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December 25, 1991, Vol 266, No. 24 >
ARTICLE | December 25, 1991

25-Year Mortality After Surgical Repair of Congenital Heart Defect in Childhood: Title and subTitle BreakA Population-Based Cohort Study FREE

Cynthia D. Morris, PhD, MPH; Victor D. Menashe, MD
[+] Author Affiliations

Reprint requests to Division of Cardiology, Oregon Health Sciences University, 3181 SW Sam Jackson Park Rd, L 463, Portland, OR 97201 (Dr Morris).


JAMA. 1991;266(24):3447-3452. doi:10.1001/jama.1991.03470240069035
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References

Objective.  —To determine long-term survival and the cause of death after repair of one of eight congenital heart defects in childhood.

Design.  —Cohort study.

Setting.  —General community.

Participants.  —All Oregon residents with one of eight congenital heart defects, which was repaired surgically between 1958 and 1989 when the patient was aged 18 years or younger, including (1) tetralogy of Fallot; (2) isolated ventricular septal defect; (3) isolated atrial septal defect; (4) coarctation of the aorta; (5) aortic valvular stenosis; (6) pulmonary valvular stenosis; (7) transposition of the great arteries; and (8) patent ductus arteriosus. Follow-up of this cohort of 2701 individuals was obtained from 94%.

Main Outcome Measure.  —Mortality from cardiac and noncardiac causes.

Results.  —Age at surgery and operative mortality have decreased significantly over the last 30 years. Late cardiac mortality at 25 years after surgery was 5% for tetralogy of Fallot and isolated ventricular septal defect, 10% for coarctation of the aorta, 17% for aortic stenosis, 5% for pulmonic stenosis, and less than 1% for patent ductus arteriosus; there were no late cardiac deaths after atrial septal defect repair. For transposition, late cardiac mortality was 15% at 15 years after the Mustard operation and was 2% at 10 years after the Senning operation.

Conclusion.  —Surgical repair of most congenital heart defects is associated with lingering cardiac mortality, particularly for aortic stenosis, coarctation, and transposition.(JAMA. 1991;266:3447-3452)

