Emmanouil Liodakis

Left Ventricular Longitudinal Function Predicts Life-Threatening Ventricular Arrhythmia and Death in Adults With Repaired Tetralogy of Fallot

Background— Sudden cardiac death and life-threatening ventricular arrhythmia remain a concern in adult patients with repaired tetralogy of Fallot. Longitudinal left ventricular (LV) function is sensitive in detecting early myocardial damage and may have prognostic implications in this setting. Methods and Results— We included 413 tetralogy of Fallot patients (age, 36±13 years; QRS duration, 148±27 milliseconds; LV ejection fraction, 55±10%). A composite end point of sudden cardiac death/life-threatening ventricular arrhythmia (sustained ventricular tachycardia, resuscitated sudden cardiac death, or appropriate implantable cardioverter-defibrillator discharge) was used. During a median follow-up of 2.9 years, 5 patients died suddenly, 9 had documented sustained ventricular tachycardia, and another 5 had appropriate implantable cardioverter-defibrillator shocks. On univariate Cox analysis, QRS duration (hazard ratio [HR], 1.02 per 1 ms; P=0.046), right atrial area (HR, 1.05 per 1 cm2; P=0.02), right ventricular fractional area change (HR, 0.94 per 1%; P=0.02), right ventricular outflow tract diameter (HR, 1.08 per 1 mm; P=0.01), mitral annular plane systolic excursion (HR, 0.84 per 1 mm; P=0.03), and LV global longitudinal 2-dimensional strain (HR, 0.87 per 1%; P=0.03) were related to the combined end point. On bivariable analysis, mitral annular plane systolic excursion and LV global longitudinal 2-dimensional strain were related to outcome independently of QRS duration (P=0.002 and P=0.01, respectively). In addition, a combination of echocardiographic variables, including right atrial area, right ventricular fractional area change, and LV global longitudinal 2-dimensional strain or mitral annular plane systolic excursion, was also found to be significantly related to outcome (P<0.001; c statistic, 0.70). Conclusions— LV longitudinal dysfunction was associated with greater risk of sudden cardiac death/life-threatening ventricular arrhythmias. In combination with echocardiographic right heart variables, also available from routine echocardiography, these measures provide important outcome information and should be considered a useful adjunct to established markers such as QRS duration in the estimation of prognosis in this challenging population.

The Prevalence and Prognostic Significance of Right Ventricular Systolic Dysfunction in Nonischemic Dilated Cardiomyopathy

Background— Cardiovascular magnetic resonance is the gold-standard technique for the assessment of ventricular function. Although left ventricular volumes and ejection fraction are strong predictors of outcome in dilated cardiomyopathy (DCM), there are limited data regarding the prognostic significance of right ventricular (RV) systolic dysfunction (RVSD). We investigated whether cardiovascular magnetic resonance assessment of RV function has prognostic value in DCM. Methods and Results— We prospectively studied 250 consecutive DCM patients with the use of cardiovascular magnetic resonance. RVSD, defined by RV ejection fraction ⩽45%, was present in 86 (34%) patients. During a median follow-up period of 6.8 years, there were 52 deaths, and 7 patients underwent cardiac transplantation. The primary end point of all-cause mortality or cardiac transplantation was reached by 42 of 86 patients with RVSD and 17 of 164 patients without RVSD (49% versus 10%; hazard ratio, 5.90; 95% confidence interval [CI], 3.35–10.37; P<0.001). On multivariable analysis, RVSD remained a significant independent predictor of the primary end point (hazard ratio, 3.90; 95% CI, 2.16–7.04; P<0.001), as well as secondary outcomes of cardiovascular mortality or cardiac transplantation (hazard ratio, 3.35; 95% CI, 1.76–6.39; P<0.001), and heart failure death, heart failure hospitalization, or cardiac transplantation (hazard ratio, 2.70; 95% CI, 1.32–5.51; P=0.006). Assessment of RVSD improved risk stratification for all-cause mortality or cardiac transplantation (net reclassification improvement, 0.31; 95% CI 0.10–0.53; P=0.001). Conclusions— RVSD is a powerful, independent predictor of transplant-free survival and adverse heart failure outcomes in DCM. Cardiovascular magnetic resonance assessment of RV function is important in the evaluation and risk stratification of DCM patients.

