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.

Overview

Congenital aortic insufficiency rarely occurs alone and is often associated with aortic stenosis or ventricular septal defect. It may occasionally be observed 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.[1]

Diagnosis

Symptoms

The majority of young patients remain asymptomatic even with severe aortic insufficiency.

Echocardiography

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.[2][3][4][5]

Treatment

Medical Management

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 left ventricular end-diastolic and end-systolic dimensions, end-diastolic and end-systolic volume indexes, mass index determined by 2D-echocardiography and wall stresses also decreased significantly with therapy. A study on benefits of ACE inhibitor in 20 children with aortic insufficiency showed that there was approximately 28% reduction in regurgitant fraction at the end of 1 year of treatment.[6]

Surgical Management

Surgery is indicated in young patients with aortic insufficiency who are:

These indications are similar to those in adults. The choice between aortic valve replacement vs aortic valve repair depends on the cause of aortic insufficiency.

Aortic Valve Repair

Aortic valve repair is effective in patients who developed aortic insufficiency after undergoing balloon valvuloplasty for aortic stenosis and in those who have prolapsed cusps in VSD. In a study on 21 patients aged between 9 months to 15years who underwent valve repair for aortic insufficiency secondary to balloon dilatation for aortic stenosis showed 100% freedom from re-operation for late failure, and 80% overall freedom from re-intervention at 3 years with significant reduction in regurgitant fraction, left ventricular end-diastolic dimension, and proximal regurgitant jet/aortic annulus diameter ratio.[7] Freedom from re-operation was 95%, 87%, and 84% at 1, 5, and 7 years, respectively in patients undergoing valve repair for cusp prolapse.[8]

Aortic Valve Replacement

Aortic valve replacement is another treatment option where mechanical or bioprosthetic valves can be used. Though use of bioprosthetic valves reduces the need for long term systemic anticoagulation, they have a high failure rate of 20% in children due to valve degeneration and calcification.[9][10] Mechanical valves with prolonged anticoagulation are preferred as they showed better outcome in yound patients with normalization of end-diastolic volume, left ventricular ejection fraction, and peak systolic strain of the left ventricular myocardium.[11]

Ross or Ross/Konno Procedure

Ross procedure is another alternative surgical procedure where the pulmonary valve is transplanted to the aortic position, and a homograft conduit is implanted from the right ventricle to the pulmonary artery. Though this procedure shows promising results for aortic valve abnormalities in some,[12][13][14] the use of this technique has been limited by high rates of pulmonary autograft failure with deterioration of right heart homografts.[15] These rates are higher in children as compared to adults. Further studies aimed at clarifying longer-term outcomes as well as preventing pulmonary homograft deteroration are needed.

To summarize, mechanical valve replacement is the preferred surgical option at present in young patients as opposed to valve repair or biological valve replacement in view of lack of evidence of long-term durability and outcomes. However, they may be appropriate for patients in whom anticoagulation are contraindicated.

2008 Focused Update Incorporated into the 2006 ACC/AHA Guidelines for the Management of Patients with Valvular Heart Disease (DO NOT EDIT)[16]

Aortic Valve Repair or Replacement in Adolescents (DO NOT EDIT)[16]

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. (Level of Evidence: C)"
"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. (Level of Evidence: C)"
"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. (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 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. (Level of Evidence: C)"
"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. (Level of Evidence: C)"

