MANAGEMENT OF CONGENITAL VALVULAR HEART DISEASE IN ADOLESCENTS AND YOUNG ADULTS

EVALUATION OF ASYMPTOMATIC ADOLESCENTS OR YOUNG ADULTS WITH AORTIC STENOSIS
CLASS I
(1) An ECG is recommended yearly in the asymptomatic adolescent or young adult with AS who has a Doppler mean gradient greater than 30 mm Hg or a peak velocity greater than 3.5 m per second (peak gradient greater than 50 mm Hg) and every 2 years if the echocardiographic Doppler mean gradient is less than or equal to 30 mm Hg or the peak velocity is less than or equal to 3.5 m per second (peak gradient less than or equal to 50 mm Hg).

(2) Doppler echocardiography is recommended yearly in the asymptomatic adolescent or young adult with AS who has a Doppler mean gradient greater than 30 mm Hg or a peak velocity greater than 3.5 m per second (peak gradient greater than 50 mm Hg) and every 2 years if the Doppler gradient is less than or equal to 30 mm Hg or the peak jet velocity is less than or equal to 3.5 m per second (peak gradient less than or equal to 50 mm Hg).

(3) Cardiac catheterization for the evaluation of AS is an effective diagnostic tool in the asymptomatic adolescent or young adult when results of Doppler echocardiography are equivocal regarding severity of AS or when there is a discrepancy between clinical and noninvasive findings regarding severity of AS.

(4) Cardiac catheterization is indicated in the adolescent or young adult with AS who has symptoms of angina, syncope, or dyspnea on exertion if the Doppler mean gradient is greater than 30 mm Hg or the peak velocity is greater than 3.5 m per second (peak gradient greater than 50 mm Hg).

(5) Cardiac catheterization is indicated in the asymptomatic adolescent or young adult with AS who develops T-wave inversion at rest over the left precordium if the Doppler mean gradient is greater than 30 mm Hg or the peak velocity is greater than 3.5 m per second (peak gradient greater than 50 mm Hg).

CLASS IIa
(1) Graded exercise testing is a reasonable diagnostic evaluation in the adolescent or young adult with AS who has a Doppler mean gradient greater than 30 mm Hg or a peak velocity greater than 3.5 m per second (peak gradient greater than 50 mm Hg) if the patient is interested in athletic participation, or if the clinical findings and Doppler findings are disparate.

(2) Cardiac catheterization for the evaluation of AS is a reasonable diagnostic tool in the asymptomatic adolescent or young adult who has a Doppler mean gradient greater than 40 mm Hg or a peak velocity greater than 4 m per second (peak gradient greater than 64 mm Hg).

(3) Cardiac catheterization for the evaluation of AS is reasonable in the adolescent or young adult who has a Doppler mean gradient greater than 30 mm Hg or a peak velocity greater than 3.5 m per second (peak gradient greater than 50 mm Hg) if the patient is interested in athletic participation or becoming pregnant, or if the clinical findings and the Doppler echocardiographic findings are disparate.

INDICATIONS FOR AORTIC BALLOON VALVOTOMY IN ADOLESCENTS AND YOUNG ADULTS
CLASS I
(1) Aortic balloon valvotomy is indicated in the adolescent or young adult patient with AS who has symptoms of angina, syncope, or dyspnea on exertion and a catheterization peak LV–to–peak aortic gradient greater than or equal to 50 mm Hg without a heavily calcified valve.

(2) Aortic balloon valvotomy is indicated for the asymptomatic adolescent or young adult patient with AS who has a catheterization peak LV–to–peak aortic gradient greater than 60 mm Hg.

(3) Aortic balloon valvotomy is indicated in the asymptomatic adoles- cent or young adult patient with AS who develops ST or T-wave changes over the left precordium on ECG at rest or with exercise and who has a catheterization peak LV–to–aortic gradient greater than 50 mm Hg.

