Title:When Pulmonary Arterial Hypertension may be Associated with Portal
Hypertension: A Case Report of Two Different Hepatic Disorders in One
Patient with Pulmonary Hypertension
Volume: 20
Issue: 1
Author(s): Ganna D. Radchenko*Yuriy M. Sirenko
Affiliation:
- State Institution National Scientific Center "Institute of Cardiology, Clinical and Regenerative Medicine named after
acad. M.D. Strazhesko" of National Academy of Medical Science, Kyiv, Ukraine
Keywords:
Reno-splenic, pulmonary arterial hypertension, hepatic disorders, prognosis, portal hypertension, biopsy.
Abstract:
Background: Pulmonary arterial hypertension (PAH) is a rare complication of hepatic
diseases with portal hypertension that, however, has a significant influence on prognosis. We
present a mini-review of how to diagnose and treat it based on a clinical case.
Case Presentation: In early childhood, a patient had portal hypertension associated
with cavernous transformation of the portal vein. It was successfully treated by reno-splenic surgery.
At the age of 20 years, this patient experienced increased dyspnea at minimal physical activity
after the hepatic biopsy due to a hepatocellular adenoma. The examination in the specialized
unit showed PAH, which was evaluated as associated with portal hypertension (PAH-PoH).
The specific two-drug combination therapy was started with prominent improvement in patient’s
state. Successful surgical tumor treatment was provided some months later. The practical and
clinical approaches to the diagnosis and treatment of PAH-PoH are discussed. It was emphasized
that not all patients with portal hypertension have pulmonary hypertension, which needs to
be treated. A lot of evidence gaps exist in management of these patients.
Conclusion: All patients, even with past history of portal hypertension, should be monitored
closely and screened for PAH earlier, for better results of treatment.
