Congenital aortic Regurgitation

Natural history and management

Mary T. Donofrio, Mary Allen Engle, John E. O'Loughlin, Michael S. Snyder, Aaron R. Levin, Kathryn H. Ehlers, Jeffrey P Gold

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Objectives and Background. Congenital aortic regurgitation is rare as an isolated lesion. We describe seven children with no physical features of the Marfan syndrome in the patients or their families and no cardiac lesions who had congenital valvular aortic regurgitation. Methods. From 1954 to the present, seven children with auscultatory and physiologic characteristics of aortic regurgitation were evaluated for a total of 108 patient-years. We report on their natural history, clinical and laboratory findings, management and outcome. Results. In five of the seven children congenital aortic regurgitation was diagnosed in infancy. In four, progressive severity of the regurgitation led to valve replacement at age 3, 10, 15 and 20 years, respectively, and to resection of an aneurysm of the ascending aorta in the 10-year old patient. Two patients had cystic medial necrosis on aortic biopsy. One of these patients died after reoperation for dissecting aneurysm of the thoracic aorta at 22 years of age; the other died after dissection and rupture of the ascending aorta at age 25 years. After obstructing pannus developed, the 3-year old patient underwent replacement of the St. Jude valve at age 10 years. The other three patients were asymptomatic at last follow-up at age 8, 10 and 20 years, respectively. Conclusions. Supportive management is recommended until it becomes necessary to intervene surgically when regurgitation becomes severe. The need for surgical treatment is indicated by the appearance of a diastolic thrill, left ventricular strain on the electrocardiogram or other evidence of left ventricular dysfunction on the echocardiogram or exercise stress testing by treadmill or radionuclide cineangiocardiography. Close follow-up of these patients is important to detect progression of aortic regurgitation, especially in the presence of cystic medial necrosis.

Original languageEnglish (US)
Pages (from-to)366-372
Number of pages7
JournalJournal of the American College of Cardiology
Volume20
Issue number2
DOIs
StatePublished - Jan 1 1992

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Aortic Valve Insufficiency
Natural History
Aorta
Necrosis
Dissecting Aneurysm
Marfan Syndrome
Left Ventricular Dysfunction
Thoracic Aorta
Reoperation
Radioisotopes
Aneurysm
Dissection
Rupture
Electrocardiography
Exercise
Biopsy

ASJC Scopus subject areas

  • Nursing(all)

Cite this

Donofrio, M. T., Engle, M. A., O'Loughlin, J. E., Snyder, M. S., Levin, A. R., Ehlers, K. H., & Gold, J. P. (1992). Congenital aortic Regurgitation: Natural history and management. Journal of the American College of Cardiology, 20(2), 366-372. https://doi.org/10.1016/0735-1097(92)90104-U

Congenital aortic Regurgitation : Natural history and management. / Donofrio, Mary T.; Engle, Mary Allen; O'Loughlin, John E.; Snyder, Michael S.; Levin, Aaron R.; Ehlers, Kathryn H.; Gold, Jeffrey P.

In: Journal of the American College of Cardiology, Vol. 20, No. 2, 01.01.1992, p. 366-372.

Research output: Contribution to journalArticle

Donofrio, MT, Engle, MA, O'Loughlin, JE, Snyder, MS, Levin, AR, Ehlers, KH & Gold, JP 1992, 'Congenital aortic Regurgitation: Natural history and management', Journal of the American College of Cardiology, vol. 20, no. 2, pp. 366-372. https://doi.org/10.1016/0735-1097(92)90104-U
Donofrio MT, Engle MA, O'Loughlin JE, Snyder MS, Levin AR, Ehlers KH et al. Congenital aortic Regurgitation: Natural history and management. Journal of the American College of Cardiology. 1992 Jan 1;20(2):366-372. https://doi.org/10.1016/0735-1097(92)90104-U
Donofrio, Mary T. ; Engle, Mary Allen ; O'Loughlin, John E. ; Snyder, Michael S. ; Levin, Aaron R. ; Ehlers, Kathryn H. ; Gold, Jeffrey P. / Congenital aortic Regurgitation : Natural history and management. In: Journal of the American College of Cardiology. 1992 ; Vol. 20, No. 2. pp. 366-372.
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