Aortic regurgitation in young patients

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editors-in-Chief: Varun Kumar, M.B.B.S.; Lakshmi Gopalakrishnan, M.B.B.S.

Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [2] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

Congenital aortic insufficiency rarely occurs alone and is often associated with aortic stenosis or ventricular septal defect. Although it may occasionally be seen in adolescents and young adults with a bicuspid aortic valve, discrete subaortic obstruction, or prolapse of one of the aortic cusp into a ventricular septal defect. Turner syndrome, osteogenesis imperfecta, tetralogy of Fallot, and truncus arteriosus are other congenital disorders that are associated with aortic insufficiency in young patients.

Rheumatic heart disease is one of the important causes for acquired aortic insufficiency in young patients in developing countries. It can also occur following an episode of infective endocarditis or as a consequence of attempts to relieve aortic stenosis by either balloon valvuloplasty or surgical valvulotomy, or when the pulmonary artery is relocated in the aortic position during repair of transposition of great vessels.

Majority of young patients remain asymptomatic even with severe aortic insufficiency. Therefore these patients should be followed-up with serial echocardiographic assessment, including measurement of ventricular dimensions, volumes, and function. This could assist in determining the timing of surgical repair. Surgery is recommended for patients with symptoms of heart failure, dyspnoea on exertion with ischemic signs and symptoms, or at the first indication of a decline in left ventricular function. As in aortic stenosis, endocarditis prophylaxis is recommended for patients with aortic regurgitation.

Treatment

Medical therapy

Vasodilators are indicated in patients who have moderate to severe aortic insufficiency, are symptomatic or in those who have concurrent hypertension. ACE inhibitors with captopril in particular may be beneficial in children with partial improvement in ventricular dilation and hypertrophy. A study on benefits of ACE inhibitor in 20 children with aortic insufficiency showed that there was 28% reduction in regurgitant fraction[1].

Surgery is indicated in young patients with aortic insufficiency who are symptomatic or having left ventricular dysfunction (ejection fraction < 50%) or increased left ventricular end-diastolic/systolic dimension (body size should be taken into account). These are similar to indications for surgery in adults.

AHA/ACC guidelines for treatment of aortic insufficiency in adolescent or young adults [2]

Class I

  1. An adolescent or young adult with chronic severe aortic insufficiency with onset of symptoms of angina, syncope, or dyspnea on exertion should receive aortic valve repair or replacement. (Level of Evidence: C)
  2. Asymptomatic adolescent or young adult patients with chronic severe aortic insufficiency with left ventricular systolic dysfunction (ejection fraction less than 0.50) on serial studies 1 to 3 months apart should receive aortic valve repair or replacement. (Level of Evidence: C)
  3. Asymptomatic adolescent or young adult patients with chronic severe aortic insufficiency with progressive left ventricualr enlargement (end-diastolic dimension greater than 4 standard deviations above normal) should receive aortic valve repair or replacement. (Level of Evidence: C)
  4. Coronary angiography is recommended before AVR in adolescent or young adult patients with aortic insufficiency in whom a pulmonary autograft (Ross operation) is contemplated when the origin of the coronary arteries has not been identified by noninvasive techniques. (Level of Evidence: C)

Class IIb

  1. An asymptomatic adolescent with chronic severe aortic insufficiency with moderate aortic stenosis (peak left ventricle–to–peak aortic gradient greater than 40 mm Hg at cardiac catheterization) may be considered for aortic valve repair or replacement. (Level of Evidence: C)
  2. An asymptomatic adolescent with chronic severe aortic insufficiency with onset of ST depression or T-wave inversion over the left precordium on electrocardiogram at rest may be considered for aortic valve repair or replacement. (Level of Evidence: C)

The Ross Procedure

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References

  1. Alehan D, Ozkutlu S (1998). "Beneficial effects of 1-year captopril therapy in children with chronic aortic regurgitation who have no symptoms". American Heart Journal. 135 (4): 598–603. PMID 9539473. Retrieved 2011-04-07. Unknown parameter |month= ignored (help)
  2. Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O'Gara PT, O'Rourke RA, Otto CM, Shah PM, Shanewise JS (2008). "2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Circulation. 118 (15): e523–661. doi:10.1161/CIRCULATIONAHA.108.190748. PMID 18820172. Retrieved 2011-04-07. Unknown parameter |month= ignored (help)

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