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Cardiac evaluation in infants and children: Features that distinguish heart disease from innocent murmurs and criteria for referral

Cardiac evaluation in infants and children: Features that distinguish heart disease from innocent murmurs and criteria for referral
  Features associated with cardiac disease Features associated innocent murmurs Criteria for referral
Features in the history
 
  • Parent or sibling with CHD*
  • Abnormal prenatal ultrasound, fetal echocardiogram, or prenatal maternal condition associated with increased risk of CHD*
  • Underlying genetic disorder associated with increased risk of CHD*
  • Age <1 year
  • Symptoms suggestive of heart disease (eg, respiratory difficulties, cyanosis, poor growth, poor feeding [in infants], diaphoresis, chest pain, syncopal episodes)Δ
  • Negative family history
  • Normal prenatal ultrasound
  • Nonsyndromic
  • Age >2 years
  • Asymptomatic
  • Abnormal fetal echocardiogram
  • Underlying genetic disorder associated with increased risk of CHD*
  • Symptoms suggestive of heart diseaseΔ
Physical examination findings:
Murmurs
  • Grade ≥3 intensity
  • Holosystolic timing
  • Maximum intensity at the left upper sternal border
  • Harsh or blowing quality
  • Increased intensity with upright position§
  • Diastolic murmur
  • Grade ≤2 intensity
  • Short systolic duration (ie, not holosystolic and not diastolic)
  • Minimal radiation
  • Musical or vibratory quality
  • Softer intensity when the patient is sitting compared with when the patient is supine§
  • Grade ≥3 intensity
  • Holosystolic timing
  • Maximum intensity at the left upper sternal border
  • Harsh or blowing quality
  • Increased intensity with upright position§
  • Diastolic murmur
Other heart sounds
  • Abnormal S2 (eg, fixed or wide splitting, single S2, loud P2)
  • Gallop rhythm (S3 or S4)
  • Systolic click
  • Friction rub
  • Quiet precordium
  • Normal S2 with normal physiologic splitting
  • No gallop, click, or rub
  • Abnormal S2 (eg, fixed or wide splitting, single S2, loud P2)
  • Gallop rhythm (S3 or S4)
  • Systolic click
  • Friction rub
Other findings
  • Abnormal vital signs (eg, tachycardia, bradycardia, >10 mmHg SBP gradient between the right arm and leg)
  • Abnormal pulses (eg, weak or absent femoral pulses, generalized decreased pulses, bounding pulses)
  • Hepatomegaly
  • Extracardiac congenital anomalies (particularly skeletal abnormalities)
  • Normal vital signs
  • Normal pulses
  • No other findings
  • >10 mmHg SBP gradient between the right arm and leg
  • Abnormal pulses (eg, weak or absent femoral pulses, generalized decreased pulses, bounding pulses)
Diagnostic test findings:§
  • Abnormal pulse oximetry¥
  • Abnormal chest radiograph (eg, cardiomegaly, increased pulmonary vascular markings, pulmonary edema)
  • Abnormal ECG (eg, LVH, RVH, abnormal axis, ischemic changes, arrhythmia, delta wave, prolonged QTc)
  • Normal pulse oximetry¥
  • Normal chest radiograph
  • Normal ECG
  • Abnormal newborn pulse oximetry screen¥
  • Cardiomegaly or pulmonary edema (without a noncardiac explanation) on chest radiograph
  • Abnormal ECG
CHD: congenital heart disease; S2: second heart sound; P2: pulmonary valve closure sound; S3: third heart sound; S4: fourth heart sound; SBP: systolic blood pressure; ECG: electrocardiogram; LVH: left ventricular hypertrophy; RVH: right ventricular hypertrophy; QTc: corrected QT interval; PPS: peripheral pulmonary stenosis.
* For a summary of maternal, prenatal, familial, and genetic factors associated with increased risk of CHD, refer to separate UpToDate content on identifying newborns with critical CHD.
¶ CHD is more likely to be diagnosed in infants <1 year; however, innocent murmurs also commonly occur at this age, particularly the PPS murmur, which is caused by turbulence across the underdeveloped branch pulmonary arteries. The PPS murmur is characterized by a grade 1 to 2, medium- to high-pitched midsystolic ejection murmur heard best at the upper left sternal border, radiating to the axilla and back. It disappears typically by age 6 to 12 months.
Δ For further discussion of symptoms associated with pediatric cardiac disease, refer to separate UpToDate content on suspected heart disease in infants and children.
Innocent murmurs also commonly occur at this location. Other features of the examination (eg, the quality of S2 and systolic clicks) help distinguish the basis of the murmur. For additional details, refer to separate UpToDate content on evaluating heart murmurs in children.
§ Most innocent murmurs (with the exception of cervical venous hum) typically become softer in the upright position.
¥ For details of pulse oximetry screening in newborns to identify critical heart disease, refer to separate UpToDate content.
‡ Chest radiograph is generally warranted only if there are respiratory symptoms and/or cyanosis. Chest radiograph and/or ECG are not required in the evaluation of a child whose history and physical examination findings are consistent with an innocent murmur. For a summary of characteristic ECG and chest radiograph findings in specific CHD lesions, refer to separate UpToDate content.
References:
  1. Frank JE, Jacobe KM. Evaluation and management of heart murmurs in children. Am Fam Physician 2011; 84:793.
  2. Kang G, Xiao J, Wang Y, et al. Prevalence and clinical significance of cardiac murmurs in schoolchildren. Arch Dis Child 2015.
  3. McCrindle BW, Shaffer KM, Kan JS, et al. Cardinal clinical signs in the differentiation of heart murmurs in children. Arch Pediatr Adolesc Med 1996; 150:169.
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