Garg Ankit, Azad Sushil, Kumar Khemendra, Bhatia Mona, Radhakrishnan S
Department of Pediatric Cardiology, Fortis Escorts Heart Institute, New Delhi, India.
Department of Radiodiagnosis, Fortis Escorts Heart Institute, New Delhi, India.
Indian J Radiol Imaging. 2022 Jan 10;31(4):837-843. doi: 10.1055/s-0041-1740541. eCollection 2021 Oct.
Hypocalcemia is a rare reversible cause of dilated cardiomyopathy in pediatric population. Myocarditis is another more frequent cause of cardiomyopathy with overlapping presenting features. Cardiac magnetic resonance imaging (CMRI) is a vital modality capable of tissue characterization for the evaluation of cardiomyopathy. The present study is the first attempt to determine if any specific characteristics on CMR exist in patients with hypocalcemic dilated cardiomyopathy. A retrospective analysis of 10 cases of hypocalcemic dilated cardiomyopathy (August 2012-August 2019), among which CMRI of nine patients were analyzed. Patients were categorized in to three categories; category 1 defined as absence of edema and late gadolinium enhancement (LGE), category 2 having edema only, and category 3 with presence of both edema and LGE. A diagnosis of myocarditis was considered if both edema and LGE were present. The mean age of the cohort was 5.5 ± 3.3 months. The mean ejection fraction of the cohort was 20.5 ± 6.85% that improved significantly to 35.22 ± 9.3% at the time of discharge. Five of nine patients had no edema or LGE (category 1), whereas two patients each were categorized into category 2 and 3. All cases in category 1 had normalized ventricular function on follow-up. One patient in category 2 had normal ejection fraction and one was lost to follow-up. Out of the two patients in category 3, there was one mortality and another was lost to follow-up. Of the six patients at follow-up (19 ± 11.0 months), the mean left ventricle ejection fraction improved to 56.5 ± 6.1%. Hypocalcemic dilated cardiomyopathy has a favorable outcome on rapid initiation of treatment. CMR can be utilized for further prognostication of these patients. Absence of edema and LGE predicts a good outcome, whereas presence of LGE and/or edema either indicates a worse prognosis or an underlying coexistent myocarditis warranting an early myocardial biopsy.
低钙血症是小儿扩张型心肌病罕见的可逆病因。心肌炎是心肌病更常见的病因,二者临床表现有重叠。心脏磁共振成像(CMRI)是评估心肌病时能够进行组织特征分析的重要检查手段。本研究首次尝试确定低钙血症性扩张型心肌病患者的CMRI是否存在任何特定特征。
对10例低钙血症性扩张型心肌病患者(2012年8月至2019年8月)进行回顾性分析,其中9例患者进行了CMRI检查。患者分为三类:1类定义为无水肿和延迟钆增强(LGE);2类仅有水肿;3类既有水肿又有LGE。若同时存在水肿和LGE,则考虑诊断为心肌炎。
该队列患者的平均年龄为5.5±3.3个月。队列的平均射血分数为20.5±6.85%,出院时显著提高至35.22±9.3%。9例患者中有5例无水肿或LGE(1类),2例患者分别归入2类和3类。1类所有病例随访时心室功能均恢复正常。2类中有1例患者射血分数正常,1例失访。3类的2例患者中,1例死亡,另1例失访。在随访的6例患者(19±11.0个月)中,平均左心室射血分数提高到56.5±6.1%。
低钙血症性扩张型心肌病若能迅速开始治疗,预后良好。CMRI可用于这些患者的进一步预后评估。无水肿和LGE预示预后良好,而LGE和/或水肿的存在则表明预后较差或存在潜在的并存心肌炎,需要早期进行心肌活检。