The document provides recommendations for various interventional and surgical procedures for different types of congenital heart defects. It recommends when closure of atrial septal defects (ASDs), ventricular septal defects (VSDs), and patent ductus arteriosus (PDA) should be performed either percutaneously or surgically based on factors like hemodynamics, symptoms, and anatomy. It also provides indications for aortic valve repair/replacement and interventions for lesions affecting the left ventricular outflow tract and pulmonary valve. Recommendations are made based on the severity of obstruction/regurgitation, symptoms, and technical factors.

1. Recommendations for Interventional and Surgical Therapy

2. Atrial Septal Defect Closures Closure of an ASD either percutaneously or surgically is indicated for right atrial and RV enlargement with or without symptoms. A sinus venosus, coronary sinus, or primum ASD should be repaired surgically rather than by percutaneous closure. Surgeons with training and expertise in CHD should perform operations for various ASD closures.

4. Atrial Septal Defect Closures Closure of an ASD, either percutaneously or surgically, may be considered in the presence of net left-to-right shunting, pulmonary artery pressure less than two thirds systemic levels, PVR less than two thirds systemic vascular resistance, or when responsive to either pulmonary vasodilator therapy or test occlusion of the defect (patients should be treated in conjunction with providers who have expertise in the management of pulmonary hypertensive syndromes). Concomitant Maze procedure may be considered for intermittent or chronic atrial tachyarrhythmias in adults with ASDs.

5. Atrial Septal Defect Closures Patients with severe irreversible PAH and no evidence of a left-to-right shunt should not undergo ASD closure. I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III

6. Recommendations for Surgical Ventricular Septal Defect Closure

7. Surgical Ventricular Septal Defect Closure Surgeons with training and expertise in CHD should perform VSD closure operations. Closure of a VSD is indicated when there is a Qp/Qs (pulmonary-to-systemic blood flow ratio) of 2.0 or more and clinical evidence of LV volume overload. Closure of a VSD is indicated when the patient has a history of IE. VSD Closure Operations

8. Surgical Ventricular Septal Defect Closure Closure of a VSD is reasonable when net left-to-right shunting is present at a Qp/Qs greater than 1.5 with pulmonary artery pressure less than two thirds of systemic pressure and pulse volume recording (PVR) less than two thirds of systemic vascular resistance. Closure of a VSD is reasonable when net left-to-right shunting is present at a Qp/Qs greater than 1.5 in the presence of LV systolic or diastolic failure. VSD closure is not recommended in patients with severe irreversible PAH. VSD Closure Operations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III

9. Interventional Catheterization for VSD Device closure of a muscular VSD may be considered, especially if the VSD is remote from the tricuspid valve and the aorta, if the VSD is associated with severe left-sided heart chamber enlargement, or if there is PAH.

10. Left Ventricular Outflow Tract Obstructed (LVOT) and Associated Lesions

13. Aortic Valve Repair/Replacement and Aortic Root Replacement Surgery to repair or replace the ascending aorta in a patient with a bicuspid aortic valve (BAV) is recommended when the ascending aorta diameter is 5.0 cm or more or when there is progressive dilatation at a rate greater than or equal to 5 mm per year. AVR is reasonable for asymptomatic patients with severe AR and normal systolic function (ejection fraction greater than 50%) but with severe LV dilatation (LV end-diastolic diameter greater than 75 mm or end-systolic dimension greater than 55 mm*). Aortic Valve Repair and Replacement *Consider lower threshold values for patients of small stature of either gender. I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B

14. Aortic Valve Repair/Replacement and Aortic Root Replacement Surgical aortic valve repair or replacement is reasonable in patients with moderate AS undergoing coronary artery bypass grafting or other cardiac or aortic root surgery. Aortic Valve Repair and Replacement I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B

16. Aortic Valve Repair/Replacement and Aortic Root Replacement e) Moderate AR undergoing coronary artery bypass grafting or other cardiac surgery. f) Severe AR with rapidly progressive LV dilation when the degree of LV dilation exceeds an end- diastolic dimension of 70 mm or end-systolic dimension of 50 mm, with declining exercise tolerance, or with abnormal hemodynamic responses to exercise. Aortic Valve Repair and Replacement

