Download congenital heart diseases

CONGENITAL HEART DISEASES
By
Nusrum Iqbal MD
Congenital Heart Diseases in Adults
•CHD occurs in 0.3% of all live births
•Most patients are recognized in infancy or childhood and appropriately treated
•Most patients with CHD who have been surgically corrected or palliated will reach
adulthood or child-bearing age
•Embryology of the formation of the heart and cardiovascular system is complex, there
are many stages at which abnormality can occur, leading to wide variety of congenital
heart abnormalities
Congenital Heart Diseases
•PATENT DUCTUS ARTERIOSUS
•COARCTATION OF AORTA
•ATRIAL SEPTAL DEFECTS
•VENTRCULAR SEPTAL DEFECTS
•TETRALOGY OF FALLOT
ETIOLOGY
The etiology of congenital cardiac disease is often unknown but the recognized
associations include
•maternal rubella infection (persistent ductus arteriosus, and pulmonary valvular and
arterial stenosis)
•maternal alcohol abuse (septal defects)
•maternal drug treatment and radiation
•genetic abnormalities (e.g. ASD)
•chromosomal abnormalities (e.g. septal defects, mitral and tricuspid valve defects with
down’s syndrome or coarctation of aorta in Turner’s syndrome (45, XO))
Anatomic-pathophysiologic categories
•Predominant left -to-right shunts
•Predominant right-to-left shunts
•Stenotic or atretic valves and hypoplastic ventricles
•Great vessel abnormalities
•Positional abnormalities
•Other congenital syndromes
SIGNS AND SYMPTOMS
•Central Cyanosis (right-to-left shunt)
•Pulmonary hypertension (Eisenmenger’s syndrome)
•Clubbing (Cyanotic conditions)
•Paradoxical embolism
•Growth retardation (cyanotic heart diseases)
•Syncope (severe right of left ventricular obs.)
•Squatting (Fallot’s tetralogy)
Patent Ductus Arteriosus
•During fetal life, before the lungs begin to mature, most of the blood from the
pulmonary artery passes to the aorta through ductus arteriosus
•PDA connects the proximal descending aorta with the pulmonary artery at its bifurcation
•Left-to-right shunt
•Marked increase in pulmonary blood flow results in left sided volume overload with
increase in the size of the left atrium, left ventricle, ascending aorta, and aortic arch
•50% of the left ventricular output may be recirculated through the lungs
Clinical Features
•Asymptomatic with small lesions
•Growth and development may be affected with the larger ductus
•Dyspnea
•Continuous “ machinery murmur” with late systolic accentuation, maximal in the 2nd
left intercostal space below the clavicle
•Frequently accompanied by thrill
•Pulses increased in volume
INVESTIGATIONS
CHEST X-RAY.
Normal with small defect
Left ventricle and atrium may be enlarged
Pulmonary vascular marking are increased
ECG.
Left ventricular hypertrophy
Left atrial enlargement
Right ventricular hypertrophy
Course and Complications
•Danger of Infective endocarditis with small defect (prophylactic antibiotics required)
•Congestive Heart Failure
•Pulmonary Vascular disease
•Eisenmenger’s syndrome is the reversible of the shunting causing central cyanosis
and the murmur gets quieter and may be confined to the systole
Treatmaent
•During the first week of life, prostaglandin synthetase inhibitor( indomethacin) may be
used
•Ligation is indicated in young and middle aged person
•Close PDA percutaneously using intravascular devices
•Antibiotic prophylaxis is recommended
COARCTATION OF AORTA
•Narrowing of the aorta at, or just distal to, the insertion of the ductus arteriosus
•Twice as commonly in men as in women
•80% of cases associated with bicuspid (and potentially stenotic) aortic valves, others are
berry aneurysm of the cerebral circulation
•Formation of collateral arterial circulation involving the periscapular and intercostal
arteries
•Decreased renal perfusion can lead to the development of systemic hypertension
CLINICAL FEATURES
•Asymptomatic for many years
•Headaches and nose bleeds (due to HTN)
•Claudication and cold legs
•Hypertension in the upper limbs
•Weak and delayed pulses in the legs(radiofemoral delay)
•Systolic murmur is heard posteriorly, over the coarctation
•Ejection systolic murmur over the aortic area due to bicuspid valves
•Localized bruits due to the collateral formation
INVESTIGATIONS
CHEST X-RAY
•Aortic knob may be enlarged
•Indentation of the descending aorta ‘3 sign’
•Notching of the undersurface of the ribs
ECG
•Normal or show left ventricular hypertrophy
ECHOCARDIOGRAPHY
MAGNETIC RESONANCE IMAGING
ANGIOGRAPHY
•MRI or Angiography is needed to define the site and extent of narrowing
