tailieunhanh - Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

(BQ) Part 2 book "Echocardiography in pediatric and congenital heart disease from fetus to adult" presents the following contents: Miscellaneous cardiovascular lesions, anomalies of ventricular myocardium, acquired pediatric heart disease, special techniques and topics. | PARTV Miscellaneous Cardiovascular Lesions CHAPTER 27 Hearts with Functionally One Ventricle Stephen P. Sanders Departments of Cardiology Pathology and Cardiac Surgery Boston Children s Hospital Boston MA USA Introduction Hearts with functionally one ventricle comprise only a few percent of congenital heart defects 1 but patients with these hearts utilize a disproportionate amount of healthcare resources because of the complexity of management and the need for repeated interventions and lifelong care 2 . Optimal management depends on early recognition and careful observation and planning. Treatment principles include meticulous protection of the pulmonary vasculature ongoing surveillance for and rapid treatment of systemic outflow obstruction and maintenance of systemic ventricular function by avoiding volume and pressure overload as much as possible. Despite attentive and intelligent management strategies the long-term outlook for these patients is guarded 3 . Palliation of patients with functionally one ventricle is likely to be effective for only two to four decades 4 . Most perhaps all such patients will eventually require an alternative management strategy such as transplantation or mechanical support. Many hearts in this category have only one ventricular sinus or body anatomically as well as functionally 5 6 . The other chamber usually present in the ventricular mass is an infundibulum or outlet chamber. These include double-inlet left ventricle DILV most double-inlet right ventricle DIRV most tricuspid atresia TA and many mitral atresia hearts. Others of this category perhaps most have two ventricular sinuses but one or both are unsuitable to function independently 7 . Most of these are discussed in other chapters and will only be listed here. Although not all cases of these defects have functionally one ventricle many do and must be identified as soon as possible to maximize outcomes. Included are variants of hypoplastic left heart syndrome Chapter 20 .