Download Congenital Heart Disease

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 rightto-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