Clinical_Rounds_in_Endocrinology_Volume_II_-_Pediatric_Endocrinology

(singke) #1

274



  1. Why are cardiac anomalies more common in patients of TS with webbed neck?


The cause for association between cardiovascular malformations and webbed
neck is not well known. However, the proposed mechanisms include compres-
sive effect of enlarged lymph sac on the developing cardiac outflow tract during
intrauterine period and dysfunction of a common gene required for lymphatic
as well as cardiovascular development.


  1. What are the cardiovascular anomalies in Turner syndrome?


Cardiovascular anomalies occur in 25–45 % patients with TS. Bicuspid aor-
tic valve (16 %) and coarctation of aorta (11 %) are the most common cardio-
vascular defects. Other cardiovascular anomalies include aortic dilatation,
aortic aneurysm, elongated transverse aortic arch, partial anomalous pulmo-
nary venous connection (PAPVC), and left-sided superior vena cava
(Fig. 8.8).

ab

Fig. 8.8 (a) Sagittal reconstructed MIP and (b) VRT images of thoracic CT angiography showing
mild dilatation of aortic root (red arrow) and ascending thoracic aorta near the aortic arch (blue
arrow) in a patient with Turner syndrome


8 Turner Syndrome
Free download pdf