Hostname: page-component-cd9895bd7-lnqnp Total loading time: 0 Render date: 2024-12-21T16:11:15.688Z Has data issue: false hasContentIssue false

The risk of having additional obstructive lesions in neonatal coarctation of the aorta

Published online by Cambridge University Press:  01 July 2011

Jami C. Levine*
Affiliation:
Department of Cardiology and the Division of Cardiovascular Surgery, Children's Hospital and the Departments of Pediatrics and Surgery, Harvard Medical School, Boston, MA
Stephen P. Sanders
Affiliation:
Division of Pediatric Cardiology, Duke University Medical Center, Durham, NC
Steven D. Colan
Affiliation:
Department of Cardiology and the Division of Cardiovascular Surgery, Children's Hospital and the Departments of Pediatrics and Surgery, Harvard Medical School, Boston, MA
Richard A. Jonas
Affiliation:
Department of Cardiology and the Division of Cardiovascular Surgery, Children's Hospital and the Departments of Pediatrics and Surgery, Harvard Medical School, Boston, MA
Philip J. Spevak
Affiliation:
Department of Pediatrics, Johns Hopkins University, Baltimore, MD, USA
*
Correspondence to: Jami C. Levine, MD, Children's Hospital, Department of Cardiology, 300 Longwood Avenue, Boston MA 02115, USA. Tel: 617-355-4902; Fax: 617-739-6282

Abstract

Infants with coarctation of the aorta may have obstructions at other sites within the left heart which are not always apparent on the initial echocardiogram. The magnitude of the risk of having the additional obstructions is not well described, with few reliable quantitative criterions for identifying patients at the highest risk. We determined the frequency of additional, late appearing, stenotic lesions within the left heart, and the predictive morphologic features on the initial cross-sectional echocardiogram.

We identified all patients with coarctation of the aorta diagnosed by 3 months of age, excluding those with complex cardiac disease or definite additional stenotic lesions at presentation, leaving 101 patients for study. At follow-up, 31 stenotic lesions were diagnosed in 23 patients, 15 of whom had at least 1 intervention. Mitral stenosis was diagnosed in 11 patients, aortic stenosis in 10, subaortic stenosis in 8, and supravalvar aortic stenosis in 2. The probability for freedom from obstructive lesions was 81% at 1 year, 74% at 3 years, and 70% at 5 years. Echocardiographic predictors of mitral stenosis included smaller mitral valvar annuluses, presence of a mean transmitral gradient between 2.5 and 5.0 mmHg, and elongation of the area of intervalvar fibrous continuity. Predictors of aortic stenosis were smaller mitral valvar annuluses, an initial aortic valvar gradient between 15 and 20 mmHg, and obliteration of the commissure between the right and non-coronary leaflets of the aortic valve. Predictors of subaortic stenosis were smaller mitral valvar annuluses and elongation of the area of intervalvar fibrous continuity. Patients with Z-scores for the diameter of the mitral valve of less than −1 were at the highest risk for manifesting obstructive lesions at any level.

Associated stenoses in the left heart are common in the setting of aortic coarctation. When Doppler data is equivocal, features of the cross-sectional echocardiogram can identify the sub-group of infants at increased risk.

