ELN and aortic stenosis: At 9 months, SVAS progressed (peak gradient 35 mmHg), while PPAS gradients regressed by >40%.<h4>Conclusion</h4>This novel exon 28 ELN frameshift expands the non-syndromic SVAS spectrum and illustrates a characteristic pattern of progressive aortic stenosis with improving PPAS, supporting ELN testing and targeted longitudinal surveillance in similar patients and families.