• BhimaniSalima AhmedSADivision of Pediatric Cardiology, Pediatric Institute, Cleveland Clinic, Cleveland, Ohio., Abdelrahman Rahmy, Suzie Kim, Justin B Jin, Kenneth Zahka, Rukmini Komarlu, Lars G Svensson, Amol Pande, Tara Karamlou, Members of Marfan Working Group, Jeevanantham Rajeswaran, Vidayasagar Kalahasti, Milind Desai, Eric Roselli, Miza Salim-Hammoud, William C Frankel, Gosta Pettersson, Eugene H Blackstone, Hani K Najm, and Jared Klein.
• Division of Pediatric Cardiology, Pediatric Institute, Cleveland Clinic, Cleveland, Ohio.
• J. Thorac. Cardiovasc. Surg. 2022 Sep 1; 164 (3): 724-740.e6.

ObjectiveSurveillance metrics in pediatric and young adult Marfan syndrome (pMFS) are challenging. We evaluated the utility of aortic root cross-sectional area/height index (CSA/Ht) on echocardiogram among pMFS patients as a risk stratification and surgical triage metric.MethodsGenotype or phenotype positive pMFS patients aged 25 years or younger seen at our center from 2001 to 2020 were identified. Time-related transition to surgery was modeled using parametric methods. Predictive utility of CSA/Ht compared with aortic root diameter (ARd) and root Z score (ARz) were modeled using nonlinear multivariable parametric and nonparametric longitudinal regression models.ResultsSeventy-nine patients (43% female) presented at median age of 5.8 years (15th-85th percentile, 0.75-17 years) with median follow-up of 4.4 years (range, 0-18.5 years). Baseline echocardiography data were: CSA/Ht, 3.9 ± 1.4 cm2/m; ARd, 2.4 ± 0.89 cm; and ARz, 2.4 ± 1.7. CSA/Ht tracked ARd better compared with ARz (r = 0.91 vs 0.24). Eighteen patients underwent surgery. Surgical procedures included at least 2 components in 17 (aortic, mitral, tricuspid, aortic root, and arch procedures) and isolated mitral valve procedures in 1 patient. Time-related transition to surgery showed a prominent early phase to 1 year post presentation, followed by a slowly increasing late phase. CSA/Ht had a more linear correlation versus ARz during periods of rapid somatic growth in surgical patients. Surgical repair occurred at CSA/Ht between 5 and 7 cm2/m.ConclusionsCSA/Ht tracked ARd well over time. CSA/Ht between 5 and 7 cm2/m might be a promising metric for surgical triage in pMFS patients. CSA/Ht surgical threshold values in pMFS patients occurred at lower than current accepted "surgical" threshold values for CSA/Ht in adult Marfan syndrome patients.Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

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