- Ductal-dependent lesions typically present with sudden-onset cardiogenic shock at 1 to 2 weeks of life and require immediate prostaglandin E1 infusion.
- Congestive heart failure typically presents in the first 6 months of infancy in children with left-to-right shunting lesions and requires immediate stabilization and medical management.
- Aortic coarctation may present with hypertension and the complications of hypertension. The median age of presentation is 5 years. Blood pressure will be higher in the upper extremities compared with the lower extremities.
- The number of survivors of cardiac surgery for congenital heart lesions is rapidly increasing, and emergency physicians should be aware of common complications, such as arrhythmias, residual or recurrent lesions, and endocarditis.
- Children commonly have benign cardiac murmurs, which are usually softer, lower pitched, and early to midsystolic compared with pathologic murmurs.
The term “congenital heart disease” encompasses a wide variety of lesions. The emergency physician must not only recognize and manage previously undiagnosed congenital heart disease but also anticipate complications in a rapidly growing population of survivors of congenital heart surgery. This chapter reviews the common presentations and management of cyanotic and acyanotic congenital heart lesions and also discusses complications seen in postoperative congenital cardiac patients.
Congenital heart lesions occur in approximately 8 in 1000 live births in the United States, which includes lesions ranging from mild to severe but does not include common lesions such as bicuspid aortic valve (1%–2% of the population) or mitral valve prolapse. Significant congenital heart lesions can go undetected by prenatal ultrasound and may not present immediately after birth.1 Overall, neither gender is predominant, but individual lesions may be more common in either males or females. The vast majority of patients will have isolated congenital heart lesions, which are multifactorial in origin. Approximately 10% of cases can be attributed to genetic causes. Many genetic syndromes (e.g., the trisomies, connective tissue disorders) and teratogens (e.g., congenital rubella infection) are associated with a higher risk of specific congenital heart lesions (Table 47–1). Most patients present during infancy (Fig. 47–1).
Table 47-1. Selected Syndromes Associated with Congenital Heart Disease2,3 |Favorite Table|Download (.pdf)
Table 47-1. Selected Syndromes Associated with Congenital Heart Disease2,3
Congenital Heart Lesions
Down syndrome (trisomy 21)
1 in 1000
Decreased tone, epicanthal folds, hypothyroidism, esophageal, and duodenal atresia
Atrial septal defect, ventricular septal defect
Klinefelter syndrome (47 XXY)
Boys only 1 in 1000
Mitral valve prolapse, left ventricular dysfunction
Noonan syndrome (AD or new mutation)
1:1000 to 1:2500
Webbed neck, wide-set eyes, epicanthal folds, short stature, lymphedema, blood dyscrasias
Pulmonic valve abnormalities, pulmonic stenosis, hypertrophic cardiomyopathy
Turner syndrome (XO syndrome)
Girls only 1:2000
Web neck, lymphedema, lack of secondary sexual characteristics, musculoskeletal and renal defects
Coarctation of the aorta, bicuspid aortic valve, secondary hypertension
DiGeorge syndrome (deletions, mutations)
Hypocalcemia, immunodeficiency, hypoparathyroidism, thymic aplasia
Conotruncal anomalies, interrupted ...
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