PPH presents roughly equally in male and female cirrhotics; 71% female in an American series and 57% male in a larger French series. Typically, patients present in their fifth decade, aged 49 +/- 11 years on average. In general, PPH is diagnosed 4–7 years after the patient is diagnosed with portal hypertension and in roughly 65% of cases, the diagnosis is actually made at the time of invasive hemodynamic monitoring following anesthesia induction prior to liver transplantation. Once patients are symptomatic, they present with right heart dysfunction secondary to pulmonary hypertension and its consequent dyspnea, fatigue, chest pain and syncope. Patients tend to have a poor cardiac status, with 60% having stage III-IV NYHA heart failure. PPH is actually independent of the severity of cirrhosis but may be more common in specific types of cirrhosis, in one series more so in Autoimmune Hepatitis and less in Hepatitis C cirrhosis, while in another it was equally distributed throughout the diagnoses.
Portopulmonary hypertension
Chronic thromboembolic pulmonary hypertension
Pulmonary hypertension
Asthma
Emphysema
Cardiac asthma
Portal hypertension
Reactive airway disease