Type
Original Articles
Copyright
Copyright © Cambridge University Press 2001

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Perry, L, Neill, C, Ferencz, C, Rubin, J, Loffredo, C. Infants with congenital heart disease: the cases In: Ferencz, C, Rubin, J, Loffredo, C, Magee, C (eds). Perspectives in Pediatric Cardiology: Epidemiology of Congenital Heart Disease. The Baltimore-Washington Infant Study 1981-1989. New York: Futura Publishing Co., Inc., 1993, Chapter 3Google Scholar
2.Ziemer, G, Jonas, R, Perry, S, Freed, M, Castaneda, A. Surgery for coarctation of the aorta in the neonate. Circulation 1986; 74: 125131Google ScholarPubMed
3.Cohen, M, Fuster, YSteele, P, Driscoll, D, McGoon, D. Coarctation of the aorta. Long-term follow-up and prediction of outcome after surgical correction. Circulation 1989; 80: 840845CrossRefGoogle ScholarPubMed
4.Brouwer, R, Erasmus, M, Ebels, T, Eijgelaar, A. Influence of age on survival, late hypertension, and recoarctation in elective aortic coarctation repair. J Thorac Cardiovasc Surg 1994; 108: 525531CrossRefGoogle ScholarPubMed
5.Trinquet, F, Vouhe, P, Vernant, F et al. , Coarctation of the aorta in infants: which operation? Ann Thorac Surg 1988; 45: 186191CrossRefGoogle ScholarPubMed
6.Nihoyannopoulos, P, Karas, S, Sapsford, R, Hallidie-Smith, K, Foale, R. Accuracy of two-dimensional echocardiography in the diagnosis of aortic arch obstruction. J Am Coll Cardiol 1987; 10:10721077CrossRefGoogle ScholarPubMed
7.Moene, R, Oppenheimer-Dekker, A, Wenink, A. Relation between aortic arch hypoplasia of variable severity and central muscular ventricular septal defects: Emphasis on associated left ventricular abnormalities. Am J Cardiol 1981; 48: 111115CrossRefGoogle ScholarPubMed
8.Bharati, S, Lev, M. The surgical anatomy of the heart in tubular hypoplasia of the transverse aorta (preductal coarctation). J Thorac Cardiovasc Surg 1986; 91: 7985CrossRefGoogle ScholarPubMed
9.Celeno, V, Peironi, D, Morera, J, Roland, J, Gingell, R. Two-dimensional echocardiographic examination of mitral valve abnormalities associated with coarctation of the aorta. Circulation 1984;69:924932CrossRefGoogle Scholar
10.Shone, J, Sellers, R, Anderson, R, Adams, P, Lillehei, C, Edwards, J. The developmental complex of ‘parachute mitral valve’, supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Catdiol 1963; X: 714725CrossRefGoogle Scholar
11.Becker, A, Becker, M, Edwards, J. Anomalies associated with coarctation of the aorta. Particular reference to infancy. Circulation 1970; 41: 10671075CrossRefGoogle ScholarPubMed
12.Rosenquist, G. Congenital mitral valve disease associated with coarctation of the aorta. A spectrum that includes parachute deformity of the mitral valve. Circulation 1974; 49: 985993CrossRefGoogle ScholarPubMed
13.Venugopalan, P, Bu'Lock, F, Joffe, H. Mitral valve hypoplasia in children with isolated coarctation of the aorta. Br Heart J 1994; 71: 358362CrossRefGoogle ScholarPubMed
14.Moene, R, Gittenberger-De Groot, A, Oppenheimer-Dekker, A, Bartelings, M. Anatomic characteristics of ventricular septal defect associated with coarctation of the aorta. Am J Cardiol 59:952955CrossRefGoogle Scholar
15.Moulaert, A, Bruins, C, Oppenheimer-Dekker, A. Anomalies of the aortic arch and ventricular septal defects. Circulation 1976; 6: 10111015CrossRefGoogle Scholar
16.Iannettoni, M, Bove, E, Mosca, R et al. , Improving results with first-stage palliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1994; 107: 934940CrossRefGoogle ScholarPubMed
17.Jamison, S, Simonds, H, Jenkins, K, Colan, S, Sanders, S. Morphology of bicommissural aortic valve in children and adolescents. J Am Coll Cardiol 1993; 21: 235A (Abstr.)Google Scholar
18.Shinebourne, E, Elseed, A. Relation between fetal flow patterns, coarctation of the aorta, and pulmonary blood flow. Br Heart J 1974; 36: A92–A99CrossRefGoogle ScholarPubMed
19.Rudolph, A, Heymann, M, Spitznas, U. Hemodynamic considerations in the development of narrowing of the aorta. Am J Cardiol 1972; 30: 514525CrossRefGoogle ScholarPubMed
20.Rosenquist, G, Clark, E, McAllister, H, Bharati, S, Edwards, J. Increased mitral-aortic separation in discrete subaortic stenosis. Circulation 1979; 60: 7073CrossRefGoogle ScholarPubMed
21.Kleinert, S, Geva, T. Echocardiographic morphometry and geometry of the left ventricular outflow tract in mixed subaortic stenosis. J Am Coll Cardiol 1993; 22: 15011508CrossRefGoogle Scholar