We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.
We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.
from Section 2 - Right-Sided Obstructive Lesions
Published online by Cambridge University Press: 09 September 2021
Similar to other variants of tetralogy of Fallot (TOF), TOF with absent pulmonary valve syndrome includes an anterior maligned ventricular septal defect, an aorta that “overrides” the septal defect, and a hypertrophied right ventricle. However, it is distinct in that there is an absent or rudimentary, incompetent pulmonary valve. The pulmonary valve, if present, may have some degree of stenosis but the dominant pathophysiology is pulmonary regurgitation. Patients usually have unobstructed flow to the pulmonary arteries and therefore do not have cyanosis or hypercyanotic spells associated with other tetralogy variants. The main and branch pulmonary arteries are generally dilated due to pulmonary regurgitation and excessive flow and can be large enough to cause a mass effect on surrounding structures, including the airways and lungs. Patients with unrepaired tetralogy with absent pulmonary valve syndrome have respiratory symptoms and cyanosis related to the degree of intracardiac shunting and airway compression. This chapter addresses the perioperative cardiorespiratory challenges of caring for an infant with this syndrome.
To save this book to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.
Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
Find out more about the Kindle Personal Document Service.
To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.
To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.
