Thoracic Aortic, Aortic Valve, and Mitral Valve Surgery in Pediatric and Young Adult Patients With Marfan Syndrome: Characteristics and Outcomes

Semin Thorac Cardiovasc Surg. 2019 Winter;31(4):818-825. doi: 10.1053/j.semtcvs.2019.06.005. Epub 2019 Jun 21.


Patients with Marfan syndrome (MFS) often require surgical intervention on the mitral valve (MV), aortic root or valve (AV), or thoracic aorta (TA) during childhood and adolescence. We aim to utilize a national database to evaluate outcomes in pediatric and young adult patients with MFS undergoing MV, AV, and aortic surgical procedures, and describe factors associated with increased mortality. The Pediatric Hospital Information System (PHIS) database, a multi-institutional administrative database of 48 pediatric hospitals, was queried for patients less than 25 years of age with a diagnosis of MFS (ICD-9 759.82) who underwent MV, AV, or thoracic aortic surgery between January 2004 and October 2015. We assessed comorbidities and complications, and performed univariate analysis to evaluate factors associated with inpatient mortality. Included were 321 hospital encounters in 294 patients. Fifty-one patients underwent 54 MV surgeries, 213 patients underwent 224 aortic/AV surgeries, and 43 patients underwent both MV and aortic/AV surgery in the same encounter. Postoperative complications were common for all surgeries (46.3% for MV procedures and 45.5% for aortic/AV procedures). Overall in-hospital mortality was 2.2% (3.7% for MV procedures, 1.8% for AV/aortic procedures, and 2.3% in the combined MV and aortic/AV procedure group). Aortic dissection or rupture was reported in 3.4%, with no in-hospital mortalities. Death after MV as well as after aortic/AV surgery was associated with younger age. Postoperative complications are common in pediatric and young adult patients with MFS after intervention on the MV, AV, and TA, although mortality is relatively low.

Keywords: Aortic; Marfan syndrome; Mitral; Pediatric.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Age Factors
  • Aorta, Thoracic / diagnostic imaging
  • Aorta, Thoracic / surgery*
  • Aortic Aneurysm, Thoracic / diagnostic imaging
  • Aortic Aneurysm, Thoracic / mortality
  • Aortic Aneurysm, Thoracic / surgery*
  • Aortic Valve / diagnostic imaging
  • Aortic Valve / surgery*
  • Child
  • Child, Preschool
  • Databases, Factual
  • Female
  • Heart Valve Diseases / diagnostic imaging
  • Heart Valve Diseases / mortality
  • Heart Valve Diseases / surgery*
  • Heart Valve Prosthesis Implantation* / adverse effects
  • Heart Valve Prosthesis Implantation* / mortality
  • Hospital Mortality
  • Humans
  • Infant
  • Male
  • Marfan Syndrome / diagnosis
  • Marfan Syndrome / epidemiology*
  • Marfan Syndrome / mortality
  • Mitral Valve / diagnostic imaging
  • Mitral Valve / surgery*
  • Mitral Valve Annuloplasty* / adverse effects
  • Mitral Valve Annuloplasty* / mortality
  • Postoperative Complications / mortality
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Treatment Outcome
  • United States
  • Vascular Surgical Procedures* / adverse effects
  • Vascular Surgical Procedures* / mortality
  • Young Adult