Cyanosis - TOF, TGA, Ebstein's anomaly

Question 1

Tetralogy of Fallot
-presentation
-pathophysiology
-investigations
-management

Answer 1

MOST COMMON CYANOTIC CONDITION
-1-2months

VSD
RVH
RVOTO, pulmonary stenosis => determines severity of cyanosis
Overriding aorta

Crying, fever => Tet spells (severe cyanosis, high RR, LOC)
Ejection systolic murmur
CXR - Boot shaped heart
ECG - RVH

Surgical repair

Question 2

Transposition of the great arteries
-presentation
-pathophysiology
-investigations
-management

Answer 2

Aorta leaves RV
Pulmonary trunk leaves LV

Cyanosis
High RR
Loud single S2 - anterior location of aorta

CXR - egg on side

Maintain PDA
Definitive - surgical correction

Question 3

Initial management of suspected cyanotic congenital heart disease

Answer 3

Supportive care
Prostaglandin E1 - alprostadil
-maintain PDA in ductal dependent congenital heart defect until definitive diagnosis and surgical correction performed

Question 4

Ebstein’s anomaly
-what is it
-presentation
-risk factors

Answer 4

Low insertion of tricuspid valve => large atrium, small ventricle
-septal and posterior leaflet of tricuspid attached to walls and septum of RV

May be caused by lithium exposure

Associated with
-patent foramen ovale
-ASD
-WPW

Cyanosis
Prominent a wave in distended JVP
Hepatomegaly, tricuspid regurg
RBBB => widely split S1, S2