Ponsiglione Andrea, Puglia Marta, Morisco Carmine, Barbuto Luigi, Rapacciuolo Antonio, Santoro Mario, Spinelli Letizia, Trimarco Bruno, Cuocolo Alberto, Imbriaco Massimo
Department of Advanced Biomedical Sciences, University "Federico II", Via Pansini, 5, 80123, Naples, Italy.
BMC Cardiovasc Disord. 2016 Nov 21;16(1):230. doi: 10.1186/s12872-016-0412-2.
Arrhythmogenic right ventricular dysplasia (ARVD), is a genetic disorder of the heart, which mainly involves the right ventricle. It is characterized by hypokinetic areas at the free wall of the right ventricle (RV) or both ventricles, where myocardium is replaced by fibrous or fatty tissue. ARVD is an important cause of ventricular arrhythmias in children and young adults. Although the transmission of the disease is based on hereditary, in young adults it may not show any symptoms. The main differential diagnoses with other frequent etiological causes of sudden arrhythmia are: idiopathic outflow tract ventricular tachycardia of the RV, myocarditis, dilated cardiomyopathy and sarcoidosis.
We describe an unusual case of a 44-year-old woman who was hospitalized for ventricular tachycardia, deep asthenia and dyspnoea with no previous history of cardiac disease. The patient had a ten-year history of palpitations, which started immediately after her last pregnancy. She was diagnosed with both acute/subacute viral myocarditis and arrhythmogenic right ventricular dysplasia, based on established clinical and cardiac MRI criteria. After the diagnosis the patient received an automatic implantable cardioverter defibrillator. Currently, she is on clinical follow-up with no apparent further complications.
Analyzing this rare case, we have shown the link between myocarditis and arrhythmogenic right ventricular dysplasia, and how important is to perform a cardiac MRI, in the context of acute myocarditis and ventricular arrhythmia.
致心律失常性右心室发育不良(ARVD)是一种心脏遗传性疾病,主要累及右心室。其特征是右心室(RV)游离壁或双心室出现运动减弱区域,此处心肌被纤维或脂肪组织替代。ARVD是儿童和年轻人室性心律失常的重要原因。尽管该病的传播基于遗传,但在年轻人中可能不表现出任何症状。与其他常见的突然心律失常病因的主要鉴别诊断包括:右室特发性流出道室性心动过速、心肌炎、扩张型心肌病和结节病。
我们描述了一例不寻常的病例,一名44岁女性因室性心动过速、严重乏力和呼吸困难入院,既往无心脏病史。患者有十年心悸病史,自上次怀孕后立即开始。根据既定的临床和心脏MRI标准,她被诊断为急性/亚急性病毒性心肌炎和致心律失常性右心室发育不良。诊断后,患者接受了植入式自动心脏复律除颤器。目前,她正在接受临床随访,没有明显的进一步并发症。
通过分析这一罕见病例,我们展示了心肌炎与致心律失常性右心室发育不良之间的联系,以及在急性心肌炎和室性心律失常的情况下进行心脏MRI检查的重要性。