18. Aortic Valve Repair/Replacement and Aortic Root Replacement AVR is not useful for prevention of sudden death in asymptomatic adults with AS who have none of the findings listed under the Class IIa/IIb indications. AVR is not indicated in asymptomatic patients with AR who have normal LV size and function. AVR and Asymptomatic Adults With AS I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III

19. Surgical Intervention for SubAS Surgical intervention is recommended for patients with SubAS and a peak instantaneous gradient of 50 mm Hg or a mean gradient of 30 mm Hg on echocardiography-Doppler. Surgical intervention is recommended for SubAS with less than a 50-mm Hg peak or less than a 30-mm Hg mean gradient and progressive AR and an LV dimension at end-systolic diameter of 50 mm or more or LV ejection fraction less than 55%. Surgical resection may be considered in patients with a mean gradient of 30 mm Hg, but careful follow-up is required to detect progression of stenosis or AR. Surgical Intervention and Surgical Resection

21. Interventional and Surgical Therapy for Supravalvular LVOT Operative intervention should be performed for patients with supravalvular LVOT obstruction (discrete or diffuse) with symptoms (i.e., angina, dyspnea, or syncope) and/or mean gradient greater than 50 mm Hg or peak instantaneous gradient by Doppler echocardiography greater than 70 mm Hg. Interventions for coronary artery obstruction in patients with SupraAS should be performed in ACHD centers with demonstrated expertise in the interventional management of such patients. Operative Intervention and Surgical Repair

24. Interventional and Surgical Treatment of Coarctation of the Aorta in Adults Percutaneous catheter intervention is indicated for recurrent, discrete coarctation and a peak-to-peak gradient of at least 20 mm Hg. Surgeons with training and expertise in CHD should perform operations for previously repaired coarctation and the following indications: a) Long recoarctation segment. b) Concomitant hypoplasia of the aortic arch. Percutaneous Catheter Intervention Stent placement for long-segment coarctation may be considered, but the usefulness is not well established, and the long-term efficacy and safety are unknown.

25. Intervention in Patients With Valvular Pulmonary Stenosis Balloon valvotomy is recommended for asymptomatic patients with a domed pulmonary valve and a peak instantaneous Doppler gradient greater than 60 mm Hg or a mean Doppler gradient greater than 40 mm Hg (in association with less than moderate pulmonic valve regurgitation). Balloon valvotomy is recommended for symptomatic patients with a domed pulmonary valve and a peak instantaneous Doppler gradient greater than 50 mm Hg or a mean Doppler gradient greater than 30 mm Hg (in association with less than moderate pulmonic regurgitation). Balloon Valvotomy and Doppler Gradients

26. Intervention in Patients With Valvular Pulmonary Stenosis Surgical therapy is recommended for patients with severe PS and an associated hypoplastic pulmonary annulus, severe pulmonary regurgitation, subvalvular PS, or supravalvular PS. Surgery is also preferred for most dysplastic pulmonary valves and when there is associated severe TR or the need for a surgical Maze procedure. Surgeons with training and expertise in CHD should perform operations for the right ventricular outflow tract (RVOT) and pulmonary valve. Balloon Valvotomy and Doppler Gradients Surgical Therapy, Training, and Expertise

27. Intervention in Patients With Valvular Pulmonary Stenosis Balloon valvotomy may be reasonable in asymptomatic patients with a dysplastic pulmonary valve and a peak instantaneous gradient by Doppler greater than 60 mm Hg or a mean Doppler gradient greater than 40 mm Hg. Balloon valvotomy may be reasonable in selected symptomatic patients with a dysplastic pulmonary valve and peak instantaneous gradient by Doppler greater than 50 mm Hg or a mean Doppler gradient greater than 30 mm Hg. Balloon Valvotomy and Doppler Gradients

28. Intervention in Patients With Valvular Pulmonary Stenosis Balloon valvotomy is not recommended for asymptomatic patients with a peak instantaneous gradient by Doppler less than 50 mm Hg in the presence of normal cardiac output. Balloon valvotomy is not recommended for symptomatic patients with PS and severe pulmonary regurgitation. Balloon valvotomy is not recommended for symptomatic patients with a peak instantaneous gradient by Doppler less than 30 mm Hg. Balloon Valvotomy and Doppler Gradients