Biomaterial Database

[Combined aortic root replacement and pectus excavatum correction in Marfan's syndrome]. 2010

M Shiraishi, and A Yamaguchi, and A Tamura, and K Naito, and H Adachi

Department of Cardiovascular Surgery, Saitama Medical Center, Jichi Medical School, Saitama, Japan.

A 53-year-old man with Marfan's syndrome was admitted for repair of annulo-aortic ectasia (58 mm). He had also severe pectus excavatum. The skin was incised along the sternal midline. The pectoral muscles were detached laterally. After the perichondrium and costal cartilages were resected bilaterally. the left-sided intercostal muscles and perichondrial sheaths were divided 3 cm lateral to the sternum. To place the retractor in parasternal position, excellent exposure of the heart and aortic root was enabled. The aortic root was replaced with a Carboseal graft. Chest wall reconstructions was completed by modified Ravitch procedure with Gore-tex sheet The patient was discharged after an uneventful recovery on postoperative day 14.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008382	Marfan Syndrome	An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE; AORTIC ANEURYSM; and AORTIC DISSECTION. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2.	Marfan Like Connective Tissue Disorder,Marfan Syndrome Type 1,Marfan Syndrome Type 2,Marfan Syndrome, Type II,Marfan Syndrome, Type I,Marfan's Syndrome,Marfans Syndrome
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D010369	Pectoralis Muscles	The pectoralis major and pectoralis minor muscles that make up the upper and fore part of the chest in front of the AXILLA.	Pectoralis Major,Pectoralis Major Muscle,Pectoralis Minor,Pectoralis Minor Muscle,Pectoral Muscle,Muscle, Pectoral,Muscle, Pectoralis,Muscle, Pectoralis Major,Muscle, Pectoralis Minor,Muscles, Pectoralis Major,Pectoral Muscles,Pectoralis Major Muscles,Pectoralis Majors,Pectoralis Minor Muscles,Pectoralis Minors,Pectoralis Muscle
D005660	Funnel Chest	A developmental anomaly in which the lower sternum is posteriorly dislocated and concavely deformed, resulting in a funnel-shaped thorax.	Pectus Excavatum,Chest, Funnel,Chests, Funnel,Excavatum, Pectus,Funnel Chests
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001011	Aorta	The main trunk of the systemic arteries.	Aortas