REFERENCES

1 +
 Report from the Joint Study on the Natural History of Congenital Heart Defects. Circulation . 1977;;55( (suppl I) ):I-1-I-87.
2 +
Hoffman JIE, Christianson R.  Congenital heart disease in a cohort of 19,502 births with long-term follow-up. Am J Cardiol . 1978;;42:641-647.
3 +
Manning JA.  Insurability and employability of young cardiac patients. Cardiovasc Clin . 1980;;11:117-127.
4 +
National Center for Health Statistics. Vital Statistics of the United States, 1986, II, Sec 6, Life Tables . Washington, DC: Public Health Service; 1988;. Dept of Health and Human Services publication PHS 88-1147.
5 +
 Report of the New England Regional Infant Cardiac Program. Pediatrics . 1980;;63:377-461.
6 +
Friedli B, Kidd BS, Mustard WT, Keith JD.  Ventricular septal defect with increased pulmonary vascular resistance. Am J Cardiol . 1974;;33:403-409.
7 +
Kirklin JW, Bargeron LM, Pacifico A.  The enlargement of small pulmonary arteries by preliminary operations. Circulation . 1977;;75:612-617.
8 +
Parsons LM, Ladusans EJ, Qureshi SA.  Growth of the pulmonary artery after neonatal balloon dilatation of right ventricular outflow tract in an infant with tetralogy of Fallot and atrioventricular septal defect. Br Heart J . 1989;;62:65-68.
9 +
Matsuda H, Hirose H, Nakano S, et al.  Age-related changes in right and left ventricular function in tetralogy of Fallot. Jpn Circ J . 1986;;50:1040-1043.
10 +
Stark J.  Do we really correct congenital heart defects? J Thorac Cardiovasc Surg . 1989;;97:1-9.
11 +
Lillehei CW, Varco RL, Cohen M, et al.  The first open heart correction of tetralogy of Fallot. Ann Surg . 1986;;204:490-501.
12 +
Fuster V, McGoon DW, Kennedy MA, Ritter DG, Kirklin JW.  Long-term evaluation (12-22 years) of open-heart surgery for tetralogy of Fallot. Am J Cardiol . 1980;;46:635-642.
13 +
James FW, Kaplan S, Chou TC.  Unexpected cardiac arrest in patients after surgical correction of tetralogy of Fallot. Circulation . 1975;;52:691-695.
14 +
Deanfield JE, Ho SY, Anderson RH, McKenna WJ, Allwork SP, Hallidie-Smith KA.  Late sudden death after repair of tetralogy of Fallot: a clinicopathologic study. Circulation . 1983;;67:626-631.
15 +
Quattlebaum TG, Varghese PJ, Neill CA, Donahoo JS.  Sudden death among post-operative patients with tetralogy of Fallot. Circulation . 1976;;54:289-293.
16 +
Chandar JS, Wolff GS, Garson A, et al.  Ventricular arrhythmias in postoperative tetralogy of Fallot. Am J Cardiol . 1990;;65:655-661.
17 +
Raj Behl PR, Sante P, Blesovsky A.  Surgical treatment of isolated coarctation of the aorta: 18 years experience. Thorax . 1987;;42:309-314.
18 +
Simsolo R, Grunfeld B, Gimenez M, et al.  Long-term systemic hypertension in children after successful repair of coarctation of the aorta. Am Heart J . 1988;;115:1268-1273.
19 +
Carpenter MA, Damman JF, Watson DD, Jedeikin R, Tompkins DG, Beller GA.  Left ventricular hyperkinesis at rest and during exercise in normotensive patients from 2 to 27 years after coarctation repair. J Am Coll Cardiol . 1985;;6:879-886.
20 +
Murphy JG, Gersh BJ, Warnes CA, et al.  The late survival after surgical repair of isolated ventricular septal defect (VSD). Circulation . 1989;;80( (suppl II) ):II-490. Abstract.
21 +
Lillehei CW, Varco RL, Cohen M, Warden HE, Patton C, Moller JH.  The first open-heart repairs of ventricular septal defect, atrioventricular communis, and tetralogy of Fallot using extracorporeal correction by cross-circulation: a 30-year follow-up. Ann Thorac Surg . 1986;;41:4-21.
22 +
Allen HD, Anderson RC, Noren GR, Moller JH.  Post-operative follow-up of patients with ventricular septal defect. Circulation . 1974;;50:465-471.
23 +
Murphy JG, Gersh BJ, McGoon MD, et al.  Long-term outlook after surgical repair of isolated atrial septal defect. N Engl J Med . 1990;;323:1645-1650.
24 +
Kopecky SL, Gersh BJ, McGoon MD, et al.  Long-term outcome of patients undergoing surgical repair of isolated pulmonary valvular stenosis. Circulation . 1988;;78:1150-1156.
25 +
Deanfield J, Camm J, Macartney F, et al.  Arrhythmia and late mortality after Mustard and Senning operations for transposition of the great arteries. J Thorac Cardiovasc Surg . 1988;;96:569-576.
26 +
Marx GR, Hougen TJ, Norwood WI, Fyler DC, Casteneda AR, Nadas AS.  Transposition of the great arteries with intact ventricular septum: results of Mustard and Senning operations in 123 consecutive patients. J Am Coll Cardiol . 1983;;1:476-483.
27 +
Duster MC, Bink-Boelkens M, Wampler D, Gillette PC, McNamara DG, Cooley DA.  Long-term follow-up of dysrhythmias following the Mustard procedure. Am Heart J . 1985;;109:1323-1326.
28 +
Clarkson PM, Barratt-Boyes BG, Neutze JM.  Late dysrhythmias and disturbances of conduction following Mustard operation for complete transposition of the great arteries. Circulation . 1976;;53:519-524.
29 +
Bender HW, Stewart JR, Merrill WH, Hammon JW Jr, Graham TP Jr.  Ten years' experience with the Senning operation for transposition of the great arteries: physiological results and late follow-up. Ann Thorac Surg . 1989;;47:218-223.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
