Case history
Case history #1
A 1-day-old infant in the general care nursery born at full term by uncomplicated spontaneous vaginal delivery is noted to have cyanosis of the oral mucosa. The baby otherwise appears comfortable. On exam, respiratory rate is 40 and pulse oximetry is 80%. A right ventricular lift is palpated, S1 is normal, S2 is single, and a harsh 3/6 systolic ejection murmur is heard at the left upper sternal border.
Case history #2
A 1-day-old infant in the general care nursery born at full term by uncomplicated spontaneous vaginal delivery is noted to have a murmur on exam. The baby otherwise appears well. On exam, respiratory rate is 40 and pulse oximetry is 96%. Precordium is normoactive. With auscultation, S1 is normal, S2 is single, and a 2/6 systolic ejection murmur is heard at the left upper sternal border.
Other presentations
Cyanosis occurs if severe right ventricular outflow tract obstruction forces blood returning to the right side of the heart to be shunted right to left across the ventricular septal defect and out to the systemic circulation, bypassing the lungs. Hypercyanotic (tet) spells may present as episodic, increasing cyanosis in a baby with TOF. The baby is typically crying and breathing deeply and rapidly, but may not be in significant respiratory distress. The typical murmur of TOF may disappear during the spell. This presentation is potentially life-threatening and requires rapid intervention. Alternatively, a baby may be severely cyanotic at birth.
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