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Pulmonary hypertension associated with postoperative biliary atresia: report of two cases
1Department of Pediatric Surgery, Osaka University Medical School, Suita City, Japan.
Insights
Pulmonary hypertension (PH) can develop years after biliary atresia surgery. Early detection and monitoring of pulmonary artery pressure are crucial for managing this serious long-term complication.
Area of Science:
- Pediatric Gastroenterology
- Cardiology
- Hepatology
Background:
- Biliary atresia is a serious condition in infants requiring surgical intervention.
- Hepatoportoenterostomy is a common surgical procedure for biliary atresia.
- Long-term complications after biliary atresia surgery require ongoing investigation.
Observation:
- Two patients who underwent hepatoportoenterostomy for biliary atresia developed pulmonary hypertension (PH) in late adolescence.
- Both patients had a history of portal hypertension and distal splenorenal shunt.
- Symptoms of dyspnea appeared around 14-15 years of age, indicating a gradual onset.
Findings:
- Cardiac catheterization revealed significantly elevated pulmonary artery pressure (PAP) unresponsive to vasodilators.
- One patient experienced respiratory infection, leading to right heart failure and death at age 20.
- Postmortem examination showed severe pulmonary artery wall thickening, confirming advanced PH.
Implications:
- Pulmonary hypertension can develop insidiously long after successful biliary atresia treatment.
- Regular monitoring of PAP and its response to vasodilators is vital for treatment decisions.
- This highlights the need for comprehensive, long-term follow-up in patients with biliary atresia.
Abstract:
The authors report on 2 patients with biliary atresia in whom pulmonary hypertension (PH) developed in the long-term follow-up after hepatoportoenterostomy. Both had portal hypertension and had undergone distal splenorenal shunt. Dyspnea developed around 14 to 15 years of age. Cardiac catheterization showed pulmonary artery pressure (PAP) of 99/37 (58) and 67/32 (48) mm Hg, respectively, which did not respond to vasodilators. One patient suffered from respiratory tract infection followed by right heart failure and subsequent death at 20 years of age. Postmortum histological findings exhibited severe thickening of the pulmonary artery wall. PH may grow insidiously even after successful hepatoportoenterostomy. Careful monitoring of PAP and hemodynamic response of PAP to vasodilators is essential for evaluating the reversibility of PH and making treatment decisions.