References

  1. Bonow RO, Carabello B, de Leon AC, Edmunds LH, Fedderly BJ, Freed MD; et al. (1998). "ACC/AHA Guidelines for the Management of Patients With Valvular Heart Disease. Executive Summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients With Valvular Heart Disease)". J Heart Valve Dis. 7 (6): 672–707. PMID 9870202.
  2. Zoghbi WA, Enriquez-Sarano M, Foster E, Grayburn PA, Kraft CD, Levine RA, Nihoyannopoulos P, Otto CM, Quinones MA, Rakowski H, Stewart WJ, Waggoner A, Weissman NJ (2003). "Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography". Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography. 16 (7): 777–802. doi:10.1016/S0894-7317(03)00335-3. PMID 12835667. Retrieved 2011-03-02. Unknown parameter |month= ignored (help)
  3. Desjardins VA, Enriquez-Sarano M, Tajik AJ, Bailey KR, Seward JB (1996). "Intensity of murmurs correlates with severity of valvular regurgitation". Am J Med. 100 (2): 149–56. PMID 8629648.
  4. Grande RD, Katz WE (2011). "Acute aortic regurgitation secondary to disk embolization of a Björk-Shiley prosthetic aortic valve". J Am Soc Echocardiogr. 24 (3): 350.e5–6. doi:10.1016/j.echo.2010.07.001. PMID 20708374.
  5. Saranteas T, Christodoulaki K, Rinaki D, Kostopanagiotou G (2011). "Transthoracic echocardiography for the identification of acute aortic regurgitation in the intensive care unit". J Cardiothorac Vasc Anesth. 25 (1): 204–5. doi:10.1053/j.jvca.2009.11.015. PMID 20117022.
  6. 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)
  7. Bacha EA, Satou GM, Moran AM, Zurakowski D, Marx GR, Keane JF, Jonas RA (2001). "Valve-sparing operation for balloon-induced aortic regurgitation in congenital aortic stenosis". The Journal of Thoracic and Cardiovascular Surgery. 122 (1): 162–8. doi:10.1067/mtc.2001.114639. PMID 11436050. Retrieved 2011-04-08. Unknown parameter |month= ignored (help)
  8. Casselman FP, Gillinov AM, Akhrass R, Kasirajan V, Blackstone EH, Cosgrove DM (1999). "Intermediate-term durability of bicuspid aortic valve repair for prolapsing leaflet". European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery. 15 (3): 302–8. PMID 10333027. Retrieved 2011-04-08. Unknown parameter |month= ignored (help)
  9. Walker WE, Duncan JM, Frazier OH, Livesay JJ, Ott DA, Reul GJ, Cooley DA (1983). "Early experience with the ionescu-shiley pericardial xenograft valve. Accelerated calcification in children". The Journal of Thoracic and Cardiovascular Surgery. 86 (4): 570–5. PMID 6621085. Unknown parameter |month= ignored (help); |access-date= requires |url= (help)
  10. Kirklin JK, Smith D, Novick W, Naftel DC, Kirklin JW, Pacifico AD, Nanda NC, Helmcke FR, Bourge RC (1993). "Long-term function of cryopreserved aortic homografts. A ten-year study". The Journal of Thoracic and Cardiovascular Surgery. 106 (1): 154–65, discussion 165–6. PMID 8320994. Unknown parameter |month= ignored (help); |access-date= requires |url= (help)
  11. Arnold R, Ley-Zaporozhan J, Ley S, Loukanov T, Sebening C, Kleber JB, Goebel B, Hagl S, Karck M, Gorenflo M (2008). "Outcome after mechanical aortic valve replacement in children and young adults". The Annals of Thoracic Surgery. 85 (2): 604–10. doi:10.1016/j.athoracsur.2007.10.035. PMID 18222274. Retrieved 2011-04-08. Unknown parameter |month= ignored (help)
  12. Ohye RG, Gomez CA, Ohye BJ, Goldberg CS, Bove EL (2001). "The Ross/Konno procedure in neonates and infants: intermediate-term survival and autograft function". The Annals of Thoracic Surgery. 72 (3): 823–30. PMID 11565665. Retrieved 2011-04-08. Unknown parameter |month= ignored (help)
  13. Laudito A, Brook MM, Suleman S, Bleiweis MS, Thompson LD, Hanley FL, Reddy VM (2001). "The Ross procedure in children and young adults: a word of caution". The Journal of Thoracic and Cardiovascular Surgery. 122 (1): 147–53. doi:10.1067/mtc.2001.113752. PMID 11436048. Retrieved 2011-04-08. Unknown parameter |month= ignored (help)
  14. Laforest I, Dumesnil JG, Briand M, Cartier PC, Pibarot P (2002). "Hemodynamic performance at rest and during exercise after aortic valve replacement: comparison of pulmonary autografts versus aortic homografts". Circulation. 106 (12 Suppl 1): I57–I62. PMID 12354710. Retrieved 2011-04-08. Unknown parameter |month= ignored (help)
  15. David TE (2009). "Ross procedure at the crossroads". Circulation. 119 (2): 207–9. doi:10.1161/CIRCULATIONAHA.108.827964. PMID 19153280. Retrieved 2011-04-08. Unknown parameter |month= ignored (help)
  16. 16.0 16.1 Bonow RO, Carabello BA, Chatterjee K; et al. (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. Unknown parameter |month= ignored (help)


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