Demonstration of peripheral pulmonary stenosis and supravalvular aortic stenosis by different cardiac imaging modalities in a patient with Williams syndrome - usefulness of noninvasive imaging studies

With technical improvement, noninvasive cardiac imaging modalities, including multislice computed tomography and magnetic resonance imaging, can offer sufficient imaging quality to differentiate cardiac anatomies. These noninvasive modalities also can identify aortic and pulmonary vascular abnormalities clearly in the same study. We report a case with severe pulmonary hypertension associated with peripheral pulmonary stenosis and supravalvular aortic stenosis in a 29-year-old woman with Williams syndrome, which was identified similarly with both invasive and noninvasive imaging modalities. The need for invasive tests can be reduced with appropriate improvement and validation of these noninvasive tests.