Amorim-Figueiredo Rosa, Pereira Lemos Ana, Rito Tiago, Conde Marta, Brito Maria João, Pinto Fátima
Pediatric Infectious Diseases Unit, Dona Estefânia Hospital, Unidade Local de Saúde São José, Academic Clinical Centre of Lisbon, 1169-045 Lisbon, Portugal.
Department of Pediatric Cardiology and Reference Center for Congenital Heart Diseases, Santa Marta Hospital, Unidade Local de Saúde São José, Academic Clinical Centre of Lisbon, 1169-024 Lisbon, Portugal.
J Cardiovasc Dev Dis. 2024 May 14;11(5):149. doi: 10.3390/jcdd11050149.
Kawasaki disease (KD) is a type of vasculitis in which giant coronary artery aneurysms (CAAs) can occur. There are no specific guidelines for managing giant CAAs that develop quickly and are at risk of rupture. Regarding cardiovascular drugs, only beta-blockers are formally recommended in the acute phase of KD.
A 6-month-old male patient with multiresistant Kawasaki disease and giant CAAs that continued to enlarge after controlling systemic inflammation was examined. The patient required three doses of intravenous immunoglobulin, methylprednisolone pulses, and anakinra and infliximab to normalize systemic inflammation. Due to the rapid increment of aneurysms' dimensions and the risk of rupture, we introduced anticoagulant therapy and propranolol plus captopril, and titration doses were introduced according to a tolerated decrease in heart rate and arterial pressure. CAAs increment stabilized and slowly reduced their dimensions.
The authors describe an atypical case of multiresistant KD with giant rapidly increasing CAAs even after controlling systemic inflammation. The introduction of a beta-blocker and an angiotensin-converting enzyme (ACE) inhibitor was demonstrated to be useful for stabilizing giant CAAs growth and reducing the potential risk of rupture.
川崎病(KD)是一种可发生巨大冠状动脉瘤(CAA)的血管炎。对于迅速发展且有破裂风险的巨大CAA,目前尚无具体的管理指南。关于心血管药物,在KD急性期仅正式推荐使用β受体阻滞剂。
对一名6个月大的男性多耐药川崎病患者进行了检查,其患有巨大CAA,在控制全身炎症后仍持续增大。该患者需要三剂静脉注射免疫球蛋白、甲泼尼龙冲击治疗以及阿那白滞素和英夫利昔单抗来使全身炎症恢复正常。由于动脉瘤尺寸迅速增加且有破裂风险,我们采用了抗凝治疗以及普萘洛尔加卡托普利,并根据心率和动脉压的耐受下降情况调整剂量。CAA的增大得到稳定,并缓慢缩小尺寸。
作者描述了一例多耐药KD的非典型病例,即使在控制全身炎症后,巨大CAA仍迅速增大。事实证明,使用β受体阻滞剂和血管紧张素转换酶(ACE)抑制剂有助于稳定巨大CAA的生长并降低潜在的破裂风险。