Critical Congenital Heart Defect (CHD) Outcomes in Children

Patients, age newborn to 21 years, followed in the High Risk Congenital Heart Defect Clinic at Children's Healthcare of Atlanta at Egleston will be asked to participate in this trial. The term "high risk" is assigned to a patient with the following diagnoses or medical problems: critical aortic stenosis, truncus arteriosus (with or without interrupted aortic arch), patients with complex single ventricle physiology (such as hypoplastic left heart syndrome, unbalanced AV canal, tricuspid atresia, transposition of the great vessels with ventricular septal defect and pulmonary stenosis, mitral atresia, pulmonary atresia with intact ventricular septum), left ventricular outflow tract obstruction requiring Konno procedure, transposition of the great vessels (with or without ventricular septal defect), pulmonary atresia with ventricular septal defect, interrupted aortic arch (with or without ventricular septal defect), anatomy requiring "double switch" procedure, aortic arch abnormality requiring transverse arch graft, total anomalous pulmonary venous return, anatomy requiring any of the following procedures: Damas-Kaye-Stansel procedure, ventricular septal defect enlargement, or resection of subpulmonic stenosis during arterial switch procedure, atrial septectomy, neonatal repositioning of the tricuspid valve for Ebstein anomaly, Ross procedure in infancy; mitral stenosis with mitral valve replacement in infancy, and any other cardiac abnormality requiring surgical repair in infancy with residual hemodynamically significant lesions. In addition, patients with infantile Marfan syndrome, or kawasaki disease with coronary artery aneurysm will also be included.

Informed consent will be obtained from the parent or legally authorized representative for all study participants. An Assent, either verbal or written, will also be obtained if the child is over the age of six (6). A sequential and unique subject number (i.e. NES.001, NES.002) will be assigned by the investigator or designee to each qualified subject after informed consent has been obtained. Once a subject number is assigned, it will not be used again by the investigator. Subjects who are asked to participate but refuse participation in the study will not be assigned a study number.

Investigational Plan:

A single-center, retrospective registry design will be utilized. After consent, the following data will be collected from each study participant's chart each time he or she attends clinic:

• Name
• Contact information (or confirm contact information)
• Names of other care providers
• Primary Diagnosis
• Secondary Diagnoses
• Age at time of visit
• Vital signs including blood pressure, heart rate, respirations, pulse oximetry
• Weight at time of visit
• If applicable, echocardiography data including m-mode, tissue doppler and color flow Doppler
• History and Physical including surgical history
• Medications
• Nutritional Status
• Growth Parameters
• If applicable, most recent standard of care laboratories
• If applicable, MRI results
• EKG findings

All procedures are standard of care. The collection of data for this registry will not effect the care provided to each patient. The individual physician has the right to diagnose and treat each patient according to standard of care procedures and his/her own medical judgement.

Participation in this registry will last as long as the patient is being followed in the high risk clinic at Children's Healthcare of Atlanta.