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Down Syndrome With Congenital Heart Malformation FREE

Sang C. Park, MD; Robert A. Mathews, MD; James R. Zuberbuhler, MD; Richard D. Rowe, MD; William H. Neches, MD; Cora C. Lenox, MD
[+] Author Affiliations

Reprint requests to Division of Pediatric Cardiology, Children's Hospital of Pittsburgh, 125 DeSoto St, Pittsburgh, PA 15213 (Dr Park).


Am J Dis Child. 1977;131(1):29-33. doi:10.1001/archpedi.1977.02120140031003.
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• Two hundred fifty-one patients with Down syndrome and congenital heart disease were seen at two institutions in recent years. Diagnosis of congenital heart disease was based on clinical (41%), catheterization (38%), surgical (11%), or autopsy data (10%). The most common lesions were endocardial cushion defect (43%), ventricular septal defect (32%), secundum atrial septal defect (10%), tetralogy of Fallot (6%), and isolated patent ductus arteriosus (4%). Thirty percent had multiple cardiac defects. The most common associated lesions were patent ductus arteriosus (16%) and pulmonic stenosis (9%). Twenty-five percent of the patients underwent cardiac surgery. Mortality in the 68 patients undergoing surgery was 26% for open heart procedures and 11% for closed heart surgery. In 32% of nonsurgically treated patients with large left-toright shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.

(Am J Dis Child 131:29-33, 1977)

REFERENCES

Polani PE:  Chromosomal abnormalities and congenital heart disease . Guys Hosp Rep 117:323-337, 1968;.
Rowe RD, Uchida IA:  Cardiac malformation in mongolism: A prospective study of 184 mongoloid children . Am J Med 31:726-735, 1961;.
Leck I:  Incidence and epidemicity of Down's Syndrome . Lancet 2:457-460, 1966;.
Stark CR, Mantel N:  Lack of seasonal or temporal-spatial clustering of Down's Syndrome births in Michigan . Am J Epidemoil 86:199-213, 1967;.
Hoffman JE:  Natural history of congenital heart disease: Problems in its assessment with special reference to ventricular septal defects . Circulation 37:97-125, 1968;.
Record RG, Smith A:  The incidence, mortality and sex distribution of mongoloid defectives . Br J Prev Soc Med 9:10-15, 1955;.
Carter CO:  A life table for mongols with the causes of death . J Ment Defic Res 2:64-74, 1958;.
Hall B:  Mongolism in newborns: A clinical and cytogenetic study . Acta Paediatr Scand 53( (suppl 154) ):5-95, 1964;.
Fabia J, Drolette M:  Life tables up to age 10 for mongols with and without congenital heart disease . J Ment Defic Res 14:235-242, 1970;.
Watson H: Classification of Heart Disease in Childhood . Scientific Council on Pediatric Cardiology of the International Society of Cardiology, 1970;.
Campbell M:  Causes of malformations of the heart . Br Med J 2:895-904, 1965;.
Cullum L, Liebman J:  The association of congenital heart disease with Down's Syndrome (mongolism) . Am J Cardiol 24:354-357, 1969;.
Shaher RM, Farina MA, Porter IH, et al:  Clinical aspects of congenital heart disease in mongolism . Am J Cardiol 29:497-503, 1972;.
Tandon R, Edwards JE:  Cardiac malformations associated with Down's syndrome . Circulation 47:1349-1355, 1973;.
Gerstmann PE, Baum D, Guntheroth WG:  Prevalence of cardiovascular disease in the retarded . JAMA 219:1171-1175, 1972;.
Chi TP, Krovetz LJ:  The pulmonary vascular bed in children with Down's syndrome . J Pediatr 86:533-538, 1975;.
Kontras SB, Boderbender JG:  Abnormal capillary morphology in congenital heart disease . Pediatrics 37:316-322, 1966;.
Ferguson CF, Kendig EL Jr:  Pediatric otolaryngology , in Disorders of the Respiratory Tract in Children , vol 2. Philadelphia, WB Saunders Co, 1972;, p 1,031.

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References

Polani PE:  Chromosomal abnormalities and congenital heart disease . Guys Hosp Rep 117:323-337, 1968;.
Rowe RD, Uchida IA:  Cardiac malformation in mongolism: A prospective study of 184 mongoloid children . Am J Med 31:726-735, 1961;.
Leck I:  Incidence and epidemicity of Down's Syndrome . Lancet 2:457-460, 1966;.
Stark CR, Mantel N:  Lack of seasonal or temporal-spatial clustering of Down's Syndrome births in Michigan . Am J Epidemoil 86:199-213, 1967;.
Hoffman JE:  Natural history of congenital heart disease: Problems in its assessment with special reference to ventricular septal defects . Circulation 37:97-125, 1968;.
Record RG, Smith A:  The incidence, mortality and sex distribution of mongoloid defectives . Br J Prev Soc Med 9:10-15, 1955;.
Carter CO:  A life table for mongols with the causes of death . J Ment Defic Res 2:64-74, 1958;.
Hall B:  Mongolism in newborns: A clinical and cytogenetic study . Acta Paediatr Scand 53( (suppl 154) ):5-95, 1964;.
Fabia J, Drolette M:  Life tables up to age 10 for mongols with and without congenital heart disease . J Ment Defic Res 14:235-242, 1970;.
Watson H: Classification of Heart Disease in Childhood . Scientific Council on Pediatric Cardiology of the International Society of Cardiology, 1970;.
Campbell M:  Causes of malformations of the heart . Br Med J 2:895-904, 1965;.
Cullum L, Liebman J:  The association of congenital heart disease with Down's Syndrome (mongolism) . Am J Cardiol 24:354-357, 1969;.
Shaher RM, Farina MA, Porter IH, et al:  Clinical aspects of congenital heart disease in mongolism . Am J Cardiol 29:497-503, 1972;.
Tandon R, Edwards JE:  Cardiac malformations associated with Down's syndrome . Circulation 47:1349-1355, 1973;.
Gerstmann PE, Baum D, Guntheroth WG:  Prevalence of cardiovascular disease in the retarded . JAMA 219:1171-1175, 1972;.
Chi TP, Krovetz LJ:  The pulmonary vascular bed in children with Down's syndrome . J Pediatr 86:533-538, 1975;.
Kontras SB, Boderbender JG:  Abnormal capillary morphology in congenital heart disease . Pediatrics 37:316-322, 1966;.
Ferguson CF, Kendig EL Jr:  Pediatric otolaryngology , in Disorders of the Respiratory Tract in Children , vol 2. Philadelphia, WB Saunders Co, 1972;, p 1,031.

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