Introduction: Patients with Marfan syndrome often require intervention on the mitral valve (MV) or thoracic aorta during childhood or adolescence but reports of outcomes in this population are limited.
Methods: The Pediatric Hospital Information System database, a multi-institutional administrative database, was queried for all patients less than 25 years old with diagnosis of Marfan syndrome (ICD-9 759.82) who underwent MV, aortic valve (AV), or thoracic aortic surgery from January 2004 to December 2016. We reported peri-operative complications, and performed univariate analysis to evaluate variables associated with inpatient mortality.
Results: Included were 321 hospital encounters in 294 patients (71% male). Ninety patients underwent 97 MV surgeries and 249 patients underwent 262 aortic/AV surgeries. In 38 cases, the patient underwent MV and aortic/AV surgery in the same hospitalization. Median age was 11.1 y (range 0.4-23.4) for those undergoing MV procedures and 15.7 y (range 0.1-24.3) for those undergoing aortic/AV procedures. Aortic dissection or rupture was reported in 11 patients (3.4%). Perioperative complications were common for all surgeries (Table). Most frequent associated complications were arrhythmia (26.1%) and post-operative hemorrhage (13.7%). Overall hospital mortality was 2.2%. Patients who died after MV surgery were younger at surgery, but not significantly (died median age 2.3 y, range 0.4-11.4; survived median age 11.2 y, range 0.4-23.4, p=0.10). Variables associated with death after aortic/AV surgery were post-operative use of ECMO (4/4 vs. 0/258, p<0.01), and younger age at surgery (died median age 10.3 y, range 5.3-14.6; survived median age 15.9 y, range 0.1-24.3, p<0.05). No case with a diagnosis of aortic dissection or rupture died.
Conclusions: Postoperative complications are common in pediatric and young adult patients with Marfan syndrome after MV, AV, and thoracic aortic surgeries, but mortality is relatively low.