Hugues Leduc

All-cause mortality and cardiovascular outcomes with prophylactic steroid therapy in Duchenne muscular dystrophy.

OBJECTIVES This study sought to determine the impact of steroid therapy on cardiomyopathy and mortality in patients with Duchenne muscular dystrophy (DMD). BACKGROUND DMD is a debilitating X-linked disease that afflicts as many as 1 in 3,500 boys. Although steroids slow musculoskeletal impairment, the effects on cardiac function and mortality remain unknown. METHODS We conducted a cohort study on patients with DMD treated with renin-angiotensin-aldosterone system antagonists with or without steroid therapy. RESULTS Eighty-six patients, 9.1 ± 3.5 years of age, were followed for 11.3 ± 4.1 years. Seven of 63 patients (11%) receiving steroid therapy died compared with 10 of 23 (43%) not receiving steroid therapy (p = 0.0010). Overall survival rates at 5, 10, and 15 years of follow-up were 100%, 98.0%, and 78.6%, respectively, for patients receiving steroid therapy versus 100%, 72.1%, and 27.9%, respectively, for patients not receiving steroid therapy (log-rank p = 0.0005). In multivariate propensity-adjusted analyses, steroid use was associated with a 76% lower mortality rate (hazard ratio: 0.24; 95% confidence interval: 0.07 to 0.91; p = 0.0351). The mortality reduction was driven by fewer heart failure-related deaths (0% vs. 22%, p = 0.0010). In multivariate analyses, steroids were associated with a 62% lower rate of new-onset cardiomyopathy (hazard ratio: 0.38; 95% confidence interval: 0.16 to 0.90; p = 0.0270). Annual rates of decline in left ventricular ejection fraction (-0.43% vs. -1.09%, p = 0.0101) and shortening fraction (-0.32% vs. -0.65%, p = 0.0025) were less steep in steroid-treated patients. Consistently, the increase in left ventricular end-diastolic dimension was of lesser magnitude (+0.47 vs. +0.92 mm per year, p = 0.0105). CONCLUSIONS In patients with DMD, steroid therapy is associated with a substantial reduction in all-cause mortality and new-onset and progressive cardiomyopathy.

Pulmonary vein isolation using a second-generation cryoballoon catheter: a randomized comparison of ablation duration and method of deflation.

Optimal cryoballoon ablation parameters for pulmonary vein (PV) isolation remain to be defined. We conducted a randomized preclinical trial to compare 2‐ versus 4‐minute ablation lesions and assess the safety of active (forced) cryoballoon deflation.

Prognostic Value of the Physical Examination in Patients With Heart Failure and Atrial Fibrillation: Insights From the AF-CHF Trial (Atrial Fibrillation and Chronic Heart Failure)

OBJECTIVES This study sought to assess the prognostic value of physical examination in a modern treated heart failure population. BACKGROUND The physical examination is the cornerstone of the evaluation and monitoring of patients with heart failure. Yet, the prognostic value of congestive signs (i.e., peripheral edema, jugular venous distension, a third heart sound, and pulmonary rales) has not been assessed in the current era. METHODS A post-hoc analysis was conducted on all 1,376 patients, 81% male, mean age 67 ± 11 years, with symptomatic left ventricular systolic dysfunction enrolled in the AF-CHF (Atrial Fibrillation and Congestive Heart Failure) trial. The prognostic value of baseline physical examination findings was assessed in univariate and multivariate Cox regression analyses. RESULTS Peripheral edema was observed in 425 (30.9%), jugular venous distension in 297 (21.6%), a third heart sound in 207 (15.0%), and pulmonary rales in 178 (12.9%) patients. Death from cardiovascular causes occurred in 357 (25.9%) patients over a mean follow-up of 37 ± 19 months. All 4 physical examination findings were associated with cardiovascular mortality in univariate analyses (all p values <0.01). In multivariate analyses, taking all 4 signs as potential covariates, only rales (hazard ratio 1.41; 95% confidence interval: 1.07 to 1.86; p = 0.013) and peripheral edema (hazard ratio: 1.25; 95% confidence interval: 1.00 to 1.57; p = 0.048) were associated with cardiovascular mortality, independent of other variables. CONCLUSIONS In the modern era, congestive signs on the physical examination (i.e., peripheral edema, jugular venous distension, a third heart sound, and pulmonary rales) continue to provide important prognostic information in patients with congestive heart failure.

Blood Pressure and Atrial Fibrillation: A Combined AF-CHF and AFFIRM Analysis.

Hypertension is an established risk factor for new‐onset atrial fibrillation (AF). However, the relationship between blood pressure and recurrent AF is less well understood.

Mini Focus Issue: Physical ExamPrognostic Value of the Physical Examination in Patients With Heart Failure and Atrial Fibrillation: Insights From the AF-CHF Trial (Atrial Fibrillation and Chronic Heart Failure)

OBJECTIVES This study sought to assess the prognostic value of physical examination in a modern treated heart failure population. BACKGROUND The physical examination is the cornerstone of the evaluation and monitoring of patients with heart failure. Yet, the prognostic value of congestive signs (i.e., peripheral edema, jugular venous distension, a third heart sound, and pulmonary rales) has not been assessed in the current era. METHODS A post-hoc analysis was conducted on all 1,376 patients, 81% male, mean age 67 ± 11 years, with symptomatic left ventricular systolic dysfunction enrolled in the AF-CHF (Atrial Fibrillation and Congestive Heart Failure) trial. The prognostic value of baseline physical examination findings was assessed in univariate and multivariate Cox regression analyses. RESULTS Peripheral edema was observed in 425 (30.9%), jugular venous distension in 297 (21.6%), a third heart sound in 207 (15.0%), and pulmonary rales in 178 (12.9%) patients. Death from cardiovascular causes occurred in 357 (25.9%) patients over a mean follow-up of 37 ± 19 months. All 4 physical examination findings were associated with cardiovascular mortality in univariate analyses (all p values <0.01). In multivariate analyses, taking all 4 signs as potential covariates, only rales (hazard ratio 1.41; 95% confidence interval: 1.07 to 1.86; p = 0.013) and peripheral edema (hazard ratio: 1.25; 95% confidence interval: 1.00 to 1.57; p = 0.048) were associated with cardiovascular mortality, independent of other variables. CONCLUSIONS In the modern era, congestive signs on the physical examination (i.e., peripheral edema, jugular venous distension, a third heart sound, and pulmonary rales) continue to provide important prognostic information in patients with congestive heart failure.

