부산대학교 도서관

Background: Patients with apical hypertrophic cardiomyopathy (ApHCM) have particularities that distinguish it from the most common hypertrophic cardiomyopathy phenotype, regarding both the clinical profiles and genetics. The relationship between myocardial fibrosis and ventricular arrhythmias in these patients is unclear. Our aim was to evaluate, in asymptomatic or mildly symptomatic western patients with ApHCM, whether there is a relationship of late gadolinium enhancement (LGE) CMR, proposed as a surrogate marker of discrete myocardial scarring, with ventricular tachycardia episodes and with major adverse cardiac events (MACE).Methods: We included prospectively a population of 48 Caucasian patients with ApHCM, consecutively enrolled. Patients underwent baseline clinical evaluation, 24-hour Holter and CMR study at 3.0-T. CMR assessment included: SSFP cine imaging for LV volumes, ejection fraction and mass and left atrial volume; LGE was obtained 10-15 mn after 0.2 mmol/kg of gadolinium chelate (gadobutrol) and LGE mass and LGE% (total LGE mass/LV mass ratio) were quantified. Patients were followed yearly for major adverse cardiac events (MACE).Results: Ten patients (20.8%) had episodes of non-sustained ventricular tachycardia (NSVT) in the Holter monitoring and 31 (64,6%) had LGE in CMR. During follow-up (29 ± 9 months), eight (16.7%) patients with MACE were identified. We found an association of NSVT with LV end-diastolic volume (OR:1.059;95% CI:1.019-1.100), end-diastolic volume index (OR:1.085;95% CI:1.002-1.174), left atrium volume (OR:1.079;95% CI:1.017-1.142), LGE mass (OR:1.763;95% CI:1.215-2.558) and LGE extent (LGE%) (OR:2.404;95% CI:1.310-4.414). A cut-off value of 6.5% allowed LGE% alone to predict NSVT presence with a sensitivity of 80%, a specificity of 91.6%, and a AUC of 87.1% (95% CI:76.6%-97.6%) on ROC analysis. After adjustment to EDVI, for each additional unit of LGE%, the risk of NSVT increased by 4-fold. Patients with MACE had larger LGE% than the ones without MACE (p = 0.03).Conclusion: In our study of a population of patients with ApHCM, the amount of late gadolinium-enhancement CMR was independently associated with NSVT and was associated with MACE.