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Cardiovascular Surgery In Children With Marfan and Marfan-like Syndromes
M. Everitt1, M. Mitchell*2, P. Kouretas*1, A. T. Yetman2. 1Primary Children's Medical Center, Salt Lake City, UT, 2The Children's Hospital, Denver, CO,
BACKGROUND: Patients with Marfan syndrome (MFS) or Loeys-Dietz syndrome (LDS) most often require cardiovascular surgery (CVS) in adulthood. There is a paucity of data on long-term outcome of patients undergoing CVS in childhood. We sought to assess the frequency, type, and outcome of CVS performed in patients < 18 years with MFS or LDS.
METHODS: Clinical databases from 2 regional Marfan subspecialty clinics were reviewed. Data was collected on all children satisfying clinical criteria for a diagnosis of MFS, or with a genetic diagnosis of LDS, who underwent CVS between 1997-2007. Age at diagnosis, at CVS and at death or last follow-up, aortic diameter at CVS (absolute & indexed values), primary indication for CVS, postoperative complications, need for reoperation, and clinical status at follow-up were recorded.
RESULTS: Between 1997-2007, 162 children (86 [53%] female) with MFS/LDS were followed serially of whom 33 (20 %) (8 [23%] female) underwent 42 surgical procedures. Initial CVS was performed 5.2 + 4.8 years after diagnosis, at a mean age of 11.4 + 6.3 years with 28 (85%) patients undergoing aortic root replacement (AR), and 5 mitral valve (MV) surgery. Indication for AR was attainment of established surgical criteria (45-50 mm [MFS]; z-score >3.5 [LDS]) in 20, rapid aortic growth in 2, aortic dissection in 3, and concomitant MV surgery being performed in 3. Absolute aortic diameters and corresponding indexed z-scores at surgery were 45.9 + 11.2 and 10.1 + 4.9 respectively. A valve-sparing aortic root replacement (VSRR) was performed in 21 (64%) patients (7 remodeling, 14 reimplantation technique) with the remaining 7 patients not eligible due to aortic dissection in 3, > moderate aortic insufficiency (AI) in 3 and patient refusal in 1. Eight patients underwent a Bentall repair with a 26 (17-29) mm mechanical valve. Concomintant surgical procedures included pectus repair in 5, ASD closure in 6, TV repair in 3, PDA ligation in 4 and hemiarch repair in 2. Of the 5 patients undergoing primary MV surgery, 4 underwent repair and 1 replacement due to extensive annular calcification. Peri-operative complications included brain death in association with carotid artery dissection in 1 patient with undiagnosed LDS presenting with extensive dissection, hemopericardium in 2, impaired LV function in 7, respiratory failure in 1 and endocarditis in 1. Patients were followed for 4.5 (0.5-10) years. Re-operation was required in 9 (27%) patients, 3.3 + 1.9 years following initial surgery for severe AI in 3, type B dissection in 1, aortic root dilation in 1, prosthetic valve dysfunction in 3, and flail MV in 1. Need for reoperation was higher in those undergoing a Bentall vs. VSRR (80% vs. 30%; p= 0.02). There were 3 late deaths. Risk of adverse outcome (death or dissection) was greater in patients with LDS vs. MFS (62% vs. 0%; p<0.001). All survivors were free of cardiovascular symptoms, and > mild MR or AI.
CONCLUSIONS: Patients with Marfan syndrome requiring CVS during childhood have a favorable long-term outcome. VSRR carries a low risk of reoperation and should be performed if possible.
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