CLASSIFICATION OF CONGENITAL HEART DISEASES

Clinical Classification of CHD

GENERAL Innocent or normal murmurs Cardiac malpositions Ventricular inversion (congenitally corrected transpositions of the great arteries) Complete heart block ACYANOTIC WITHOUT A SHUNT (Normal or decreased pulmonary arterial flow) Malformations originating in right side of heart (from most proximal to most distal) Malformations originating in left side of heart (from most proximal to most distal)

 ACYANOTIC WITH A SHUNT (LEFT TO RIGHT,increased pulmonary arterial flow) Shunt at arterial level Shunt at ventricular level Shunt between aortic root and right side of heart Shunt at aortopulmonary level Shunts at more than one level CYANOTIC Increased pulmonary arterial blood flow Normal or decreased pulmonary arterial blood flow Dominant left ventricle Dominant right ventricle With pulmonary hypertension Without pulmonary hypertension Normal or nearly normal ventricles

INNOCENT MURMURS
50% of normal children have an innocent heart murmur. No symptoms & signs Due to turbulent blood flow at the origin of the great vessels Better heard in children than in adults due to: Thin chest wall in children More angulated great vessels in children More dynamic circulation in children

Types of innocent murmurs Stills murmur: Due to blood flow across the aortic valve. Heard best over the right upper sternal border. It is a systolic ejection murmur, vibratory, musical in quality. Seen in infancy to adolescence Physiologic systolic ejection murmur; Second most common. Hear best at the left mid to upper sternal border. Heard best with diaphragm due to high frequency Supraclavicular arterial bruit: Above and not below the clavicle. Louder on right due to brachiocephalic arteries branching Neonatal peripheral pulmonary stenosis(PPS): Birth to 3-6 months. Heard best at base of the heart, both axillae and the back. Due to relative small size of branch pulmonary arteries and the angle of bifurcation of the Pas
Cervical venous hum; Continuous hum. Heard over right upper sternal border. Better in sitting, disappear in supine and when pressure is applied to neck to reduce venous blood return.

CARDIAC MALPOSITIONS
DEXTROCARDIA heart on right side 4 common types: 1.Classic mirror image 2.Normal heart displaced to right 3.Congenitally corrected TGA with situs solitus 4.Situs ambiguus with splenic syndromes MESOCARDIA heart in midline of thorax

VENTRICULAR INVERSION
Congenitally corrected TGAs Visceroatrial relationship is normal but there is ventricular inversion

COMPLETE HEART BLOCK

Atrial and ventricular activities are independent of each other Different from acquired CHB Normal QRS duration,Ventricular rate faster (50-80) & variable to various physiological conditions

MALFORMATIONS ORIGINATING IN LEFT HEART

Aortic stenosis Coarctation of aorta Congenital mitral incompetence Primary endocardial firbroelastosis Congenital obstruction to left atrial flow -Cor triatum -Mitral stenosis -Pulmonary vein stenosis Congenital aortic incompetence

MALFORMATIONS ORIGINATING FROM RIGHT HEART

Pulmonary stenosis Idiopathic dilatation of pulmonary artery Congenital pulmonary valve incompetence Primary pulmonary hypertension Hypoplastic right ventricle

ACYANOTIC WITH SHUNT

SHUNT AT ATRIAL LEVEL ASD (isolated) ASD with mild pulmonic stenosis Total anomalous pulmonary venous connection with low pressure pulmonary vascular resistance Partial anomalous pulmonary venous connection with intact ASD ASD with Mitral stenosis ( Lutembachers syndrome)

SHUNT AT VENTRICULAR LEVEL VSD (isolated) VSD with mild PS ( Acyanotic Fallots tetralogy) VSD with right ventricular origin of both great arteries VSD with aortic incompetence VSD with left ventricular to right atrial shunt VSD with complete interruption of aortic arch

SHUNT BETWEEN AORTIC ROOT & RIGHT HEART Coronary arteriovenous fistula Ruptured sinus of Valsalvas aneurysm
SHUNT AT AORTICOPULMONARY LEVEL PDA Aorticolpulmonary septa defect Anomalous origin of left coronary artery from pulmonary artery Truncus arteriossus with large pulmonary arteries & low pulmonary vascular resistance

SHUNT AT MORE THAN ONE LEVEL Complete endocardial cushion defect VSD with PDA VSD with ASD

CYANOTIC WITH INCREASED PULMONARY VASCULARITY

With RVH: D-TGA TAPVR with obstruction DORV with subpulmonary VSD (Taussig-Bing anomaly) PPHN With LVH or BVH : Persistant truncus arteriosus (type I) Single ventricle (without PS) TGA & VSD Polysplenia syndrome

CYANOTIC WITH DECREASED PULMONARY VASCULARITY

With RVH: TOF DORV with PS Asplenia syndrome With RBBB: Ebsteins anomaly With LVH: Pulmonary atresia Tricuspid atresia With BVH: TGA with PS Persistant truncus arteriosus (type II or III) Single ventricle & PS

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