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Left isomerism with absence of the spleen and obstructed pulmonary venous drainage—an uncommon association

Published online by Cambridge University Press:  19 August 2008

Marco Zimarino ,
Salvatore Gerboni  and
Fernando M. Picchio
Marco Zimarino
Affiliation:
From the Institute of Cardiology, Section of Pediatric Cardiology, University of Chieti, Chieti
Salvatore Gerboni
Affiliation:
Section of Pediatric Cardiology, University of Bologna, Chieti
Fernando M. Picchio*
Affiliation:
From the Institute of Cardiology, Section of Pediatric Cardiology, University of Chieti, Chieti
*
Dr. Fernando M. Picchio Istituto di Cardiologia, Sezione di Cardiologia Pediatrica, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy.
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Summary

Cardiac and extracardiac anomalies tend to follow characteristic patterns when there is an isomeric arrangement of the heart and lungs. We present a case diagnosed correctly during prenatal life as having isomerism of the left atrial appendages in which some of the associated features were most unexpected, namely absence of the spleen and obstructed totally anomalous pulmonary venous connection. This association proved misleading for us in the management of the patient. We found it difficult to believe our findings in the light of our past knowledge, and this resulted in a delay in the referral for surgery. The final outcome was unfortunately fatal. In the management of isomerism of any type, therefore, a wide spectrum of combinations and “crossover” of cardiac anomalies must be anticipated, emphasizing the need to assess individually the arrangement of the different organs of the body.

Keywords

Left isomerismabsence of the spleenobstructed pulmonary venous connection
Type
Brief Reports
Information
Cardiology in the Young , Volume 4 , Issue 2 , April 1994 , pp. 160 - 163
Copyright
Copyright © Cambridge University Press 1994

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