Pediatrics, Chakri Naruebodindra Medical Institute, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Samut Prakan, Bang phli, Thailand.
Pediatrics, Chakri Naruebodindra Medical Institute, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Samut Prakan, Bang phli, Thailand
BMJ Case Rep. 2024 Jun 12;17(6):e259272. doi: 10.1136/bcr-2023-259272.
Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation. After birth, he developed severe respiratory failure, congestive heart failure and airway obstruction because an enlarged left atrium from severe mitral regurgitation compressed the distal left main bronchus. There is limited experience in surgical management of this condition in Thailand, and the patient's mitral valve was too small for replacement. Therefore, he was treated with medication to control heart failure and supported with positive pressure ventilation to promote growth. We have followed the patient until the current time of writing this report at the age of 2 years, and his outcome is favourable regarding heart failure symptoms, airway obstruction, growth and development. This case describes a challenging experience in the non-surgical management of MA with severe regurgitation, which presented at birth.
先天性二尖瓣环异常（MA）是一种罕见的先天畸形。我们报告了一例 MA 病例，该病例发生在一名新生儿中，其表现为严重二尖瓣反流导致的胎儿水肿。出生后，他因严重二尖瓣反流导致的左心房增大压迫远端左主支气管而出现严重呼吸衰竭、充血性心力衰竭和气道阻塞。在泰国，这种情况下的手术治疗经验有限，并且患者的二尖瓣太小，无法进行置换。因此，他接受了药物治疗以控制心力衰竭，并使用正压通气来促进生长。我们一直随访该患者，直至撰写本报告时，他已经 2 岁了，其心力衰竭症状、气道阻塞、生长发育情况均良好。本例描述了一种在出生时即出现严重反流的 MA 的非手术治疗具有挑战性的经验。
