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Circulation, Vol 90, 2731-2742, Copyright © 1994 by American Heart Association
L Fananapazir, ND Epstein, RV Curiel, JA Panza, D Tripodi and D McAreavey
BACKGROUND: We previously reported that 6 to 12 weeks of dual-chamber (DDD)
pacing results in clinical and hemodynamic improvement in obstructive
hypertrophic cardiomyopathy (HCM). This study examines the long-term
results of DDD pacing in obstructive HCM. METHODS AND RESULTS: DDD devices
were implanted in 84 patients (mean age, 49 +/- 16 years) with obstructive
HCM and severe drug-refractory symptoms. At a mean follow-up of 2.3 +/- 0.8
years (maximum, 3.5 years), the New York Heart Association (NYHA)
functional class had improved significantly (1.6 +/- 0.6 versus 3.2 +/-
0.5, P < .00001). Symptoms were eliminated in 28 patients (33%),
improved in 47 patients (56%), but remained unchanged in 7 patients (8%).
Two patients died suddenly (97% cumulative 3-year survival rate). In 74
patients with significant left ventricular outflow tract (LVOT) obstruction
at rest, the LVOT gradients were significantly reduced at follow-up (27 +/-
31 versus 96 +/- 41 mm Hg, P < .00001). Symptoms and provokable LVOT
gradients were also reduced in all 10 patients without significant resting
but with provokable LVOT obstruction. Persistence of the LVOT obstruction
and symptoms was attributed to inability to pre-excite the interventricular
septum (n = 8) and onset of atrial fibrillation (n = 7). Fifty patients had
two cardiac catheterization evaluations, 3 +/- 1 and 16 +/- 4 months after
implantation of a pacemaker. In this subgroup, the NYHA functional class
improved from 3.2 +/- 0.5 at baseline to 1.8 +/- 0.7 at the initial
evaluation (P < .00001), but with a further significant improvement at
the second evaluation: 1.4 +/- 0.6, P < .001. This symptomatic
improvement was associated with progressive reduction of LVOT gradient at
the two evaluations: baseline, 100 +/- 47 mm Hg; first evaluation, 41 +/-
36 mm Hg (P < .0001); and second evaluation, 29 +/- 34 mm Hg (P <
.01). Despite the presence of left bundle branch block, DDD pacing reduced
LVOT obstruction significantly in 15 patients (LVOT gradient, baseline 89
+/- 36 mm Hg versus 18 +/- 26 mm Hg at follow-up, P < .0001). There was
a weak but significant correlation between the reduction in LVOT gradients
accomplished by AV pacing before implantation of DDD device and the
eventual reduction in LVOT gradients recorded at the follow-up evaluation
(r = .38, P = .0017). Echocardiography demonstrated significant thinning of
the anterior septum and distal anterior LV wall in the absence of
deterioration of LV systolic function. CONCLUSIONS: (1) Although most of
the improvement of symptoms and hemodynamic indexes occurs during the first
few months of DDD pacing, further changes are often observed a year later;
(2) DDD pacing is associated with an excellent prognosis in a subgroup of
severely disabled patients, many of whom present with syncope or
presyncope; (3) baseline pacing studies are not essential to identify
patients who may benefit from pacing; (4) preexisting left bundle branch
block is compatible with severe LVOT obstruction, and DDD pacing is also
beneficial in this subgroup; (5) DDD pacing reduces both resting and
provokable LVOT obstruction; (6) additional therapy, for example,
radiofrequency ablation of the AV node, may be necessary in some patients
either to preexcite the interventricular septum or to control atrial
fibrillation; and (7) although LV hypertrophy has been considered a primary
feature of HCM, pacing appears to reverse LV wall thickness in a
significant subset of adult HCM patients.
ARTICLES
Long-term results of dual-chamber (DDD) pacing in obstructive hypertrophic cardiomyopathy. Evidence for progressive symptomatic and hemodynamic improvement and reduction of left ventricular hypertrophy
Inherited Cardiac Diseases Section, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892.
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