TY  - CHAP
M1  - Book, Section
TI  - THE ADULT WITH EBSTEIN's ANOMALY OF THE TRICUSPID VALVE
A1  - Kay, W. Aaron
A1  - Roble, Sharon
A1  - Zaidi, Ali N.
A2  - Daniels, Curt J.
A2  - Zaidi, Ali N.
PY  - 2016
T2  - Color Atlas and Synopsis of Adult Congenital Heart Disease
AB  - A  32-year-old  woman  presented  for  evaluation  of  decreasing  exercise  tolerance  and  a  loud  heart  murmur.  On  physical  examination,  she  had  a  fixed  split  second  heart  sound  with  a  normal  pulmonary  component.  She  had  a  holosystolic  murmur  at  the  mid-left  sternal  border  that  did  not  radiate  and  increased  with  inspiration.  An  electrocardiogram  (ECG)  and  echocardiogram  were  performed.  ECG  demonstrated  right  atrial  enlargement  and  a  right  bundle  branch  block.  Echocardiogram  showed  apical  displacement  of  the  septal  and  posterior  leaflets  of  her  tricuspid  valve  resulting  in  severe  tricuspid  regurgitation.  Her  clinical  findings  were  consistent  with  Ebstein's  anomaly.  Further  testing  included  a  functional  vo2  exercise  stress  test.  With  exercise,  she  was  found  to  have  significant  oxygen  desaturation  to  the  mid-80s  on  room  air.  A  subsequent  transesophageal  echocardiogram  showed  a  patent  foramen  ovale  (PFO)  with  bidirectional  shunting,  severe  tricuspid  regurgitation,  and  an  apically  displaced  septal  leaflet  of  the  tricuspid  valve  consistent  with  Ebstein's  anomaly.  Because  of  her  significant  hypoxia  with  exercise,  she  underwent  a  catheter-based  closure  of  her  PFO.  A  repeat  exercise  study  following  PFO  closure  showed  normal  oxygen  saturations  with  exercise  and  improved  exercise  capacity.  She  was  followed  with  serial  imaging  that  continued  to  demonstrate  the  severe  tricuspid  regurgitation  and  gradual  worsening  in  her  functional  capacity.  She  developed  atrial  flutter  with  rapid  ventricular  rate  for  which  she  needed  to  be  cardioverted.  She  was  managed  with  antiarrhythmic  medications  and  has  had  no  further  arrhythmia  recurrences.  She  finally  underwent  surgical  management  in  the  form  of  a  tricuspid  valve  replacement  with  a  bioprosthetic  valve  and  plication  of  the  atrialized  ventricle.  The  patient  had  an  uneventful  postoperative  recovery  with  significant  improvement  in  her  functional  capacity.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - accesscardiology.mhmedical.com/content.aspx?aid=1128634127
ER  -