Adult Congenital Heart Disease Registry (QuERI)

Inclusion Criteria: Cohort 1 (historic high risk) 1. Male and female adults (≥ 18 years of age) 2. Patients with documented history (at least one year) of an isolated, repaired congenital heart defect such as ASD, VSD, PDA, AVC (AVSD) 3. History of a large defect prior to closure as evidenced by any one of the following: * Size of: ASD \> 2 cm; VSD \> 1 cm; PDA \> 0.6 cm * Shunt 2:1 or greater * Pre-operative PH (PAS \> 40 mmHg) or documented shunt- related heart failure (radiographic evidence) * Pre-operative atrial fibrillation or flutter 4. High risk features (any one the following): * Age \> 40 years * Later surgical repair: i. ≥ 2 years of age for PDA or VSD ii. ≥ 1 year of age for AVC iii. ≥ 10 years of age for ASD * Sinus venosus defect * Primum defect * WHO functional class \> 1 * Atrial fibrillation or flutter 5. Echocardiographic evidence of high risk features. Any one of the following: * Degree of TR that is mild or greater * Right ventricular (RV) systolic dysfunction * Evidence of RV dilatation: Any one of the following: i. Evidence of RV dilation by general, qualitative assessment ii. RV end diastolic diameter \> 3.2 cm on apical 4 chamber view at the tips of the tricuspid valve iii. RV to LV ratio at end-diastole \> 0.6 (RV and LV end diastolic dimensions measured from apical 4 chamber view, 1 cm apical of the respective valve annuli) * Any abnormality in the motion of the inter-ventricular septum 6. Ability and desire to execute the consent for follow up Inclusion Criteria: Cohort 2 (current high risk) 1. Male and female adults (≥ 18 years of age) 2. Patients with documented history (at least one year) of an isolated, repaired congenital heart defect such as ASD, VSD, PDA, AVC (AVSD) 3. Current (within the last 12 months) evidence of 1 or more of the 7 following criteria: * Desaturation on exercise (92% or less) * 6 MWD \<380 m * PFT demonstrating DLC \<70% predicted \& FEV1\>70% predicted * ECG demonstrating i) RAD and ii) RVH or RAE * Physical findings of edema accompanied by elevated JVP and +HJR * CXR evidence of enlarged main and/or hilar pulmonary arterial shadows in association with right ventricular enlargement * Elevated biomarks (BNP or NT-proBNP above upper limit of normal) 4. High risk features (any one of the following:) * Age \> 40 years * Later surgical repair: i. ≥ 2 years of age for PDA or VSD ii. ≥ 1 year of age for AVC iii. ≥ 10 years of age for ASD * Sinus venosus defect * Primum defect * WHO functional class \> 1 * Atrial fibrillation or flutter 5. Echocardiographic evidence of high risk features. Any one of the following: * Degree of TR that is mild or greater * Right ventricular (RV) systolic dysfunction * Evidence of RV dilatation: Any one of the following: i. Evidence of RV dilation by general, qualitative assessment ii. RV end diastolic diameter \> 3.2 cm on apical 4 chamber view at the tips of the tricuspid valve iii. RV to LV ratio at end-diastole \> 0.6 (RV and LV end diastolic dimensions measured from apical 4 chamber view, 1 cm apical of the respective valve annuli) * Any abnormality in the motion of the inter-ventricular septum 6. Ability and desire to execute the consent for follow up Exclusion Criteria: 1. Poor mental function, drug or substance (e.g., alcohol) abuse, or unstable psychiatric illness, which, in the opinion of the investigator, may interfere with optimal participation in the study 2. Diagnosis of PAH (defined as RHC demonstrating mPAP ≥ 25 mm Hg and PCWP ≤ 15 and PVR \> 3 WU or PVR (indexed) \> 4 WU or treatment with PAH specific therapy) after surgical repair and prior to visit 1 3. Prior inclusion in this registry