Cyanotic Congenital

Heart Diseases
Dr. Ravi Gadani
MS, FMAS
Cyanotic congenital heart diseases
• Pt becomes cyanosed soon after birth
Cyanosis
• Cyanosis in children happens when there is clinically apparent amount
of desaturated hemoglobin

• Usually requires 3-4 grams/ dL of reduced hemoglobin

• This usually correspond to oxygen saturation of 70-80%

• Therefore mild desaturation may clinically be missed

 Cyanosis

Pulmonary Cardiac Others

Intrapulmonary Intracardiac
Airway Dz
Shunting Shunting
 Right-to-Left Shunt TOF

Mechanisms of
Mixing Single Ventricle
Cardiac Cyanosis

Recirculation TGA
 TOF

Transposition of
Great Arteries

Cyanotic Heart Truncus

Diseases Arteriosus

Tricuspid Atresia

Total Anomalous
Pulmonary
Venous Return
Fallots tetralogy
• Combination of 4 abnormalities- tetralogy
• Stenson 1671
• Understood by Fallots- 1888
• Constitutes 50% of all cyanotic heart diseases
Tetralogy of Fallot (TOF)
1. Pulmonary stenosis
(obstruction of outflow
tract of rt ventricle)
2. VSD
3. Overriding aorta
4. RV hypertrophy
Pathology
• Ventricular obstruction- infundibular or valvular
• Rt ventricular obstruction increases the rt ventricular systolic pressure
• Equal to the left ventricular pressure
• VSD is large 2-3cm
Pathology
• Rt Ventricular venous blood shunted to aorta– cyanosis
• Rt ventricular pressure never exceeds lt ventricular pressure—cardiac
enlargement and failure not seen
• Pulmonary flow decreased –severe anoxia
• 1/3 rd pt are cyanotic at birth and do not survive infancy if not
operated
Clinical features
• All pts symptomatic
• Dyspnoea on exertion
• After walking short distance pt squats- pathognomic
• Signs
• Varying degree of cyanosis
• Clubbing
• Heart normal size
• Systolic murmur 3-4 ICS left side with thrill
• Second pulmonic sound decreased, aortic sound increased
Investigations
• Chest Xray- non specific
• ECHO- diagnostic
• Cardiac catheterization-
• rt to lt shunt
• Decreased blood flow in lungs
• Equal pressure in ventricles
• Selective angiocardigraphy-
• status of pulmonary arteries and aorta
Treatment
• Life saving anastomotic procedure
• Shunt procedures of ill babies <4months
• Blalock shunt- left subclavian with left pulmonary artery
• Waterson- ascending aorta with rt pulmonary artery
• Aorto pulmonary anastomosis with goretex graft
• Potts- descending aorta with lt pulmonary artery
Surgical Palliation
Surgical Management -corrective
• VSD closure
• transatrial / trans ventricular access if possible
• Patch closure of VSD
• Relief of RV outflow obstruction
• Infundibular area widened with annular patch
Late complications
• Conduction abnormalities
• RBBB
• Left anterior hemi-block
• Atrial flutter and fibrillation
• Ventricular tacharrhythmias
Transposition of Great Arteries (TGA)
• Aorta originating from the right
ventricle, and pulmonary artery
originating from the left ventricle
• Accounts for 5-7% of all
congenital heart disease
Transposition of Great Arteries (TGA)
• Incompatible with life unless communication with pulmonary and
systemic circulation
• Patent ductus arteriosus
• ASD
• VSD
• Only if above three than baby survives
Clinical features
• Infants deeply cyanotic at birth
• Anoxic spells of unconciousness
• Cardiac failure
• Dypnoea on exertion
• Child survives more than 2yrs –clubbing, polycythemia
TGA -Acute Management
• PGE-1 with no supplemental O2
• Maintain ductus arteriosus patency, this will increase the effective pulmonary
blood flow, and thence increase the left atrial pressure, therefore inhance the
left to right shunt at the atrial level
• Balloon atrial septostomy
• Life saving procedure in the presence of inadequate atrial septal defect
Corrective operation
• Total corrective transposition done within 1st two yrs of life
• Switching over the aorta and pulmonary artery and transposition of
abnormal coronary arteries