1. Cyanotic Heart Disease; Overview of ManagementBy
Dr. Ahmad Shaker
MD Cardiology

2. Definition
Cyanotic Heart Disease is a defect or group of defects in the structure or function of the heart or the great vessels, present at birth, consisting of abnormal blood flow from the right to the left part of the circulatory system (either at the level of the atria, the ventricles, or the great vessels).
This abnormal communication (called right-to-left shunt) results in poor oxygenation of the body and therefore cyanosis

3. Causes of Cyanotic Heart Disease:
–Tetralogy of Fallot
–Transposition of the great arteries (D-TGA)
–Single ventricle
–Truncus arteriosus
–Total anomalous pulmonary venous connections
–Ebstein’s anomaly
–Eisenmenger’s disease

4. Ductal Independent Mixing Lesions:
-Truncus Arteriosus .
-d-Transposition of Great Arteries.
-Total Anomalous Pulmonary Venous Connection without Obstruction (TAPVC).
Ductal-Dependent Pulmonary Blood flow:
-Tricuspid Atresia
-Pulmonic Atresia with Intact Ventricular Septum.
-Tetralogy of Fallot.
-Ebstein’s Anomaly.

5. Increased Pulmonary Blood Flow Decreased Pulmonary Blood Flow
Lesions with Ductal Dependent Systemic Blood Flow:
-Hypoplastic Left Heart Syndrome (HLHS).
-Interrupted Aortic Arch.
-Total Anomalous Pulmonary Venous Connection with Obstruction.
Increased Pulmonary Blood Flow
Decreased Pulmonary Blood Flow
Obstruction to Systemic Blood Flow
Total Anomalous Pulmonary Venous Return
Truncus Arteriosis
Tetralogy of Fallot
Transposition of the Great Arteries
Tricuspid Atresia
Hypoplastic Left Heart Syndrome

6. Symptoms & Signs Cyanosis. Dyspnea.
Failure to thrive, or failure to grow properly
Fatigue
Squatting position after physical activity to relieve breathlessness.
Hypoxic spells, characterized by:
-Anxiety Hyperventilation.
-Sudden increase in cyanosis.
Syncope.
Chest pain, Arrythmias.
CHF.

7. Diagnosis
Clinical:
-Upper left sternal border ejection murmur of RV outflow tract obstruction TOF.
-Newborns present with severe cyanosis and a continuous murmur of ductal flow pulmonary valve atresia and ductus arteriosus-dependent pulmonary blood flow .
-Present immediately after birth with severe cyanosis that progresses rapidly to metabolic acidosis TGA.

8. Days to weeks after birth with heart failure and mild hypoxemia, A hyperdynamic precordium, wide pulse pressure, a normal S1 with a frequent ejection click, and a loud, usually single S2 are characteristic persistant truncus.
In persistent truncus, Heart murmurs vary and may include a flow murmur at the base, a loud regurgitant murmur at the lower left sternal border, and a mid-diastolic mitral flow murmur. With truncal valve insufficiency, a high-pitched diastolic murmur over the mid-sternum is present

9. ECG”

10. -Right axis deviation and RV hypertrophy on ECG ---- TFO.

11. -Combined ventricular hypertrophy ----- Truncus.
-Right axis deviation and Right Ventricular Hypertrophy ---- Total APVD.
-Superior axis and L V Hypertrophy ----
TV atresia.
-LV hypertrophy, leftward axis --- Pulmonic Atresia with Intact Ventricular Septum
-Right Bundle Branch Block, Delta Waves due to WPW syndrome --- Ebestine’s Anomaly.

12. TGA
Right ventricular hypertrophy, right axis deviation .

13. X-Ray:

14. TFO (Coure in Sabou).
Small heart and a concave main pulmonary artery segment with lung oligemia, Right aortic arch is present in 25%

15. Cardiomegally with narrow base, lung hyperemia ---- TGA (Egg on side).

22. Anomalus Pulmonary Venous Drainage

34. Echocardiography

35. A Segmental Approach to cardiac Situs & malposition;
1- Atrial Situs:
Veseral Situs & visceroatrial concordance.
Atrial morphology (situs solitus or inversus).
venous inflow pattern.
2- Ventricular localization:
ventricular morphology.
atrioventricular concordance.
Base-to-apex axis (Levo or Dextrocardia).
3- Greate Artery Connections & its identifications.
4- Ventriculoarterial concordance.

38. Echocardiographic characteristics of Rt & Lt ventricles;
Rt Ventricle
Lt Ventricle
*Trabiculated.
*Three papillary.
*Chorade insertes into
ventricular septum.
*Infundibular muscle band.
*Moderator band.
*Triangular cavity shape.
*Tricusped valve with relatively
apical isersion.
*Smooth.
*Two papillary.
*Ellipsoidal in shape.
*Mitral valve with 2 leaflets &
relatively basal insersion.

41. Tetralogy of Fallot

42. Ebstein’s Anomaly

43. Ebstein’s Anomaly

44. Tricusped Atresia

45. APVD

46. TGA

47. Truncus Arteriosus

48. Cathetrization

49. Tetralogy of Fallot

53. Trancus Arteriosus

54. Complications Heart failure and hypoxic spells.
Polycythemia and increased coagulation.
Stroke.
Infective endocarditis.
Brain abscess.
Hemoptysis.
Impaired growth.
Pulmonary hypertension.

