Long term follow-up of children with Down syndrome with cardiac lesions

P. Mathew, D. Moodie, R. Sterba, Eliot Rosenkranz, E. Rosenkranz, A. Homa

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Two hundred and eighty four patients with Down Syndrome (DS) were seen between 1951-1989 with 114 (40.1%) having a cardiac murmur at presentation. A definitive cardiac diagnosis was established in 47 (41%) patients, of which 38 had long term follow-up. Fifteen (33%) patients had atrioventricular canals. There were 21 males and 17 females, with a mean age of 5.3 years. Fifteen (39%) patients were in functional class (FC) I, 16 (42%) in FC II, six (15%) in FC III, and one patient in FC IV at the time of presentation. There were 18 survivors (13 in the surgical group and five in the nonsurgical group) and 20 nonsurvivors (four in the surgical group and 16 in the nonsurgical group). Causes of death in the nonsurgical group included congestive heart failure, pneumonia, and pulmonary vascular disease, and occured at a mean age of 8.4 years. Post-operative complications accounted for deaths in three of the four surgical patients. The survivors in the surgical group are presently in FC I/II. In the nonsurgical group, there was increased mortality, especially in those who presented in an earlier era, and a deterioration in functional class on follow-up due to the development of pulmonary vascular disease. Our data suggest that a) patients with Down syndrome and heart disease are helped by cardiac surgery with stabilization and improvement of their functional class; b) deterioration in functional class is seen in patients with Down syndrome with cardiac lesions who are managed surgically and c) mortality remains high in such patients treated nonsurgically due to development of pulmonary vascular disease and congestive heart failure. Therefore, early diagnosis and surgical correction where appropriate is a key issue in the management of children with Down syndrome.

Original languageEnglish
Pages (from-to)569-574
Number of pages6
JournalClinical Pediatrics
Volume29
Issue number10
StatePublished - Dec 1 1990
Externally publishedYes

Fingerprint

Down Syndrome
Vascular Diseases
Lung Diseases
Survivors
Heart Failure
Heart Murmurs
Mortality
Thoracic Surgery
Early Diagnosis
Cause of Death
Heart Diseases
Pneumonia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Mathew, P., Moodie, D., Sterba, R., Rosenkranz, E., Rosenkranz, E., & Homa, A. (1990). Long term follow-up of children with Down syndrome with cardiac lesions. Clinical Pediatrics, 29(10), 569-574.

Long term follow-up of children with Down syndrome with cardiac lesions. / Mathew, P.; Moodie, D.; Sterba, R.; Rosenkranz, Eliot; Rosenkranz, E.; Homa, A.

In: Clinical Pediatrics, Vol. 29, No. 10, 01.12.1990, p. 569-574.

Research output: Contribution to journalArticle

Mathew, P, Moodie, D, Sterba, R, Rosenkranz, E, Rosenkranz, E & Homa, A 1990, 'Long term follow-up of children with Down syndrome with cardiac lesions', Clinical Pediatrics, vol. 29, no. 10, pp. 569-574.
Mathew P, Moodie D, Sterba R, Rosenkranz E, Rosenkranz E, Homa A. Long term follow-up of children with Down syndrome with cardiac lesions. Clinical Pediatrics. 1990 Dec 1;29(10):569-574.
Mathew, P. ; Moodie, D. ; Sterba, R. ; Rosenkranz, Eliot ; Rosenkranz, E. ; Homa, A. / Long term follow-up of children with Down syndrome with cardiac lesions. In: Clinical Pediatrics. 1990 ; Vol. 29, No. 10. pp. 569-574.
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