CLASS IIa
(1) Aortic balloon valvotomy is reasonable in the asymptomatic adolescent or young adult patient with AS when catheterization peak LV–to–peak aortic gradient is greater than 50 mm Hg and the patient wants to play competitive sports or desires to become pregnant.

(2) In the adolescent or young adult patient with AS, aortic balloon valvotomy is probably recommended over valve surgery when balloon valvotomy is possible. Patients should be referred to a center with expertise in balloon valvotomy.

CLASS III
(1) Aortic balloon valvotomy should not be performed when the asymptomatic adolescent or young adult patient with AS has a catheterization peak LV–to–peak aortic gradient less than 40 mm Hg without symptoms or ECG changes.

AORTIC REGURGITATION
CLASS I
(1) An adolescent or young adult with chronic severe AR* with onset of symptoms of angina, syncope, or dyspnea on exertion should receive aortic valve repair or replacement.

(2) Asymptomatic adolescent or young adult patients with chronic severe AR* with LV systolic dysfunction (ejection fraction less than 0.50) on serial studies 1 to 3 months apart should receive aortic valve repair or replacement.

(3) Asymptomatic adolescent or young adult patients with chronic severe AR* with progressive LV enlargement (end-diastolic dimension greater than 4 standard deviations above normal) should receive aortic valve repair or replacement.

(4) Coronary angiography is recommended before AVR in adolescent or young adult patients with AR in whom a pulmonary autograft (Ross operation) is contemplated when the origin of the coronary arteries has not been identified by noninvasive techniques.

CLASS IIb
(1) An asymptomatic adolescent with chronic severe AR* with moderate AS (peak LV–to–peak aortic gradient greater than 40 mm Hg at cardiac catheterization) may be considered for aortic valve repair or replacement.

(2) An asymptomatic adolescent with chronic severe AR* with onset of ST depression or T-wave inversion over the left precordium on ECG at rest may be considered for aortic valve repair or replacement.

MITRAL REGURGITATION
CLASS I
(1) MV surgery is indicated in the symptomatic adolescent or young adult with severe congenital MR* with NYHA functional class III or IV symptoms.

(2) MV surgery is indicated in the asymptomatic adolescent or young adult with severe congenital MR* and LV systolic dysfunction (ejection fraction less than or equal to 0.60).

CLASS IIa
1. MV repair is reasonable in experienced surgical centers in the asymptomatic adolescent or young adult with severe congenital MR* with preserved LV systolic function if the likelihood of successful repair without residual MR is greater than 90%.

CLASS IIb
1. The effectiveness of MV surgery is not well established in asymptomatic adolescent or young adult patients with severe congenital MR* and preserved LV systolic function in whom valve replacement is highly likely.

MITRAL STENOSIS
CLASS I
(1) MV surgery is indicated in adolescent or young adult patients with congenital MS who have symptoms (NYHA functional class III or IV) and mean MV gradient greater than 10 mm Hg on Doppler echocardiography.

CLASS IIa
(1) MV surgery is reasonable in adolescent or young adult patients with congenital MS who have mild symptoms (NYHA functional class II) and mean MV gradient greater than 10 mm Hg on Doppler echocardiography.

(2) MV surgery is reasonable in the asymptomatic adolescent or young adult with congenital MS with pulmonary artery systolic pressure 50 mm Hg or greater and a mean MV gradient greater than or equal to 10 mm Hg.

CLASS IIb
(1) The effectiveness of MV surgery is not well established in the asymptomatic adolescent or young adult with congenital MS and new-onset atrial fibrillation or multiple systemic emboli while receiving adequate anticoagulation.

EVALUATION OF TRICUSPID VALVE DISEASE IN ADOLESCENTS AND YOUNG ADULTS
CLASS I
(1) An ECG is indicated for the initial evaluation of adolescent and young adult patients with TR, and serially every 1 to 3 years, depending on severity.

(2) Chest X-ray is indicated for the initial evaluation of adolescent and young adult patients with TR, and serially every 1 to 3 years, depending on severity.

