There is a flow reversal indeed. However, in this case the hepatic flow reversal is pre-systolic due to powerful atrial contraction in the setting of stiff RV. TR here is not severe. This is similar to a large Ar wave in pulmonary veins doppler in patients with stiff LV.
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Fabry's CM is an X-linked inherited deficiency of alpha-galactosidase A which results in systemic sphingolipid accumulation.
Severity of manifestation depends on degree of X-chromosome inactivation => female carriers can have a wide range of phenotypes! doi.org/10.1016/j.jcmg…
This 67 yo pt presented with severe HCM phenocopy and strong family history of confirmed Anderson-Fabry's CM. She was determined to have a GLA mutation and successfully started on treatment with Fabrazyme.
Note the diffuse asymmetric LVH (wall thickness 24mm) with marked apical hypertrophy (30mm). Despite normal LVEF, her GLS was markedly reduced at -5.8%. CMR demonstrated 20% LGE and short T1 time (indicative of fat accumulation)
1/5 An interesting illustrative case of the day. 62-year-old man with mid ventricular HCM associated with a large apical aneurysm #EchoFirst@AAH_StLukesCV
2/5 CW Doppler trace shows triphasic flow: 1. MR 2. Mid ventricular obstruction 3. Outflow from the apex towards mid LV cavity during IVRT.
3/5 This characteristic flow pattern is also visualized on 2D color flow (still images below).
1/7 This 56 yo was the first patient I saw in clinic this morning. EKG reminded of my friend professor Sakamoto, who first described giant negative T waves in 1976 of apical HCM. #echofirst
2/8 I am beckoned by the famous lyrics written by Pete Seegar in 1955 “When will we ever learn?” and subsequently popularized by Peter, Paul and Mary when it became a favorite anti-war song in the 60s. We, cardiologists, are indeed at war against CVD, of which is 80% preventable
3/8 “When will we ever learn?” While we are rightfully proud of our excellence in medical, surgical and catheter based therapies, we need to be reminded that each patient represents our collective failure in prevention.