Gherardo Finocchiaro

Etiology of Sudden Death in Sports: Insights From a United Kingdom Regional Registry

BACKGROUNDnAccurate knowledge of causes of sudden cardiac death (SCD) in athletes and its precipitating factors is necessary to establish preventative strategies.nnnOBJECTIVESnThis study investigated causes of SCD and their association with intensive physical activity in a large cohort of athletes.nnnMETHODSnBetween 1994 and 2014, 357 consecutive cases of athletes who died suddenly (mean 29 ± 11 years of age, 92% males, 76% Caucasian, 69% competitive) were referred to our cardiac pathology center. All subjects underwent detailed post-mortem evaluation, including histological analysis by an expert cardiac pathologist. Clinical information was obtained from referring coroners.nnnRESULTSnSudden arrhythmic death syndrome (SADS) was the most prevalent cause of death (nxa0= 149 [42%]). Myocardial disease was detected in 40% of cases, including idiopathic left ventricular hypertrophy (LVH) and/or fibrosis (nxa0= 59, 16%); arrhythmogenic right ventricular cardiomyopathy (ARVC) (13%); and hypertrophic cardiomyopathy (HCM) (6%). Coronary artery anomalies occurred in 5% of cases. SADS and coronary artery anomalies affected predominantly young athletes (≤ 35 years of age), whereas myocardial disease was more common in older individuals. SCD during intense exertion occurred in 61% of cases; ARVC and left ventricular fibrosis most strongly predicted SCD during exertion.nnnCONCLUSIONSnConditions predisposing to SCD in sports demonstrate a significant age predilection. The strong association of ARVC and left ventricular fibrosis with exercise-induced SCD reinforces the need for early detection andxa0abstinence from intense exercise. However, almost 40% of athletes die at rest, highlighting the need forxa0complementary preventive strategies.

Long-Term Evolution and Prognostic Stratification of Biopsy-Proven Active Myocarditis

Background— Active myocarditis is characterized by large heterogeneity of clinical presentation and evolution. This study describes the characteristics and the long-term evolution of a large sample of patients with biopsy-proven active myocarditis, looking for accessible and valid early predictors of long-term prognosis. Methods and Results— From 1981 to 2009, 82 patients with biopsy-proven active myocarditis were consecutively enrolled and followed-up for 147±107 months. All patients underwent clinical and echocardiographic evaluation at baseline and at 6 months. At this time, improvement/normality of left ventricular ejection fraction (LVEF), defined as a LVEF increase > 20 percentage points or presence of LVEF≥50%, was assessed. At baseline, left ventricular dysfunction (LVEF<50%) and left atrium enlargement were independently associated with long-term heart transplantation–free survival, regardless of the clinical pattern of disease onset. At 6 months, improvement/normality of LVEF was observed in 53% of patients. Persistence of New York Heart Association III to IV classes, left atrium enlargement, and improvement/normality of LVEF at 6 months emerged as independent predictors of long-term outcome. Notably, the short-term reevaluation showed a significant incremental prognostic value in comparison with the baseline evaluation (baseline model versus 6 months model: area under the curve 0.79 versus 0.90, P=0.03). Conclusions— Baseline left ventricular function is a marker for prognosis regardless of the clinical pattern of disease onset, and its reassessment at 6 months appears useful for assessing longer-term outcome.

Cardiopulmonary responses and prognosis in hypertrophic cardiomyopathy: a potential role for comprehensive noninvasive hemodynamic assessment.

OBJECTIVESnThis study sought to discover the key determinants of exercise capacity, maximal oxygen consumption (oxygen uptake [Vo2]), and ventilatory efficiency (ventilation/carbon dioxide output [VE/Vco2] slope) and assess the prognostic potential of metabolic exercise testing in hypertrophic cardiomyopathy (HCM).nnnBACKGROUNDnThe intrinsic mechanisms leading to reduced functional tolerance in HCM are unclear.nnnMETHODSnThe study sample included 156 HCM patients consecutively enrolled from January 1, 2007 to January 1, 2012 with a complete clinical assessment, including rest and stress echocardiography and cardiopulmonary exercise test (CPET) with impedance cardiography. Patients were also followed for the composite outcome of cardiac-related death,xa0heart transplant, and functional deterioration leading to septal reduction therapy (myectomy or septal alcohol ablation).nnnRESULTSnAbnormalities in CPET responses were frequent, with 39% (nxa0= 61) of the sample showing a reduced exercise tolerance (Vo2 maxxa0<80% of predicted) and 19% (nxa0= 30) characterized by impaired ventilatory efficiency (VE/Vco2 slope >34). The variables most strongly associated with exercise capacity (expressed in metabolic equivalents), were peak cardiac index (rxa0= 0.51, pxa0< 0.001), age (rxa0= -0.25, pxa0< 0.01), male sex (rxa0= 0.24, pxa0= 0.02), and indexed right ventricular end-diastolic area (rxa0= 0.31, pxa0= 0.002), resulting in an R(2) of 0.51, pxa0< 0.001. Peak cardiac index was the main predictor of peak Vo2 (rxa0= 0.61, pxa0< 0.001). The variables most strongly related to VE/VCO2 slope were E/E (r = 0.23, p = 0.021) and indexed left atrial volume index (LAVI) (r = 0.34, p = 0.005) (model R(2) = 0.15). The composite endpoint occurred in 21 (13%) patients. In an exploratory analysis, 3xa0variables were independently associated with the composite outcome (mean follow-up 27 ± 11 months): peak Vo2xa0<80% of predicted (hazard ratio: 4.11; 95% confidence interval [CI]: 1.46 to 11.59; pxa0= 0.008), VE/Vco2 slope >34 (hazard ratio: 3.14; 95% CI: 1.26 to 7.87; pxa0= 0.014), and LAVI >40 ml/m(2) (hazard ratio: 3.32; 95% CI: 1.08 toxa010.16; pxa0= 0.036).nnnCONCLUSIONSnIn HCM, peak cardiac index is the main determinant of exercise capacity, but it is not significantly related to ventilatory efficiency. Peak Vo2, ventilatory inefficiency, and LAVI are associated with an increased risk of major events in the short-term follow-up.

