A Case of Neonatal Marfan Syndrome Requiring Double Atrioventricular Valve Replacement due to Rapid Exacerbation of the Repaired Valves

Yuki OGATA; Yoshiya SHIGEHISA; Yushi YAMASHITA; Tomoyuki MATSUBA; Kenji TOYOKAWA; Hideaki KANDA; Keisuke KAWAIDA; Shinya KURAMOTO; Yoshiharu SOGA; Yutaka IMOTO

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A Case of Neonatal Marfan Syndrome Requiring Double Atrioventricular Valve Replacement due to Rapid Exacerbation of the Repaired Valves

VernacularTitle:両房室弁形成後の逆流急性増悪のために2弁置換を行った新生児Marfan症候群の1例
Author: Yuki OGATA ¹ ; Yoshiya SHIGEHISA ² ; Yushi YAMASHITA ² ; Tomoyuki MATSUBA ¹ ; Kenji TOYOKAWA ² ; Hideaki KANDA ² ; Keisuke KAWAIDA ² ; Shinya KURAMOTO ² ; Yoshiharu SOGA ² ; Yutaka IMOTO ³
Author Information

1. Cardiovascular Surgery, Kagoshima City Hospital
2. Cardiovascular Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
3. Cardiovascular Surgery, Fujimoto General Hospital
Keywords: neonatal Marfan syndrome; mitral valve regurgitation; mitral and tricuspid valvuloplasty; ECMO; double atrioventricular valve replacement
From:Japanese Journal of Cardiovascular Surgery 2023;52(2):77-82
CountryJapan
Language:Japanese
Abstract: Neonatal Marfan syndrome is the most severe form of Marfan syndrome usually showing critical cardio-respiratory symptoms from the neonatal period or early infancy. We report a boy with this syndrome who presented with heart failure at 3 months of age and was referred to our department at 6 months old after intense medical treatment. He had enophthalmos, funnel chest, arachnodactyly, and Steinberg's thumb sign, but had no family history of Marfan syndrome or other cardiac diseases. Left ventricular dilatation, severe mitral regurgitation and moderate tricuspid regurgitation were noted on echocardiography. Mitral valvuloplasty and tricuspid annuloplasty were performed, and the regurgitation improved to trivial and mild level, respectively. However, rapid exacerbation of mitral regurgitation occurred, and the patient fell into circulatory collapse which needed circulatory support with extracorporeal membrane oxygenator (ECMO) on 18th postoperative day. In the emergency operation, the previous surgical procedures on the mitral valve were intact and we thought that rapid progression of the mitral annular dilatation and valve expansion to be the cause of exacerbation. Mitral valve replacement (Regent® 21 mm aortic) was performed, and the cardiac function improved, but ECMO was still needed because of the depressed respiratory function. Furthermore, tricuspid regurgitation due to annular dilatation and valve expansion was aggravated rapidly which needed tricuspid valve replacement (ATS® 20 mm mitral) 9 days after the mitral valve replacement. ECMO was ceased on the 37th day and the patient was extubated on 71st day. He was discharged from the hospital 5 months after the first operation. One year has passed after discharge, and he is doing well with anticoagulation. In the treatment of neonatal Marfan syndrome, surgical procedure for valve repair is still controversial and it should be remembered that rapid exacerbation of the atrioventricular valve can occur even after satisfactory valve repair and there should be no hesitation regarding surgical intervention when needed.