Logeswaran T, Akintürk H, Müller M, Rueblinger L, Gummel K, Klingel K, Jux C, Steinbrenner B, Schranz D
Department of Pediatric Cardiology, Intensive Care Medicine and Congenital Heart Disease, Pediatric Heart Center, Justus Liebig University, Giessen, Germany.
Department of Pediatric Cardiac Surgery and Congenital Heart Disease, Pediatric Heart Center, Justus Liebig University, Giessen, Germany.
Front Pediatr. 2025 Jun 6;13:1579212. doi: 10.3389/fped.2025.1579212. eCollection 2025.
The incidence of parvovirus B19 (B19 V)-associated myocarditis progressing to dilated cardiomyopathy (DCM) is on the rise. We hypothesize that a comprehensive treatment regimen enables cardiac regeneration in young patients with life-threatening B19 V myocarditis.
Four patients with clinical and imaging evidence of DCM were referred due to suspected myocarditis. An endomyocardial biopsy (EMB) confirmed the diagnosis. The diastolic dysfunction associated with heart failure and reduced left ventricular ejection fraction (HFrEF) was established invasively. Before surgical pulmonary artery banding (PAB), a transcatheter procedure was performed to create a restrictive atrial defect (rASD).
The drug-treated patients (ages 15-26 months) had a mean LV-EF of 22.5% (20%-25%), a left ventricular end-diastolic diameter (LVEDD) of 49 (45-51) mm (Z-score >5), and elevated LVED pressures (>18 mmHg). EMB revealed B19V-associated acute/subacute or chronic active myocarditis with characteristics of DCM. Drug therapy, including immunoglobulins and creating a rASD, resulted in clinical improvement and enhanced right ventricular function. However, LV enlargement and dysfunction persisted. Four weeks after surgical PAB, all patients showed improvement and were discharged home. The pressure gradient across the PAB ranged from 40 to 45 mmHg, and LVEDD decreased to a mean z-score of +3.5. Within three to six months, LVEDD normalized, and LV-EF increased to a mean of 63% (range: 57%-68%). Clinical and cardiac improvements were sustained over a median follow-up of 7.5 years.
A holistic treatment approach allows functional regeneration in B19 V myocarditis with obvious end-stage DCM. Restrictive ASD creation is required before surgical PAB when HFrEF is associated with a diastolic dysfunction component.
细小病毒B19(B19V)相关心肌炎进展为扩张型心肌病(DCM)的发病率正在上升。我们假设一种综合治疗方案能够使患有危及生命的B19V心肌炎的年轻患者实现心脏再生。
4例有DCM临床和影像学证据的患者因疑似心肌炎前来就诊。心内膜心肌活检(EMB)确诊了诊断。通过侵入性方法确定了与心力衰竭相关的舒张功能障碍和左心室射血分数降低(HFrEF)。在进行外科肺动脉环扎术(PAB)之前,先进行经导管操作以制造限制性房间隔缺损(rASD)。
接受药物治疗的患者(年龄15 - 26个月)平均左心室射血分数(LV - EF)为22.5%(20% - 25%),左心室舒张末期内径(LVEDD)为49(45 - 51)mm(Z值>5),左心室舒张末期压力升高(>18 mmHg)。EMB显示为B19V相关的急性/亚急性或慢性活动性心肌炎,并具有DCM的特征。包括免疫球蛋白和制造rASD在内的药物治疗使临床症状得到改善,并增强了右心室功能。然而,左心室扩大和功能障碍仍然存在。外科PAB术后四周,所有患者均有改善并出院。PAB两端的压力阶差为40至45 mmHg,LVEDD降至平均Z值+3.5。在三到六个月内,LVEDD恢复正常,LV - EF平均增加到63%(范围:57% - 68%)。在中位随访7.5年期间,临床和心脏状况持续改善。
整体治疗方法可使伴有明显终末期DCM的B19V心肌炎实现功能再生。当HFrEF伴有舒张功能障碍成分时,在外科PAB之前需要制造限制性ASD。