 Report from the Joint Study on the Natural History of Congenital Heart Defects. Circulation . 1977;;55( (suppl I) ):I-1-I-87.
2 +
Hoffman JIE, Christianson R.  Congenital heart disease in a cohort of 19,502 births with long-term follow-up. Am J Cardiol . 1978;;42:641-647.
3 +
Manning JA.  Insurability and employability of young cardiac patients. Cardiovasc Clin . 1980;;11:117-127.
4 +
National Center for Health Statistics. Vital Statistics of the United States, 1986, II, Sec 6, Life Tables . Washington, DC: Public Health Service; 1988;. Dept of Health and Human Services publication PHS 88-1147.
5 +
 Report of the New England Regional Infant Cardiac Program. Pediatrics . 1980;;63:377-461.
6 +
Friedli B, Kidd BS, Mustard WT, Keith JD.  Ventricular septal defect with increased pulmonary vascular resistance. Am J Cardiol . 1974;;33:403-409.
7 +
Kirklin JW, Bargeron LM, Pacifico A.  The enlargement of small pulmonary arteries by preliminary operations. Circulation . 1977;;75:612-617.
8 +
Parsons LM, Ladusans EJ, Qureshi SA.  Growth of the pulmonary artery after neonatal balloon dilatation of right ventricular outflow tract in an infant with tetralogy of Fallot and atrioventricular septal defect. Br Heart J . 1989;;62:65-68.
9 +
Matsuda H, Hirose H, Nakano S, et al.  Age-related changes in right and left ventricular function in tetralogy of Fallot. Jpn Circ J . 1986;;50:1040-1043.
10 +
Stark J.  Do we really correct congenital heart defects? J Thorac Cardiovasc Surg . 1989;;97:1-9.
11 +
Lillehei CW, Varco RL, Cohen M, et al.  The first open heart correction of tetralogy of Fallot. Ann Surg . 1986;;204:490-501.
12 +
Fuster V, McGoon DW, Kennedy MA, Ritter DG, Kirklin JW.  Long-term evaluation (12-22 years) of open-heart surgery for tetralogy of Fallot. Am J Cardiol . 1980;;46:635-642.
13 +
James FW, Kaplan S, Chou TC.  Unexpected cardiac arrest in patients after surgical correction of tetralogy of Fallot. Circulation . 1975;;52:691-695.
14 +
Deanfield JE, Ho SY, Anderson RH, McKenna WJ, Allwork SP, Hallidie-Smith KA.  Late sudden death after repair of tetralogy of Fallot: a clinicopathologic study. Circulation . 1983;;67:626-631.
15 +
Quattlebaum TG, Varghese PJ, Neill CA, Donahoo JS.  Sudden death among post-operative patients with tetralogy of Fallot. Circulation . 1976;;54:289-293.
16 +
Chandar JS, Wolff GS, Garson A, et al.  Ventricular arrhythmias in postoperative tetralogy of Fallot. Am J Cardiol . 1990;;65:655-661.
17 +
Raj Behl PR, Sante P, Blesovsky A.  Surgical treatment of isolated coarctation of the aorta: 18 years experience. Thorax . 1987;;42:309-314.
18 +
Simsolo R, Grunfeld B, Gimenez M, et al.  Long-term systemic hypertension in children after successful repair of coarctation of the aorta. Am Heart J . 1988;;115:1268-1273.
19 +
Carpenter MA, Damman JF, Watson DD, Jedeikin R, Tompkins DG, Beller GA.  Left ventricular hyperkinesis at rest and during exercise in normotensive patients from 2 to 27 years after coarctation repair. J Am Coll Cardiol . 1985;;6:879-886.
20 +
Murphy JG, Gersh BJ, Warnes CA, et al.  The late survival after surgical repair of isolated ventricular septal defect (VSD). Circulation . 1989;;80( (suppl II) ):II-490. Abstract.
21 +
Lillehei CW, Varco RL, Cohen M, Warden HE, Patton C, Moller JH.  The first open-heart repairs of ventricular septal defect, atrioventricular communis, and tetralogy of Fallot using extracorporeal correction by cross-circulation: a 30-year follow-up. Ann Thorac Surg . 1986;;41:4-21.
22 +
Allen HD, Anderson RC, Noren GR, Moller JH.  Post-operative follow-up of patients with ventricular septal defect. Circulation . 1974;;50:465-471.
23 +
Murphy JG, Gersh BJ, McGoon MD, et al.  Long-term outlook after surgical repair of isolated atrial septal defect. N Engl J Med . 1990;;323:1645-1650.
24 +
Kopecky SL, Gersh BJ, McGoon MD, et al.  Long-term outcome of patients undergoing surgical repair of isolated pulmonary valvular stenosis. Circulation . 1988;;78:1150-1156.
25 +
Deanfield J, Camm J, Macartney F, et al.  Arrhythmia and late mortality after Mustard and Senning operations for transposition of the great arteries. J Thorac Cardiovasc Surg . 1988;;96:569-576.
26 +
Marx GR, Hougen TJ, Norwood WI, Fyler DC, Casteneda AR, Nadas AS.  Transposition of the great arteries with intact ventricular septum: results of Mustard and Senning operations in 123 consecutive patients. J Am Coll Cardiol . 1983;;1:476-483.
27 +
Duster MC, Bink-Boelkens M, Wampler D, Gillette PC, McNamara DG, Cooley DA.  Long-term follow-up of dysrhythmias following the Mustard procedure. Am Heart J . 1985;;109:1323-1326.
28 +
Clarkson PM, Barratt-Boyes BG, Neutze JM.  Late dysrhythmias and disturbances of conduction following Mustard operation for complete transposition of the great arteries. Circulation . 1976;;53:519-524.
29 +
Bender HW, Stewart JR, Merrill WH, Hammon JW Jr, Graham TP Jr.  Ten years' experience with the Senning operation for transposition of the great arteries: physiological results and late follow-up. Ann Thorac Surg . 1989;;47:218-223.

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