Table 3. Continued Variable Description CLINICAL STATEMENTS AND GUIDELINES Table 4. ACHD AP Classification (CHD Anatomy + Physiological Stage = ACHD AP Classification) CHD Anatomy* I: Simple Native disease Isolated small ASD Isolated small VSD Mild isolated pulmonic stenosis Repaired conditions Previously ligated or occluded ductus arteriosus Repaired secundum ASD or sinus venosus defect without significant residual shunt or chamber enlargement Repaired VSD without significant residual shunt or chamber enlargement II: Moderate Complexity Repaired or unrepaired conditions Aorto-left ventricular fistula Anomalous pulmonary venous connection, partial or total Anomalous coronary artery arising from the pulmonary artery Anomalous aortic origin of a coronary artery from the opposite sinus AVSD (partial or complete, including primum ASD) Congenital aortic valve disease Congenital mitral valve disease Coarctation of the aorta Ebstein anomaly (disease spectrum includes mild, moderate, and severe variations) Infundibular right ventricular outflow obstruction Ostium primum ASD Moderate and large unrepaired secundum ASD Moderate and large persistently patent ductus arteriosus Pulmonary valve regurgitation (moderate or greater) Pulmonary valve stenosis (moderate or greater) Peripheral pulmonary stenosis Sinus of Valsalva fistula/aneurysm Sinus venosus defect Subvalvar aortic stenosis (excluding HCM; HCM not addressed in these guidelines) Supravalvar aortic stenosis Straddling atrioventricular valve Repaired tetralogy of Fallot VSD with associated abnormality and/or moderate or greater shunt III: Great Complexity (or Complex) Cyanotic congenital heart defect (unrepaired or palliated, all forms) Double-outlet ventricle Fontan procedure Interrupted aortic arch Mitral atresia Single ventricle (including double inlet left ventricle, tricuspid atresia, hypoplastic left heart, any other anatomic abnormality with a functionally single ventricle) (Continued) III: Great Complexity (or Complex) (Continued) Pulmonary atresia (all forms) TGA (classic or d-TGA; CCTGA or l-TGA) Truncus arteriosus Other abnormalities of atrioventricular and ventriculoarterial connection (ie, crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion) Physiological Stage A NYHA FC I symptoms No hemodynamic or anatomic sequelae No arrhythmias Normal exercise capacity Normal renal/hepatic/pulmonary function B NYHA FC II symptoms Mild hemodynamic sequelae (mild aortic enlargement, mild ventricular enlargement, mild ventricular dysfunction) Mild valvular disease Trivial or small shunt (not hemodynamically significant) Arrhythmia not requiring treatment Abnormal objective cardiac limitation to exercise C NYHA FC III symptoms Significant (moderate or greater) valvular disease; moderate or greater ventricular dysfunction (systemic, pulmonic, or both) Moderate aortic enlargement Venous or arterial stenosis Mild or moderate hypoxemia/cyanosis Hemodynamically significant shunt Arrhythmias controlled with treatment Pulmonary hypertension (less than severe) End-organ dysfunction responsive to therapy D NYHA FC IV symptoms Severe aortic enlargement Arrhythmias refractory to treatment Severe hypoxemia (almost always associated with cyanosis) Severe pulmonary hypertension Eisenmenger syndrome Refractory end-organ dysfunction *This list is not meant to be comprehensive; other conditions may be important in individual patients. 