RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164076681
T1 LEOPARD Syndrome
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164076681
RD 2024/04/16
AB It  is  an  acronym  that  stands  for  Lentigines  (multiple),  Electrocardiographic  conduction  abnormalities,  Ocular  hypertelorism,  Pulmonary  stenosis,  Abnormal  genitalia,  Retardation  of  growth,  and  Deafness  (sensorineural).  It  is  a  complex  congenital  dysmorphogenetic  disorder  characterized  by  skin  lesions  (lentigines),  severe  cardiac  anomalies  (dysrhythmias,  pulmonary  valve  stenosis),  ocular  hypertelorism,  abnormalities  of  genitalia,  sensorineural  deafness,  and  severe  retardation  of  growth.  The  second  most  common  defects  are  ventricular  septal  hypertrophy  and  pulmonary  (or  subpulmonary)  valve  stenosis.  The  major  perioperative  complications  are  severe  arrhythmias  and/or  cardiac  decompensation.  The  presence  of  nonobstructive  asymmetric  anteroseptal  hypertrophic  cardiomyopathy  has  been  reported.