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. 1993 Jan;69(1):71–79. doi: 10.1136/hrt.69.1.71

Incidence and prognosis of congenital aortic valve stenosis in Liverpool (1960-1990).

D J Kitchiner 1, M Jackson 1, K Walsh 1, I Peart 1, R Arnold 1
PMCID: PMC1024922  PMID: 8457400

Abstract

OBJECTIVE--To determine the incidence and prognosis of congenital aortic valve stenosis in the five Health Districts of Liverpool that make up the Merseyside area. DESIGN--The records of the Liverpool Congenital Malformations Registry and the Royal Liverpool Children's Hospital identified 239 patients (155 male, 84 female) born with aortic valve stenosis between 1960 and 1990. Patients were traced to assess the severity of stenosis at follow up. Information on the severity at presentation and all subsequent events was obtained. RESULTS--Congenital aortic valve stenosis occurred in 5.7% of patients with congenital heart disease born in the Merseyside area. The median age at presentation was 16 months (range 0-20 years). Stenosis was mild at presentation in 145 patients, moderate in 33, severe in one and critical in 21 and 39 had a bicuspid valve without stenosis. Additional cardiac lesions were significantly more common in children presenting under one year of age and in those with critical stenosis. The median duration of follow up was 9.2 years (range 1-28 years) and seven patients were lost to follow up. 81 operations were performed in 60 patients. The reoperation rate was 28.3% after a median duration of 8.7 years (range 2.5-18 years). 15% of patients who presented with mild stenosis subsequently required operation compared with 67% of those with moderate stenosis. There were no sudden unexpected deaths and no deaths after aortic valvotomy, except in those presenting with critical stenosis. Mortality was 16.7% but patients presenting with critical aortic stenosis had a much worse prognosis. Actuarial and hazard analysis showed that the survival and absence of serious events (aortic valve surgery or balloon dilatation, endocarditis, or death) were significantly better in patients who presented with mild aortic stenosis than in those who presented with moderate aortic stenosis. 75% of patients presenting with mild stenosis had not progressed to moderate stenosis after 10 years of follow up. CONCLUSIONS--Congenital aortic valve stenosis may be progressive even when it is mild at presentation. Patients presenting with mild stenosis, however, have a significantly better prognosis than those presenting with moderate stenosis. An accurate clinical and echocardiographic assessment of the severity of aortic valve stenosis at presentation provides a good guide to prognosis into early adult life.