COURSE AND COMPLICATIONS
•May be asymptomatic
•Stroke
•Congestive Heart Failure
•Premature coronary artery disease
•50% of the patients died by about the age of 30 years and 90% by 60 years
•Proximal aortic rupture
•Aortic dissection
•Cerebral hemorrhage due to the rupture of berry aneurysm
•Infective endocarditis
Management
•Repair is indicated if the coarctation is severe (gradient >25-30mmHg)
•Primary balloon dilatation has been used successfully
•Treatment of Hypertension
•Baseline MRI after surgery
•Close clinical follow up
ATRIAL SEPTAL DEFECT
This condition is often first diagnosed in adults
More common in women than in men
Left-to-right shunt
Types
Ostium secundum defect (center) fossa ovalis
•the most common defect
Ostium primum defect (lower part )AV septum
•associated with cleft mitral valve (ant. Leaflet)
Sinus venosus defect (superior and posterior)
•associated with an anomalous drainage of one or more pulmonary veins
CLINICAL FEATURES
•Asymptomatic for many years
•Dyspnea
•Chest infections
•Cardiac failure
•Arrhythmias (atrial fibrillation)
•Weakness
•Palpitations
•Wide fixed splitting of the second heart sound
•Systolic flow murmur over the pulmonary valve
•Diastolic flow murmur over the tricuspid area due to large shunt
INVESTIGATIONS
CHEST X-RAY
•Prominent pulmonary artery and pulmonary plethora
•Right ventricular enlargement
ECG
•Right bundle branch block
•Right axis deviation
ECHOCARDIOGRAM
DOPPLER
Course and Complications
•Most patients are asymptomatic or minimally symptomatic
•Young women tolerate pregnancy
•Congestive heart failure
•Atrial fibrillation
•Pulmonary vascular disease with pulmonary hypertension
•Cyanosis
•50% survival beyond the age of 40 years
•Paradoxical embolization
Management
•Operative patch closure
•Percutaneous closure with variety of devices
•Treatment of atrial fibrillation
•Anticoagulation
•Prophylactic antibiotics are recommended only for the first 6 months after surgery
VENTRCULAR SEPTAL DEFECT
•Most common congenital cardiac malformation (1:500 live births)
•Occurs as an isolated abnormality or in association with other syndromes
•Left-to-right shunt
•Incomplete septation of the ventricles
•Two portion, membranous and muscular
•Defect usually are perimembranous
CLINICAL FEATURES
•Patient may be asymptomatic
•Pansystolic murmur heard best along the left sternal border in the 3rd or 4th intercostal
space
•Murmur is usually loud with the small defect
•signs and symptoms of CHF
•Fatigue and dyspnea
•Hyperdynamic, displaced apex beat
•There may be a diastolic flowing rumble
•Cyanosis in later stages
•Right ventricular parasternal heave
•Loud P2 component of the 2nd heart sound
INVESTIGATIONS
CHEST X-RAY
•Normal, LVE, LAE, RVE
•Prominent pulmonary vasculature and peripheral pruning of the pulm. Vasculature
ECG
•LVH or Biventricular hypertrophy
ECHOCARDIOGRAPHY & DOPPLER
REFERRAL TO CARDIOLOGIST
Course and Complications
Infective Endocarditis
Congestive Heart Failure
Eisenmenger’s syndrome
MANAGEMENT
•With smaller VSD, antibiotic prophylaxis is recommended
•Closure of the defect is recommended as long as the shunt is from left-to-right
•Repaired VSD donot require antibiotic prophylaxis except during first six months
postoperatively
TETRALOGY OF FALLOT
Most common cyanotic congenital heart abnormality
Four features
•a ventricular septal defect
•positioning of the aorta above the VSD (overriding aorta)
•right ventricular hypertrophy
•right ventricular outflow obstruction
Combination of lesions leads to a high right ventricular pressure and right-to-left
shunting of blood
CLINICAL FEATURES
•Dyspnea
•Fatigue
•Syncope
•Deep cyanosis
•Squatting
•Parasternal sustained heave
•Systolic ejection murmur
•2nd heart sound is soft
•Central cyanosis
•Growth retardation
•Finger clubbing
INVESTIGATIONS
CHEST X-RAY
•Large right ventricle and a small pulmonary artery
•Boot-shaped heart
ECG
•Right ventricular hypertrophy
•RBBB or varrying degree of AV block after repair
ECHOCARDIOGRAPHY
Course and Complications
•Most patients used to die in the 2nd decade of life before surgical interventions
•Arrhythmias
•Right heart failure
MANAGEMENT
Surgical repair can be performed successfully
The operations consists
•patching the ventricular septal defect
• relieving the right outflow tract obstruction
The patients should receive antibiotic prophylaxis postoperatively
CLINICAL FEATURES
•Dyspnea
•Fatigue
•Syncope
•Deep cyanosis
•Squatting
•Par