Endocardial acceleration (sonR) vs. ultrasound-derived time intervals in recipients of cardiac resynchronization therapy systems.

AIMS: Optimization of cardiac resynchronization therapy (CRT) requires the gathering of cardiac functional information. An accurate timing of the phases of the cardiac cycle is key in the optimization process. METHODS AND RESULTS: We compared Doppler echocardiography to an automated system, based on the recording of sonR (formerly endocardial acceleration), in the detection of mitral and aortic valves closures and measurements of the duration of systole and diastole. We prospectively studied, under various conditions of cardiac stimulation, 75 recipients of CRT systems (69% men), whose mean age was 72 ± 9.2 years, left ventricular ejection fraction 35 ± 11%, baseline QRS duration 154 ± 29 ms, and New York Heart Association functional class 3.0 ± 0.7. We simultaneously recorded (i) sonR, detected by a non-invasive piezoelectric micro-accelerometer sensor clipped onto an electrode located in the parasternal region, (b) electrocardiogram, and (c) Doppler audio signals, using a multichannel data acquisition and analysis system. The correlation between timing of mitral and aortic valve closure by sonR vs. Doppler signals was examined by linear regression analysis. Correlation coefficients and the average absolute error were calculated. A concordance in the timing of the mitral (r = 0.86, error = 9.7 ms) and aortic (r = 0.93, error = 9.7 ms) valves closure was observed between the two methods in 94% of patients. Similarly, sonR and the Doppler-derived measurements of systolic (r = 0.85, error = 13.4 ms) and diastolic (r = 0.99, error = 12 ms) interval durations were concordant in 80% of patients. CONCLUSION: A high concordance was found between sonR and the cardiac ultrasound in the timings of aortic and mitral valve closures and in the estimation of systolic and diastolic intervals durations. These observations suggest that sonR could be used to monitor cardiac function and adaptively optimize CRT systems.

Hypertrophic cardiomyopathy: distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy.

BACKGROUND: Hypertrophic cardiomyopathy is an autosomal-dominant disorder in which 10 genes and numerous mutations have been reported. The aim of the present study was to perform a systematic screening of these genes in a large population, to evaluate the distribution of the disease genes, and to determine the best molecular strategy in clinical practice. METHODS AND RESULTS: The entire coding sequences of 9 genes (MYH7, MYBPC3, TNNI3, TNNT2, MYL2, MYL3, TPM1, ACTC, andTNNC1) were analyzed in 197 unrelated index cases with familial or sporadic hypertrophic cardiomyopathy. Disease-causing mutations were identified in 124 index patients ( approximately 63%), and 97 different mutations, including 60 novel ones, were identified. The cardiac myosin-binding protein C (MYBPC3) and beta-myosin heavy chain (MYH7) genes accounted for 82% of families with identified mutations (42% and 40%, respectively). Distribution of the genes varied according to the prognosis (P=0.036). Moreover, a mutation was found in 15 of 25 index cases with "sporadic" hypertrophic cardiomyopathy (60%). Finally, 6 families had patients with more than one mutation, and phenotype analyses suggested a gene dose effect in these compound-heterozygous, double-heterozygous, or homozygous patients. CONCLUSIONS: These results might have implications for genetic diagnosis strategy and, subsequently, for genetic counseling. First, on the basis of this experience, the screening of already known mutations is not helpful. The analysis should start by testing MYBPC3 and MYH7 and then focus on TNNI3, TNNT2, and MYL2. Second, in particularly severe phenotypes, several mutations should be searched. Finally, sporadic cases can be successfully screened.

The Kora Pacemaker is Safe and Effective for Magnetic Resonance Imaging.

BACKGROUND: The impact of magnetic resonance imaging (MRI) on pacemakers is potentially hazardous. We present clinical results from a novel MRI conditional pacing system with the capability to switch automatically to asynchronous mode in the presence of a strong magnetic field. AIMS: The IKONE (Assessment of the MRI solution: KORA 100™ and Beflex™ pacing leads system) study is an open-label, prospective, multicenter study aimed at confirming the safety and effectiveness of the system, when used in patients undergoing MRI of anatomical regions excluding the chest. METHODS: Primary eligibility criteria included patients implanted with the system, with or without a clinically indicated MRI. The primary endpoint was to confirm no significant change in pacing capture thresholds at 1 month after an MRI, with an absolute difference of ≤0.75 V between the pre- and 1-month post-MRI for both atrial and ventricular capture thresholds. RESULTS: Out of 33 patients enrolled (mean age: 72.8 ± 11.4 years, 70% male, implant indication or device), 29 patients implanted with the MRI conditional system underwent an MRI 6-8 week postimplant. The study reached its primary endpoint: the mean absolute difference in pacing capture threshold at 1-month post-MRI versus pre-MRI was less than 0.75 V in the atrium (Δ = 0.18 ± 0.16 V, P-value < 0.001) and in the ventricle (Δ = 0.18 ± 0.22 V, P-value < 0.001). There were no adverse events related to the MRI procedure nor were there reports of patient symptoms or discomfort associated. MR image quality was of diagnostic quality in all patients. CONCLUSION: Lead electrical performance as measured by difference in capture thresholds were not impacted by MRI. This first clinical evaluation of a novel MRI conditional system demonstrates it is safe and effective for use in out-of-chest, 1.5-T MR imaging.

Does the pretransplant UNOS status modify the short- and long-term cardiac transplant prognosis?

BACKGROUND: We compared the morbidity and mortality rates of patients who had urgent heart transplantation or transplantation after bridging with a ventricular assist device, with the rates of patients whose clinical stability allowed them to wait at home. METHODS: From March 1985 to December 2000, 404 patients underwent heart transplantation in a single center. There were 273 patients with UNOS status 2 (US 2), 103 patients with UNOS Status 1A (US 1A), and 28 patients with UNOS Status 1B (US 1B). We compared the groups retrospectively with respect to pretransplantation status and operative results. RESULTS: Despite more severely impaired hemodynamics and a significantly higher preoperative infection rate in US 1A and 1B patients, there were no statistically significant differences in survival rates among the three groups. Donor sex and age, cytomegalovirus and toxoplasmosis, mismatch rate, ischemic time, method of myocardial protection, and operative technique did not differ statistically among the three groups. Length of intensive care unit stay, postoperative morbidity, first year postoperative rejection rate, and graft occlusive vascular disease rate were statistically similar among the three groups. Although pretransplantation cancer assessment was less complete in US 1A and 1B than in US 2 patients, the late-cancer rate was not statistically different among the three groups. CONCLUSIONS: These data suggest that urgently transplanted patients have both early and long term morbidity and mortality similar to those of patients waiting for transplantation at home or with a ventricular assist device.