Patients with Alagille syndrome require specialized care in complex cardiac cases

Patients with Alagille syndrome who also have pulmonary atresia or severe stenosis and major aortopulmonary collateral arteries (MAPCAs) face a higher risk of mortality, according to a study.

Specifically, the study found that individuals with Alagille syndrome had a hazard ratio of 2.8, meaning they were 2.8 times more likely to face a higher risk of death compared to those without Alagille syndrome in the study population.

The study, spanning from November 2001 to December 2021, examined 780 unique patients who underwent surgery for MAPCAs and biventricular heart disease. The annual intake of new patients remained relatively stable for the initial 15 years, followed by a substantial surge in recent years. Notably, nearly 40% of patients had undergone surgery prior to referral, a trend more prevalent in recent cases.

Of the 780 patients, a complete repair was successfully achieved in 704 individuals, constituting a 90% success rate. The median right ventricular to aortic pressure ratio was calculated at 0.34, indicating a favorable outcome.

Over the course of a decade, the cumulative incidence of mortality stood at 15%, with no discernible variance based on the era of surgery, as determined by statistical analysis (P = 0.53). However, multivariable Cox regression identified specific risk factors associated with a higher risk of mortality. Alagille syndrome, preoperative respiratory support, and palliative first surgery at the authors’ center emerged as significant contributors.

Reference
McElhinney DB, Asija R, Zhang Y, et al. 20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries. J Am Coll Cardiol. 2023;82(12):1206-1222. doi: 10.1016/j.jacc.2023.06.041. PMID: 37704311.