1. Cyanotic Heart Lesions
Case presentation (pink kid turns blue, blue ki)d comes out
What would you think about
Where does cyanotic heart disease come in?
ing Dr. Meadows
How would you approach this patient?
Indications/Contraindications
Which kids to start PGE
Neonatal Intensive Care Nursery
Night Curriculum Series

2. Cyanosis Arterial saturation less than 90% and a PO2 less than 60 torr
In all cyanotic heart lesions the amount of cyanosis seen is dependent on the amount of pulm blood flow
Decreased PBF- increased cyanosis
Increased PBF- minimal cyanosis but CHF may develop
With 100% oxygen
PO2 >250 is not congenital heart disease
PO2 <100 is cardiac disease
Turn into questions

3. 5 “T’s” Most common cyanotic lesions of the newborn
Tetralogy of Fallot
Transposition of the Great Arteries
Truncus Arteriosus
Total Anomalous Venous Return
Tricuspid Atresia
Make this a question and animate it

4. General Sources of Arterial Saturation
Decreased Pulmonary Blood Flow
Admixture Lesions
Increased Pulmonary Blood Flow
Tetralogy of Fallot
Transposition of Great Vessels
Truncious Arteriosus
Tricuspid Atresia
Anomalous pulmonary venous return
Pulmonary Atresia

5. PGE For cyanotic heart lesions with reduced blood flow
RE-opens PDA and prevents it from closing
Allows partially desaturated systemic arterial blood to enter the pulmonary artery and be oxygenated
Initial dose 0.1mg/kg/min
Side effects- apnea, fever, hypotension
Ask what is PGE? How does it work? And What are potential Complications of it?

6. Complete Transposition of the Great Arteries
5% of all CHD
Boys 3:1
Most common cyanotic condition that requires hospitalization in the first two weeks of life

7. Complete Transposition of the Great Arteries
Aorta arises from the right ventricle
Pulmonary artery arises from the left ventricle

8. Complete Transposition of the Great Arteries
Complete separation of the 2 circuits
Hypoxemic blood circulating in the body
Hyperoxemic blood circulating in the pulmonary circuit

9. Complete Transposition of the Great Arteries
Defect to permit mixing of 2 circulations- ASD, VSD, PDA.
VSD is present in 40% of cases
Necessary for survival

10. Clinical Symptoms Depend on anatomy present
No mixing lesion and restrictive PFO
Profound hypoxia
Rapid deterioration
Death in first hours of life
Absent respiratory symptoms or limited to tachypnea
Single second heart sound, no murmurs
animate

11. Clinical Symptoms Mixing lesion present (VSD or large PDA)
Large vigorous infant
Cyanotic
Little to no resp distress
Most likely to develop CHF in first 3-4 months of life
excessive sweating (a cold, clammy sweat often noted during feeding); poor feeding, slow weight gain, irritability or lethargy, and/or rapid breathing
animate

12. CXR Egg shaped cardiac silhouette Narrow superior mediastinum
Describe the findings on this CXR, and animate

13. Management Prostaglandin to establish patency of the ductus arteriosus
Increases shunting from aorta into the pulmonary artery
Increases pulmonary venous return distending the left atrium
Facilitates shunting from the left to the right atrium of fully saturated blood across the foramen ovale.
Ask what physiologically happens

14. Management
Therapeutic balloon atrial septostomy (Rashkind Procedure) if surgery is not going to be performed immediately
Improves mixing and pulmonary venous return at the atrial level

15. Treatment
Surgery consists of switching the right and left sided structures at the atrial level, at the ventricular level, or at the great artery level.

