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Partial abnormal pulmonary venous return in Turner syndrome
A G van Wassenaer1, L J Lubbers, G Losekoot
1Section of Pediatric Cardiology, Academisch Medisch Centrum, Amsterdam, The Netherlands.
European Journal of Pediatrics
|November 1, 1988
Summary
Partial anomalous pulmonary venous return, a heart defect, is described in three patients with Turner syndrome. This unique combination of heart anomalies and Turner syndrome may be specifically linked.
Area of Science:
- Cardiology
- Genetics
- Pediatric Medicine
Background:
- Turner syndrome is a genetic condition affecting females.
- Congenital heart defects are common in Turner syndrome.
- Partial anomalous pulmonary venous return (PAPVR) is a rare heart anomaly.
Observation:
- Three cases of PAPVR were identified in patients with Turner syndrome.
- Two patients had specific venous drainage anomalies (right superior vena cava, left innominate vein).
- All three patients had an intact atrial septum.
Findings:
- PAPVR occurred in conjunction with other cardiac conditions, including coarctation of the aorta and subaortic stenosis.
- The specific pattern of venous drainage and intact atrial septum in these cases is noteworthy.
- The co-occurrence suggests a potential specific association between PAPVR and Turner syndrome.
Implications:
- This finding may indicate a specific predisposition to PAPVR in individuals with Turner syndrome.
- Further research is needed to confirm this association and elucidate underlying mechanisms.
- Understanding this link can improve diagnostic and management strategies for patients with Turner syndrome and congenital heart disease.