MRI characteristics and clinical value of hypertrophic cardiomyopathy with scar-like late enhancement / 中华放射学杂志

Objective To explore the prevalence,MRI characteristics and clinical evaluation of hypertrophic cardiomyopathy(HCM) patients with infarct-like late enhancement(LGE).Methods HCM patients were diagnosed via cardiac magnetic resonance(CMR) from October,2009 to December,2013.1 411 HCM patients were diagnosed via CMR,465 patients with LGE,of which 24 patients with infarct-like LGE (primarily in the subendocardium).Clinical and MRI data of patients demonstrating infarct-like LGE were retrospectively analyzed.All the HCM patients with LGE were followed up in the clinic or by telephone interview.Major adverse cardiovascular events(MACE) were defined as malignant ventricular arrhythmia events,including sudden cardiac death,ventricular tachycardia/fibrillation,implantable cardioverter defibrillator(ICD) discharge and heart failure events,including death from heart failure,heart transplantation.The correlation between left ventricle ejection fraction and subendocardial LGE volume fraction was evaluated by Pearson correlation analysis.Comparison of subendocardial LGE volume fraction between obstructed and non-obstructed left ventricular outflow tract (LVOT) group was performed using independent sample t test.Prognosis of patients with infarct-like LGE and other LGE patterns was compared using Kaplan-Meier curves.Results The prevalence of infarct-like LGE were 0.05％(24/465) among patients with LGE.Mean left venricle ejection fraction(LVEF) was (56.20±3.60)％ and mean LGE volume fraction was (14.52 ± 12.73)％.According to the American Heart Association (AHA) 17-segment model,infarct-like LGE was most frequent in 14 segment(10 patients),followed by 9,15 (8 patients,respectively) and 3,8,16 (7 patients,respectively) segments,mainly distributed in left ventricular septum and apical portion.EF value was inversely correlated with mean LGE volume fraction(r=-0.85,P＜0.05).Mean follow up time was (4.25t± 1.35) years for all the HCM patients with LGE,and event-free survival rate was lower in HCM patients with infarct-like LGE than patients with other LGE patterns(P＜0.001).Conclusions Infarct-like LGE is a special manifestation in HCM patients,which shows worse prognosis than other LGE patterns.Infarct-like LGE volume fraction is associated with LVEF and LVOT.

Right Ventricular Cardiomyopathy Meeting the Arrhythmogenic Right Ventricular Dysplasia Revised Criteria? Don't Forget Sarcoidosis!

A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.

Miocardiopatía arritmogénica del ventrículo derecho: Breve revisión de la literatura a propósito de un caso / Arrhythmogenic right ventricular cardiomyopathy: Case report and a brief literature review

Hombre de 51 años admitido en el hospital por presentar palpitaciones y mareos de 2 h de evolución. El electrocardiograma demostró taquicardia regular de QRS ancho y frecuencia cardíaca de 250 lpm, con eje superior y morfología de bloqueo completo de rama izquierda sin descompensación hemodinámica. Se administraron dosis de carga y mantenimiento con amiodarona, revirtiendo a ritmo sinusal. El estudio electrofisiológico demostró el origen ventricular de la taquicardia y su inducibilidad. En la angiografía coronaria no se observaron lesiones significativas en los vasos epicárdicos. Se realizó un ecocardiograma Doppler que presentó cavidades con diámetros y función sistólica y diastólica dentro de los parámetros normales. Ante la sospecha de enfermedad estructural miocárdica se llevó a cabo una resonancia magnética cardíaca contrastada con realce tardío que demostró alteración estructural del ventrículo derecho con incremento de la trabeculación e infiltración fibrograsa parietal y deterioro moderado de su función sistólica, y deterioro leve de la función sistólica del ventrículo izquierdo, lo cual permitió realizar el diagnóstico de miocardiopatía arritmogénica del ventrículo derecho por presentar 2 criterios mayores. Se decidió implantar un cardiodesfibrilador automático, para prevenir la muerte súbita. El paciente evolucionó de manera favorable y fue dado de alta.

A 51-year-old man was admitted to this hospital because of palpitations and a feeling of dizziness for a period of 2h. The electrocardiogram revealed a regular wide-QRS complex tachycardia at a rate of 250 beats per minute, with superior axis and left bundle branch block morphology without hemodynamically decompensation, the patient was cardioverted to sinus rhythm after the administration of a loading and maintenance dose of amiodarone. The elechtrophysiological study showed the ventricular origin of the arrhythmia. In order to diagnose the etiology of the ventricular tachycardia we performed a coronary arteriography that showed normal epicardial vessels, thus ruling out coronary disease. Doppler echocardiography revea- led systolic and diastolic functions of both left and right ventricles within normal parameters, and normal diameters as well. A cardiac magnetic resonance with late enhancement was done, showing structural abnormalities of the right ventricle wall with moderate impairment of the ejection fraction, and a mild dysfunction of the left ventricle. The diagnosis of arrhythmogenic right ventricular cardiomyopathy was performed as 2 major Task Force criteria were met. We implanted an automatic cardioverter defibrillator as a prophylactic measure. The patient was discharged without complications.