Actively Enrolling Cohorts
- Male or Female, aged 0-30 years old at time of diagnosis
- Diagnosis and/or management of AAOCA at a CHSS member institution from January 1, 1998
- Surgical repair performed from January 21, 2009 (only applicable for surgically managed AAOCA cases) with a complete surgical note
- Structurally normal heart or with small, hemodynamically insignificant lesion, including: patent ductus arteriosus, atrial septal defect, ventricular septal defect, mild pulmonic valvar stenosis, or bicuspid aortic valve without aortic stenosis
- Anomalous coronary from the pulmonary artery, coronary artery atresia, or other coronary artery anomalies (e.g., coronary-cameral fistula, coronary aneurysms, myocardial bridging)
- Hemodynamically significant structural heart disease, except as outlined above
- Diagnosis of or referral with complete AVSD at a CHSS institution
- Admitted to a CHSS institution for surgery after January 1, 2012
- Age ≤ 365 days at admission for surgery
- Atrioventricular and Ventriculoarterial concordance (includes Tetralogy of Fallot and Double Outlet Right Ventricle)
- Partial or Transitional AVSD (Separate AV valve orifices, restrictive VSD or intact ventricular septum)
- Total or Partial Anomalous Pulmonary Venous Drainage (TAPVC or PAPVC)
- Aortic Atresia
- First intervention at a non-CHSS institution
- Diagnosis of Ebstein Anomaly with medical or surgical treatment
- Diagnosis made on or after January 1, 2014
- Age ≤ 21 years at diagnosis
- Treatment for Ebstein Anomaly at a CHSS member institution
- Subjects with concomitant anomalies including atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and pulmonary atresia will be included
- English, Spanish or French speakers
- Atrioventricular-ventriculoarterial (AV-VA) discordance
- Hypertrophic cardiomyopathy
- Partial Anomalous Pulmonary Venous Connection
- Atrioventricular septal defect
- Diagnosis of Tricuspid Atresia with normally related great arteries
- Age < 3 months at time of diagnosis
- First intervention at a CHSS institution
- Patients with Atrioventricular (AV) or Ventriculoarterial (VA) Discordance
- First intervention at non-CHSS institution
- Age > 3 months at time of diagnosis
Enrollment on Hold
- Age > 30 days at admission to a CHSS institution
- Date of admission AFTER December 31, 2004
- AV & VA concordance whose LVOTO precludes an adequate systemic cardiac output through the aortic valve (include Critical LVOTO due to either aortic valve stenosis OR anatomically normal but hypoplastic left heart)
NOTE: Patients with a VSD will be included
- First intervention at a non-CHSS institution
- AV or VA discordance
- Atrioventricular Septal Defect
- Valved Conduit implant at age <2years at member institution.
- Date of first implant AFTER January 1, 2002 Note: Patients who have 1.5 ventricle repair (e.g. CCTGA) are included
- Survival to hospital discharge after conduit insertion
- First pulmonary ventricle-pulmonary artery conduit placement
- Informed consent from parents/guardian
- Single Ventricle RV-PA conduit, e.g. Norwood RV-PA conduit
- Non-Valved Conduit
- VSD Fenestrated or not closed
From 2002 to 2014, 632 neonates with PC were enrolled prospectively from 29 institutions.
AVA & AVS
- Neonates younger than 30 days at the time of admission
- First procedure at a CHSS member institution
- Concordant atrioventricular and ventriculoarterial connections and either Aortic Valve Atresia (AVA) or critical Aortic Valve Stenosis (AVS).
- AVA is defined as the absence of blood flow across the aortic valve, as determined by color Doppler echocardiography.
- AVS is critically important left ventricular outflow tract obstruction or hypoplasia, with demonstrated patency of the aortic valve and either moderately or severely reduced left ventricular function at admission or presence of duct-dependent systemic perfusion.
- First procedure at a non-CHSS institution
- Age >30 days at the time of admission
- Atrioventricular or ventriculoarterial discordance
- Any AVA or AVS that does not meet the morphologic criteria
From 1994 to 2000, 566 neonates with AVA were enrolled prospectively from 26 institutions, and 421 neonates with AVS were enrolled prospectively from 28 institutions.
- To determine outcomes for neonates with coarctation of the aorta.
- To analyze the importance of different treatment strategies.
From 1990 to 1993, 893 neonates with CoA were enrolled prospectively from 32 institutions.
Note: There is no follow-up phase being conducted for this cohort.
PAIVS & PSIVS
- To determine the proportion of neonates reaching defined end-states (i.e. Bi-ventricular Repair, Fontan, Heart Transplant, One-and-a-half ventricle repair or death).
- To identify risk factors associated with end-state, including Bi-ventricular Repair Fontan, Heart Transplant, One-and-a-half ventricle repair or death).
From January 1987 to April 1997, 408 neonates with PAIVS admitted to a CHSS institution within 30 days after birth were prospectively enrolled in a multi-institutional study.
PAIVS was defined as no communication between the right ventricle (RV) and pulmonary trunk and absence of ventricular septal defect (VSD) as determined by echocardiographic, catheterization, or surgical findings.
- To determine outcomes and associated patient and management factors for neonates with pulmonary stenosis with intact ventricular septum.
- To observe the transition from surgical to percutaneous management.
From 1987 to 1997, 448 neonates with PAIVS or PSIVS were enrolled prospectively from 33 institutions.
- To determine outcomes for neonates with interruption of the aortic arch (IAA).
- To determine what patient management variables improve outcomes.
From 1987 to 1997, 470 neonates with IAA were enrolled prospectively from 33 institutions.
- To assemble a multi-institutional inception cohort of neonates with Transposition of the Great Arteries.
- To compare survival outcomes of neonates age 2 weeks presenting with various forms of TGA.
From 1985 to 1989, 890 neonates with TGA were enrolled from 24 institutions.