Hypoplastic Left Heart Syndrome Current Considerations and Expectations

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients

Society for Cardiovascular Magnetic Resonance/European Society of Cardiovascular Imaging/American Society of Echocardiography/Society for Pediatric Radiology/North American Society for Cardiovascular Imaging Guidelines for the Use of Cardiac Magnetic Resonance in Pediatric Congenital and Acquired Heart Disease: Endorsed by The American Heart Association

Cardiovascular magnetic resonance has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of cardiovascular magnetic resonance in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking. This guidelines document outlines the use of cardiovascular magnetic resonance in this patient population for a significant number of heart lesions in this age group and although admittedly, is not an exhaustive treatment, it does deal with an expansive list of many common clinical issues encountered in daily practice

Heart and large vessel interaction in congenital heart disease, assessed by magnetic resonance imaging

The main objective of the current thesis is to assess aortic wall elasticity and aortic dimensions and their impact on aortic valve competence and LV function in patients with a bicuspid aortic valve, transposition of the great arteries, tetralogy of Fallot and patients after the Ross procedure, with the use of MRI. In addition, MRI was also used to test whether a similar interaction is present between pulmonary artery dynamics and the right ventricle in the above mentioned entities. Chapter 2 reviews the 5 most common entities of inherited connective tissue disorders and classical congenital heart disease with intrinsic aortic wall abnormalities, with description of the potential role of MRI in their evaluation and management. Chapter 3 describes in vivo validation, as well as testing of reproducibility of aortic pulse wave velocity as assessed with MRI, as indicator of aortic elasticity. Chapter 4 studies aortic elasticity, aortic valve competence and LV function in non-stenotic bicuspid aortic valve patients. Chapters 5 and 6 describe aortic elasticity, aortic valve competence and LV function, and the results of pulmonary flow dynamics in relationship with right ventricular function in patients after the arterial switch operation, respectively. Chapter 7 addresses aortic dimensions and elasticity in conjunction with aortic valve and LV function in patients with repaired tetralogy of Fallot after pulmonary valve replacement. Chapter 8 describes the outcome of patients who have previously undergone the Ross procedure in our institution for a dysfunctioning aortic valve. Chapter 9 studies aortic elasticity, aortic valve competence and LV function in patients after the Ross procedure. Finally, Chapter 10 describes the results of pulmonary flow dynamics in relationship with right ventricular function in patients after the Ross procedure. In Chapter 11 all studies presented in this thesis are summarized and future directions are discussed.Nederlandse Hartstichting, Astra Zeneca B.V., Bayer B.V., Philips Medical Systems, Boehringer-Ingelheim B.V., J.E. Jurriaanse Stichting, Guerbet Nederland B.V., Laboratorium voor Klinische en Experimentele Beeldverwerking (LKEB)UBL - phd migration 201

Image-based computational hemodynamics of distal aortic arch recoarctation following the Norwood procedure

Currently, few applications of computational hemodynamics predict outcomes after palliative surgery for congenital heart defects such as hypolastic left heart syndrome in infants. Here, we report on three-dimensional imaging methods for modeling recoarctation that commonly occurs in the distal aortic arch following the Norwood procedure. We used computer-aided design to simulate seven stages of increasing stenosis, analyze the resulting hemodynamics, and visualize the impairment of blood flow. A disproportionate drop in pressure occurred when stenosis reached 20% of the cross-sectional area of the descending aorta. The corresponding decrease in blood flow in the descending aorta was relatively small, however. Accordingly our findings suggest a reasonable approach to this phenomenon is watchful waiting rather than immediate surgical intervention. Such patient-specific predictions appear to be valuable for minimizing the risks of corrective surgery. These methods also may be applied to procedures for all eviating other congenital heart defects.6 page(s