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Non-ischemic left ventricular scar: an emerging cause of ventricular arrhythmias and sudden death in the young and athlete
Full text linkBACKGROUND: The clinical relevance of isolated non-ischemic left-ventricular (LV) scar as a cause of ventricular arrhythmias and sudden death in the young and athlete remains to be established. Contrast-enhanced cardiac magnetic resonance (CE-CMR) is increasingly used in the clinical work-up of athletes with apparently idiopathic ventricular arrhythmias (VA) and can reveal LV scar in the form of late gadolinium enhancement (LGE).
AIMS: we aimed to: 1) evaluate the incidence and causes (with particular reference to non-ischemic LV scar) of out-of-hospital cardiac arrest (OHCA) in people 1-40 year-old in the Padua province in the modern era; 2) assess the clinical profile and outcome of athletes referred to our Institution for non-ischemic LV scar as suggested by LGE at CE-CMR, which was performed for evaluation of apparently idiopathic VA and/or ECG abnormalities; 3) evaluate whether a strategy consisting of 24-hours 12-leads ambulatory ECG monitoring as first-line investigation and CE-CMR as second line investigation may improve our ability to identify concealed LV scar in apparently healthy athletes.
METHODS: we performed three different studies: 1) we recorded all cases of OHCA which occurred during the period 2011-2015. The study population included all residents in the province of Padua 1-40 year-old who suffered OHCA, either resuscitated or not, of presumed cardiovascular origin. The cause of OHCA was ascertained clinically in survivors and at postmortem investigation in victims of sudden death; 2) we compared the clinical profile and outcome of 35 athletes with VA and isolated non-ischemic (subepicardial/midmyocardial) LGE on CE-CMR suggesting myocardial scar with 38 athletes with VA and no LGE and 40 healthy control athletes; 3) we offered a 12-leads 24-hours ambulatory ECG monitoring to apparently healthy athletes ≥16 year-old, who performed at least 6 hours of physical exercise per week and who have been considered eligible at preparticipation screening within 1 year. Athletes were selected to undergo CE-CMR if they showed >29 premature ventricular beats (PVBs) of “uncommon morphology” (i.e. excluding those with a morphology suggestive of right ventricular outflow tract or fascicular origin) or repetitive VA (excluding isolated couplets with a morphology suggestive of right ventricular outflow tract origin).
RESULTS: the main results for each study were: 1) the incidence of OHCA in the Padua province was 3.5/100.000 residents/year and was significantly lower among screened athletes than among non-athletes (1.1/100.000/year vs. 3.9/100.000/year, p<0,001). A final diagnosis was reached in 40 (83%) subjects while in 8 (17%) victims of sudden death the autopsy was not performed. The most frequent diagnosis were coronary artery atherosclerosis (23%) and structurally normal heart (23%) while cardiomyopathies accounted for 10% of cases. The most frequent cardiomyopathy (3 of 7 cases) was the non-ischemic LV scar; 2) a “stria” LGE pattern with subepicardial/midmyocardial distribution, mostly involving the lateral LV wall, was found in 27 (77%) of athletes with VA versus 0 controls (p<0.001). All athletes with “stria” LGE pattern showed VA with a predominant right-bundle-branch-block morphology (suggesting LV origin) but only 5/27 (19%) showed hypokinesis of the lateral LV wall at echocardiography. During a follow-up of 38±25 months, 6/27 (22%) athletes with a “stria” LGE pattern experienced major arrhythmic events (including 1 sudden death) compared with none of athletes with no or LGE-“spotty” pattern; 3) PVBs were rare in apparently healthy athletes (median 1/day) and their number and complexity significantly correlated with age. The prevalence of frequent or repetitive PVBs with “uncommon” morphology was 28/384 (7.3%) apparently healthy athletes. In this group, 3/28 (11%) showed non-ischemic LV scar at CE-CMR.
CONCLUSIONS: the non-ischemic LV scar with a “stria” pattern may be associated with life-threatening VA and sudden death in the young athlete and cannot be simply dismissed as a sign of a previous healed myocarditis. Because of its subepicardial/midmyocardial location, the LV scar is often undetectable by echocardiography, and athletes with PVBs with right-bundle-branch-block morphology, particularly if exercise-induced or associated with ECG abnormalities, should undergo CE-CMR to exclude an underlying pathological myocardial substrate
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