Book contents
- Congenital Cardiac Anesthesia
- Congenital Cardiac Anesthesia
- Copyright page
- Dedication
- Contents
- Contributors
- Introduction
- Chapter 1 A Congenital Heart Disease Primer
- Section 1 Left-to-Right Shunts
- Section 2 Right-Sided Obstructive Lesions
- Section 3 Left-Sided Obstructive Lesions
- Section 4 Complex Mixing Lesions
- Section 5 Single-Ventricle Physiology
- Section 6 Heart Failure, Mechanical Circulatory Support, and Transplantation
- Section 7 Miscellaneous Lesions and Syndromes
- Chapter 40 Idiopathic Pulmonary Hypertension
- Chapter 41 Pulmonary Hypertension and Congenital Heart Disease
- Chapter 42 Pulmonary Hypertension and Prematurity
- Chapter 43 Pulmonary Hypertension and Moyamoya Disease
- Chapter 44 Vascular Ring
- Chapter 45 Pericardial Effusion
- Chapter 46 Kawasaki Disease
- Chapter 47 VACTERL Syndrome
- Chapter 48 Hurler Syndrome
- Chapter 49 Long QT Syndrome
- Chapter 50 Marfan Syndrome
- Index
- References
Chapter 50 - Marfan Syndrome
from Section 7 - Miscellaneous Lesions and Syndromes
Published online by Cambridge University Press: 09 September 2021
- Congenital Cardiac Anesthesia
- Congenital Cardiac Anesthesia
- Copyright page
- Dedication
- Contents
- Contributors
- Introduction
- Chapter 1 A Congenital Heart Disease Primer
- Section 1 Left-to-Right Shunts
- Section 2 Right-Sided Obstructive Lesions
- Section 3 Left-Sided Obstructive Lesions
- Section 4 Complex Mixing Lesions
- Section 5 Single-Ventricle Physiology
- Section 6 Heart Failure, Mechanical Circulatory Support, and Transplantation
- Section 7 Miscellaneous Lesions and Syndromes
- Chapter 40 Idiopathic Pulmonary Hypertension
- Chapter 41 Pulmonary Hypertension and Congenital Heart Disease
- Chapter 42 Pulmonary Hypertension and Prematurity
- Chapter 43 Pulmonary Hypertension and Moyamoya Disease
- Chapter 44 Vascular Ring
- Chapter 45 Pericardial Effusion
- Chapter 46 Kawasaki Disease
- Chapter 47 VACTERL Syndrome
- Chapter 48 Hurler Syndrome
- Chapter 49 Long QT Syndrome
- Chapter 50 Marfan Syndrome
- Index
- References
Summary
Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder caused by FBN1 gene mutations on chromosome 15, resulting in defective fibrillin-1 matrix glycoproteins manifesting as tissue abnormalities. Aberrant protein expressions increase and worsen with age and are most notoriously manifested in the musculoskeletal, cardiovascular, and ophthalmic systems. Cardinal clinical features include aortic root dilatation and ectopia lentis. Other clinical manifestations may include pectus excavatum or carinatum, scoliosis, dural ectasia, pulmonary involvement (emphysema, lung cysts, spontaneous pneumothorax), retrognathia, malar hypoplasia, and joint abnormalities. Aortic root dilatation is seen in approximately 50% of young children with MFS, with the risk of aortic rupture increasing during the teenage years. The Nuss procedure, a minimally invasive approach involving the placement of a retrosternal bar to correct the depressed anterior chest wall, has become the technique of choice for correction of pectus excavatum deformity. This chapter describes the anesthetic and perioperative management of a patient with MFS undergoing a Nuss procedure for correction of a pectus deformity.
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- Congenital Cardiac AnesthesiaA Case-based Approach, pp. 386 - 392Publisher: Cambridge University PressPrint publication year: 2021