U.O.C. di Aritmologia, Dipartimento Medico Chirurgico di Cardiologia Pediatrica, Ospedale

Pediatrico Bambino Gesù, Palidoro-Fiumicino, Roma, Italy

R. Palmieri , M.S. Silvetti, A. Ammirati, C. Di Mambro, S. Placidi, D. Righi, F. Drago.

CRT in CHD and pediatric patients

• Advances in cardiac surgery have led to an increased survival of patients with congenital

heart disease (CHD)

• Progressive heart failure is a major cause of death during late follow-up of patients with

complex CHD and ventricular dyssynchrony appears to be very common

• The current inclusion criteria for CRT in adult populations may not be directly applied to

pediatric patients because they are a very heterogeneous group.

• In contrast to the vaste experience with biventricular stimulation gathered in adults with

HF, the safety and efficacy of CRT in patients CHD and RV dysfunction has not been fully

established.

• Evidence is limited to case reports, retrospective analyses of heterogeneous populations,

and small crossover trials conducted in the immediate post-operative period..

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CRT in CHD and pediatric patients

1) DUBIN et al (J Am Coll Cardiol. 2005 Dec 20;46(12):2277-83).

A multi-center , retrospective evaluation of CRT in 103 pt from 22 institutions

2) CECCHIN et al (J Cardiovasc Electrophysiol. 2009 Jan;20(1):58-65. Epub 2008 Sep 3)

A single Institution, retrospective evaluation of CRT in 60 consecutive patients

3) JANOUSEK et al (Heart. 2009 Jul;95(14):1165-71. Epub 2009 Mar 22)

A multi-center european, retrospective evaluation of CRT in 109 pt from 17 institutions

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Beyond case reports and small case series, data on mid-and long-term outcome, as well as survival and

complication rates of CRT in paediatric patients are limited to three retrospective studies including

patients with all anatomical subtrates and aetiologies.

• CRT may be a promising therapy to improve the clinical outcome of CHD

and paediatric patients

• LV and single ventricle group have the best response to CRT

• Patients with NYHA class II benefiting the most of the CRT

• An optimal implant technique is very important in the CRT response

• Patients with ventricular dyssynchrony associate with pacing show major

clinical improvement and reverse remodelling with CRT

• The combination of CRT with surgery aimed at decreasing AV-valve

regurgitation may be a valuable strategy to improve CRT response

CRT in CHD and pediatric patients

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OUR STUDY

We retrospectively reviewed all data about 20 consecutive CHD and pediatric patients (12 male and 8 female)

who underwent biventricular pacing between 2006 and 2012 in our institution.

For individualized VV interval optimization 3DE full-volume datasets of the left ventricle were obtained and

analyzed to derive a systolic dyssynchrony index (SDI).

The implantation approach for CRT was transvenous in 5 patients (25%), epicardial in 13 patients (65%) and

hybrid in 2 patients (10%). No complications occurred.

Response to CRT therapy was predefined as a minimum 5% proportional increase in EF over baseline

measurements

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Study population

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20

13 AVB without structural heart defects 7 AVB and CHD

12 CAVB 1

JET

1

AV

C

4

TOF

2

VSD

•All patients had advanced or complete AV block (CAVB).

• 11 patients showed severe systolic left ventricular dysfunction (LVD) without CHD

� 10 CAVB and chronic right ventricular pacing

� 1 after ablate and pace of chronic untreatable congenital Junctional Ectopic

Tachycardia

• 2 CAVB without LVD and “de novo” CRT-P

• 7 patients with CHD and LVD

Multi parametric echocardiographic indices

• Interventricular dyssynchrony:

- IVMD

• Intraventricular dyssynchrony:

- M-mode Pitzalis

- SDI: 3D volumetric (TmSv-16-SD%)

• Atrio-ventricular dyssynchrony:

- morphology of the pulsed Doppler mitral valve

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ECO 3-D/ 4D e CRT(Real time 3-D Echo)

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� Simultaneous study of the 'ENTIRE VENTRICLE’ (longitudinal, radial and circumferential contraction)

to identify GLOBAL DYSSYNCHRONY during the SAME BEAT heart

� SYSTOLIC DYSSYNERGY INDEX: dispersion of the time needed to reach the minimum volume for each of the 16 segments

vn < 3.5% (+ or - 1.8) normal> 15.6% (+ or - 1) severe LV dysfunction

� Reproducibility

� Verifiability

� Universal validity

Results

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Left isomerism, VSD, CMD

SDI LV/RV -40msSDI LV/RV 0 ms

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Tetralogy of Fallot, AVB, LV failure

LV/RV – 72 ms SDI LV/RV - 20ms

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cAVB, PMK VVI

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SDI LV/RV - 20msSDI LV/RV 0 ms

Conclusions

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� Patients with CAVB (isolated or with other

CHD), permanent PM and LVD show good

response to CRT-P upgrading.

� CRT-D is a preferred options in case of

severe LVD (2 deaths!)

� 3D echo is a useful tool to optimize PM

programming in CRT patients.

Tetralogy of Fallot, LV failure

LV-72 ms LV-20ms

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Thank you!