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Published on: December 11, 2017
Effects of dual-chamber pacing for pediatric patients with hypertrophic obstructive cardiomyopathy
1Children's Heart Center, Egleston Children's Hospital, Emory University, Atlanta, Georgia 30328, USA.
Insights
Dual-chamber pacing (DCP) effectively treats pediatric hypertrophic obstructive cardiomyopathy (HOCM) by reducing outflow tract gradients and improving symptoms. This therapy is beneficial for selected children unresponsive to medication.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Electrophysiology
Background:
- Hypertrophic obstructive cardiomyopathy (HOCM) is a genetic heart condition causing left ventricular outflow tract (LVOT) obstruction.
- Medications are often insufficient for symptomatic pediatric HOCM.
- Dual-chamber pacing (DCP) has shown promise in adults with HOCM.
Purpose of the Study:
- To evaluate the efficacy of temporary and permanent dual-chamber pacing (DCP) in pediatric patients with hypertrophic obstructive cardiomyopathy (HOCM).
- To assess the impact of DCP on left ventricular outflow tract (LVOT) gradients and patient symptoms.
Main Methods:
- Ten pediatric patients (age 1-17.5 years) with HOCM and LVOT gradient >= 40 mm Hg were studied.
- Temporary pacing was assessed during cardiac catheterization.
- Seven patients with hemodynamic improvement underwent permanent DCP implantation and were followed with questionnaires, Doppler, treadmill tests, and repeat catheterization.
Main Results:
- Temporary pacing showed significant reduction in LVOT gradient, left ventricular systolic pressure, and pulmonary capillary wedge pressure in 70% of patients.
- Permanent DCP led to significant symptom improvement (dyspnea, exercise intolerance) and sustained reduction in LVOT gradient at follow-up (23 months).
- Congenital mitral valve abnormalities and rapid atrial rates were identified as factors precluding effective pacing in some patients.
Conclusions:
- Permanent dual-chamber pacing (DCP) is an effective treatment for selected pediatric patients with hypertrophic obstructive cardiomyopathy (HOCM).
- Careful patient selection is crucial, as certain conditions like congenital mitral valve abnormalities can limit pacing effectiveness.
Objectives:
The effects of both temporary and permanent dual-chamber pacing (DCP) were evaluated in symptomatic pediatric patients with hypertrophic obstructive cardiomyopathy (HOCM) unresponsive to medications.
Background:
Permanent DCP pacing can reduce left ventricular outflow tract (LVOT) gradient and relieve symptoms in adult patients with HOCM.
Methods:
Ten patients (mean [+/-SD] age 11.1 +/- 6 years, range 1 to 17.5) with HOCM and a Doppler LVOT gradient > or = 40 mm Hg were studied. The seven patients showing hemodynamic improvement during temporary pacing at cardiac catheterization underwent surgical implantation of a permanent DCP system. The effects of permanent pacing were evaluated using a questionnaire, Doppler evaluation, treadmill testing and repeat cardiac catheterization.
Results:
At initial cardiac catheterization, three patients failed to respond to temporary pacing (inadequate pace capture in two; congenital mitral valve abnormality in one). The remaining seven patients (70%, 95% confidence interval 38.0% to 91.7%, mean age 13 +/- years, range 4 to 17.5) showed a significant reduction (p < 0.05) in LVOT gradient, left ventricular systolic pressure and pulmonary capillary wedge pressure. After pacemaker implantation, these seven patients reported a significant reduction in dyspnea on exertion and exercise intolerance. Serial Doppler evaluation showed a significant reduction in LVOT gradient. Follow-up catheterization at 23 +/- 4 months in six patients (one patient declined restudy) showed a persistent decrease in LVOT gradient (53 +/- 13 vs. 16 +/- 11 mm Hg), left ventricular systolic pressure (149 +/- 16 vs. 108 +/- 14 mm Hg) and pulmonary capillary wedge pressure (18 +/- 2 vs. 12 +/- 4 mm Hg) versus preimplantation values.
Conclusions:
Permanent DCP is an effective therapy for selected pediatric patients with HOCM. Rapid atrial rates and intrinsic atrioventricular conduction, as well as congenital mitral valve abnormalities, may preclude effective pacing in certain patients.
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