16. Tetraology of Fallot VSD RVOT Obstruction RVH Overriding aorta
Ask what are the 4 compents and animate

17. Two Important Abnormalities
NonRestrictive VSD
Large enough to equalize pressures in both ventricles
Degree of RVOT
Because of the non-restrictive VSD, systolic pressures in the RV and the LV are identical.
Depending on the degree of RVOT obstruction, and L-R, bidirectional, or r-L shunt is present.
Example:
Mild RVOT- there is a left to right shunt and infant is acyanotic
Severe RVOT- there is a predominant R-L shunt and the infant is cyanotic
Degree of cyanosis depends on the degree of right outflow tract obstruction

18. Clinical Presentation of Cyanotic TOF
Cyanosis, clubbing, dyspnea on exertion, squatting, hypoxic spells.
Loud systolic ejection murmur, systolic thrill at middle LSB
Soft murmurs are associated with less blood flow and more hypoxia

19. CXR- boot shaped
Ask what are the classic CXR findings

20. Hypoxic Spell (“TET Spell”)
Peak incidence of 2-4 months
Characterized by:
Hyperapnea (Rapid and deep respirations)
Irritability and prolonged crying
Inc cyanosis
Decreased heart murmur

21. Pathophysiology
Lower SVR or inc resistance of RVOT can increase the R-L shunt
Stimulates the respiratory center to produce hyperapnea
Results in an increase in systemic venous return
In turn, increases R-L shunt through VSD

22. TET Spell Treatment Hold infant in knee-chest position Morphine
Sodium bicarbonate to treat acidosis- decreases resp stimulating effect of acidosis
Vasoconstrictor (phenylephrine)
Propranolol

23. Treatment Early surgical repair depending on pt’s weight
VSD is closed and obstructing ventricular muscle is removed

24. Total Anomalous Pulmonary Venous Return
The pulmonary veins drain into the RA or its venous tributaries rather than the LA
A interatrial communication (ASD or PFO) is necessary for survival
Pulmonary venous return reaches the RA
Systemic and pulmonary venous blood are completely mixed

25. 4 Types Supracardiac
Common pulmonary vein drains into the SVC via the left SVC and left innominate vein.

26. 2. Cardiac
The common PV drains into the coronary sinus

27. 3. Infracardiac
The common PV drains into the portal vein, ductous venosus, hepatic vein, or IVC.
Infracardiac-type TAPVC. Pulmonary venous blood draining through the liver to reach the IVC and right atrium.

28. 4. Mixed
A combination of the other types

29. Clinical Signs for Unobstructed Veins
Mild cyanosis, signs of CHF in infancy, history of pneumonia
Widely split S2, Grade 2-3/6 systolic murmur heard at the ULSB
CXR- marked cardiomegaly

30. Clinical Signs for Obstructed Veins
Profound desaturation
Acidosis
PGE1 administration does not improve oxygenation because elevated pulmonary pressures in the right side of the heart (due to obstructed pulmonary outflow) will result in right to left shunting across an open ductus further decreasing arterial saturation.

31. Treatment Digitalis and diuretics to treat heart failure
Intubation and inc PEEP for those with severe pulm over load
Corrective surgery

32. Tricuspid Atresia Tricuspid valve is absent RV and PA are hypoplastic
Associated defects- ASD, VSD, or PDA (necessary for survival)
Dilation of LA and LV
Essentially single
ventricle physiology
LA and LV are large since they handle both systemic and pulmonary venous return

33. Clinical Signs Severe cyanosis, poor feeding, tachypnea
Single S2, grade 3/6 systolic murmur at LLSB if VSD is present
CXR- boot shaped heart

34. Treatment PGE IV infusion Blalock-Taussig shunt in infancy
systemic to pulmonary arterial shunt
Provide stable blood flow to the lungs
A gortex tube is sewen between the subclavian artery and the right pulmonary artery
PGE infusion in severely cyanotic lesions to maintain patency of PDA

35. Bidirectional Glenn
Superior vena cava is connected to the pulmonary arteries
IVC continues to be connected to the heart

36. Fontan Procedure Redirects IVC to lungs
Now oxygen-poor blood from upper and lower body flows through the lungs without being pumped and corrects cyanosis
The single ventricle is only responsible for supplying blood to the body.

37. Truncus Arteriosus A single trunk leaves the heart
Gives rise to pulm, systemic, and coronary circulations
Large VSD is always present

38. Clinical Signs Cyanosis immediately after birth Early signs of CHF
2-4/6 systolic murmur at LSB suggestive of VSD

39. Treatment Anticongestive medications (diuretics and digitalis)
Corrective surgery
VSD is closed
Pulmonary artery is separated from the truncus
Continuity is then established between the right ventricle and the pulmonary artery utilizing a valved homograft conduit

40. References?