Heart rate and adverse outcomes in patients with atrial fibrillation: A combined AFFIRM and AF-CHF substudy.

BACKGROUND An elevated resting heart rate has been associated with adverse cardiovascular outcomes. Its prognostic value has not specifically been examined in patients with atrial fibrillation. OBJECTIVE The purpose of this study was to assess the relationship between resting heart rate measured in sinus rhythm and in atrial fibrillation and subsequent hospitalizations and death. METHODS An analysis of individual patient-level data from subjects enrolled in the AFFIRM and AF-CHF trials was conducted to determine the impact of resting heart rate on hospitalizations and mortality. Separate analyses were performed in atrial fibrillation and sinus rhythm. A total of 7159 baseline ECGs (4848 in atrial fibrillation, 2311 in sinus rhythm) were analyzed in 5164 patients (34.8% female, age 68.2 ± 8.3 years). RESULTS During mean follow-up of 40.8 ± 16.3 months, 1016 patients died (668 cardiovascular deaths), and 3150 required at least 1 hospitalization (2215 cardiovascular). An elevated baseline heart rate in sinus rhythm was associated with increased all-cause mortality [hazard ratio (HR) 1.24 per 10 bpm increase, 95% confidence interval (CI) 1.14-1.36, P < .0001]. In contrast, a baseline heart rate in atrial fibrillation was not associated with mortality. However, compared to heart rates 90-114 bpm in atrial fibrillation, a heart rate >114 bpm was independently associated with all-cause (HR 1.18, 95% CI 1.06-1.31, P = .0018) and cardiovascular (HR 1.25, 95% CI 1.10-1.42, P = .0005) hospitalizations. CONCLUSION In patients with a history of atrial fibrillation, an elevated baseline heart rate in sinus rhythm is independently associated with mortality. In contrast, the baseline heart rate in atrial fibrillation is not associated with mortality but predicts hospitalizations.

Systolic blood pressure and mortality in patients with atrial fibrillation and heart failure: insights from the AFFIRM and AF-CHF studies

To investigate the association between baseline systolic blood pressure levels and mortality in patients with AF with or without LV dysfunction. Hypertension leads to cardiovascular disease but, in specific groups, low blood pressure has been associated with a paradoxical increase in mortality. In patients with AF and heart failure, the relationship between blood pressure and death remains largely unknown.

Impaired arm development after Blalock–Taussig shunts in adults with repaired tetralogy of Fallot

BACKGROUND Many adults with repaired tetralogy of Fallot have had prior Blalock-Taussig shunts. These shunts may theoretically hinder growth and development of the ipsilateral arm. METHODS We prospectively enrolled consecutive patients with tetralogy of Fallot in a cross-sectional study to measure arm length and assess handgrip strength. Bilateral handgrip strength was quantified by a dynamometer in a standing position after instructing patients to clench each hand tightly in succession. The maximum force achieved, in kilograms, was measured. RESULTS A total of 80 consecutive adults with tetralogy of Fallot, aged 36.0 ± 12.5 years, 49% female, were prospectively enrolled. Thirty-eight (47.5%) patients had prior Blalock-Taussig shunts at a median age of 1.0 year. Twenty-one (55.3%) were left-sided and 23 (60.5%) were classic shunts. All but six patients with right-sided shunts and one without a prior shunt were right-handed. The shunts were present for a median of 4.0 years prior to takedown during corrective surgery. The arm ipsilateral to the shunt was significantly shorter than the contralateral arm (71.5 ± 6.1 versus 73.6 ± 5.6 cm, P<0.0001). Handgrip strength was significantly weaker on the ipsilateral versus contralateral side (median [IQR], 26.5 [14.0-41.5] versus 31.0 [18.0-46.0] kg, P<0.0001) and the ipsilateral-to-contralateral handgrip ratio was lower with classic versus modified shunts (median [IQR], 1.05 [1.02-1.14] versus 1.19 [1.07-1.33] kg, P=0.0541). CONCLUSION In patients with tetralogy of Fallot, Blalock-Taussig shunts may impair normal development of the ipsilateral arm with repercussions in adulthood that include shorter limb length and reduced handgrip strength. These changes are most pronounced in patients with classic end-to-side anastomoses.

Treatment Failure With Rhythm and Rate Control Strategies in Patients With Atrial Fibrillation and Congestive Heart Failure: An AF-CHF Substudy

Rate and rhythm control strategies for atrial fibrillation (AF) are not always effective or well tolerated in patients with congestive heart failure (CHF). We assessed reasons for treatment failure, associated characteristics, and effects on survival.

ECG Features Associated With Adverse Cardiovascular Outcomes in Patients With Atrial Fibrillation: A Combined AFFIRM and AF-CHF Analysis.

The association between standard parameters from a simple 12‐lead ECG (i.e., QRS duration and PR, JT, and QT intervals) and adverse cardiovascular outcomes (cardiovascular mortality, all‐cause mortality, arrhythmic mortality, and hospitalizations) in patients with a history of atrial fibrillation (AF) has not been previously studied.