Key result
Severe cardiopulmonary complications occur in over a third of patients with congenital portocaval shunts, requiring continuous targeted PAH therapy combined with surgical shunt treatment.
Why the study?
Severe cardiopulmonary complications including pulmonary arterial hypertension occur in over a third of patients with congenital portosystemic shunts and are the leading cause of death.
Patients with CPSS-associated PAH require comprehensive specialized care, including continuous targeted PAH therapy and surgical CPSS treatment.
Answers are grounded in this paper and cite their sources.
Alerts to cardiopulmonary risks in congenital portocaval shunts; leaves open optimal management without controlled data.
Pulmonary arterial hypertension (PAH) associated with portal hypertension, or portopulmonary hypertension, is a severe, life-threatening complication of portal hypertension and/or portocaval shunt surgery. Congenital portocaval shunts (CPSSs) are rare vascular anomalies of the portal system, leading to severe pathophysiological reactions and multisystem damage, including PAH, liver nodules, cognitive, metabolic, immune, hematological and hormonal disorders. Severe cardiopulmonary complications are detected in more than a third of patients with CPSSs, which is the main cause of their death. The article describes the pathophysiology, clinical characteristics, diagnostic features and possibilities of modern targeted therapy for CPSS-associated PAH. Patients with CPSS-associated PAH require comprehensive specialized care in an expert center. For long-term successful management of patients, continuous targeted therapy for PAH, in combination with surgical treatment of CPSSs, is crucial.
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Миклашевич et al. (2024) conducted a review in Pulmonary arterial hypertension associated with congenital portocaval shunts. Targeted therapy for PAH and surgical treatment of CPSSs was evaluated. Severe cardiopulmonary complications occur in over a third of patients with congenital portocaval shunts, requiring continuous targeted PAH therapy combined with surgical shunt treatment.
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