RT Book, Section
A1 Huang, Jennifer
A1 Broberg, Craig
A2 Daniels, Curt J.
A2 Zaidi, Ali N.
SR Print(0)
ID 1128634065
T1 THE ADULT WITH A SINGLE VENTRICLE AND FONTAN PALLIATION
T2 Color Atlas and Synopsis of Adult Congenital Heart Disease
YR 2016
FD 2016
PB McGraw-Hill Education
PP New York, NY
SN 9780071749435
LK accesscardiology.mhmedical.com/content.aspx?aid=1128634065
RD 2024/04/25
AB A  20-year-old  man  with  a  single  morphologic  left  ventricle  due  to  tricuspid  atresia  and  ventricular  inversion  with  transposed  great  arteries,  with  transposed  great  arteries  presents  with  stunted  growth  and  maturation.  He  initially  had  a  pulmonary  artery  band  (Figure  7-1)  placed  when  he  was  2  months  old  with  an  atrial  septectomy  at  9  months.  At  age  3  years  he  underwent  a  surgical  Fontan  palliation,  which  involved  redirection  of  the  superior  vena  cava  (SVC)  and  inferior  vena  cava  (IVC),  by  way  of  a  lateral  tunnel,  to  the  right  pulmonary  artery,  confluent  with  the  left.  The  main  pulmonary  artery  (MPA)  was  excised.  Therefore,  systemic  venous  flow  was  redirected  to  passively  flow  to  the  right  and  left  pulmonary  vascular  beds,  bypassing  the  heart  entirely  (Figure  7-2).  He  had  an  uneventful  recovery  in  early  childhood  until  he  was  diagnosed  with  protein-losing  enteropathy  (PLE)  at  the  age  of  6  years.  Subsequently,  at  age  10,  further  surgery  was  done  to  remove  the  lateral  tunnel  and  replace  this  with  an  extracardiac  conduit  between  the  IVC  and  pulmonary  artery.  Despite  surgical  revision  and  medical  therapy,  he  continued  to  have  evidence  of  PLE  complicated  by  a  small  stroke  and  evidence  of  mild  liver  fibrosis.  He  was  started  on  warfarin.  By  age  12,  he  had  evidence  of  delayed  maturation  and  stunted  growth  (Figure  7-3),  as  a  complication  of  PLE.  He  had  chronic  hypoproteinemia,  hypocalcemia,  and  hyponatremia.  Measured  testosterone  and  growth  hormone  levels  were  normal.  He  was  considered  for  heart  transplantation,  but  instead  medical  therapy  was  continued,  including  subcutaneous  heparin,  testosterone,  and  spironolactone.