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We sought to couple current cardiac magnetic resonance (CMR) thresholds of right ventricular (RV) size and function with longitudinal trajectories of RV recovery, after pulmonary valve replacement (PVR). We aimed to identify optimal timing of PVR and couple CMR-based metrics with contemporaneous echocardiographic metrics. From June 2002 to January 2019, 174 patients with severe pulmonary regurgitation and peak RV outflow tract gradient <30 mm Hg underwent PVR at Cleveland Clinic. Mean age was 35 ± 16 years and 60 (34%) had concomitant tricuspid valve surgery. RV end diastolic area index (RVEDAi) and function metrics were measured by offline image review on preoperative and 794 postoperative echocardiograms. Contemporaneous RV end diastolic volume index (RVEDVi) was assessed on CMR and correlated to RVEDAi. Multiphase nonlinear mixed-effects models were used to analyze the longitudinal change in RV size and function after PVR. RVEDAi was correlated with RVEDVi (P < 0.0001, r = 0.59). RVEDAi decreased slowly over 10 years following PVR. An inflection point at 24 cm 2 /m 2 was noted at 1 year post-PVR and was associated with failure of RV reverse remodeling and RVEDVi ≥150 mL/m 2 . Compared to patients with preoperative RVEDVi ≥150 mL/m 2 , patients with RVEDVi <150 mL/m 2 had accelerated recovery of longitudinal trajectories of RV size and function metrics on echocardiograms. Reverse remodeling of RV following PVR is an ongoing process. Current accepted threshold values for PVR are associated with greatest RV recovery, suggesting that earlier PVR is warranted. Echocardiography can potentially be utilized in lieu of CMR for surveillance and interventional triage., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Patients with complex systemic and pulmonary venous anatomy, common atrioventricular canal defects and conotruncal anomalies have traditionally been routed to univentricular palliation and labeled as "unseptatable." This report describes our initial experience in septation/biventricular conversion ("ventricular switch"), utilizing the left ventricle (LV) as the subpulmonary ventricle, essentially recapitulating the physiology of congenitally corrected transposition of the great arteries. Five consecutive patients with challenging anatomic configuration underwent septation. All patients were severely cyanotic and had important functional limitations. All patients required complex atrial septation. Ventricular septation was precluded by fixed pulmonary vascular resistance in 2 patients. Systemic venous return was diverted to the morphologic LV as part of physiological 2V (n = 4) or 1.5 V repair (n = 1). Median conversion age was 9 years (range 11 months-46 years). Four patients had 12 previous cardiac surgical procedures in preparation for univentricular repair elsewhere. Three dimensional-printed heart models evaluated feasibility of septation. All patients are alive at a median follow-up of 0.6 years (range 0.08-2.7 years). Median hospital stay was 13 (range 10-60) days. LV recruitment improved functional status and significantly increased systemic oxygen saturation in all patients (79 ± 7% vs 95 ± 5%, P = 0.003). We report a novel paradigm for successfully utilizing both ventricles with the morphologic LV as the subpulmonary ventricle, in a complex population thought to be unseptatable. This approach is versatile and can likely be extrapolated to other complex anatomic configurations. Although we utilized this strategy in patients of variable age, earlier ventricular switch may yield the best results., (Copyright © 2020. Published by Elsevier Inc.)

The objective of this study is to simulate regionalization of congenital heart surgery (CHS) in the United States and assess the impact of such a system on travel distance and mortality. Patients ≤18 years of age who underwent CHS were identified in 2012 State Inpatient Databases. Operations were stratified by the Risk Adjustment for Congenital Heart Surgery, version 1 (RACHS-1) method, with high risk defined as RACHS-1 levels 4-6. Regionalization was simulated by progressive closure of hospitals, beginning with the lowest volume hospital. Patients were moved to the next closest hospital. Analyses were conducted (1) maintaining original hospital mortality rates and (2) estimating mortality rates based on predicted surgical volumes after absorbing moved patients. One hundred fifty-three hospitals from 36 states performed 1 or more operation (19,064 operations). With regionalization wherein, all hospitals performed >310 operations, 37 hospitals remained, from 12.5% to 17.4% fewer deaths occurred (83-116/666), and median patient travel distance increased from 38.5 to 69.6 miles (P < 0.01). When only high-risk operations were regionalized, 3.9-5.9% fewer deaths occurred (26-39/666), and the overall mortality rate did not change significantly. Regionalization of CHS in the United States to higher volume centers may reduce mortality with minimal increase in patient travel distance. Much of the mortality reduction may be missed if solely high-risk patients are regionalized., (Copyright © 2019 Elsevier Inc. All rights reserved.)