Faculty Collaboration Database

Simplified mitral valve repair in pediatric patients with connective tissue disorders. J Thorac Cardiovasc Surg 2017 Feb;153(2):399-403

Date

01/21/2017

Pubmed ID

28104197

DOI

10.1016/j.jtcvs.2016.09.039

Scopus ID

2-s2.0-85009953717 (requires institutional sign-in at Scopus site)   14 Citations

Abstract

BACKGROUND: In pediatric patients with connective tissue disorders (CTDs), early cardiac presentation often involves severe mitral regurgitation (MR) associated with severe bileaflet prolapse and, less frequently, aortic root enlargement. We adopted a simplified repair to address MR and prevent systolic anterior motion (SAM) in this unique group of patients.

MATERIALS AND METHODS: Retrospective review of clinical and echocardiographic data of all pediatric patients (age < 18 years) with CTD and MR undergoing simplified repair at 3 institutions (2000-2014).

RESULTS: Eighteen children who underwent surgery for severe MR and bileaflet prolapse were identified. All were treated with ring annuloplasty and Alfieri edge-to-edge repair. Median age and weight were 8.2 years (range, 0.4-17.2 years) and 24.9 kg (5.6-63.3 kg), respectively. Median left ventricular end diastolic dimension median z score was 4.9 (2.1-11.9). One patient died (5.6%), and there were no other major complications. Among survivors, 94.4% had mild regurgitation or less, with no stenosis or SAM at median clinical follow-up of 2.4 years (range, 0-13.9 years). Median left ventricular end-diastolic dimension z score regressed to 1.3 (-0.5 to 4.3).

CONCLUSIONS: In pediatric patients with CTD and severe MR, a simplified approach is associated with intermediate-term competence, absence of SAM or significant stenosis, and regression of left ventricular enlargement.

Author List

Vricella LA, Ravekes WA, Arbustini E, Jaquiss RD, Mavroudis C, Dietz HC, Jacobs ML, Hibino N, Cameron DE

Author

Robert D. Jaquiss MD Chief, Center Associate Director, Professor in the Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Child
Child, Preschool
Connective Tissue Diseases
Echocardiography
Female
Follow-Up Studies
Forecasting
Humans
Infant
Male
Mitral Valve
Mitral Valve Annuloplasty
Mitral Valve Insufficiency
Retrospective Studies
Time Factors
Treatment Outcome