TY  - CHAP
M1  - Book, Section
TI  - THE ADULT WITH D-TRANSPOSITION OF THE GREAT ARTERIES (D-TGA)
A1  - Cribbs, Marc G.
A1  - Zaidi, Ali N.
A2  - Daniels, Curt J.
A2  - Zaidi, Ali N.
Y1  - 2016
N1  - 
T2  - Color Atlas and Synopsis of Adult Congenital Heart Disease
AB  - A  29-year-old  man  was  born  a  "blue  baby"  and  found  to  have  d-transposition  of  the  great  arteries  (D-TGA).  A  balloon  atrial  septostomy  (BAS)  was  performed  as  an  infant,  followed  by  the  Mustard  (atrial  baffle)  procedure.  He  did  well  for  several  years  and  did  not  require  further  surgical  or  percutaneous  interventions.  He  was  lost  to  follow-up  for  several  years  and  ultimately  presented  to  a  local  emergency  room  at  24  years  of  age,  with  atrial  flutter  and  a  rapid  ventricular  rate.  He  was  electrically  cardioverted  back  to  sinus  rhythm.  At  the  time,  an  echocardiogram  demonstrated  mild  systemic  (right  ventricular)  dysfunction  and  concerns  for  narrowing  of  the  superior  vena  cava  (SVC)  limb  of  the  systemic  venous  baffle.  Cardiac  magnetic  resonance  imaging  (MRI)  subsequently  demonstrated  a  mildly  dilated  and  hypertrophied  systemic  right  ventricle  (RV)  with  mild  global  systolic  dysfunction  (estimated  RV  ejection  fraction  [EF]  of  43%).  The  thin-walled  left  ventricle  (LV)  had  normal  size  and  function  (LVEF  of  63%).  He  was  taken  to  the  cardiac  catheterization  laboratory  where  SVC  baffle  limb  stenosis  was  confirmed.  A  36-mm  max  LD  stent  expanded  to  34  mm  was  deployed  to  relieve  the  baffle  stenosis.  An  electrophysiology  (EP)  study  was  also  performed  and  revealed  both  atrial  flutter  and  ventricular  tachycardia.  Given  this,  a  transvenous  implantable  cardioverter-defibrillator  (ICD)  was  placed  for  primary  prevention  of  sudden  cardiac  death  (SCD).  He  did  well  from  24  to  29  years  of  age  with  no  major  concerns  and  maintained  regular  follow-up  with  the  Adult  Congenital  Heart  Disease  (ACHD)  clinic.  He  maintained  a  regular  job,  got  married,  had  two  children,  and  exercised  regularly.  Closer  to  his  29th  birthday,  he  noted  that  he  was  becoming  "fatigued"  with  running  2  to  3  miles.  He  had  rare  episodes  of  palpitations  associated  with  lightheadedness  though  he  never  had  frank  syncope  or  an  ICD  discharge.  A  Cardio-Pulmonary  Exercise  Test  (CPET)  was  done  to  screen  for  desaturations  and  arrhythmias  with  exercise.  This  revealed  a  baseline  oxygen  saturation  of  92%  on  room  air  with  significant  desaturation  to  86%  while  running.  There  were  no  rhythm  concerns.  A  repeat  echocardiogram  was  done  that  showed  a  systemic  venous  baffle  leak.  He  underwent  cardiac  catheterization  with  removal  of  the  ICD  leads,  placement  of  a  covered  stent  to  relieve  the  baffle  leak,  and  ICD  lead  replacement.  Since  this  procedure,  he  has  been  doing  well.  He  remains  fully  saturated,  continues  to  work,  and  exercises  often  with  no  concerns.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/16
UR  - accesscardiology.mhmedical.com/content.aspx?aid=1128633879
ER  -