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 48 - Hurler Syndrome
from Section 7 - Miscellaneous Lesions and Syndromes
Published online by Cambridge University Press: 09 September 2021
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
Summary
The mucopolysaccharidoses are a diverse group of lysosomal storage diseases that present multiple challenges to the anesthesiologist. Key features include difficult mask ventilation, difficult intubation, cardiac disease, coronary ischemia, obstructive sleep apnea, and skeletal abnormalities. Careful and thorough preoperative evaluation is crucial for anesthetic planning and safe management. It should emphasize airway management along with the cardiovascular, pulmonary, and neurologic systems. Cardiac valvulopathies and ventricular dysfunction are common and must be managed appropriately. Postoperative consideration should be given to caring for these patients in a high-acuity environment depending on the type of surgery and the severity of the underlying disease.
Keywords
- Type
- Chapter
- Information
- Congenital Cardiac AnesthesiaA Case-based Approach, pp. 367 - 373Publisher: Cambridge University PressPrint publication year: 2021
References
Suggested Reading
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