30 yo W with 3 years of progressive SOB

Original case provided by Lauren Brown, MD.
Edits and graphics by Yilin Zhang, MD.



30yo woman with progressive dyspnea on exertion x 3 years. She is originally from Nepal and moved to the US 2 years ago.  She reports associated orthopnea, palpitations and chest pain. She denies fevers, chills, weight changes. She reports joint pains x several years. She denies any significant family history and denies tobacco, EtOH or other substance use.

On exam, she is afebrile, HR 80, BP 90/60, SpO2 99% on RA. Her JVP is 10 cm with a laterally displaced PMI. She has an irregularly irregular heart rhythm and a 3/6 holosystolic and diastolic murmur.

CXR is shown below:

What is the most likely diagnosis?