Congenital Heart Disease

• congenital heart disease can be organized into three major categories:

Malformations causing a left-to-right shunt

Malformations causing a right-to-left shunt

Malformations causing an obstruction

A shunt is an abnormal communication between chambers or blood vessels

• right-to-left shunt:

pulmonary/venous ---- Systemic/arterial

Deoxygenated ------ Oxygenated --- cyanosis

Hypoxia ----- clubbing (hypertrophic osteoarthropathy)

paradoxical embolism

• left-to-right shunt:

Systemic/arterial ---- pulmonary/venous

Oxygenated ----------- deoxygenated--- no cyanosis

right ventricular hypertrophy

Pulmonary congestion ------ hypertension

Rt-to-left shunt (Eisenmenger syndrome)--- cyanosis

• left-to-right shunt:

- m.C

- ASD, VSD (m.c) , PDA, AVSD

• right-to-left shunt:

Tetralogy of Fallot ------- m.c

Transposition of the Great Arteries

persistent truncus arteriosus

Tricuspid atresia

total anomalous pulmonary venous connection

• Obstructive Lesions:

aortic or pulmonary valve stenosis or atresia

coarctation of the aorta

subpulmonary stenosis in TOF

ASD

• Defect in septum primum or secundum

Mortality is low, and long-term survival is comparable to that of the normal population.

Asymptomatic until adulthood

Patent Foramen Ovale

• foramen ovale/ostium secundum permits continued right-to-left shunting of blood during intrauterine development.

• the unsealed flap can open if right-sided pressures become elevated (coughing, /sneezing) can produce brief periods of right-to-left shunting

Ventricular Septal Defect

• Membranous (m.c), muscular, infundibular

• The functional consequences of a VSD depend on the size

Patent Ductus Arteriosus

• ductus arteriosus---pulmonary artery---aorta

• During intrauterine life, it permits blood flow from the pulmonary artery to the aorta, thereby bypassing the unoxygenated lungs.

• + prostaglandin, immuture

• Used to save life in pulmonary or oartic valve obstruction or atresia

atrioventricular (AV) septal defect

• Endocardial cushion defect

• Down syndrome

Tetralogy of Fallot

(1) VSD

(2) subpulmonary stenosis/infundibulum

(3) an aorta that overrides the VSD

(4) Right ventricular hypertrophy

Transposition of the Great Arteries

• Aorta---anterior---right ventricle

• pulmonary artery---posterior---left ventricle

• Parallel instead of series

• incompatible with postnatal life unless a shunt exists for adequate mixing of blood.

persistent truncus arteriosus

• failure of separation of truncus into aorta and pulmonic trunck .

• The truncus overrides both ventricles.

• Always accompanied by a membranous VSD.

total anomalous pulmonary venous return (TAPVR)

• pulmonary veins----innominate vein/coronary sinus

• Patent foramen ovale/ or ASD always present

Coarctation of the Aorta

• Narrowing

• 2 types

1- infantile: + PDA---- early after birth--- cyanosis in lower ½ of body

2- adult: - PDA---- late presentation---- HTN in upper extremities

Aortic Stenosis and Atresia

• Valvular--- hypoplastic left heart syndrome

• Supravalvular

• Subvalvular----- sudden death with exertion