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Sudden death during a change in treatment for pulmonary hypertension

Published online by Cambridge University Press:  22 April 2005

Henrik Brun
Affiliation:
Department of Paediatrics, National Hospital (Rikshospitalet), Oslo, Norway
Henrik Holmström
Affiliation:
Department of Paediatrics, National Hospital (Rikshospitalet), Oslo, Norway
Erik Thaulow
Affiliation:
Department of Paediatrics, National Hospital (Rikshospitalet), Oslo, Norway

Abstract

We describe an infant with an atrioventricular septal defect and separate valvar orifices for the right and left ventricles, the so-called primum defect, who presented with serious pulmonary hypertension. Treatment with sildenafil was changed to intravenous epoprostenol due to lack of any measurable effects of the initial therapy as shown by echocardiography. The girl died three days after the change in treatment. We suggest that a mismatch between ventilation and perfusion contributed significantly to her death.

Type
Brief Report
Copyright
© 2005 Cambridge University Press

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References

Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99: 18581865.Google Scholar
Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation 1999; 99: 11971208.Google Scholar
Yamaki S, Abe A, Sato K, Takahashi T. Microatelectasis in patients with secundum atrial septal defect and its relation to pulmonary hypertension. Jpn Circ J 1997; 61: 384389.Google Scholar
Castro PF, Bourge RC, McGiffin DC, et al. Intrapulmonary shunting in primary pulmonary hypertension: an observation in two patients treated with epoprostenol sodium. Chest 1998; 114: 334336.Google Scholar
Bush A, Busst CM, Knight WB, Shinebourne EA. Effects of infusion of prostacyclin on anatomical intrapulmonary right to left shunt: a useful model of human hypoxic vasoconstriction? Clin Sci 1989; 76: 143149.Google Scholar
Falk A, Lookstein RA, Mitty HA. Flolan infusion interruption: a lethal complication during venous access. J Vasc Interv Radiol 2001; 12: 667668.Google Scholar
Andrews R, Tulloh R, Magee A, Anderson D. Atrial septal defect with failure to thrive in infancy: hidden pulmonary vascular disease? Pediatr Cardiol 2002; 23: 528530.Google Scholar
Haworth SG. Pulmonary vascular disease in secundum atrial septal defect in childhood. Am J Cardiol 1983; 51: 265272.Google Scholar