1. Coarctation of The Aorta (COA)

2. 5 – 7 % of CHD .
Constriction of the Aorta of varying degree which may occur at any point from the transverse arch to the iliac bifurcation .
M : F  2 : 1 .
May be a feature of Turner syndrome .
Associated with bicuspid Aortic valve in >70 % of cases

3. Types & Pathophysiology :
Discrete juxtaductal obstruction ( adult type ).
98 % of cases .
Occurs just below the origin of L subclavian artery , at the origin of Ductus Arteriosus .
LV hypertrophy & LV HT .
L  R Ductal shunting occurs following birth .

4. Preductal (infantile type ) :
Tubular hypoplasia of transverse aorta , starting at one of the head &neck vessels & extending to Ductal area .
RV  PDA  descending Aorta (R  L shunt ):
palpable femoral pulse , differential cyanosis .
Sever pul. HT & high pul. Vascular resistance & HF .
BP  elevated in vessels proximal to coarctation .
BP & pulse pressure  lower below coarctation .
With time  collateral circulation (early adulthood)

6. Clinical Manifestations :
After infancy , most patients are a symptomatic .
Some children , adolescents weakness or pain (or both ) in lower extremities after exercise .
HT on routine physical examination .
Classic signs :
Disparity in pulsation & BP in the arms & legs
Weak or absent pulse(up to 40 % ) in LL , bounding in UL & neck , lower BP in LL relative to UL
Radial – femoral delay .

7. 90 % of patients have systolic HT in UL > 95th percentile for age .
Precordial impulse &heart sounds are normal .
Systolic thrill in supra sternal notch  bicuspid AS .
Short systolic murmur along L sternal border at 3rd, 4th ICS well transmitted to L infra scapular area, neck.
Murmur of AS  3rd R ICS .
In older patients  systolic or continuous murmur heard L & R sides of chest laterally & Posteriorly associated with a palpable thrill .

8. In neonates or infants with sever COA :
Sign of lower body hypo perfusion (differential cyanosis) .
Acidosis & sever HF
O/E  cardiomegaly
systolic murmur , heard along L sternal
border .
loud S2 .

9. Investigations : CXR : finding depends on :
Age , effect of HT , collateral circulation s
Infants with sever COA  cardiomegaly & pul. Congestion .
After 1st decade :
Mild – moderate cardiomegaly ( LV) .
Notching of inferior border of ribs (late childhood ).
Descending Aorta has an area of post steno tic dilatation .

11. ECG : Neonates & young infants  R or biventricular hypertrophy .
Young children  normal .
Older patients  LV hypertrophy .

12. Cardiac Catheterization & selective L ventriculography &Aortography :
Echo :
Visualize the segment of coarctation , & look for associated lesion .
Cardiac Catheterization & selective L ventriculography &Aortography :
Visualization of collateral blood vessels .

13. Treatment :
PGE2 infusion for neonates with sever COA & management of associating HF .
Surgical repair soon after the diagnosis & stabilization of the general condition .
Timing of surgery should not be delayed after 2nd decade because of associating LV dysfunction .

14. Prognosis & complications :
CVA  severe neurologic damage or death.
Premature coronary artery disease , HF , hypertensive encephalopathy , intracranial Hg  complication of HT .
Infective endocarditis or endarteritis  adult
Aneurysms of the descending Aorta & of collaterals .
HF & hypo perfusion (life threatening ) .
Complication of associating lesions .
Abnormalities involving L subclavian artery .

15. Tetra logy of Fallot (TOF)

16. 5 – 7 % of CHD .
Consist of :
Obstruction of RV outflow ( RV infundibulum, PS ) .
VSD .
Dextro position of the Aorta with Septal over –ride .
RV hypertrophy .

17. Pathophysiology

18. Clinical Manifestations :
In infancy :
Mild RV outflow obstruction  HF ( L  R shunt across VSD ) with no cyanosis at birth .
( cyanosis occurs later in 1st year of life ).
Sever degree of RV outflow obstruction  neonatal cyanosis , sever collapse following closure of Ductus Arteriosus .

19. Older Children :
Cyanosis with dusky blue skin , gray sclerae & engorged blood vessels .
Clubbing of fingers & toes .
Dyspnea on exertion & tendency to squat .
Paroxysmal hyper cyanotic attack ( hypoxic , blue or tet spells) :
Common in 1st 2 years .
Hyperpneic , restless , cyanosis increase , gasping respiration & syncope may result .

20. Occurs in the morning after initial awakening or after a vigorous crying .
Lasts from few min. to few hrs., rarely fatal .
Disappearance or decrease in the intensity of murmur
Mild attacks  weakness & sleep .
Severe attacks  unconsciousness , convulsion ,hemi paresis .
Prolonged attacks  sever systemic hypoxia & metabolic acidosis .
Growth & developmental delay .

21. Physical signs : precordial bulge with sub sternal impulse  RVH .
Systolic thrill along L sternal border in 3rd &4th para sternal space .
Systolic murmur : ejection in type at the upper sternal border , holo systolic at the lower sternal border .
S2 either single or pulmonic component is soft.
Continuous murmur in presence of collaterals .

22. Investigations CXR : ECG: Normal in size , boot shaped .
Clear hilar & pulmonary vascularity (oligaemic lung ).
R sided Aortic arch in % of cases .
ECG:
RVH , P- pulmonale , RAD .

24. Echo :
Cardiac Catheterizations :

25. Treatment :
Aim  provides an immediate increase in pul. Blood flow to prevent the sequel of sever hypoxia .
Lines of therapy :
Maitainence of normal body temperature & blood glucose .
IV administration of PGE  potent relaxant of Ductal smooth muscle .
Prevention & prompt treatment of dehydration .
Iron therapy : improves exercise tolerance & decrease the frequency of spells .

26. Oral propranolol decrease severity & frequency of hyper cyanotic spells .
Surgical treatment as soon as spells begins .
Palliative systemic –to-pulmonary artery shunt .
( Blalock – Taussig shunt ).
Corrective open heart surgery .
Treatment of hyper cyanotic spells :
Placement of the infant on the abdomen in the knee – chest position .
O2, rehydration, morphine (not > 0.2 mg/kg S.C ).
Correction of metabolic acidosis  NaHCO3 .
Drugs increasing systemic vascular resistance (phenylephrine, methoxamines) B-blockers .

27. Complications :
Polycythemia & dehydration  cerebral thrombosis & CVA .
Brain abscess .
Bacterial endocarditis ,
HF unusual unless in pink TOF & following surgery .
Growth retardation & pubertal delay .
Complications of hyper cyanotic spells .
Post – operative complications  chylothorax , diaphragmatic paralysis , Horner syndrome .