Long-term management of patients with hypoplastic left heart syndrome: the diagnostic approach at All Children's Hospital.

Improved survival in children with hypoplastic left heart syndrome has created a sub-population of children and young adults who are living with functionally univentricular physiology. Routine surveillance with comprehensive screening for structural cardiac disease, functional cardiac disease, arrhythmias, thromboembolic disease, and associated dysfunction of end organs is important. Future directives will better define the plans of care for routine surveillance in patients with hypoplastic left heart syndrome.

Magnetic resonance angiography detection of vascular aneurysms in patients with kawasaki disease and coronary artery aneurysms.

Kawasaki disease is a systemic panvasculitis that causes coronary artery aneurysms in approximately 15% to 25% of untreated patients. Systemic vascular aneurysms may also occur in medium-sized arteries throughout the body and may lead to increased morbidity and mortality in patients with Kawasaki disease. We report a case of diffuse systemic aneurysm formation in a 2-year-old patient with Kawasaki disease with coronary artery aneurysms. Full-body magnetic resonance angiography (MRA) imaging was utilized both in the acute phase and again prior to cardiac catheterization performed at 6 months from the acute illness. The initial MRA detected aneurysmal dilatation of the common and internal iliac arteries bilaterally in the acute phase. Subsequent MRA performed prior to cardiac catheterization 6 months later demonstrated resolution of the iliac artery lesions. Full-body MRA may be useful in screening for associated systemic vascular aneurysms in patients with Kawasaki disease and associated coronary artery aneurysms.

Myocardial function following cold ischemic storage is improved by cardiac-specific overexpression of A1-adenosine receptors.

Cold ischemic storage of hearts for transplantation is limited to 4-6 h, and therefore the development of strategies to extend preservation time may increase the donor pool of hearts. Overexpression of A1-adenosine receptors (A1AR) can protect hearts from acute ischemic injury, and the purpose of this study was to test the hypothesis that overexpression of A1AR will improve tolerance to longer periods of cold ischemic preservation. Hearts from 18 wild type and 16 transgenic mice with overexpression of A1AR (A1AR Trans) were isolated and perfused, and then subjected to 18 h of preservation in 5 degrees C University of Wisconsin solution followed by 2 h of reperfusion. Left ventricular end diastolic pressure and left ventricular developed pressure were measured as indices of ventricular function. Cell viability was assessed by determination of infarct size and myocardial cell apoptosis. A1AR Trans hearts showed improved function following 18 h of ischemia, as shown by lower end diastolic pressure (p < 0.05) and higher recovery of left ventricular developed pressure (p < 0.05) during reperfusion. A1AR Trans hearts had markedly reduced infarct size (p < 0.05) and decreased apoptosis (p < 0.05). Overexpression of cardiac A1AR imparts cardioprotection during long-term cold ischemic preservation.