Major Recommendations (DONOT EDIT)
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The American College of Cardiology/American Heart Association (ACC/AHA) classification of the recommendations for patient evaluation and treatment (classes I-III) and the levels of evidence (A-C) are defined at the end of the "Major Recommendations" field.
Clinical Features and Evaluation of the Unoperated Patient
Recommendations for Cardiac Catheterization
Class I
- Cardiac catheterization to assess the operability of adults with ventricular septal defect (VSD) and pulmonary arterial hypertension (PAH) should be performed in an adult congenital heart disease (ACHD) regional center in collaboration with experts. (Level of Evidence: C)
Class IIa
- Cardiac catheterization can be useful for adults with VSD in whom noninvasive data are inconclusive and further information is needed for management. Data to be obtained include the following:
- Quantification of shunting. (Level of Evidence: B)
- Assessment of pulmonary pressure and resistance in patients with suspected PAH. Reversibility of PAH should be tested with various vasodilators. (Level of Evidence: B)
- Evaluation of other lesions such as aortic regurgitation (AR) and double-chambered right ventricle. (Level of Evidence: C)
- Determination of whether multiple VSDs are present before surgery. (Level of Evidence: C)
- Performance of coronary arteriography is indicated in patients at risk for coronary artery disease. (Level of Evidence: C)
- VSD anatomy, especially if device closure is contemplated. (Level of Evidence: C)
Management Strategies
Recommendation for Medical Therapy
Class IIB
- Pulmonary vasodilator therapy may be considered for adults with VSDs with progressive/severe pulmonary vascular disease (refer to the National Guideline Clearinghouse [NGC] summary of the ACC/AHA guideline, Pulmonary Hypertension/Eisenmenger Physiology). (Level of Evidence: B)
Recommendations for Surgical Ventricular Septal Defect Closure
Class I
- Surgeons with training and expertise in congenital heart disease (CHD) should perform VSD closure operations. (Level of Evidence: C)
- 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 left ventricular (LV) volume overload. (Level of Evidence: B)
- Closure of a VSD is indicated when the patient has a history of infective endocarditis (IE). (Level of Evidence: C)
Class IIa
- Closure of a VSD is reasonable when net left-to-right shunting is present at a pulmonary blood flow/systemic blood flow (Qp/Qs) greater than 1.5 with pulmonary artery pressure less than two thirds of systemic pressure and PVR less than two thirds of systemic vascular resistance. (Level of Evidence: B)
- 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. (Level of Evidence: B)
Class III
- VSD closure is not recommended in patients with severe irreversible PAH. (Level of Evidence: B)
Recommendation for Interventional Catheterization
Class IIb
- 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. (Level of Evidence: C)
Key Issues to Evaluate and Follow-Up
Recommendations for Surgical and Catheter Intervention Follow-Up
Class I
- Adults with VSD with residual heart failure, shunts, PAH, aortic regurgitation (AR), or right ventricular outflow tract (RVOT) or left ventricular outflow tract (LVOT) obstruction should be seen at least annually at an ACHD regional center. (Level of Evidence: C)
- Adults with a small residual VSD and no other lesions should be seen every 3 to 5 years at an ACHD regional center. (Level of Evidence: C)
- Adults with device closure of a VSD should be followed up every 1 to 2 years at an ACHD center depending on the location of the VSD and other factors. (Level of Evidence: C)
Recommendation for Reproduction
Class III
- Pregnancy in patients with VSD and severe PAH (Eisenmenger syndrome) is not recommended owing to excessive maternal and fetal mortality and should be strongly discouraged. (Level of Evidence: A)
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