Left isomerism with absence of the spleen and obstructed pulmonary venous drainage: an uncommon association
Left isomerism with absence of the spleen and obstructed pulmonary venous drainage—an uncommon association
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ABSTRACT: 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.
ABSTRACT: 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.
Cardiology in the Young 03/1994; 4(02):160 – 163. · 0.95 Impact Factor
