RT Book, Section
A1 Kay, W. Aaron
A1 Daniels, Curt J.
A1 Zaidi, Ali N.
A2 Daniels, Curt J.
A2 Zaidi, Ali N.
SR Print(0)
ID 1128634301
T1 EISENMENGER SYNDROME IN THE ADULT WITH CONGENITAL HEART DISEASE
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=1128634301
RD 2024/04/19
AB A  35-year-old-young  man  from  Guatemala  presented  with  chronic  cough  and  progressive  fatigue.  He  was  told  that  he  had  a  "hole  in  his  heart"  when  he  was  a  child  but  never  underwent  surgical  repair.  His  oxygen  saturations  at  rest  were  84%,  heart  rate  of  106  bpm,  and  a  BP  of  118/78  mm  Hg.  He  had  equal  saturations  in  all  4  extremities.  He  had  a  loud  second  heart  sound,  and  a  III/VI  holosystolic  murmur  at  the  left  sternal  border  (LSB)  with  a  mild  right  parasternal  heave  on  palpation  and  clear  lungs.  He  had  only  trivial  edema  around  his  ankles.  On  echocardiography  he  was  found  to  have  a  large  perimembranous  ventricular  septal  defect  (VSD)  with  predominantly  right-to-left  shunting  with  color  flow  Doppler  assessment  (Figures  10-1A,  10-1B,  and  10-2).There  was  no  evidence  of  any  right  ventricular  outflow  tract  obstruction.  He  underwent  a  cardiac  catheterization  and  was  found  to  have  irreversible  pulmonary  vascular  disease  with  a  pulmonary  vascular  resistance  of  14  Wood  units  (WU)  and  no  reversibility  with  a  vasodilator  challenge  (Figure  10-3).  He  was  not  a  candidate  for  late  surgical  closure  of  the  defect.  He  was  treated  with  pulmonary  vasodilators  for  symptomatic  relief.  His  symptoms  of  chronic  cough  did  improve.  He  also  had  significant  erythrocytosis  and  an  elevated  hematocrit.  He  did  not  manifest  any  symptoms  of  hyperviscosity  and  therefore  did  not  undergo  any  phlebotomy.