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General Anesthesia for Cardiac Catheterization in a Child with Trisomy 14 Mosaicism

Kunst, Gudrun MD, DEAA; Gillbe, C MB, ChB, FRCA

doi: 10.1213/01.ANE.0000156687.85500.4B
Letters to the Editor: Letters & Announcements

Department of Anaesthetics, King’s College Hospital, (Kunst)

Department of Anaesthesia, Royal Brompton Hospital, London, United Kingdom (Gillbe)

Trisomy 14 mosaicism is an extremely rare genetic disorder first described in the mid 1970s (1). Only 18 live-born infants and children have been presented in nonanesthetic journals (2–4). For general anesthesia relevant clinical findings in patients with trisomy 14 mosaicism include growth and psychomotor retardation, micrognathia, a short neck, and congenital heart disease including tetralogy of Fallot, atrial septal defect, ventricular septal defect, and patent ductus arteriosus (3). Therefore, the anesthesiologist needs to be aware of two major anesthetic challenges: difficult airway (5,6) and cardiac abnormalities (7).

A 12-mo-old female patient with trisomy 14 mosaicism and tetralogy of Fallot was scheduled for cardiac catheterization. Weight and height were below the third centile. Examination revealed micrognathia, a small right ear, bilateral coloboma of the iris, clubbing of her fingers, and tachypnea, and mild crackles were heard at both lung bases (Fig. 1). Oxygen saturation by pulse oximetry was 93% on air and she did not show with cyanotic spells. She had a 3–4/6 ejection systolic murmur all over her precordium. She was premedicated with oral midazolam and general anesthesia was induced with sevoflurane. After IV atracurium, laryngoscopy revealed a grade III Cormack and Lehane (8,9) score. Therefore a bougie was inserted below the epiglottis before an endotracheal tube was inserted over the bougie. General anesthesia was maintained with sevoflurane, air, and oxygen. The patient was hemodynamically stable during the 90-min procedure. Cardiac catheterization revealed increased right ventricular pressures and ventriculography confirmed tetralogy of Fallot. Removal of the tracheal tube at the end of the procedure was uneventful, as was recovery from general anesthesia.

Figure 1

Figure 1

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We thank the patient’s mother for her permission by written consent to use a photograph of her daughter.

Gudrun Kunst, MD, DEAA

Department of Anaesthetics

King’s College Hospital

C. Gillbe, MB, ChB, FRCA

Department of Anaesthesia

Royal Brompton Hospital

London, United Kingdom

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© 2005 International Anesthesia Research Society