A case of mitral valve replacement and left atrial plication for infantile Marfan syndrome with giant left atrium due to mitral valve regurgitation

• Published in: Kyobu geka. The Japanese journal of thoracic surgery Vol. 52; no. 7; p. 579
• Main Authors: Kamikubo, Y, Murashita, T, Yamauchi, H, Yasuda, K, Matano, J, Myojin, K
• Format: Journal Article
• Language: Japanese
• Published: Japan 01.07.1999
• Subjects: Child, Preschool; Heart Atria - pathology; Heart Atria - surgery; Heart Valve Prosthesis Implantation; Humans; Male; Marfan Syndrome - complications; Mitral Valve Insufficiency - pathology; Mitral Valve Insufficiency - surgery; Respiratory Insufficiency - etiology
• ISSN: 0021-5252

In Marfan syndrome, there is a subset, called infantile Marfan syndrome, in which the disease is diagnosed during infancy and cardiac lesions including mitral regurgitation and aortic root dilatation tend to be deteriorated rapidly. In infantile Marfan syndrome, respiratory function is sometimes impaired when skeletal abnormalities such as scoliosis and pectus excavatum are severe. In this report, we describe a 2-year and 4-months old boy with infantile Marfan syndrome who presented with severe mitral regurgitation and the collapsed left lung. In addition to the impairment of respiratory function due to severe scoliosis, the left lung was collapsed because of the compression of the left bronchus by the enlarged left atrium. The patient required mitral valve replacement concomitantly with left atrial plication, resulting in the decompression of the left bronchus and the re-expansion of the left lung. Characteristics and surgical management of infantile Marfan syndrome are discussed in this report.