John M. Karamichalis’s research while affiliated with New York Presbyterian Hospital and other places

Objective: Ebstein's anomaly is a rare congenital heart malformation for which surgical and medical management are still controversial. The cone repair has transformed surgical outcomes in many of these patients. We aimed to present our results on the outcomes of patients with Ebstein's anomaly who underwent a cone repair or tricuspid valve replacement. Methods: A total of 85 patients who underwent a cone repair (mean age, 16.5 years) or tricuspid valve replacement (mean age, 40.8 years) between 2006 and 2021 were included. Univariate, multivariate, and Kaplan-Meier analyses were used to evaluate operative and long-term outcomes. Results: Residual/recurrent greater than mild-to-moderate tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement (36% vs 5%; P = .010). However, at last follow-up, the risk of greater than mild-to-moderate tricuspid regurgitation was not different between groups (35% in the cone group vs 37% in the tricuspid valve replacement group; P = .786). The tricuspid valve replacement group had a higher risk of tricuspid valve reoperation (37% vs 9%; P = .005) and tricuspid stenosis (21% vs 0%; P = .002) compared with the cone repair group. Kaplan-Meier freedom from reintervention was 97%, 91%, and 91% at 2, 4, and 6 years after cone repair, respectively, and 84%, 74%, and 68% at 2, 4, and 6 years after tricuspid valve replacement, respectively (P = .0191). At last follow-up, right ventricular function was significantly worse from baseline in the tricuspid valve replacement group (P = .0294). There were no statistical differences between age-stratified cohorts or surgeon volume in the cone repair group. Conclusions: The cone procedure offers excellent results, with stable tricuspid valve function and low reintervention and death rates at last follow-up. The rate of greater than mild-to-moderate residual tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement, but this did not expose the patient to a higher risk of reoperation or death at last follow-up. Tricuspid valve replacement was associated with a significantly higher risk of tricuspid valve reoperation and tricuspid valve stenosis, and worse right ventricular function at last follow-up.

OBJECTIVES To examine cases of operative mortality at a single quaternary academic center for patients undergoing relatively lower risk (STAT Mortality Category 1-3) procedures, as a means of identifying systemic weaknesses and opportunities for quality improvement. METHODS A retrospective review of all operative mortality events for patients who underwent a STAT Mortality Category 1, 2, or3 index procedure (2009-2020) at our institution was performed. After a detailed chart review was performed by two independent faculty for each case, factors and/or system deficiencies that contributed to mortality were identified. RESULTS 42 mortalities were identified. 37 patients(88%) had at least one STS-designated risk factor, including prior cardiac operations(48%), extracardiac malformations(43%), preoperative ventilation(33%). Eight patients(19%) had non-STS-designated preoperative patient-level variables considered as at potential risk, including severe ventricular dysfunction, pulmonary hypertension, lung hypoplasia, and undiagnosed severe coronary abnormalities. Four patients (10%) had no identified preoperative risk factors. After detailed chart review, five broad categories were identified: patient-related factors (n=33;78%), postoperative infection (n=13;31%), postoperative residual lesions (n=7;17%), Fontan physiology failure (n=4;10%) and unexplained left ventricular failure after tetralogy of Fallot repair (n=3;7%). 74% of patients had at least one preoperative, intraoperative or postoperative system deficiencies. 50% of surgery were urgent or emergent. CONCLUSIONS Operative mortality after STAT Mortality Category 1-3 procedures is related to the presence of multifactorial risk patterns (STS and non-STS designated patient-level risk factors and variables, broad risk categories, system deficiencies, emergent surgery). A multidisciplinary approach to care, with early recognition and treatment of modifiable additional burdens could reduce this risk.