Korean Circ J.  2022 Dec;52(12):865-875. 10.4070/kcj.2022.0143.

Late Outcomes of Pediatric and Congenital Heart Disease Patients Following Cardiac Resynchronization Therapy

• Ahn JE
• Kim ST
• Kwon HW
• Lee SY
• Kim GB
• Kwak JG
• Kim WH
• Song MK
• Bae EJ

• KMID: 2536788
• DOI: http://doi.org/10.4070/kcj.2022.0143

Abstract

Background and Objectives
Cardiac resynchronization therapy (CRT) is an effective treatment for heart failure. However, in pediatric and congenital heart disease (CHD) patients, current adult indications cannot be directly applied because of heterogeneity in anatomy and diagnosis. Therefore, CRT responses and clinical outcomes in these patients were investigated to derive possible candidates for CRT.
Methods
This study retrospectively analyzed 16 pediatric and CHD patients who underwent CRT implantation at a single center in early (0.7±0.2 year) and late (4.7±0.3 years) follow-up period after CRT.
Results
The median age at CRT implantation was 2.5 (0.3–37.2) years, and median follow-up duration was 6.3 (0.1–13.6) years. Thirteen had non-transvenous CRT. Two had congenital complete atrioventricular (AV) block with previous right ventricular pacing, 5 had dilated cardiomyopathy (DCM) with left bundle branch block, and 9 had CHD. The mean ejection fraction of the systemic ventricle increased from 28.1±10.0% to 44.3±21.0% (p=0.003) in early and 51.8±16.3% (p=0.012) in late outcome. The mean functional class improved from 3.1±0.9 to 1.8±1.1 after CRT (p=0.003). Twelve patients (75%) showed improvement in ventricular function or functional class after CRT. Proportion of responders differed between patients without CHD (2/2 patients with complete AV block and 5/5 with DCM, 100%) and those with CHD (5/9, 56%), although statistical significance was not reached (p=0.088).
Conclusions
CRT improved ventricular function and functional status according to the underlying condition in pediatric and CHD patients. However, further large and longer-term studies are needed to establish the guideline for the patient selection of CRT in these patients.

Keyword

Cardiac resynchronization therapy; Congenital heart disease; Pediatrics; Heart failure

Figure

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  Figure 1 Early and late changes of ventricular ejection fraction (A) and left ventricular end-diastolic dimension (B) before and after cardiac resynchronization therapy implantation.cAVB = complete atrioventricular block; DCM = dilated cardiomyopathy; LBBB = left bundle branch block; LV = left ventricle.

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  Figure 2 Early and late changes of QRS duration before and after cardiac resynchronization therapy implantation.cAVB = complete atrioventricular block; DCM = dilated cardiomyopathy; LBBB = left bundle branch block.

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  Figure 3 Early and late changes of New York Heart Association or modified Ross functional class.cAVB = complete atrioventricular block; DCM = dilated cardiomyopathy; LBBB = left bundle branch block.

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  Figure 4 An 8-month-old boy with right isomerism, RV type single ventricle, severe atrioventricular valve underwent CRT implantation and concomitant atrioventricular valve replacement for severe ventricular dysfunction and valve regurgitation. Chest X-ray shows cardiomegaly before CRT (A), which improved 13 months after atrioventricular valve replacement and CRT implantation (B). The CRT ventricular leads were implanted at the apex of the ventricle and the RV outflow tract, respectively. Improvement of ventricular mechanical dyssynchrony and ventricular function in echocardiography after 3 months of CRT. The ejection fraction before CRT was 34%, which increased to 51% 3 months after CRT implantation and atrioventricular replacement. In addition, shortening of QRS duration from 162 ms to 108 ms supported the improvement of electrical dyssynchrony. (C) Before CRT; (D) After CRT.CRT = cardiac resynchronization therapy; RV = right ventricle.

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