Early and Late Effects of Cardiac Resynchronization Therapy in Adult Congenital Heart Disease

Abstract

BackgroundThere are limited data about cardiac resynchronization therapy (CRT) in adult congenital heart disease. We aimed to assess early and late outcomes of CRT among patients with adult congenital heart disease.Methods and ResultsWe retrospectively studied 54 patients with adult congenital heart disease (median age, 46 years; range, 18–73 years; 74% men) who received CRT implantation (biventricular paced >90%) between 2004 and 2017. Clinical and echocardiographic data were analyzed at baseline and early (mean±SD: 1.8±0.8 years) and late (4.7±0.8 years) follow‐up after CRT. Compared with baseline, CRT was associated with significant improvement at early follow‐up in New York Heart Association functional class, QRS duration, and cardiothoracic ratio (P<0.05 for all); improvement in New York Heart Association class was sustained at late follow‐up. Among patients with a systemic left ventricle (LV; n=39), there was significant increase in LV ejection fraction and reduction in LV end‐systolic volume at early and late follow‐up (P<0.05 for both). For patients with a systemic right ventricle (n=15), there was a significant early but not late reduction in systemic right ventricular basal and longitudinal diameters. Eleven patients died, and 2 had heart transplantation unrelated to systemic ventricular morphological characteristics. Thirty‐five patients (65%) responded positively to CRT, but only baseline QRS duration was predictive of a positive response.Conclusions CRT results in sustained improvement in functional class, systemic LV size, and function. Patients with a systemic LV and prolonged QRS duration, independent of QRS morphological characteristics, were most likely to respond to CRT.

Cardiac Resynchronization Therapy for Adult Patients With a Failing Systemic Right Ventricle: A Multicenter Study

“…Recent survival data show a 5‐year survival rate of ≈80% for adult patients with CHD undergoing CRT. 22 , 23 The 5‐year survival rate in the present study is 85%, which is in line with a recent single‐center study including 31 patients with SRV. 24 Our data indicate that CRT, regardless of precise lead location, is a promising long‐term therapy in patients with SRV with ventricular pacing–induced dyssynchrony.…”

Cardiac resynchronization therapy for the failing systemic right ventricle: A systematic review

“…More recently, Karpawich et al used an invasive measure of contractility (dP/dt-max) with a catheter within the SRV as a pre-implant screening tool in CHD patients [22]. Six DTGA and 2 CCTGA adult patients (age 25 [24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39]) showed an initial dP/dt-max improvement of ≥15% and received CRT. During a median follow-up time of 2.7 years, hemodynamic benefits of CRT persisted in all patients.…”

“…Less favorable long-term outcomes were reported by another single-center study conducted by Yin et al who also focused on exclusively adult SRV patients. After long-term follow-up (4-5 years), 40% of the patients were identified as CRT responders, defined as ≥5% absolute increase in SRV function or FAC [27].…”

JCS 2022 Guideline on Management and Re-Interventional Therapy in Patients With Congenital Heart Disease Long-Term After Initial Repair

“…We can apply the CRT method for general adult cardiology to CHD patients with a systemic LV showing LV intraventricular dyssynchrony. 248 The circumstances are different for those with right ventricular (RV) conduction delay and RV intraventricular dyssynchrony, because the architecture and contractile sequence of the RV differ from those of the LV. CRT for the RV appears to be less effective than for the LV.…”

“…CRT for the RV appears to be less effective than for the LV. 248 There have been several reports stating the results of CRT for the systemic RV and subpulmonary RV. Further accumulation of cases is mandatory to establish better treatment strategies.…”