Hepato- and Porto-pulmonary Hypertension

Abstract

Porto-pulmonary hypertension and hepato-pulmonary syndrome are infrequent complications of chronic hepatic diseases and portal hypertension but their prognostic role is important, limiting the survival of the patients. They are related to the portal hypertension but their mechanisms are different. Porto-pulmonary hypertension represents the association between portal hypertension and pulmonary arterial hypertension, increased pulmonary vascular resistance > 240 dyne∙s∙cm−5 or >3 u Wood, normal pulmonary wedge pressure and normal left ventricular end diastolic pressure. The occurrence of the porto-pulmonary hypertension is related to the hyperdynamic circulation due to the porto-systemic anastomoses. Hepato-pulmonary syndrome associates chronic liver disease or portal hypertension with increased alveolar- arterial gradient in O2 (P(A-a)O2 ≥ 15 mmHg or >20 mmHg for patients >65 years old while breathing ambient air), hypoxemia (PaO2 < 80 mmHg) and intrapulmonary vascular dilation proven by contrast-enhanced echocardiography or lung perfusion scanning. The diagnosis can be often confused with concomitant pulmonary or heart diseases. Both complications are difficult to deal with. In porto-pulmonary hypertenion the efficiency of the pulmonary vasodilatation treatment is uncertain. Moderate to severe porto-pulmonary hypertension is a contraindication for hepatic transplant. The hepatic transplant represents the elective treatment for hepato-pulmonary syndrome especially in patients in which hypoxemia is improved by the administration of 100% O2. 

Appendices

Self Study

1.1 Questions

• 1. A 57 years old woman with post necrotic hepatic cirrhosis and portal hypertension is admitted in the hospital for dyspnoea to minimal efforts. Electrocardiography: sinus tachycardia, right ventricular hypertrophy. Transthoracic echocardiography: right ventricular and right atrial dilation, mPAP 50 mmHg. Child Pugh score is B. Her treatment involves Propranolol 60 mg/day, Spironolactone 150 mg/day, Isodinitmononitrate 40 mg/day. What is the best therapeutic decision?

  • (a) Including the patient on the liver transplant list

  • (b) Sildenafil

  • (c) Bosentan

  • (d) Anticoagulation

  • (e) Changing propranolol with bisoprolol

• 2. What is true about the hepato-pulmonary syndrome?

  • (a) The prevalence in chronic liver disease is 50%

  • (b) It occurs especially in patients with posthepatitis cirrhosis

  • (c) Sildenafil is the most useful treatment

  • (d) It improves after hepatic transplantation

  • (e) The gold standard test for diagnosis is right cardiac catheterization

Answers

• 1. A 57 years old woman with post necrotic hepatic cirrhosis and portal hypertension is admitted in the hospital for dyspnoea to minimal efforts. Electrocardiography: sinus tachycardia, right ventricular hypertrophy. Transthoracic echocardiography: right ventricular and right atrial dilation, mPAP 50 mmHg. Child Pugh score is B. Her treatment involves Propranolol 60 mg/day, Spironolactone 150 mg/day, Isodinitmononitrate 40 mg/day. What is the best therapeutic decision?

  • (b) Medium and severe POPH is a contraindication for the liver transplantation. Bosentan worsens the hepatic function. The anticoagulation increases the hemorrhagic risk in a patient with hepatic cirrhosis. The non selective beta blockers are indicated in PH and not the selective one.

• 2. What is true about the hepato-pulmonary syndrome?

  • (d) The prevalence of the HPS in chronic liver disease is 1% and 30% in those proposed for hepatic transplantation. It doesn’t matter the etiology of the liver disease. Sildenafil can be used in POPH not in HPS. The gold standard test for diagnosis is MAA scan.

Future Perspectives

The effort must be do to better understand the pathophysiology of POPH and HPS. There is a possible genetic determination of the occurrence of the POPH and HPS. Future studies must bring additional data with regard to genetic status involved in POPH and HPS. The early diagnosis is very important for improving the treatment and therefore we need new methods.