Mukhtar Gousia, Sasidharan Bijulal, Krishnamoorthy Kavassery Mahadevan, Kurup Harikrishnan K N, Gopalakrishnan Arun, Sasikumar Deepa, Sarma Sankara, Valaparambil Ajit Kumar, Sivasubramonian Sivasankaran
Department of Cardiology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Achutha Menon Centre for Health Science Studies, Thiruvananthapuram, Kerala, India.
Ann Pediatr Cardiol. 2023 May-Jun;16(3):175-181. doi: 10.4103/apc.apc_149_22. Epub 2023 Sep 8.
Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome.
We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5-9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up ( = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias ( value- 0.05). The 5-year survival rate of DCM patients was 59%.
A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts.
扩张型心肌病（DCM）是儿童心力衰竭（HF）的重要病因。儿科DCM的研究仍然极为匮乏。本研究的主要目的是评估儿科DCM的临床特征和预后，次要目的是研究预后的预测因素。
我们纳入了1990年至2020年间就诊于我院且年龄小于18岁的所有心肌病患者。在30年的研究期间，我们共识别出233例儿科心肌病患者。其中119例（51%）为DCM。对这个回顾性队列进行分析，以研究其预后及可能的预后预测因素。近8%在新生儿期发病，37%在婴儿期发病。最常见的表现形式是劳力性呼吸困难（71%）。93例患者以心力衰竭形式就诊（78%）。舒张期左心室尺寸z评分的中位数为4.3（范围2.5 - 9.06）。左心室（LV）射血分数的中位数为31%。该队列中72%的患者使用血管紧张素转换酶抑制剂，40%使用醛固酮拮抗剂，47%使用β受体阻滞剂。三分之一的患者有综合征、代谢、遗传或任何继发性病因。27例患者符合可能的急性心肌炎诊断的三级临床分类。平均随访3.29年期间，27%的患者失访。在其余接受随访的患者（n = 86）中，39例（45%）死亡，31例（36%）康复，16例（18%）存在持续性左心室功能障碍。心力衰竭是最常见的死亡原因。该队列中有8例患者（4.2%）出现血栓栓塞现象。9例有持续性室性心律失常，6例有房性/交界性心律失常。在研究的各种危险因素中，只有婴儿期发病与死亡或室性心律失常有显著相关性（P值 = 0.05）。DCM患者的5年生存率为59%。
我们研究人群中有相当比例（36%）的患者左心室功能恢复。只有婴儿期发病与死亡或室性心律失常有显著相关性。我们DCM队列的预后与其他人群队列相似。