The prevalence and significance of a short QT interval in 18 825 low-risk individuals including athletes

Objectives The short QT syndrome is a cardiac channelopathy characterised by accelerated repolarisation which manifests as a short QT interval on the ECG. The definition of a short QT interval is debated, ranging from <390 to ≤320u2005ms, and its clinical significance in healthy young individuals is unknown. We assessed the prevalence and medium-term significance of an isolated short QT interval in a diverse young British population. Methods Between 2005 and 2013, 18u2005825 apparently healthy people aged 14–35u2005years underwent cardiovascular evaluation with history, physical examination and ECG. QT intervals were measured by cardiologists using 4 recommended guidelines (Seattle 2013, Heart Rhythm Society 2013, European Society of Cardiology 2010 and American Heart Association 2009). Results The prevalence of a short QT interval was 0.1% (26 patients, ≤320u2005ms), 0.2% (44 patients, ≤330u2005ms), 7.9% (1478 patients, <380u2005ms), 15.8% (2973 patients, <390u2005ms). Male gender and Afro-Caribbean ethnicity had the strongest association with short QT intervals. Athletes had shorter QT intervals than non-athletes but athletic status did not predict short QT intervals. Individuals with short QT intervals ≤320u2005ms did not report syncope or a sinister family history, and during a follow-up period of 5.3±1.2u2005years, there were no deaths in this group. Conclusions The prevalence of a short QT interval depends on the recommended cut-off value. Even at values ≤320u2005ms, there was an excellent medium-term prognosis among 14 people followed. We conclude that a definition of ≤320u2005ms is realistic to prevent overdiagnosis and excessive investigations.

Prevalence and clinical correlates of right ventricular dysfunction in patients with hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HC) is a disease that mainly affects the left ventricle (LV), however recent studies have suggested that it can also be associated with right ventricular (RV) dysfunction. The objective of this study was to determine the prevalence of RV dysfunction in patients with HC and its relation with LV function and outcome. A total of 324 consecutive patients with HC who received care at Stanford Hospital from 1999 to 2012 were included in the study. A group of 99 prospectively recruited age- and gender-matched healthy volunteers were used as controls. RV function was quantified using the RV fractional area change, tricuspid annular plane systolic excursion (TAPSE), and RV myocardial performance index (RVMPI). Compared with the controls, the patients with HC had a higher RVMPI (0.51 ± 0.18 vs 0.25 ± 0.06, p <0.001) and lower TAPSE (20 ± 3 vs 24 ± 4, p <0.001). RV dysfunction based on an RVMPI >0.4 and TAPSE <16xa0mm was found in 71% and 11% of the HC and control groups, respectively. Worst LV function and greater pulmonary pressures were independent correlates of RV dysfunction. At an average follow-up of 3.7 ± 2.3xa0years, 17 patients had died and 4 had undergone heart transplantation. LV ejection fraction <50% and TAPSE <16xa0mm were independent correlates of outcome (hazard ratio 3.98, 95% confidence interval 1.22 to 13.04, pxa0= 0.02; and hazard ratio 3.66, 95% confidence interval 1.38 to 9.69, pxa0= 0.009, respectively). In conclusion, RV dysfunction based on the RVMPI is common in patients with HC and more frequently observed in patients with LV dysfunction and pulmonary hypertension. RV dysfunction based on the TAPSE was independently associated with an increased likelihood of death or transplantation.

Anterior T-Wave Inversion in Young White Athletes and Nonathletes: Prevalence and Significance.