55. Prognosis
Most children with congenital heart defects can be helped by surgery even if the defect is severe.   
 Death rates attributable to congenital cardiovascular defects are only about two per 100,000, but they are considerably higher for infants under one year old.   
For infants under one year, the death rate is about 60 per 100,000.

56. Treatment: *‘tet’ spells: treatment
1. ‘knee-chest position’ or over parent's shoulder with
knees bent
2. supplemental oxygen (effectiveness is questionable in
the absence of pulmonary blood flow)
3. sedation: intravenous or subcutaneous morphine,
0.1 mg/kg
4. intravascular volume expansion
5. prolonged cyanosis: an alpha agonist (phenylephrine,
5-10 mcg/kg IV)
6. for prevention of spells: propranolol (0.5-1 mg/kg po
QID)

57. *The infants need to be monitored because of the
resultant polycythemia, which may lead to
hyperviscosity. Hyperviscous blood flows poorly
through the circulatory bed and results in poor
tissue perfusion.
*If blood flow to the systemic or pulmonic circulation
is not sufficient to sustain life, prostaglandin E1
(PGE1) (0.05 to 0.1 µg/kg/min IV) can be
administered to maintain patency of the ductus
arteriosus.
*When a PGE1 infusion is being administered, blood
pressure must be monitored and hypotension
corrected.

58. Tetralogy of Fallot
*Corrective operations are often performed by 18
months of age or earlier if the child has recurrent
hypoxic episodes or progressive cyanosis.
*If pulmonary stenosis is severe, and supplemental
blood flow through the ductus arteriosus is required
to support oxygenation during the neonatal period.
*A palliative shunt is often placed, the most common
of which is the Blalock-Taussig shunt. The shunting
procedure involves anastomosis of the subclavian
artery to the pulmonary artery, which will direct blood
from the systemic circuit into the pulmonary bed and
improve pulmonary blood flow.

59. *Corrective surgery involves closing the VSD with a
patch, relieving the right ventricular outflow
obstruction, and closing any previous palliative
shunts.
*Early corrective surgery is preferable to a palliative
procedure and can be performed in infancy if the
pulmonary arteries are of sufficient size and the
coronary artery connection is in a normal position.
*Without surgery, life expectancy is markedly
reduced. Even with successful surgery, heart block,
aneurysm formation, and late sudden death are
possible.

60. Transposition of the Great Vessels
*The arterial switch procedure is the surgical
intervention of choice, since it returns blood flow to
its normal pattern. This procedure involves cutting
the great vessels above the valves and switching
their positions with reimplantation of the coronary
arteries.
*When corrective surgery is not possible, a palliative
balloon or surgical septostomy is performed.
*If a balloon septostomy is not possible or not
effective, a surgical septostomy (Blalock-Hanlon
operation) can be performed.

61. *Corrective surgery involves partitioning the atrium and dividing the chamber into a front and a back section (Atrial Switch).
*Systemic venous blood is redirected in front of the partition toward the left ventricle and pulmonary venous blood is directed behind the partition toward the right ventricle.
*This partition can be made of a synthetic material (Mustard procedure) or of the child’s atrial septum (Senning procedure).
*The most common long-term complications of the Mustard and Senning procedures are arrhythmias

62. Truncus Arteriosus *Palliative pulmonary banding can be performed to
improve the condition and development of
pulmonary vascular disease until reparative
procedures can be performed.
*Since banding increases mortality and complicates
of corrective surgery, the corrective surgery is usually
attempted in infancy. Correction involves closing
the VSD so the left ventricle empties into the runcus.
*The pulmonary arteries are removed from the truncus
and a conduit is inserted between the right ventricle
and the pulmonary arteries (Rastelli procedure).

63. Total Anomalous Pulmonary Venous Return
*Surgery is usually indicated soon after the diagnosis
is confirmed. This operation involves the
anastomosis of the pulmonary veins to the left
atrium, closure of the ASD, and division of the
anomalous connection.
*Without surgery, the prognosis is poor.
*Even if surgery is performed within days of birth,
infants who have severe cyanosis and poor cardiac
output before repair have high postoperative
mortality. Connections above the diaphragm have a
better prognosis than connections below the
diaphragm.

64. Tricuspid Atresia *If pulmonary blood flow is minimal, palliative
surgery is frequently necessary soon after birth. A
connection is usually made between the subclavian
artery and the pulmonary artery (Blalock-Taussig).
*Enlargement of the interatrial defect may be
necessary to facilitate flow to the left atrium from the
right.
*Corrective surgery usually involves a Fontan
procedure, an anastomosis between the pulmonary
artery and the right atrium. Any previous shunt
procedure will be closed and the ASD patched.

65. Hypoplastic Left Heart Syndrome
*Staged surgical repair of HLHS is still in its infancy and the
mortality rate is high, but it does offer some hope for survival.
*The first surgery—the Norwood procedure—is performed
during the neonatal period. A portion of the main pulmonary
artery is anastomosed to the aorta, a shunt is performed to
increase the pulmonary blood flow, and a large atrial septal
defect is created.
*In the second stage, the volume load on the right ventricle is
reduced.
*The final repair is a modified Fontan procedure, in which
deoxygenated blood from the lower part of the body is directed
to the right pulmonary artery shunt to mix with blood returning
from the head and upper body and flow into the systemic
circulation.
*Heart transplantation is also an option for infants with HLHS.

66. Thank You