Clinical utility and prognostic value of left atrial volume assessment by cardiovascular magnetic resonance in non-ischaemic dilated cardiomyopathy

Echocardiographic studies have shown that left atrial volume (LAV) predicts adverse outcome in small heart failure (HF) cohorts of mixed aetiology. However, the prognostic value of LAV in non‐ischaemic dilated cardiomyopathy (DCM) is unknown. Cardiovascular magnetic resonance (CMR) allows accurate and reproducible measurement of LAV. We sought to determine the long‐term prognostic significance of LAV assessed by CMR in DCM.

Peak oxygen uptake correlates with disease severity and predicts outcome in adult patients with Ebstein's anomaly of the tricuspid valve.

BACKGROUND Ebsteins anomaly of the tricuspid valve often results in biventricular dysfunction and functional deterioration. Little is known about the relation between exercise capacity, disease severity and outcome in adults with Ebsteins anomaly. METHODS Data on all patients with Ebsteins anomaly of the tricuspid valve who underwent cardiopulmonary exercise testing in our tertiary center were collected. The relation between exercise parameters, anatomic severity (Glasgow outcome score) and the combined end-point of death, non-elective hospitalization and surgical repair was studied using Cox regression analysis. RESULTS A total of 51 adult patients fulfilled inclusion criteria (49% male, mean age 37.8±13.6 years). Mean peak oxygen uptake (peak VO2) was 63.2±18.7% of predicted, the slope of ventilation per unit of carbon dioxide output (VE/VCO2 slope) 37.4±11.4, heart rate reserve (HRR) 23.6±22.7 bpm. A significantly lower peak VO2 was found in patients with a higher Glasgow outcome score, higher cardiothoracic ratio and documented atrial shunt. Peak VO2 (HR for value <60% of predicted 3.47, 95% CI: 1.28–9.44, p=0.015) and HRR (HR for value <25 bpm 3.07, 95% CI: 1.24–7.61, p=0.016) were significant predictors of outcome, the former being the strongest on multivariable analysis. CONCLUSIONS Reduced exercise capacity in patients with Ebsteins anomaly relates to severity of the underlyingdisease and is a strong and independent predictor of outcome. Cardiopulmonary exercise testing should be incorporated in the follow-up and risk stratification of patients with this relatively uncommon and challenging cardiac defect.

Prevalence and prognostic implication of restenosis or dilatation at the aortic coarctation repair site assessed by cardiovascular MRI in adult patients late after coarctation repair

BACKGROUND Cardiovascular magnetic resonance (CMR) is ideal for assessing patients with repaired aortic coarctation (CoA). Little is known on the relation between long-term complications of CoA repair as assessed by CMR and clinical outcome. We examined the prevalence of restenosis and dilatation at the repair site and the long-term outcome in patients with repaired CoA. METHODS AND RESULTS CMR imaging and clinical data for adult CoA patients (247 patients aged 33.0 ± 12.8 years, 60% male), were analyzed. The diameter of the aorta at the repair site was measured on CMR and its ratio to the aortic diameter at the diaphragm (repair site-diaphragm ratio, RDR) was calculated. Restenosis (RDR≤70%) was present in 31% of patients (and significant in 9% [RDR<50%]), and dilatation (RDR>150%) in 13.0%. A discrete aneurysm at the repair site was observed in 9%. Restenosis was more likely after resection and end-end anastomosis, whereas dilatation after patch repair. Systemic hypertension was present in 69% of patients. Of the hypertensive patients, blood pressure (133 ± 20/73 ± 10 mm Hg) was well controlled in 93% with antihypertensive therapy. Mortality rate over a median length of 5.9 years was low (0.69% per year, 95% CI: 0.33-1.26), but significantly higher than age-matched healthy controls (standardised mortality ratio 2.86, CI 1.43-5.72, p<0.001). CONCLUSION Restenosis or dilatation at the CoA repair site as assessed by CMR is not uncommon. Medium term survival remains good, however, albeit lower than in the general population. Life-long follow-up and optimal blood pressure control are likely to secure a good longer term outlook in these patients.

Detrimental impact of socioeconomic status on exercise capacity in adults with congenital heart disease.

