[Portopulmonary hypertension: Updated review]

Nielzer Rodríguez-Almendros1, Liz N Toapanta-Yanchapaxi2, Jonathan Aguirre Valadez2

  • 1Departamento de Hipertensión Pulmonar y Función Ventricular Derecha, UMAE Cardiología, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, México, D.F., México.

Insights

Portopulmonary hypertension (PPH), a rare condition, is poorly understood in Mexico despite high rates of chronic liver disease. Early diagnosis via echocardiogram and right cardiac catheterisation is vital for liver transplant candidates.

Area of Science:

  • Cardiology
  • Hepatology
  • Pulmonology

Background:

  • Portopulmonary hypertension (PPH) is a rare complication of chronic liver disease, with unknown prevalence in Mexico.
  • PPH is the fourth most frequent subtype of pulmonary arterial hypertension.
  • Chronic liver diseases are highly prevalent in Mexico, suggesting a potential underdiagnosis of PPH.

Purpose of the Study:

  • To highlight the diagnostic challenges and clinical significance of Portopulmonary hypertension (PPH) in Mexico.
  • To emphasize the impact of PPH on orthotopic liver transplantation (OLT) outcomes.
  • To discuss current therapeutic strategies and future considerations for PPH management.

Main Methods:

  • Diagnosis relies on echocardiogram and right cardiac catheterisation in patients with suspected pulmonary hypertension or awaiting OLT.
  • Assessment of PPH severity is crucial for determining OLT candidacy and perioperative risk.
  • Review of existing literature on PPH diagnosis, management, and transplantation outcomes.

Main Results:

  • PPH significantly increases perioperative mortality in OLT candidates.
  • Specific therapies can improve outcomes for OLT candidates with moderate to severe PPH.
  • PPH can be an absolute contraindication for OLT.

Conclusions:

  • Accurate diagnosis and management of PPH are critical for improving outcomes in liver disease patients, especially OLT candidates.
  • Therapeutic interventions are essential for managing PPH and optimizing OLT success.
  • The role of combined lung-liver or heart-lung-liver transplantation for PPH requires further investigation.

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