Cyanotic Congenital Cardiac Defects

Cyanotic cardiac defect is a group-type of congenital heart defects (CHDs),In a specific topic congenital heart defects which are associated with Cyanosis that occurs due to deoxygenated blood bypassing the lungs and entering the systemic circulation or a mixture of oxygenated and unoxygenated blood entering the systemic circulation. It is caused by structural defects of the heart (ie:right-to-left , bidirectional shunting, malposition of the great arteries), or any condition which increases pulmonary vascular resistance. The result being the development of collateral circulation is called as Cyanotic Congenital Cardiac Defects.

Condition includes

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

Increased pulmonary blood flow

Transposition of the great arteries

Truncus arteriouses

Total anomalous pulmonary venous connection 

Normal to decreased pulmonary blood flow

          I. Dominant left ventricle

(a) Tricuspid atresia

(b) Ebstein’s anomaly of the tricuspid valve

         II. Dominant right ventricle

(a)  Normal or low pulmonary arterial pressure Tetralogy of Fallot

(b)  Elevated pulmonary arterial pressure Ventricular septal defect with reversed shunt (Esenmenger’s complex)

Cyanosis AT birth (2 As, 5 Ts)

        Artresia of aorta

        Atresia of pulmonary artery

        Tricupid atresia

        Truncus arterious

        Transposition of vessels

        Tetralogy of Fallot

        Total anomalous pulmonary venous drainage

Common lesions (if child survives infancy)

Non-Cyanotic Congenital Cardiac Defects :  Right Ventricular Hypertrophy : Atrial Septal Defect,Pulmonary Stenosis

Left Ventricular Hypertrophy : Patent Ductus Arteriosus, Ventricular Septal Defect, Coarctation

Cyanotic Congenital Cardiac Defects     :   Tetralogy of Fallot

Single gene defects and congential heart disease

Noonan’s syndrome       :  Hypertrophic cardiomyopathy

Pulmonary Stenosis

Atrial Septal Defect

Marfan’s syndrome        :  Aortic root dilatation

Mitral Valve Prolapse

Holt-Oram syndrome     :   Atrial Septal Defect

Ventricular Septal Defect (occasional)

Down’s syndrome

40% have congenital heart disease

40% of these are A-V septal defects

Other Lesions

Ventricular Septal Defect   :         30%

Atrial Septal Defect               :         10%

Tetrology of Fallot                 :         5%

Patent Dutus Arteriosus       :         5%

 

	ASD	PS	PDA	VSD	Coarctation
As.anomalies	APVD	VSD ASDAortic root ano.	Isolated (usually)	PS	PDA BicuspidAort.  Valve Aneu.of sinus of Valsalva
Signs	Sys.mur.S2Wide split	Mild Harsh sys.mur.thrill click P2 delay severe No click P2 Absent	Cont. Machi-nery mur. Diff. cyanosis	Pansys. Mur. Thrill 4th LIS	Delayed femorals Reversed Hills Suzmans Ejection click Sys.mur
ECG	RBBB	RVH plus strain	LVH	LVH or LVH plus RVH	LVH
Radiology	Hilar dance	Oligemia Post-sten.dil.	Prominent pul.conus	Vascularity plus RVH	Rib notching Reversed 3 sign
Other features	Only cong. Lesion in which acquired lesion may be present	Moon face (at times)	Compen.channel in Tetra logy Tricuspid atresia Transp.	Loudness and length of murmur inversely prop.to size of defect	4-5 times more in males lower limbus less developed

Incidence of congenital heart disease

0.8 percent of all live births (Excluding MVP and bicuspid aortic valve)

Manifest with failure to survive to adulthood or progressive hemodynamic changes

Biscupid aortic valve (2 percent of population)

Mitral valve prolapse (5 percent of population)

Most common congenital heart diseases in adult are stenosis of bicuspid aortic valve and Atrial septal defect.

Common Congenital Heart Diseases at Birth

i.      Ventricular septal defect (30percent)

ii.     Atrial septal defect

iii.     Patent ductus arteriouses

iv.     Pulmonic stenosis

v.      Coarctation of aorta

vi.      Aortic stenosis

vii.     Tetralogy of fallot

viii.    Transposition of great vessels

ix.      Persistent truncus arteriousus

x.       Tricuspid atresia

xi.       Double outlet right ventricle

xii.      Annomalous pulmonary venous connections

Treatment options in Cyanotic Congenital Cardiac Defects

Morphine during Tet spells to decrease associated infundibular spasm.
Prophylactic: Propranolol/Inderall
Prostaglandin E (to keep the ductus arteriosus patent)
Prophylactic antibiotic to prevent endocarditis
Surgery