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Background: Myotonic dystrophy type 1 (DM1) is a neurologic disorder with known cardiac involvement, including left ventricular systolic dysfunction (LVSD), heart failure (HF), atrioventricular and intraventricular conduction system disease, and sudden death. We studied the prevalence of these conditions and associated findings in a large population with DM1., Methods: History, physical examination, genetic testing, and electrocardiography were performed on 406 patients with DM1, and cardiac imaging was performed on 180 (44.3%) of these patients., Results: Left ventricular systolic dysfunction and clinical HF were found in 34 (18.9%) of 180 and in 23 (5.7%) of 406 of enrolled subjects, respectively, yielding an overall prevalence of LVSD/HF in 41 (10.1%) of 406. Increasing age, male sex, electrocardiographic conduction abnormalities, presence of atrial and ventricular arrhythmias, and implanted devices were all significantly associated with LVSD/HF, whereas cytosine-thiamine-guanine repeat length and neuromuscular severity score were not. The interval≥240 milliseconds (relative risk 4.1, 95% CI 1.7-9.6, P=.001) and QRS duration≥120 milliseconds (relative risk 4.2, 95% CI 2.0-8.5, P<.001) were significant predictors of LVSD/HF. The presence of LVSD/HF was also significantly associated with all-cause death (relative risk 3.9, 95% CI 2.3-6.4, P<.001) and cardiac death (relative risk 5.7, 95% CI 2.6-12.4, P<.001)., Conclusions: A significant prevalence of LVSD/HF exists in patients with DM1. The presence of LVSD/HF in DM1 is significantly associated with all-cause and cardiac death., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Background: Myotonic dystrophy type 1 (DM1) is a neurological disorder with known cardiac involvement, including conduction disturbances, arrhythmias, and ventricular dysfunction. We studied which clinical and electrocardiographic features are associated with structural cardiac abnormalities., Methods: History, physical examination, electrocardiography, and genetic testing were performed on 382 patients with DM1, and cardiac imaging was performed on 100 of these patients., Results: Clinical congestive heart failure was found in 7 of the 382 patients (1.8%). Structural cardiac abnormalities determined with cardiac imaging included left ventricular hypertrophy (19.8%), left ventricular dilatation (18.6%), left ventricular systolic dysfunction (14.0%), mitral valve prolapse (13.7%), regional wall motion abnormality (11.2%), and left atrial dilatation (6.3%). Left ventricular systolic dysfunction was associated with increasing age (relative risk [RR], 1.9 per decade; 95% CI, 1.1-3.2; P =.02), cytosine-thymine-guanine (CTG) repeat length (RR, 2.8 per 500 repeats; 95% CI, 1.3-6.3; P =.01), P-R >200 ms (RR, 14.7; 95% CI, 3.0-73.1; P =.001), and QRS >120 ms (RR, 5.7; 95% CI, 1.5-21.8; P =.01). P-R >200 ms was predictive of regional wall motion abnormalities. QRS >120 ms correlated with regional wall motion abnormalities and left atrial dilatation., Conclusions: Several clinical and electrocardiographic findings in patients with DM1 are significantly associated with structural heart abnormalities. These results suggest an underlying genetic and pathophysiologic correlate that may lead to cardiac disease in these patients.

Objectives: This study was designed to determine echocardiographic follow-up results of the Ross procedure in older adult patients with aortic valve disease., Background: The excellent long-term results of the Ross procedure from several institutions have indicated that the pulmonary autograft may be the best available substitute for the diseased aortic valve in children and adolescents. The advantages of this operation include optimal hemodynamics and elimination of thromboembolic complications. These features may benefit older adult patients as well., Methods: We reviewed data from 49 consecutive patients who had a Ross procedure between 1991 and 1996. Preoperative and postoperative Doppler echocardiographic studies were available for 44 patients (22 men, 22 women; mean [+/-SD] age 36 +/- 14 years) who were grouped into <40 (n = 25) and > or =40 years old (n = 19). Measurements included left ventricular diastolic volume (LVDV), mass, and ejection fraction (EF); a peak pressure gradient across autograft in the aortic position and homograft in the pulmonary position; and valvular regurgitation., Results: The mean length of echocardiographic follow-up was 36 +/- 16 months. Postoperatively, there was a reduction in LVDV and left ventricular mass in both age groups: 153 +/- 99 mL to 111 +/- 72 mL (P =.015) and 210 +/- 93 g to 152 +/- 54 g (P =.002) for younger patients, 174 +/- 115 mL to 126 +/- 43 mL (P =.17) and 233 +/- 71 g to 215 +/- 65 g (P =.19) for older patients. No significant change in EF was noted in the younger age group. However, in the older age group a significant decrease to EF <25% was found in 2 patients 1 year after surgery. Moderate autograft regurgitation was also detected in 2 patients: 1 in each age group. Pressure gradients across the autograft remained within the normal range in both age groups. Two younger patients had severe homograft stenosis with peak gradients of 100 and 62 mm Hg. The older patients did not demonstrate homograft dysfunction., Conclusions: The Ross procedure can be performed in selected older adults with aortic valve disease and provides durable valves in both aortic and pulmonic positions for at least 3 years after surgery but may result in less favorable left ventricular remodeling compared with that in the younger patients. Further follow-up will be necessary to determine the long-term outcome of the Ross procedure in this older adult patient population.