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The Case
A full-term infant with a prenatal diagnosis of heterotaxy presents with respiratory distress and hypoxia with a postnatal diagnostic radiograph revealing a significant differential in lung expansion.
Prenatal and Birth Histories
Born to a 27-year-old gravida 5, para 4 woman who presented in labor.
Estimated gestational age: 38 weeks based on last menstrual period.
Prenatal diagnosis of heterotaxy, with absent right eye, and concern for esophageal atresia or tracheoesophageal fistula because of absent stomach bubble on ultrasonography. Fetal echocardiography was performed, which showed levocardia, an interrupted inferior vena cava with a large azygous continuation to the superior vena cava.
Prenatal maternal laboratory findings: Blood type B positive, Coombs negative; rubella immune; group B Streptococcus–unknown; hepatitis B surface antigen–negative; HIV–negative; syphilis treponemal screening test–negative.
Delivery: The infant was born via vaginal delivery in vertex position. Apgar scores were 4, 5, and 7 at 1, 5, and 10 minutes, respectively. She had a weak cry and poor tone at delivery. Continuous positive airway pressure of 5 cm H2O was initiated. Fraction of inspired oxygen (Fio2) was slowly increased to 100% over the next 3 minutes after birth to maintain adequate oxygen saturation. At 7 minutes, the infant was noted to have poor respiratory effort and positive pressure ventilation was initiated. At 10 minutes after birth, she continued to have intermittent apnea and persistent hypoxia, leading to endotracheal intubation with minimal subsequent improvement of oxygen saturation. She was transported to the NICU without difficulty.
Presentation
Progression (Day 1)
Heart rate: 160 beats/min
Respiratory rate: 46 breaths/min
Blood pressure: 79/67 mm Hg
Oxygen saturation: 90% on 100% Fio2 delivered via mechanical ventilation
Temperature: 97.0°F (36.1°C)
Physical Examination (Day 1)
Measurements: Weight 3,690 g (84th percentile), length 47 cm (17th percentile), head circumference 32.5 cm (14th percentile).
General: Female infant in open warmer, orally intubated, and receiving mechanical ventilation.
Skin: …
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