History And Physical Examination Findings That Should Raise Suspicion For CHD In An Infant Or Child With Tachypnea, Cyanosis, And/Or Shock

History of poor feeding for several days to weeks preceding illness: Less than 3 ounces or greater than 40 minutes per feed or poor weight gain

 

Absence of fever in history or in ED: Temperature less than 38.3°C (100.4°F)

 

Upper extremity hypertension or decreased lower extremity blood pressure as compared to upper extremity: Should be apparent by 5 days of age

 

Oxygen desaturation out of proportion of what would be expected based on physical examination or differential between upper and lower extremity: Less than 95% in both extremities or greater than 4% difference between upper and lower extremity

 

Abnormal femoral pulses: Absent or diminished relative to the brachial pulses

 

Signs of congestive heart failure: Facial edema, hepatomegaly (especially greater than 2.5 cm below costal margin), pulmonary edema

 

Abnormal cardiac examination, especially 1 of the 6 cardinal signs:

Pansystolic murmur

Intensity of the murmur greater than or equal to grade 3

Point of maximal intensity at the upper left sternal border

Harsh quality of murmur

Early mid-systolic click

Abnormal second heart sound

Non-Cardiogenic Etiologies In The Differential Diagnosis Of CHD

Respiratory:

Bronchiolitis

Pneumonia

Spontaneous pneumothorax

Laryngomalacia

Pulmonary hemangioma

Cystic adenomatoid malformation

Reactive airways disease

 

Hemodynamic:

Sepsis

Anaphylaxis

 

Gastrointestinal:

Gastroesophageal reflux

Tracheoesophageal fistula

 

Other:

Non-accidental trauma

 

Toxic/Metabolic:

Methemoglobinemia

Toxic ingestion

Congenital adrenal hyperplasia

Congenital Heart Disease In Children: Key Points

1. Because sepsis is strongly suspected in any neonate presenting to the emergency department in distress, a complete diagnostic evaluation for infection should be performed, and antibiotic therapy should be initiated concomitant with the evaluation for CHD.

2. Despite recent advances in antenatal ultrasound, the majority of patients with CHD are diagnosed after delivery.

3. Functional closure of the DA may be delayed in children with CHD. As a result, patients with ductal-dependent lesions will become symptomatic after discharge from the neonatal nursery.

4. The timing of presentation of CHD is bimodal. Patients with ductal-dependent defects will present in the first month of life, while patients with predominantly left-to-right shunting lesions will present between 2-6 months of age.

5. A ductal-dependent CHD should be considered in any neonate less than 1 month of age who presents with sudden cardiovascular collapse. These lesions have the highest incidence of mortality prior to surgical correction.

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Congenital Heart Disease In Children: Risk Management Pitfalls

1. “If the patient had CHD, the diagnosis would have been made in utero or prior to leaving the newborn nursery.” Recent studies estimate that greater than 60% of CHD cases are not diagnosed on antenatal ultrasound. Most neonates with CHD are clinically asymptomatic immediately after birth. Even when abnormalities such as a murmur are detected on physical examination, many will not be directly referred to a pediatric cardiologist.

2. “The patient is greater than 1 week old, so the diagnosis cannot be a ductal-dependent CHD. The DA should have closed days ago.” Patients with ductal-dependent cardiac disease, especially ductal-dependent systemic blood flow, may have persistence of DA patency beyond 24 hours of life as a result of increased DA blood flow. Cardiovascular collapse in a child less than 1 month of age should be considered as a CHD with ductal-dependent systemic blood flow until proven otherwise.

3. “The infant is febrile. We should evaluate and treat for sepsis. CHD is not likely.” While sepsis is more common and should be suspected in any neonate presenting to the emergency department with tachypnea, cyanosis, or shock, a diagnosis of CHD should not be excluded based solely on the presence of fever. In fact, the presentation of a patient with CHD may be precipitated by an infectious process.

4. “The blood pressure and SpO2 obtained in triage are normal. I’m reassured.” Infants presenting to the emergency room in distress should have blood pressure and SpO2 measurements performed on the right upper extremity and one lower extremity. A discrepancy between the two extremities is suggestive of CHD.

5. “No one can hear a murmur on this infant. Therefore, CHD is not likely.” In many patients with CHD, especially those presenting with marked tachycardia, a murmur will not be present or will be difficult to auscultate. The presence of a murmur should raise suspicions for CHD, but the absence of a murmur does not exclude the diagnosis.

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Congenital Heart Disease In Children: Case Study

Case Study:

It’s January, the middle of “RSV season,” when a 2-week-old infant is brought to the emergency department by his mother because he was “breathing fast all night.” His birth history is unremarkable, and his mother claims that he had been doing well until yesterday breastfeeding, urinating, and stooling regularly. In triage, physical examination is remarkable for an irritable child with a respiratory rate (RR) of 60 breaths per minute, a heart rate (HR) of 170 beats per minute, right upper extremity blood pressure of 70/50 mmHg, axillary temperature of 37°C (99°F), and oxygen saturation as determined by pulse oximetry of 85%. Skin and mucous membranes appear dry. Extremities are cool with a capillary refill time of 4 seconds. Nasal flaring and moderate intercostals retractions are noted, and expiratory wheezing is heard bilaterally. No murmur is noted, and the liver edge is 3 cm below the right costal margin.

Supplemental oxygen is administered by a facemask and SpO2 increases to 90%. The initial concerns are RSV bronchiolitis or sepsis. An inhaled albuterol treatment is started, a capillary blood gas is obtained, intravenous access is promptly accomplished, and he receives two 20 mL/kg fluid boluses with no improvement. In fact, vital signs have deteriorated to RR 60, HR 180, BP 62/38, SpO2 86%, and crackles are auscultated on repeat examination of lungs. Extremities remain cool and mottled, and liver is now 5 cm below costal margin. A chest x-ray shows a normal sized heart, prominent pulmonary vascular markings, and mild bilateral hyperinflation. Arterial blood gas results are reported as follows: pH 7.26, pCO2 20 torr, and pO2 60 torr. At this point, one of the residents asks, “Could this patient have congenital heart disease?”

Conclusion to the above case study…

The patient presented in the opening vignette was ultimately diagnosed with critical aortic stenosis. Presentation to the emergency department occurred following delayed closure of the DA. The abrupt development of symptoms and presence of hepatomegaly provided early clues to the diagnosis, but the patient’s clinical deterioration following treatment with inhaled albuterol and aggressive intravenous fluid boluses increased the likelihood of CHD. The patient was subsequently endotracheally intubated, administered PGE via continuous infusion, and transferred to a tertiary care facility for surgical correction.

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