Acute Rupture of Chordae Tendineae of the Mitral Valve in Infants -A Nationwide Survey in Japan Exploring a New Syndrome

2014 Fiscal Year Final Research Report

• Project/Area Number: 24591594
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Pediatrics
• Research Institution: National Cardiovascular Center Research Institute
• Principal Investigator: SHIRAISHI Isao 独立行政法人国立循環器病研究センター, 病院, 部長 (80295659)
• Co-Investigator(Renkei-kenkyūsha): IKEDA Yoshihiko 国立研究開発法人, 国立循環器病研究センター病理部, 医長 (30393242)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Keywords: 臨床医学 / 小児科 / 循環器 / 急性心不全 / 僧帽弁閉鎖不全

Outline of Final Research Achievements

Ninety-five children with rupture of mitral chordae tendineae were identified in nationwide surveys of Japan diagnosed from 1995 to 2013. Eighty-one (85%) patients were between 4 and 6 months of age. The underlying conditions included Kawasaki disease (10 cases), and maternally derived anti-SSA antibodies (2 cases). Surgery was performed in 80 patients (94 operations), and the final operations included plasty of mitral chordae in 52 cases and mechanical valve replacement in 26 cases. The histopathologic examinations (n=28) revealed inflammatory reactions with predominant mononuclear cell infiltration in 18 cases (64%). Eight patients (8.4%) died and significant neurological complications persisted in 10 cases (11%).
Acute heart failure attributable to rupture of the mitral chordae tendineae in infants should be recognized as a significant cardiovascular disorder that may cause sudden onset of cardiogenic shock and death in infants.

Free Research Field

小児科学