplant

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Portopulmonary hypertension is one of the main pulmonary conditions affecting patients with liver disease and/or portal hypertension. Other conditions include hepatopulmonary syndrome and hepatic hydrothorax. Portopulmonary hypertension is caused by pulmonary vasoconstriction and increased pulmonary vascular resistance. It develops as a result of portal hypertension with or without liver disease and is associated with a higher morbidity and mortality. However, portopulmonary hypertension is usually asymptomatic; the most common symptoms are dyspnea, fatigue, and peripheral edema. All liver transplant candidates should be screened for potential portopulmonary hypertension because its coexistence can affect survival rates after transplant. All patients with cirrhosis who present with dyspnea should also be screened. Transthoracic echocardiography is a noni nvasive, useful method for screening, but right heart-sided catheterization remains the criterion standard for diagnosis. Portopulmonary hypertension carries a poor prognosis without liver transplant, and its severe form is considered to be a contraindication for liver transplant. Treating patients with pulmonary arterial hypertension-specific therapies before liver transplant for moderate and severe portopulmonary hypertension appears to be beneficial.

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Section: Epidemiologymentioning

confidence: 99%

Section: Pathophysiology and Pathogenesismentioning

confidence: 99%

Section: Pathophysiology and Pathogenesismentioning

confidence: 99%