BACKGROUNDnAnterior T-wave inversion (ATWI) on electrocardiography (ECG) in young white adults raises the possibility of cardiomyopathy, specifically arrhythmogenic right ventricular cardiomyopathy (ARVC). Whereas the 2010 European consensus recommendations for ECG interpretation in young athletes state that ATWI beyond lead V1 warrants further investigation, the prevalence and significance of ATWI have never been reported in a large population of asymptomatic whites.nnnOBJECTIVESnThis study investigated the prevalence and significance of ATWI in a large cohort of young, white adults including athletes.nnnMETHODSnIndividuals 16 to 35 years of age (nxa0= 14,646), including 4,720 females (32%) and 2,958 athletes (20%), were evaluated by using a health questionnaire, physical examination, and 12-lead ECG. ATWI was defined as T-wave inversion inxa0≥2 contiguous anterior leads (V1 to V4).nnnRESULTSnATWI was detected in 338 individuals (2.3%) and was more common in women than in men (4.3% vs. 1.4%, respectively; pxa0< 0.0001) and more common among athletes than in nonathletes (3.5% vs. 2.0%, respectively; pxa0<xa00.0001). T-wave inversion was predominantly confined to leads V1 to V2 (77%). Only 1.2% of women and 0.2% of men exhibited ATWI beyond V2. No one with ATWI fulfilled diagnostic criteria for ARVC after further evaluation. During a mean follow-up of 23.1 ± 12.2 months none of the individuals with ATWI experienced an adverse event.nnnCONCLUSIONSnATWI confined to leads V1 to V2 is a normal variant or physiological phenomenon in asymptomatic white individuals without a relevant family history. ATWI beyond V2 is rare, particularly in men, and may warrant investigation.

Long-term outcomes of septal reduction for obstructive hypertrophic cardiomyopathy.

BACKGROUNDnSurgical myectomy and alcohol septal ablation (ASA) aim to decrease left ventricular outflow tract (LVOT) gradient in hypertrophic cardiomyopathy (HCM). Outcome of myectomy beyond 10 years has rarely been described. We describe 20 years of follow-up of surgical myectomy and 5 years of follow-up for ASA performed for obstructive HCM.nnnMETHODSnWe studied 171 patients who underwent myectomy for symptomatic LVOT obstruction between 1972 and 2006. In addition, we studied 52 patients who underwent ASA for the same indication and who declined surgery. Follow-up of New York Heart Association (NYHA) functional class, echocardiographic data, and vital status were obtained from patient records. Mortality rates were compared with expected mortality rates of age- and sex-matched populations.nnnRESULTSnSurgical myectomy improved NYHA class (2.74±0.65 to 1.54±0.74, p<0.001), reduced resting gradient (67.4±43.4mmHg to 11.2±16.4mmHg, p<0.001), and inducible LVOT gradient (98.1±34.7mmHg to 33.6±34.9mmHg, p<0.001). Similarly, ASA improved functional class (2.99±0.35 to 1.5±0.74, p<0.001), resting gradient (67.1±26.9mmHg to 23.9±29.4mmHg, p<0.001) and provoked gradient (104.4±34.9mmHg to 35.5±38.6mmHg, p<0.001). Survival after myectomy at 5, 10, 15, and 20 years of follow-up was 92.9%, 81.1%, 68.9%, and 47.5%, respectively. Of note, long-term survival after myectomy was lower than for the general population [standardized mortality ratio (SMR)=1.40, p<0.005], but still compared favorably with historical data from non-operated HCM patients. Survival after ASA at 2 and 5 years was 97.8% and 94.7%, respectively. Short-term (5 year) survival after ASA (SMR=0.61, p=0.48) was comparable to that of the general population.nnnCONCLUSIONnLong-term follow-up of septal reduction strategies in obstructive HCM reveals that surgical myectomy and ASA are effective for symptom relief and LVOT gradient reduction and are associated with favorable survival. While overall prognosis for the community HCM population is similar to the general population, the need for surgical myectomy may identify a sub-group with poorer long-term prognosis. We await long-term outcomes of more extensive myectomy approaches adopted in the past 10 years at major institutions.

Cost Implications of Using Different ECG Criteria for Screening Young Athletes in the United Kingdom

BACKGROUNDnHigh false-positive rates and cost of additional investigations are an obstacle to electrocardiographic (ECG) screening of young athletes for cardiac disease. However, ECG screening costs have never been systematically assessed in a large cohort of athletes.nnnOBJECTIVEnThis study investigated the costs of ECG screening in athletes according to the 2010 European Society of Cardiology (ESC) recommendations and the Seattle and refined interpretation criteria.nnnMETHODSnBetween 2011 and 2014, 4,925 previously unscreened athletes aged 14 to 35 years were prospectively evaluated with history, physical examination, and ECG (interpreted with the 2010 ESC recommendations). Athletes with abnormal results underwent secondary investigations, the costs of which were based on U.K. National Health Service Tariffs. The impact on cost after applying the Seattle and refined criteria was evaluated retrospectively.nnnRESULTSnOverall, 1,072 (21.8%) athletes had an abnormal ECG on the basis of 2010 ESC recommendations; 11.2% required echocardiography, 1.7% exercise stress test, 1.2% Holter, 1.2% cardiac magnetic resonance imaging, and 0.4% other tests. The Seattle and refined criteria reduced the number of positive ECGs to 6.0% and 4.3%, respectively. Fifteen (0.3%) athletes were diagnosed with potentially serious cardiac disease using all 3 criteria. The overall cost of de novo screening using 2010 ESC recommendations was

Effect of Sex and Sporting Discipline on LV Adaptation to Exercise

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Myocarditis in Clinical Practice

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