0 Transposition of the Great Arteries (TGA)



Transposition of the Great Arteries (TGA)

Types :
  • Complete
  • With Communication
  • Corrected

1. Complete : has 2 circuits 
  • Oxygenated - Pulmonary circuit
  • Un-Oxygenated - Systemic circuit
Therefore O2 therapy will not help as there is no path for O2 to reach systemic circuit.
Hence the child dies without t/t in 1st wk of life due to Hypoxia

T/t - 
  • PGE1 infusion to keep Ductus open (i.e to keep PDA)
  • Emergency Surgery ( Create artificial ASD with balloon - Balloon atrial septostomy )
Definitive Surgery at later stage : Arterial Switch Surgery ( Jatene Procedure )


2. With Communication : 
  • With ASD - Small patent foramen ovale , Not helpful - hence cyanosis in 1st wk of life
  • With VSD and PDA - good mixing of blood , therefore only mild cyanosis at 6-12 months of life.



3. Corrected : Physiologically behaves Normal.

Ventriculo-Arterial disconcordance with Atrioventricular disconcordance (See the image below)
  • Ventriculo-Arterial disconcordance means - Aorta arise from RV ; PA arise from LV
  • AtrioVentricular dysconcordance means - Rt Arium opens into LV ; Lt Atrium opens into RV
Image Courtesy - DrAyushGoel


MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel

0 Duct dependant heart defects


Duct dependant heart defects

i.e - good with Patent ductus arteriosus but deteriorates with its closure

 Left Sided Lesions : (depend on ductus for systemic blood flow) 
  • Congenital AS
  • Coarctation of Aorta
  • Interrupted Aortic Arch
  • Hypoplastic Left heart syndrome
if ductus closes - Presents as Shock
Therefore give Prostaglandins infusion (PG E1)


 Severe Right Sided Lesion (Depend on ductus for pulmonary blood flow) 
i.e After birth - blood flow to lungs only if Ductus is open - if closes then Cyanosis. 
  • Critical PS
  • Tricuspid Atresia
  • Tetrology of fallot

 TGA i.e Aorta from right and pulmonary trunk from left 

Thus systemic circuit will have only unoxygenated blood
and Pulmonary circuit will have only oxygenated blood

thus it depends on Ductus for Oxygen.



Note : Truncus Arteriosus (i.e Pulm Art and Aorta are 1) is ductal independant.



MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel

1 Chest X-Ray PA view - Cardiac Borders



MCQ 117

Q. Which of the following, per se, does not form Left cardiac border on CXR PA view ?

a. Left atrium
b. Left Ventricle
c. Aortic arch/knob
d. Pulmonary trunk


ANSWER:
Ans : A

The cardiovascular silhouette , or cardiac shadow, is the contour of the heart and great vessels seen on chest radiographs.
Its right border is formed by the SVC, the right atrium, and the IVC. 
Its left border is formed by the aortic arch (which produces the aortic knob ), the pulmonary trunk, the left auricle, and the left ventricle. 
Its inferior border is formed by the right ventricle, and 
the left atrium shows no border.
Please note 
  1. Left auricle is Left atrial appendage which is a muscular pouch connected to left atrium.
  2. Silhouette sign is a misnomer, it should technically be " Loss of silhouette ".






MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel

0 Medical One Liners 131 - 140



131. Cat eye syndrome :
  • Partial trisomy of chromosome 22
  • Vertical oval pupil due to inferior iris coloboma
132. Single S2 in Tetrology of fallot.

133. All newborns have right ventricular hypertrophy and right axis deviation.

134. AA which have a pKa of 4 - aspartic acid and glutamic acid

135. Topoisomerase I (relaxase) 
  • makes ssDNA cuts, 
  • requires no ATP, 
  • relaxes supercoils, and 
  • acts as the swivel in front of the replication fork
136. 10 ATPs are generated per acetylCoA. (3NADH + 1FADH2 + 1GTP) {NADH = 2.5ATP; FADH2 = 1.5ATP}

137. Ketogenic AAs - Leucine and Lysine

138. Thiamine pyrophosphate (TPP) associated enzymes :
  • Alpha-ketoglutarate dehydrogenase
  • Pyruvate dehydrogenase
  • Transketolase
139. DNA replication occurs during S phase of the cell cycle.

140. Rise in conjugated (direct) bilirubin seen in Rotor syndrome and Dubin–Johnson syndrome.



MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
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