Terlipressin as rescue therapy in hypercyanotic spells of Fallot tetralogy

• Published in: Anales de pediatría (Barcelona, Spain : 2003) Vol. 73; no. 1; pp. 35 - 38
• Main Authors: Azpilicueta Idarreta, M, Ibarra de la Rosa, I, Tejero Hernández, M A, Ulloa Santamaría, E, Jaraba Caballero, S, Frías Pérez, M A
• Format: Journal Article
• Language: Spanish
• Published: Spain 01-07-2010
• Subjects: Cyanosis - drug therapy; Cyanosis - etiology; Humans; Hypoxia - drug therapy; Hypoxia - etiology; Infant; Lypressin - analogs & derivatives; Lypressin - therapeutic use; Male; Tetralogy of Fallot - complications; Vasoconstrictor Agents - therapeutic use
• ISSN: 1695-9531
• DOI: 10.1016/j.anpedi.2010.03.014

Hypercyanotic spells, very common in Fallot tetralogy, are a medical emergency and can cause neurological damage or even death. They are produced by a dramatic decrease in pulmonary blood flow, with increased right-left shunt and a drop in systemic arterial saturation. Treatment includes alpha.-agonists (such as methoxamine or phenylephrine), which increase peripheral resistance, forcing the entry of blood through the infundibulum. However, they are not available in our environment. An alternative, still not described, could be terlipressin, a potent vasoconstrictor. We report a three months old infant, with Fallot tetralogy and hypoplastic pulmonary branches, who developed a severe hypoxaemic crisis during postoperative palliative surgery (Blalock-Taussig), no responsive to standard therapy. The administration of terlipressin in three hypercyanotic spells, was effective and reversed them, with a significant and sustained increase in arterial saturation. Although there is still no evidence, terlipressin may be an alternative to alpha.-agonists in these patients.