Introduction: Mitral stenosis (MS) indicates elevation of transvalvular diastolic pressure gradient which is the most often caused by rheumatic heart disease. Congenital mitral stenosis is far less common, especially in adult population. (1-3) Management of those patients is challenging due to lack of data.

Clinical case: We present a case of 29-year-old patient with known significant allegedly rheumatic mitral stenosis scheduled for percutaneous mitral balloon valvuloplasty (PMBV). After additional workup we have reclassified stenosis as moderate, caused by attachment of chordae to a single papillary muscle - defect known as a parachute mitral valve. It is the least common form of congenital MS, usually accompanied by the other left sided outflow lesions (Shone complex). Our patient had an isolated form. She was completely asymptomatic, although planning pregnancy which is hemodynamic challenge, even with a moderate MS. Patient was definitely discouraged from PMBV and referred to adult congenital heart disease center for another opinion.

Discussion: We will discuss treatment options for women of child bearing potential with congenital mitral stenosis. Congenital form of MS in adult population is a very rare disease which challenges our knowledge based mainly on managing rheumatic MS.

Conclusion: Define of etiology end severity of valvular lesion is essential, especially when invasive management is planned.