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Modifying Longitudinal Growth Trajectories Of Aortic Structures In Pediatric Marfan Syndrome: Impact Of And Interactions Between Medications And Surgery
Tara Karamlou, MD, MSc, Amol Pande, PhD, Salima Bhimani, MD, Suzie Kim, BS, Abdelrahman Rahmy, BS, Justin Jin, MD, Kenneth Zahka, MD, Rukmini Komarlu, MD, Lars Svensson, MD, PhD, Vidyasagar Kalahasti, MD, Milind Desai, MD, Eric Roselli, MD, Hani K. Najm, MD, MSc, Gosta Pettersson, MD, PhD.
Cleveland Clinic, Cleveland, OH, USA.

Objective(s): Efficacy of beta blockers (BBs) and angiotensin-receptor blockers (ARBs), accounting for interactions with surgery on longitudinal aortic root (AR) growth in pediatric Marfan Syndrome (pMFS) is unknown. We evaluated how the initiation and dosage adjustments of medications over time, accounting for surgical intervention, impacted AR growth.
Methods: pMFS ≤age 26 years 2001-2020 were identified. Serial echocardiograms (N=809) underwent protocolized review. BBs, ARBs and weight-based dose adjustments were tracked. Main outcome was AR cross sectional area/height ratio (ARCSA/Ht). We utilized a novel mathematical model that simultaneously accounts for medication profiles measured on a continuum and relates these estimates to distinct echocardiographic assessments.
Results: Of 78 patients with median age 5.5 years (y) (25/75%: 1.8-12.5y): 31 patients were on BB+ARBís, 19 only ARB, 13 only BB, and 15 patients neither. Surgery occurred in 18 patients. Median age at medication initiation was 7.7y (25/75%: 3.5-15.2y). Average medication changes/patient was 3.7. Patient factors, medications, and surgical intervention differentially impacted ARCSA/Ht across time with surgery predominating after 4 years (Figure 1a). Importantly, BB+ARBs stabilized ARCSA/Ht among those with higher initial ARCSA/Ht values. (Figure 1b). However, ARCSA/Ht progressed rapidly among those having surgery at 9 years despite medical therapy.
Conclusions: Combination therapy with BB+ARB stabilizes ARCSA/Ht and delays surgical intervention, but is a marker for worse phenotype. A subgroup of patients will progress in the mid-term and require definitive surgery.


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