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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 2 مورد

Suspected cyanotic congenital heart disease (CHD) in the newborn: Rapid overview of initial evaluation and management

Suspected cyanotic congenital heart disease (CHD) in the newborn: Rapid overview of initial evaluation and management
Clinical findings
Presentation:
  • If the PDA is widely patent: Cyanosis may be the only abnormality noted
  • As the PDA closes within the first few days after birth: The neonate's clinical status can deteriorate rapidly; the neonate may present with life-threatening cardiorespiratory instability (ie, cardiogenic shock, cardiac arrest, severe cyanosis refractory to oxygen therapy, respiratory failure)
Examination findings:
  • Abnormal heart sounds (eg, S3 gallop, click, or single S2)
  • Pathologic murmurs (loud, harsh, pansystolic, diastolic)
  • Hepatomegaly
  • Diminished or absent lower extremity pulses (suggests CoA or IAA)
  • BP ≥10 mmHg higher in the arms than legs (suggests CoA or IAA)
Evaluation
Cardiology consult – Urgent consultation with a pediatric cardiologist is warranted in all newborns with suspected cyanotic CHD
Initial tests:
  • Pulse oximetry – SpO2 should be measured in both preductal (right hand) and postductal (either foot) locations
  • Upper and lower BP measurements – A BP gradient of ≥10 mmHg higher in the arms than legs suggests CoA or IAA
  • Chest radiograph – May identify a pulmonary cause of cyanosis (eg, pneumonia, pneumothorax); CHD is suggested based upon the size and shape of the cardiac silhouette, pulmonary vascular markings, and/or situs of the aortic arch
  • ECG – The ECG can be normal in some cyanotic CHD lesions during the neonatal period; abnormalities suggestive of CHD include left axis deviation for age, right atrial enlargement, left ventricular hypertrophy, or marked right ventricular hypertrophy
  • Evaluation for sepsis and other potential causes – In addition to the cardiac evaluation, newborns with concerning cardiorespiratory findings should undergo evaluation for other potential causes. In most cases, it is appropriate to perform a sepsis evaluation and administer empiric antibiotics pending culture results*.
Echocardiography – Provides detailed information on cardiac anatomy and function. Obtain if:
  • Clinical findings suggest cardiac disease (eg, differential cyanosis [ie, pre/postductal SpO2 difference of ≥4%], BP or pulse differential between upper and lower extremities, pathologic murmur, cardiomegaly on chest radiograph), or
  • Initial evaluation does not reveal another clear cause for the newborn's cyanosis (eg, lung disease)
Management
Transport to a center with pediatric cardiology expertise:
  • Transport arrangements should be made as soon as the diagnosis is suspected
  • May need to perform initial interventions prior to transport (eg, obtain vascular access, start vasoactive medications and/or PGE1 infusion); these decisions are generally made in consultation with the medical team at the accepting hospital
Supportive care:
  • Provide supportive respiratory care; intubate and initiate mechanical ventilation, if necessary
  • Obtain vascular access (through umbilical vessels, if feasible) and initiate continuous cardiorespiratory monitoring
  • Treat hypoglycemia, acidosis, and electrolyte abnormalities, if present
Manage shock:
  • For newborns with hypotension or other signs of shock, administer an initial fluid bolus of 5 to 10 mL/kg isotonic crystalloid (normal saline); repeat, if necessary; discontinue if the patient deteriorates during fluid administration
  • For fluid-refractory shock, start an IV inotropic agent:
    • Dopamine: Start at 5 mcg/kg per min and titrate up based on response; maximum dose: 15 mcg/kg per min, or
    • Epinephrine: Start at 0.05 mcg/kg/min and titrate up based on response; maximum dose: 1 mcg/kg per min
Empiric antibiotic therapy pending cultures* – For term newborns, the usual regimen is:
  • Ampicillin: 100 mg/kg per dose IV every 8 hours
  • Gentamicin: 4 mg/kg per dose IV every 24 hours
Prostaglandin therapy for newborns with ductal-dependent lesions:
  • Dose – Dosing for PGE1 (alprostadil) is as follows:
    • If echocardiography shows large PDA, start with a low dose: 0.01 mcg/kg per min IV
    • If the PDA is restrictive or the status of the PDA is unknown, start with standard dose: 0.05 mcg/kg per min IV
  • Adverse effects – PGE1 can cause apnea and hypotension; these risks increase with increasing PGE1 doses
  • Transport on PGE1 – Consider intubation prior to transport due to risk of apneaΔ
  • Deterioration after starting PGE1 – This suggests one of several rare congenital CHD defects associated with pulmonary venous obstruction (eg, obstructed TAPVC) or a restrictive atrial septum; urgent consultation with a pediatric cardiologist is advised
This table provides a rapid overview of the initial evaluation and stabilization of neonates with suspected cyanotic CHD. These lesions include, but are not limited to:
  • Right-sided obstructive lesions: TOF, PA/IVS, critical PS, tricuspid atresia, severe Ebstein anomaly
  • Left-sided obstructive lesions: HLHS, critical CoA, IAA, critical AS
  • Others: D-TGA, truncus arteriosus, TAPVC

This table is intended for use with other UpToDate content. For additional details, refer to UpToDate topic on initial evaluation and management of cyanotic CHD in the newborn and separate topics on each specific CHD lesion.

AS: aortic stenosis; BP: blood pressure; CHD: congenital heart disease; CoA: coarctation of the aorta; D-TGA: dextro transposition of the great arteries; ECG: electrocardiogram; IAA: interrupted aortic arch; IV: intravenous; PA/IVS: pulmonary atresia with intact ventricular septum; PDA: patent ductus arteriosus; PGE1: prostaglandin E1; PS: pulmonic stenosis; SpO2: peripheral oxygen saturation; TAPVC: total anomalous pulmonary venous connection; TOF: tetralogy of Fallot.

* When evaluating a neonate with suspected CHD, there are many other diagnostic considerations, including sepsis. The differential diagnosis varies depending on the presentation (eg, shock, severe cyanosis, respiratory distress). In most cases, it is appropriate to evaluate for sepsis and administer empiric antibiotic therapy pending culture results unless another specific etiology is promptly identified. Refer to UpToDate topics on evaluation of sepsis, shock, and cyanosis in newborns for additional details.

¶ PGE1 (alprostadil) infusion should be started promptly if a ductal-dependent CHD lesion is confirmed on echocardiography. If echocardiography is not readily available and there is strong clinical suspicion for ductal-dependent CHD based on the initial evaluation, PGE1 infusion should be started empirically while awaiting echocardiographic confirmation. Most forms of cyanotic CHD are ductal-dependent.

Δ When it is necessary to transfer a neonate receiving PGE1 to another medical facility with pediatric cardiology expertise, the care team should anticipate the potential for apnea during transport. At the author's institution, our usual practice is to electively intubate and mechanically ventilate infants prior to transport if they are receiving PGE1 because of the risk for apnea. However, practice varies and other tertiary care transport teams may not routinely intubate neonates in these circumstances.
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