Abstract
BackgroundOnce surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction.MethodsA retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair.ResultsOf 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14–57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died.A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60o, relative risk of dehiscence was 3.2 (CI 1.3–4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3–100, p < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p = 0.04), and were older (median 38 vs 19 years, p = 0.01). TR %, chamber area ratios and LV parameters were not different.ConclusionsComprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.
Highlights
Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy
Cohort description Between 2009 and May 2018, a total of 26 patients older than 12 years had undergone cone reconstruction of the native Ebstein tricuspid valve in our unit, having met conventional criteria for surgery. Six of these 26 patients were not examined with preoperative cardiovascular magnetic resonance (CMR), and have been excluded from this analysis
CMR examinations The CMR examinations were undertaken at a median age (13–56) years, and the surgery was undertaken at an age of (14–57) years
Summary
Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. Ebstein anomaly of the tricuspid valve involves failure of delamination of the septal leaflet, and to a variable extent, failure of delamination of the inferior and anterior leaflets [1]. The anomaly shows remarkable morphologic and physiologic heterogeneity. It is strongly associated with right ventricular myocardial disease, conduction tissue anomalies and left ventricular (LV) noncompaction [2]. Hughes et al Journal of Cardiovascular Magnetic Resonance (2019) 21:34 leaflet and the remnants of the septal and inferior leaflets, a reduction posterior commissuroplasty, a limited right ventricular plication over the diaphragmatic surface if indicated, and a partial ring or deVega annuloplasty, to support the repair. The success of da Silva’s surgery [4] and subsequently that of other groups [5,6,7] has led to increasing uptake of this procedure in many centres, worldwide
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