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Pulmonary atresia with intact ventricular septum and antenatal left ventricular failure

  • Saad Khoshhal (a1), George G. S. Sandor (a1) and Walter J. Duncan (a1)

Abstract

Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.

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Corresponding author

Correspondence to: Walter Duncan MD, Department of Pediatrics, Division of Cardiology, British Columbia Children's Hospital, 4480 Oak St., Vancouver, B.C. V6H 3V4, Canada. Tel: +1 604 875 2855; Fax: +1 604 875 3463; E-mail: wduncan@cw.bc.ca

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References

Freedom RM. General morphological considerations. In: Freedom RM (ed.). Pulmonary Atresia with Intact Ventricular Septum. Futura Publishing, Mount Kisco, NY, 1989, pp 1736.
Daubeney PEF, Delany DJ, Anderson RH, et al., for the United Kingdom and Ireland Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum. Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study. J Am Coll Cardiol 2002; 39: 16701679.
Galindo A, Drant SE. Pulmonary atresia with intact ventricular septum. PA-IVS: diagnostic and interventional cardiac catheterization in the neonate. Prog Pediatr Cardiol 2001; 13: 177182.
Powell AJ, Mayer JE, Lang P, Lock JE. Outcome in infants with pulmonary atresia, intact ventricular septum and right ventricle-dependent coronary circulation. J Am Coll Cardiol 2000; 86: 12721274.
Laks H, Plunkett MD. Surgical management of pulmonary atresia with intact ventricular septum. Prog Pediatr Cardiol 2001; 13: 183197.

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