Incidentally detected situs ambiguous in adults
Jae-Gyung Kim1, Ho-Joong Youn, Gee-Hee Kim
1Division of Cardiology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul, Korea.
Journal of Cardiovascular Ultrasound
|January 20, 2012
Summary
Situs ambiguous with polysplenia, a rare congenital anomaly, was identified in two adult patients presenting with cardiac issues. This condition involves spleen and organ arrangement abnormalities, presenting unique features in each case.
Area of Science:
- Cardiology
- Medical Genetics
- Developmental Biology
Background:
- Situs ambiguous is a rare congenital anomaly characterized by abnormal arrangement of visceral organs.
- Polysplenia, a component of situs ambiguous, involves the presence of multiple spleens.
- Congenital anomalies of the great vessels and abdominal organs can present in adulthood, often discovered incidentally during cardiac evaluations.
Observation:
- Two adult patients, a 42-year-old male and a 52-year-old female, were diagnosed with situs ambiguous and polysplenia.
- The male patient presented with atrial fibrillation and had left-sided inferior vena cava (IVC), interrupted hepatic IVC segment with hemiazygos continuation, multiple spleens, and intestinal malrotation.
- The female patient, hospitalized for infective endocarditis post-pacemaker implantation, exhibited multiple spleens, left-sided stomach, bilateral liver with midline gallbladder, and left-sided IVC.
Findings:
- Both cases demonstrated situs ambiguous with polysplenia, confirming its occurrence in adult cardiac patients.
- Distinctive anatomical variations were noted in each patient, highlighting the spectrum of this congenital anomaly.
- Associated cardiac conditions included atrial fibrillation and sick sinus syndrome, requiring interventions like catheter ablation and pacemaker implantation.
Implications:
- This case series underscores the importance of recognizing situs ambiguous with polysplenia in adult patients, even when presenting for unrelated cardiac conditions.
- Awareness of these anomalies is crucial for accurate diagnosis, surgical planning, and management of associated cardiovascular and gastrointestinal issues.
- Further research into the genetic and developmental underpinnings of situs ambiguous with polysplenia may elucidate its varied presentations and long-term outcomes.
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