A cyanotic heart defect is a group-type of congenital heart defects (CHDs). The patient appears blue (cyanotic), due to deoxygenated blood bypassing the lungs and entering the systemic circulation. This can be caused by right-to-left or bidirectional shunting, or malposition of the great arteries. Cyanotic heart defects, which account for approximately 25% of all CHDs, include: Tetralogy of Fallot (ToF) Total anomalous pulmonary venous connection Hypoplastic left heart syndrome (HLHS) Transposition of the great arteries (d-TGA) Truncus arteriosus (Persistent) Tricuspid atresia Interrupted aortic arch Pulmonary atresia (PA) Pulmonary stenosis (critical) Eisenmenger syndrome(Reversal of Shunt due to Pulmonary Hypertension) . Patent ductus arteriosus may cause cyanosis in late stage.

1. Cyanotic Heart Disease;
Overview of Management
By
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. 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