OBJECTIVES To evaluate the relationship between socioeconomic status (SES), access to physical activity resources, urban-rural dwelling, levels of pollution and exercise capacity in adult congenital heart disease (ACHD) patients. BACKGROUND Exercise intolerance is prevalent in ACHD and the contributing factors are poorly understood. METHODS A total of 1268 ACHD patients living in England who underwent cardiopulmonary exercise testing at our center were included. Neighborhood deprivation (English Indices of Deprivation), urban-rural dwelling, availability of green space, distance to the closest gym/fitness center and levels of pollution were estimated based on administrative data. RESULTS Urban-rural dwelling, availability of green space and levels of pollution were unrelated to exercise capacity. Lower SES was associated with a significantly lower peak oxygen consumption (P<0.002) and heart rate reserve (P<0.004). This association was non-linear and most pronounced in ACHD patients with cardiac defects of medium complexity living in the most socioeconomically disadvantaged communities. Low SES was associated with higher prevalence of diabetes (P=0.015) and smoking (P=0.01). Coronary artery disease was rare in this young population and low SES was found to be related to exercise capacity independently of the presence of coronary artery disease. CONCLUSIONS Living in poorer areas is associated with exercise intolerance in contemporary ACHD patients. Although low SES is linked to traditional cardiovascular risk factors, the deleterious effects of SES on exercise capacity seem to be only partially mediated via coronary artery disease. Reducing social inequalities in ACHD patients may have a positive effect on quality of life and long-term prognostic implications.

Body mass index in adult congenital heart disease

Objective Abnormal body mass index (BMI) is associated with higher mortality in various cardiovascular cohorts. The prognostic implications of BMI in adults with congenital heart disease (ACHD) are unknown. We aim to assess the distribution of BMI and its association with symptoms and survival in the ACHD population. Methods We included 3069 ACHD patients (median age 32.6 years) under follow-up at our institution between 2001 and 2015. Patients were classified based on BMI as underweight (<18.5), normal weight (18.5–25), overweight (25–30) or obese (>30), and symptoms, exercise capacity and mortality were assessed. Results Overall, 6.2% of patients were underweight, 51.1% had normal weight, 28.2% were overweight and 14.6% were obese. Higher BMI values were associated with lower all-cause and cardiac mortality on univariable Cox analysis, and this effect persisted after adjustment for age, defect complexity, cyanosis and objective exercise capacity. Higher BMI was especially associated with better prognosis in symptomatic ACHD patients (HR 0.94 (95% CI 0.90 to 0.98), p=0.002) and those with complex underlying cardiac defects (HR 0.96 (95% CI 0.91 to 0.997), p=0.048) In patients with a complex cardiac defect who had repeated weight measurements, weight loss was also associated with a worse survival (HR 1.82 (95% CI 1.02 to 3.24), p=0.04). Conclusions ACHD patients with a higher BMI had a lower mortality. The association between BMI and mortality was especially pronounced in symptomatic patients with complex underlying cardiac defects, suggesting that cardiac cachexia may play a role. Indeed, weight loss in complex ACHD patients was linked to an even higher mortality.

Prognostic significance of midwall fibrosis in dilated cardiomyopathy

Methods Consecutive patients with DCM referred for CMR between 2002-2008 were prospectively enrolled. The diagnosis of DCM was made using clinical, CMR and coronary angiographic findings. Patients with ischemic heart disease, primary valvar disease and infiltrative CM were excluded. LGE-CMR at 1.5T (Siemens Sonata or Avanto, Germany) was performed in 2 phase-encoding directions. The presence of midwall LGE was determined by a specialist blinded to all outcome data. The primary endpoint was all-cause mortality. The secondary endpoint was a composite of cardiovascular (CV) mortality or cardiac transplantation.

The relation between significant stenosis or dilatation at the repair site and outcome in a contemporary cohort of patients with repaired aortic coarctation as assessed using Cardiovascular Magnetic Resonance.

Summary Complications of restenosis or dilatation at the site of repair may occur late after initial repair of coarctation of the aorta (CoA). Cardiovascular magnetic resonance (CMR) is becoming the choice of imaging of patients after CoA. Using CMR, we studied the relation between these complications and outcome inn patients with previous CoA repair, and found that significant stenosis or dilatation at the repair site is a predictor of adverse outcome. Background Cardiovascular magnetic resonance (CMR) is becoming the method of choice for assessing repaired CoA patients, especially for identifying important long-term sequelae of restenosis or dilatation at the repair site. We examined the prevalence of restenosis and dilatation at the site of aortic coarctation (CoA) repair and their relation to long-term outcome in patients with repaired CoA.