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Diastolic dysfunction is common and contributes to many clinical manifestations in hypertrophic cardiomyopathy (HCM). 4-dimensionnal (4D) flow cardiac magnetic resonance (CMR) sequence can assess left ventricular mitral filling. The purpose of this study was to determine if the peak early filling rate normalized to the filling volume (PEFR/FV), estimated from 4D-flow sequence can accurately assess impaired left ventricular mitral filling and predict clinical outcomes. This monocentric study included 44 HCM patients from a prospective HCM register and 44 healthy volunteers, who all underwent CMR with 4D-Flow sequence. Median follow-up time of HCM cohort patients was 2.3 years (interquartile range: 1.7 to 3.3 years). The predefined primary endpoint was a composite criteria that included syncope, onset of atrial fibrillation, admission for congestive heart failure with administration of diuretics, decision to perform septal reduction therapy due to persistent symptomatic obstruction, stroke, ventricular tachycardia or fibrillation and cardiac death. PEFR/FV was significantly lower in HCM cohort compared to healthy volunteers (P < 0.001). PFR/FV significantly correlated with BNP (r = −0.31, P < 0.001) and with E/E' ratio (r = −0.56, p < 0.001). PEFR/FV according to NYHA status in HCM cohort was statistically different (P = 0.001). Primary endpoint occurred in 14 patients (32%). In univariate and bivariate adjusted cox-model analysis PEFR/FV < 2.61 s -1 was significantly associated with primary endpoint with Hazard Ratio ranging from [HR = 9.46 (95% CI 2.61–45.17, P < 0.001) to 15.21 (95%CI 3.51–80.22, P < 0.001)] according to succesive bivariate models. 4D-Flow can accurately assess left ventricular mitral filling with a good predictive value of clinical outcomes in HCM patients. Relation of PEFR/FV calculated with 4D flow in HCM. [ABSTRACT FROM AUTHOR]

Hypertrophic cardiomyopathy (HCM) is considered to be the first cause of sudden death in young subjects, including competitive athletes, often in the absence of significant symptoms. However, most of these data come from tertiary centers and the clinical profile of these patients, as well as their management in real life in France, remain poorly understood. To evaluate the management of young adults with sarcomeric HCM and the predictive factors for major cardiac event (MCE), patients < 25 years included in the national HCM register "REMY" of the French Society of Cardiology from 2006 to 2018 were identified. At inclusion was calculated the European 5-year sudden cardiac death (SCD) score and identified the presence of the 5 major classical SCD risk factors (MRF). At follow-up, MCE [SCD or implantable cardioverter defibrillator (ICD) shock, sustained ventricular tachycardia (SVT), atrial fibrillation (AF), stroke, hospitalization for heart failure (HFH) or syncope] were notified. Among 61 included patients [20.5 ± 3(16–25) years, 16(26%) women], 92% were in NHYA class I/II, 18(30%) obstructive, 37(86%) showed MRI fibrosis, 6(12%) abnormal exercise blood pressure response, 25% a family history of SCD, 13(21%) had a prophylactic ICD. The score (4.8 ± 3.2%) was < 4% in 53% and ≥ 6% in 25% of patients. After 4.4 ± 2.2years, 15(25%) patients (7 women) presented 18 MCE: 3 SCD (17%), 5 SVT (28%), 5 AF and 1 stroke (33%), 1 HFH (5%), 3 unexplained syncopal episodes (17%); 10 patients received an ICD (4 in secondary prevention). 61% patients with score> 6% had an ICD. Female sex was predictive of MCE (P =.02), with a tendency for obstruction (P =.09), but not late gadolinium enhancement, prophylactic ICD or MRF. Thus, in young adults with HCM, MCE are common, especially in women, more often rhythmic. Prophylactic ICD is frequent, especially if the score exceeds 6%, but MCE are difficult to predict using the current criteria. [ABSTRACT FROM AUTHOR]