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Selected References

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  1. Ankeney J. L., Tzeng T. S., Liebman J. Surgical therapy for congenital aortic valvular stenosis. A 23 year experience. J Thorac Cardiovasc Surg. 1983 Jan;85(1):41–48. [PubMed] [Google Scholar]
  2. BRAUNWALD E., GOLDBLATT A., AYGEN M. M., ROCKOFF S. D., MORROW A. G. Congenital aortic stenosis. I. Clinical and hemodynamic findings in 100 patients. II. Surgical treatment and the results of operation. Circulation. 1963 Mar;27:426–462. doi: 10.1161/01.cir.27.3.426. [DOI] [PubMed] [Google Scholar]
  3. Balaji S., Keeton B. R., Sutherland G. R., Shore D. F., Monro J. L. Aortic valvotomy for critical aortic stenosis in neonates and infants aged less than one year. Br Heart J. 1989 Apr;61(4):358–360. doi: 10.1136/hrt.61.4.358. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Bernhard W. F., Keane J. F., Fellows K. E., Litwin S. B., Gross R. E. Progress and problems in the surgical management of congenital aortic stenosis. J Thorac Cardiovasc Surg. 1973 Sep;66(3):404–419. [PubMed] [Google Scholar]
  5. Campbell M. The natural history of congenital aortic stenosis. Br Heart J. 1968 Jul;30(4):514–526. doi: 10.1136/hrt.30.4.514. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Cohen L. S., Friedman W. F., Braunwald E. Natural history of mild congenital aortic stenosis elucidated by serial hemodynamic studies. Am J Cardiol. 1972 Jul 11;30(1):1–5. doi: 10.1016/0002-9149(72)90116-6. [DOI] [PubMed] [Google Scholar]
  7. Currie P. J., Hagler D. J., Seward J. B., Reeder G. S., Fyfe D. A., Bove A. A., Tajik A. J. Instantaneous pressure gradient: a simultaneous Doppler and dual catheter correlative study. J Am Coll Cardiol. 1986 Apr;7(4):800–806. doi: 10.1016/s0735-1097(86)80339-4. [DOI] [PubMed] [Google Scholar]
  8. DeBoer D. A., Robbins R. C., Maron B. J., McIntosh C. L., Clark R. E. Late results of aortic valvotomy for congenital valvar aortic stenosis. Ann Thorac Surg. 1990 Jul;50(1):69–73. doi: 10.1016/0003-4975(90)90090-s. [DOI] [PubMed] [Google Scholar]
  9. Dickinson D. F., Arnold R., Wilkinson J. L. Ventricular septal defect in children born in Liverpool 1960 to 1969. Evaluation of natural course and surgical implications in an unselected population. Br Heart J. 1981 Jul;46(1):47–54. doi: 10.1136/hrt.46.1.47. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. EDWARDS J. E. The congenital bicuspid aortic valve. Circulation. 1961 Apr;23:485–488. doi: 10.1161/01.cir.23.4.485. [DOI] [PubMed] [Google Scholar]
  11. Emanuel R., Withers R., O'Brien K., Ross P., Feizi O. Congenitally bicuspid aortic valves. Clinicogenetic study of 41 families. Br Heart J. 1978 Dec;40(12):1402–1407. doi: 10.1136/hrt.40.12.1402. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Fisher R. D., Mason D. T., Morrow A. G. Results of operative treatment in congenital aortic stenosis. Pre- and postoperative hemodynamic evaluations. J Thorac Cardiovasc Surg. 1970 Feb;59(2):218–224. [PubMed] [Google Scholar]
  13. Galan A., Zoghbi W. A., Quiñones M. A. Determination of severity of valvular aortic stenosis by Doppler echocardiography and relation of findings to clinical outcome and agreement with hemodynamic measurements determined at cardiac catheterization. Am J Cardiol. 1991 May 1;67(11):1007–1012. doi: 10.1016/0002-9149(91)90175-k. [DOI] [PubMed] [Google Scholar]
  14. Gunning J. F., Oakley C. M. Aortic-valve disease in Turner's syndrome. Lancet. 1970 Feb 21;1(7643):389–391. doi: 10.1016/s0140-6736(70)91519-9. [DOI] [PubMed] [Google Scholar]
  15. HUGENHOLTZ P. G., LEES M. M., NADAS A. S. The scalar electrocardiogram, vectorcardiogram, and exercise electrocardiogram in the assessment of congenital aortic stenosis. Circulation. 1962 Jul;26:79–91. doi: 10.1161/01.cir.26.1.79. [DOI] [PubMed] [Google Scholar]
  16. Hegrenaes L., Hatle L. Aortic stenosis in adults. Non-invasive estimation of pressure differences by continuous wave Doppler echocardiography. Br Heart J. 1985 Oct;54(4):396–404. doi: 10.1136/hrt.54.4.396. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Hoffman J. I. The natural history of congenital isolated pulmonic and aortic stenosis. Annu Rev Med. 1969;20:15–28. doi: 10.1146/annurev.me.20.020169.000311. [DOI] [PubMed] [Google Scholar]
  18. Hossack K. F., Neutze J. M., Lowe J. B., Barratt-Boyes B. G. Congenital valvar aortic stenosis. Natural history and assessment for operation. Br Heart J. 1980 May;43(5):561–573. doi: 10.1136/hrt.43.5.561. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Hsieh K. S., Keane J. F., Nadas A. S., Bernhard W. F., Castaneda A. R. Long-term follow-up of valvotomy before 1968 for congenital aortic stenosis. Am J Cardiol. 1986 Aug 1;58(3):338–341. doi: 10.1016/0002-9149(86)90073-1. [DOI] [PubMed] [Google Scholar]
  20. Jones M., Barnhart G. R., Morrow A. G. Late results after operations for left ventricular outflow tract obstruction. Am J Cardiol. 1982 Sep;50(3):569–579. doi: 10.1016/0002-9149(82)90326-5. [DOI] [PubMed] [Google Scholar]
