NEW YORK — Partial anomalous pulmonary venous return was detected by cardiac magnetic resonance imaging in 7 of 39 adolescent and young adult women with Turner's syndrome whose charts were retrospectively evaluated.
The finding suggests that careful screening for the partial anomalous pulmonary venous return (PAPVR) heart defect is indicated in the Turner's syndrome population, along with appropriate cardiac referral and management, Dr. Iris Gutmark-Little said at the joint meeting of the Lawson Wilkins Pediatric Endocrine Society/European Society for Pediatric Endocrinology.
Results from the study also suggest that cardiac magnetic resonance (CMR) imaging is a more sensitive modality than echocardiography for the detection of PAPVR in patients with Turner's syndrome, said Dr. Gutmark-Little of Cincinnati Children's Hospital Medical Center.
The 39 patients were the first to be screened with CMR after Cincinnati Children's began using the modality routinely in all Turner's syndrome patients during the early teen years. The patients also underwent echocardiographic evaluation.
A total of seven patients (18%) were found to have PAPVR by cardiac magnetic resonance imaging, six of them following a normal echocardiogram. Aberrant drainage of the right upper pulmonary veins was seen in five patients, of whom three also had involvement of at least a portion of the right middle lobe vein. The other two had the defect in the left upper pulmonary vein.
The 18% PAPVR prevalence seen here is similar to the 13% found in a previous study of adult Turner's syndrome patients (Circulation 2004;110:1694–700), she noted.
In one patient, PAPVR was associated with clinically significant enlargement of the right ventricle, with a pulmonary-to-systemic blood flow ratio (Qp:Qs) of 1.9:1. She required surgical repair. The other six patients had Qp:Qs ratios ranging from 1.24:1 to 1.62:1 (mean, 1.5:1), and did not require intervention.
There were no differences in age, height, karyotype, or right ventricular ejection fraction between the 7 patients with PAPVR and the 32 without, nor did the two groups differ in peripheral lymphedema, neck webbing, renal malformations, coarctation, or bicuspid aortic valve. This finding differs from previous studies that have linked some of these features to PAPVR in Turner's syndrome patients. It's possible that the small sample size may have missed the associations, Dr. Gutmark-Little commented.
After her presentation, an audience member asked Dr. Gutmark-Little if she would recommend cardiac magnetic resonance imaging in asymptomatic Turner's syndrome patients. She replied, “Not necessarily primarily for detecting PAPVR, but I think it's important to use CMR during the teen years in order to look at aortic size and function.”
Dr. Gutmark-Little stated that she had no financial disclosures.
A four-chamber MRI shows a dilated right ventricle as a result of a right-sided PAPVR lesion in an adolescent female Turner's syndrome patient.
A three-dimensional MRI shows a right-sided PAPVR lesion in an adolescent female Turner's syndrome patient. The right middle– and right upper–lobe anomalous pulmonary veins are seen draining into the superior vena cava. This patient had a nearly 2:1 left-to-right shunt, resulting in right ventricular dilatation. This necessitated surgical repair of her PAPVR.
Source Images courtesy Cincinnati Children's Hospital