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08:34
Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
Partial anomalous pulmonary venous return is common in Turner syndrome
Iris Gutmark-Little1, Kan N Hor, James Cnota
1Division of Endocrinology, Cincinnati Children's Hospital, Cincinnati, OH, USA. iris.little@cchmc.org
Journal of Pediatric Endocrinology & Metabolism : JPEM
|August 11, 2012
Summary
Partial anomalous pulmonary venous return (PAPVR) is common in Turner syndrome (TS) and can be hemodynamically significant. Cardiac MRI is crucial for diagnosis in TS patients, as transthoracic echocardiogram may miss this condition.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Cardiovascular disease is prevalent in over 50% of Turner syndrome (TS) patients.
- Advanced imaging reveals a wider spectrum of cardiac anomalies in TS.
Purpose of the Study:
- To determine the prevalence and hemodynamic significance of partial anomalous pulmonary venous return (PAPVR) in adolescents and young adults with TS.
- To compare transthoracic echocardiogram (TTE) and cardiac magnetic resonance (CMR) for PAPVR diagnosis in TS.
- To investigate the association between PAPVR and TS phenotype.
Main Methods:
- Retrospective review of 39 young TS patients who underwent both TTE and CMR.
- Analysis of diagnostic accuracy of TTE versus CMR for PAPVR detection.
- Assessment of hemodynamic parameters (Qp:Qs, RV/LV volumes) and clinical features.
Main Results:
- PAPVR was diagnosed in 18% (7/39) of TS patients.
- CMR identified PAPVR in 6 patients missed by initial TTE.
- One patient with PAPVR required surgical repair due to significant right ventricular enlargement and Qp:Qs ratio of 1.9:1.0.
- Hemodynamic parameters differed significantly between TS patients with and without PAPVR.
Conclusions:
- PAPVR is highly prevalent and potentially hemodynamically significant in Turner syndrome.
- Transthoracic echocardiogram may underestimate PAPVR prevalence in TS.
- Routine screening and timely management are essential for TS patients with PAPVR.
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