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Dual chamber pacemaker therapy for mid-cavity obstructive hypertrophic cardiomyopathy
D Begley1, S Mohiddin, L Fananapazir
1Inherited Heart Diseases Section, Cardiology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892, USA.
Insights
Dual chamber (DDD) pacemaker therapy significantly improved symptoms and reduced pressure gradients in patients with mid-cavity obstructive hypertrophic cardiomyopathy (HCM). This therapy offers a viable alternative for patients unsuitable for traditional cardiac surgery.
Area of Science:
- Cardiology
- Cardiovascular Medicine
- Medical Devices
Background:
- Intracavitary left ventricular (LV) obstruction is a key factor in hypertrophic cardiomyopathy (HCM) outcomes.
- Mid-cavity obstructive HCM, sometimes with LV aneurysm, presents challenges for standard surgical intervention.
- This specific obstruction pattern affects a minority of HCM patients and may indicate a poorer prognosis.
Purpose of the Study:
- To evaluate the long-term efficacy of dual chamber (DDD) pacemakers in managing mid-cavity obstructive HCM.
- To assess the impact of DDD pacing on symptoms, functional class, and hemodynamic parameters in this patient subset.
Main Methods:
- A study involving 14 patients with mid-cavity obstructive HCM (mean age 34 years).
- Patients received DDD pacemakers and were assessed via cardiac catheterization before and after therapy (6 months to 1 year follow-up).
- Drug therapy was discontinued during the evaluation period.
Main Results:
- All patients experienced symptomatic improvement, with a significant reduction in NYHA functional class (from 2.8 to 1.9, P < 0.0005).
- Intracavitary LV pressure gradients decreased significantly (from 84 to 43 mmHg, P < 0.0005).
- A significant reduction in apical LV systolic pressure and a trend towards increased exercise tolerance were observed.
Conclusions:
- Chronic DDD pacing provides substantial symptomatic and hemodynamic benefits for patients with mid-cavity obstructive HCM.
- This therapy is a valuable option for patients with this specific HCM subtype, where surgical options are less defined.
Abstract:
Intracavitary LV obstruction is an important determinant of clinical outcome in hypertrophic cardiomyopathy (HCM). In a minority of patients the obstruction is at the level of the papillary muscles. Mid-cavity obstructive HCM may be associated with a distal LV aneurysm and a worse prognosis. It is often not amenable to standard cardiac surgeryfor LV outflow obstruction. The long-term effects (mean follow-up 4.8+/-2.9 years) of dual chamber (DDD) pacemaker therapy in 14 patients with mid-cavity obstructive HCM (mean age 34+/-16 years, range 15-65 years) were studied. Patients were evaluated by cardiac catheterization at baseline and 6 months to 1 year after receiving DDD pacemakers off all drug therapy. Symptoms were improved in all patients and NYHA functional class reduced from 2.8+/-0.1 to 1.9+/-0.4 (P < 0.0005). Intracavitary LV pressure gradients was reduced significantly (43+/-36 vs 84+/-31 mmHg at baseline, P < 0.0005). There was a significant associated reduction in apical LV systolic pressure (152+/-37 vs 188+/-34 mmHg, P < 0.001). In addition, there was a trend towards increased exercise tolerance (445+/-123 vs 396+/-165). Cardiac output and LV filling pressures were unchanged. In conclusion, chronic DDD pacing results in significant symptomatic and hemodynamic improvement in this uncommon but important subset of patients with obstructive HCM in whom the role of cardiac surgery is less well defined compared with the more typical outflow tract location of LV obstruction.
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