Article Text

  1. W. N. Evans1,
  2. G. A. Mayman1,
  3. R. J. Acherman1,
  4. A. Rothman1
  1. 1Children's Heart Center and University of Nevada, School of Medicine, Las Vegas, NV.


Background Literature reports limited experience with serial brain natriuretic peptide (BNP) levels, as a clinical marker, in pediatric patients with congenital heart disease either preoperatively or postoperatively.

Objective To validate the clinical usefulness of rapid, serial BNP level monitoring in patients with complex congenital heart disease.

Methods We describe an infant whose elevated BNP levels were the most sensitive marker of ventricular overload after Norwood I.

Case An asymptomatic 3.3 kg male infant, delivered vaginally at term with an uncomplicated prenatal history, underwent echocardiography within 24 hours of age for cardiac murmur evaluation. Echocardiographic findings were consistent with hypoplastic left heart syndrome, including situs solitus, a 2.5 mm ascending aortic root, moderate tricuspid valve insufficiency, a 5 mm interatrial communication, normal pulmonary venous return, and a restrictive patent ductus arteriosus. After neonatal stabilization, the patient underwent a classic Norwood I operation using bovine pericardial arch augmentation and a 3.5 mm Gore-Tex systemic-to-pulmonary artery shunt. During follow-up, echocardiography showed normal systemic right ventricular systolic function, moderate tricuspid valve insufficiency, no neoaortic valve insufficiency, pulsatile abdominal aorta Doppler flow profile, a peak Doppler flow velocity of < 2 m/s across the augmented-arch-descending aorta anastomosis, and a well-functioning systemic-to-pulmonary artery shunt. Repeated BNP levels were consistently elevated (> 400 pg/mL). The patient was scheduled for cardiac catheterization to evaluate the aortic arch repair and determine suitability for early cavopulmonary anastomosis.

Discussion History, physical examination, pulse oximetry, electrocardiography, and echocardiography are the “gold standard” methods for outpatient management of complex congenital heart disease. In this case, the lack of congestive heart failure resulted in a negative medical history. In addition, the echocardiogram demonstrated good systemic ventricular function and no significant Doppler flow disturbance at the site of the arch repair. Elevated BNP levels, however, suggested systemic ventricular overload. Such monitoring proved to be the most sensitive indicator of ventricular overload.

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