(3) Doppler echocardiography is indicated for the initial evaluation of adolescent and young adult patients with TR, and serially every 1 to 3 years, depending on severity.

(4) Pulse oximetry at rest and/or during exercise is indicated for the initial evaluation of adolescent and young adult patients with TR if an atrial communication is present, and serially every 1 to 3 years, depending on severity.

CLASS IIa
(1) If there is a symptomatic atrial arrhythmia, an electrophysiology study can be useful for the initial evaluation of adolescent and young adult patients with TR.

(2) Exercise testing is reasonable for the initial evaluation of adolescent and young adult patients with TR, and serially every 1 to 3 years.

CLASS IIb
(1) Holter monitoring may be considered for the initial evaluation of asymptomatic adolescent and young adult patients with TR, and serially every 1 to 3 years.

INDICATIONS FOR INTERVENTION IN TRICUSPID REGURGITATION
CLASS I
(1) Surgery for severe TR is recommended for adolescent and young adult patients with deteriorating exercise capacity (NYHA functional class III or IV).

(2) Surgery for severe TR is recommended for adolescent and young adult patients with progressive cyanosis and arterial saturation less than 80% at rest or with exercise.

(3) Interventional catheterization closure of the atrial communication is recommended for the adolescent or young adult with TR who is hypoxemic at rest and with exercise intolerance due to increasing hypoxemia with exercise, when the tricuspid valve appears difficult to repair surgically.

CLASS IIa
(1) Surgery for severe TR is reasonable in adolescent and young adult patients with NYHA functional class II symptoms if the valve appears to be repairable.

(2) Surgery for severe TR is reasonable in adolescent and young adult patients with atrial fibrillation.

CLASS IIb
(1) Surgery for severe TR may be considered in asymptomatic adolescent and young adult patients with increasing heart size and a cardiothoracic ratio of more than 65%.

(2) Surgery for severe TR may be considered in asymptomatic adolescent and young adult patients with stable heart size and an arterial saturation of less than 85% when the tricuspid valve appears repairable.

(3) In adolescent and young adult patients with TR who are mildly cyanotic at rest but who become very hypoxemic with exercise, closure of the atrial communication by interventional catheterization may be considered when the valve does not appear amenable to repair.

(4) If surgery for Ebstein’s anomaly is planned in adolescents and young adult patients (tricuspid valve repair or replacement), a preoperative electrophysiological study may be considered to identify accessory pathways. If present, these may be considered for mapping and ablation either preoperatively or at the time of surgery.

EVALUATION OF PULMONIC STENOSIS IN ADOLESCENTS AND YOUNG ADULTS
CLASS I
(1) An ECG is recommended for the initial evaluation of pulmonic stenosis in adolescent and young adult patients, and serially every 5 to 10 years for follow-up examinations.

(2) Transthoracic Doppler echocardiography is recommended for the initial evaluation of pulmonic stenosis in adolescent and young adult patients, and serially every 5 to 10 years for follow-up examinations.

(3) Cardiac catheterization is recommended in the adolescent or young adult with pulmonic stenosis for evaluation of the valvular gradient if the Doppler peak jet velocity is greater than 3 m per second (estimated peak gradient greater than 36 mm Hg) and balloon dilation can be performed if indicated.

CLASS III
(1) Diagnostic cardiac catheterization is not recommended for the initial diagnostic evaluation of pulmonic stenosis in adolescent and young adult patients.

CLASS IIb
(1) Balloon valvotomy may be reasonable in asymptomatic adolescent and young adult patients with pulmonic stenosis and an RV–to–pulmonary artery peak-to-peak gradient 30 to 39 mm Hg at catheterization.

CLASS III
(1) Balloon valvotomy is not recommended in asymptomatic adolescent and young adult patients with pulmonic stenosis and RV–to– pulmonary artery peak-to-peak gradient less than 30 mm Hg at catheterization.




JACC Vol. 52, No. 13, 2008
September 23, 2008: e1-142