Type of structural heart lesionPrevalence among children with congenital heart disease (%)Symptoms and clinical courseCharacteristics
Ventricular septal defect20 to 25Small defects: usually asymptomaticSmall defects: loud holosystolic murmur at LLSB (may not last throughout systole if defect is very small)
Medium or large defects: CHF, symptoms of bronchial obstruction, frequent respiratory infections
Medium and large defects: increased right-to-left ventricular impulses; thrill at LLSB; split or loud single S2; holosystolic murmur at LLSB without radiation; grade 2 to 5; may also hear a grade 1 or 2 mid-diastolic rumble
Atrial septal defect8 to 13Usually asymptomatic and incidentally found on physical examination or echocardiography; large defects can be present in infants with CHFGrade 2 or 3 systolic ejection murmur best heard at ULSB; wide split fixed S2; absent thrill; may have a grade 1 or 2 diastolic flow rumble at LLSB
Patent ductus arteriosus6 to 11May be asymptomatic; can cause easy fatigue, CHF, and respiratory symptomsContinuous murmur (grade 1 to 5) in ULSB (crescendo in systole and decrescendo into diastole); normal S1; S2 may be “buried” in the murmur; thrill or hyperdynamic left ventricular impulse may be present
Tetralogy of Fallot10Onset depends on severity of pulmonary stenosis; cyanosis may appear in infancy (2 to 6 months of age) or in childhood; other symptoms include hypercyanotic spells or decreased exercise toleranceCentral cyanosis; clubbing of nail beds; grade 3 or 4 long systolic ejection murmur heard at ULSB; may have holosystolic murmur at LLSB; systolic thrill at ULSB; normal to slightly increased S1; single S2
Pulmonary stenosis7.5 to 9Usually asymptomatic but may have symptoms secondary to pulmonary congestionSystolic ejection murmur (grade 2 to 5); heard best at ULSB radiating to infraclavicular regions, axillae, and back; normal or loud S1; variable S2; systolic ejection click may be heard at left sternal border and may vary with respiration
Coarctation of the aorta5.1 to 8.1Newborns and infants may present with CHF; older children are usually asymptomatic or may have leg pain or weaknessSystolic ejection murmur best heard over interscapular region; normal S1 and S2; decreased or delayed femoral pulse; may have increased left ventricular impulse
Aortic stenosis5 to 6Usually asymptomatic; symptoms may include dyspnea, easy fatigue, chest pain, or syncope; newborns and infants may present with CHFSystolic ejection murmur (grade 2 to 5) best heard at upper right sternal border with radiation to carotid arteries; left ventricular heave; thrill at ULSB or suprasternal notch
Transposition of the great arteries5Variable presentation depending on type; may include cyanosis or CHF in first week of lifeCyanosis; clubbing of nail beds; single S2; murmur may be absent or grade 1 or 2 nonspecific systolic ejection murmur; may have a grade 3 or 4 holosystolic murmur at LLSB and mid-diastolic murmur at apex
Total anomalous pulmonary venous connection2 to 3Onset of CHF at 4 to 6 weeks of ageGrade 2 or 3 systolic ejection murmur at ULSB; grade 1 or 2 mid-diastolic flow rumble at LLSB; wide split fixed S2
Tricuspid atresia1.4Early-onset cyanosis or CHF within the first month of lifeCyanosis; clubbing of nail beds; normal pulses; single S2; holosystolic murmur at LLSB or midsternal border; murmur may be absent; mid-diastolic flow murmur at apex may be present
Hypoplastic left heart syndromeRareMay be asymptomatic at birth, with cyanosis and CHF developing with duct closureHyperdynamic precordium; single S2; nonspecific grade 1 or 2 systolic ejection murmur along left sternal border
Truncus arteriosusRareOnset of CHF in first few weeks of life; minimal cyanosisIncreased cardiac impulses; holosystolic murmur (ventricular septal defect); mid-diastolic rumble