Hostname: page-component-8448b6f56d-mp689 Total loading time: 0 Render date: 2024-04-19T01:17:08.344Z Has data issue: false hasContentIssue false
  • English
  • Français

Cardiac features in the presymptomatic period in a neonate with anomalous left coronary artery arising from the pulmonary trunk

Published online by Cambridge University Press:  24 May 2005

Isabel Bolt ,
Johannes Spalinger  and
Jean-Pierre Pfammatter
Isabel Bolt
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Berne, Switzerland
Johannes Spalinger
Affiliation:
Division of Pediatric Gastroenterology, University Children's Hospital, Berne, Switzerland
Jean-Pierre Pfammatter
Affiliation:
Division of Pediatric Cardiology, University Children's Hospital, Berne, Switzerland
Get access Rights & Permissions [Opens in a new window]

Abstract

We describe a neonate presenting with a cholestatic jaundice due to Alagille syndrome. On echocardiography as part of the diagnostic work-up, we noted a slight dilation of the normally functioning left ventricle at the initial examination in the third week of life. Over the next 2 weeks, serial echocardiograms showed slowly progressive dilation and dysfunction of the left ventricle, together with a persistent mild elevation of levels of cardiac Troponin-I in the serum. These findings, unrelated to the underlying Alagille syndrome, prompted cardiac catheterisation, which confirmed that the main stem of the left coronary artery was originating from the pulmonary trunk. Surgery was successfully performed still in the presymptomatic period. The association of Alagille syndrome with anomalous left coronary artery arising from the pulmonary trunk is most unusual. We emphasise the cardiac findings prior to detection of the anomalous origin of the coronary artery.

Keywords

Alagille syndromenewbornTroponin-I
Type
Brief Report
Information
Cardiology in the Young , Volume 13 , Issue 6 , December 2003 , pp. 579 - 581
Copyright
© 2003 Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. Am Heart J 1933; 8: 787–801.Google Scholar
McElhinney DB, Krantz ID, Bason L, et al. Analysis of cardiovascular phenotype and genotype-phenotype correlation in individuals with a JAG1 mutation and/or Alagille syndrome. Circulation 2002; 106: 2567–2574.Google Scholar
Johnsrude CL, Perry JC, Cecchin F, et al. Differentiating anomalous left main coronary artery originating from the pulmonary artery in infants from myocarditis and dilated cardiomyopathy by electrocardiogram. Am J Cardiol 1995; 75: 71–74.Google Scholar
Chang R, Allada V. Electrocardiographic and echocardiographic features that distinguish anomalous origin of the left coronary artery from the pulmonary artery from idiopathic dilated cardiomyopathy. Pediatr Cardiol 2001; 22: 3–10.Google Scholar
Azakie A, Russel JL, McCrindle BW, et al. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome. Ann Thorac Surg 2003; 75: 1535–1541.Google Scholar
Schwartz ML, Jonas RA, Colan SD. Anomalous origin of the left coronary artery from the pulmonary artery: recovery of left ventricular function after dual coronary repair. J Am Coll Cardiol 1997; 30: 547–553.Google Scholar