  21. Karl T. R., Sano S., Brawn W. J., Mee R. B. Critical aortic stenosis in the first month of life: surgical results in 26 infants. Ann Thorac Surg. 1990 Jul;50(1):105–109. doi: 10.1016/0003-4975(90)90099-r. [DOI] [PubMed] [Google Scholar]
  22. Kenna A. P., Smithells R. W., Fielding D. W. Congenital heart disease in Liverpool: 1960--69. Q J Med. 1975 Jan;44(173):17–44. [PubMed] [Google Scholar]
  23. Kennedy K. D., Nishimura R. A., Holmes D. R., Jr, Bailey K. R. Natural history of moderate aortic stenosis. J Am Coll Cardiol. 1991 Feb;17(2):313–319. doi: 10.1016/s0735-1097(10)80092-0. [DOI] [PubMed] [Google Scholar]
  24. Lakier J. B., Lewis A. B., Heymann M. A., Stanger P., Hoffman J. I., Rudolph A. M. Isolated aortic stenosis in the neonate. Natural history and hemodynamic considerations. Circulation. 1974 Oct;50(4):801–808. doi: 10.1161/01.cir.50.4.801. [DOI] [PubMed] [Google Scholar]
  25. Leech G., Mills P., Leatham A. The diagnosis of a non-stenotic bicuspid aortic valve. Br Heart J. 1978 Sep;40(9):941–950. doi: 10.1136/hrt.40.9.941. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Miller M. J., Geffner M. E., Lippe B. M., Itami R. M., Kaplan S. A., DiSessa T. G., Isabel-Jones J. B., Friedman W. F. Echocardiography reveals a high incidence of bicuspid aortic valve in Turner syndrome. J Pediatr. 1983 Jan;102(1):47–50. doi: 10.1016/s0022-3476(83)80284-4. [DOI] [PubMed] [Google Scholar]
  27. Mills P., Leech G., Davies M., Leathan A. The natural history of a non-stenotic bicuspid aortic valve. Br Heart J. 1978 Sep;40(9):951–957. doi: 10.1136/hrt.40.9.951. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Mody M. R., Mody G. T. Serial hemodynamic observations in congenital valvular and subvalvular aortic stenosis. Am Heart J. 1975 Feb;89(2):137–143. doi: 10.1016/0002-8703(75)90037-x. [DOI] [PubMed] [Google Scholar]
  29. Odemuyiwa O., Bourke J. P., Peart I., Been M., Heads A., Hall R. J. Valvar stenosis: a comparison of clinical assessment, echocardiography, Doppler ultrasound and catheterisation. Int J Cardiol. 1990 Jan;26(1):59–65. doi: 10.1016/0167-5273(90)90248-4. [DOI] [PubMed] [Google Scholar]
  30. Radford D. J., Bloom K. R., Izukawa T., Moes C. A., Rowe R. D. Echocardiographic assessment of bicuspid aortic valves. Angiographic and pathological correlates. Circulation. 1976 Jan;53(1):80–85. doi: 10.1161/01.cir.53.1.80. [DOI] [PubMed] [Google Scholar]
  31. Roberts W. C. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol. 1970 Jul;26(1):72–83. doi: 10.1016/0002-9149(70)90761-7. [DOI] [PubMed] [Google Scholar]
  32. Roberts W. C. The structure of the aortic valve in clinically isolated aortic stenosis: an autopsy study of 162 patients over 15 years of age. Circulation. 1970 Jul;42(1):91–97. doi: 10.1161/01.cir.42.1.91. [DOI] [PubMed] [Google Scholar]
  33. Rocchini A. P., Beekman R. H., Ben Shachar G., Benson L., Schwartz D., Kan J. S. Balloon aortic valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Am J Cardiol. 1990 Mar 15;65(11):784–789. doi: 10.1016/0002-9149(90)91388-m. [DOI] [PubMed] [Google Scholar]
  34. Sandor G. G., Olley P. M., Trusler G. A., Williams W. G., Rowe R. D., Morch J. E. Long-term follow-up of patients after valvotomy for congenital valvular aortic stenosis in children: a clinical and actuarial follow-up. J Thorac Cardiovasc Surg. 1980 Aug;80(2):171–176. [PubMed] [Google Scholar]
  35. Selzer A. Changing aspects of the natural history of valvular aortic stenosis. N Engl J Med. 1987 Jul 9;317(2):91–98. doi: 10.1056/NEJM198707093170206. [DOI] [PubMed] [Google Scholar]
  36. Shub C., Tajik A. J., Holmes D. R., Jr, Reeder G. S., Freeman W. K., Ilstrup D. M., Smith H. C. Doppler echocardiography in aortic stenosis: feasibility and clinical impact. Int J Cardiol. 1990 Jul;28(1):57–66. doi: 10.1016/0167-5273(90)90009-t. [DOI] [PubMed] [Google Scholar]
  37. Sullivan I. D., Wren C., Bain H., Hunter S., Rees P. G., Taylor J. F., Bull C., Deanfield J. E. Balloon dilatation of the aortic valve for congenital aortic stenosis in childhood. Br Heart J. 1989 Feb;61(2):186–191. doi: 10.1136/hrt.61.2.186. [DOI] [PMC free article] [PubMed] [Google Scholar]
  38. Turley K., Bove E. L., Amato J. J., Iannettoni M., Yeh J., Cotroneo J. V., Galdieri R. J. Neonatal aortic stenosis. J Thorac Cardiovasc Surg. 1990 Apr;99(4):679–684. [PubMed] [Google Scholar]
  39. Wheller J. J., Hosier D. M., Teske D. W., Craenen J. M., Kilman J. W. Results of operation for aortic valve stenosis in infants, children, and adolescents. J Thorac Cardiovasc Surg. 1988 Sep;96(3):474–477. [PubMed] [Google Scholar]
  40. Yeager M., Yock P. G., Popp R. L. Comparison of Doppler-derived pressure gradient to that determined at cardiac catheterization in adults with aortic valve stenosis: implications for management. Am J Cardiol. 1986 Mar 1;57(8):644–648. doi: 10.1016/0002-9149(86)90851-9. [DOI] [PubMed] [Google Scholar]

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