Congenital Heart Diseases

Dr. Vineet Chauhan

M.S.,F.M.A.S.,F.I.A.G.E.S.
 Congenital heart disease (CHD)
• A cause of heart failure in children
• Consequences depend on lesion:
– Location
– Size of defects
– Nature of abnormality
 Causes of CHD
• 80% unknown
• Infection – rubella
• Chromosomal
Turners syndrome XO – coarctation of aorta,
Downs trisomy 21 – ASD/VSD/PDA
• Poorly defined familial susceptibility
 Acyanotic CHD
• Arterial saturation remains normal so body –never cyanotic
• Patent Ductus Arteriosus
• Coarctation of Aorta
• Atrial Septal defect
• Ventricular Septal defect
 Patent Ductus Arteriosus
• Incidence – 1/ 4000 births, common in females
• Aetiology- PDA develops from 6th left aortic arch
• Connects pulmonary artery with aorta by passing the collapsed
lungs
• At birth-lung expands blood passes to Pulmonary artery and
PDA collapses and becomes fibrosed –ligamentum arteriosum
• PGE1 prevents closure
Patent Ductus Arteriosus
 Fetal Circulation
• Placenta oxygenates blood
and returns to right atrium
(RA) via IVC.
• Preferentially shunts
across Foramen Ovalis to
Left Atrium.
• LV ejects most oxygenated
blood to carotids and
coronaries.
 Fetal Circulation
• Superior vena cava (SVC) returns deoxygenated blood to Right Atrium
where it mixes with oxygenated blood from the placenta.
• Preferentially enters R Ventricle.
• RV ejects into Pulmonary Artery.
• No pulmonary capillary flow, so PA is shunted into the descending aorta
via the ductus arteriosus.
 Pathology
• Aortic pressure high- flow of blood from aorta to Pulmonary
artery via PDA
• Large Ductus – 50 -70 % shunting
• Dialated blood vessels of lungs (pulmonary plethora)
• Increased pulsations (hilar dance)
• Additional blood in lungs –more blood in left heart –left
ventricular hypertrophy
 Clinical features
• Depends on size of Ductus
• Asymptomatic to serious heart failure
• Heart failure
– Failure to thrive
– Difficulty in breast feeding
– Breathlessness
• Physical signs
– Continuous murmur accentuated in systole and decreased on diastole
– Pulse collapsing nature, wide pulse pressure
– Thursting left ventrical apical pulse
Complication- cardiac failure, bacterial endocarditis
 Investigation
• Chest X-Ray: normal
• Flouroscopy- hilar dance
• ECG- changes of LVH
• 2-D Echo- diagnostic
• Cardiac catherization-localizes the shunt
• Aortography- diagnostic
 Treatment
• Indomethacin, a prostaglandin E1 inhibitor may close a PDA in
preterm babies

• Surgical treatment at age of 2-3 yrs

• Left posterolateral thoracotomy by ligation, clipping
Patent Ductus Arteriosis
Patent Ductus Arteriosis
 Coarctation of aorta
• Frequent in males: m:f :: 2:1
• Aetiology-Narrowing of aorta beyond the origin of subclavian
artery
• Theory-extension of the fibrotic process of PDA into
ligamentum arteriosum
Coarctation of aorta
 Pathology
• Constriction 2-4 cm below the origin of L subclavian artery
• Lumen -1-3 mm
• Distal to coarctation- aorta dilated
• Dilated intercostal arteries provide collateral circulation
• Notching of ribs by arteries
• Rarely arteries become aneurysmal and rupture
 Pathology cont.
• Two types
• Post ductal type or adult coarctation: beyond the PDA
– common
• Pre ductal type or infantile type-
– PDA remains patent
– Deoxygenated blood from pulmonary arteries –aorta( distal to
coarctation)
– Lower trunk and extremities –cyanosed
– Head neck upper extremities pink- differential cyanosis
 Clinical Features
• Hypertension above obstruction
• Hypotension below obstruction
• Headche , dizziness, epistaxis, throbbing and pulsation of arteries
of head and neck systolic murmur, left ventricular failure
• Weak femoral pulsation, cold lower extremities, intermittent
claudication
• Complications-LVF, intracranial hemorrhage, intrathoracic
hemorrhage and rarely rupture of aorta
 Investigation
• Chest X Ray- notching of ribs
• 2Decho, ECG
• Cardiac catheterization and aortography
Coarctation of the Aorta
 Treatment
• Infants- with CCF: operation urgently
• Ideal age – 5-7yrs
• Operative Rx- Posterolateral thoracotomy
• Excision of segment
• End to end anastomosis
• Dacron graft in 10% cases as intervening segment
 Atrial Septal Defect
• 6% of all congenital heart diseases
• Defect allows the blood to flown from left atrium to right
atrium-rt side of heart and lungs are over filled and left side
receives less blood
• Most common is a patent foramen ovale (overlap of septum
primum/septum secundum)
• Late effect – pulmonary hypertension
Atrial septal defect
 Types-1.Secundum defect
• Secundum defect-
– Commonest variety
– Defect at centre
– Embryologically failure of development
• Symptoms
– Uncommon in first few yrs of life
– Fatigue, palpitation and exertional dyspnoea
– Systolic murmur at pulmonary area
– Second pulmonic sound widely split
• Treatment –
• Direct suturing of the defect with prolene
• If gap big – dacron prosthetic patch
 Type 2. Ostium primum defect
• Ostium primum
– Infrequent defect
– Associated with incomplete formation of mitral or tricuspid valves
• Surgery-
– 4-6yrs
– Suturing of the mitral valve cleft from the ventricular septum to the
mitral orifice with interrupted sutures
– Septal defect is closed with patch of pericardium or dacron
– Tricuspid valve usually not ammenable to suturing
 Type 3. Anomalous drainage of PV
• Anomalous drainage of pulmonary veins along with ASD
– Failure of incorporation of sinus venosus into atrium proper
– Rt pulmonary vein enters superior venacava or rt atrium or inferior
vena cava
– Lt pulmonary vein –superior venacava
• Treatment
– Single vein – no treatment
– Both veins – pulmonary veins transposed to lt atrium with closure of
atrial defect with prosthetic patch
 Ventricular septal defect
• 20 -30% of congenital heart diseases
• Associated with complex diseases like fallots tetralogy
• Defects-
– Single/ multiple
– Membranous / fibrous part of septum
– Anterior or posterior defects – crista supraventricularis
• Posterior defects near the mitral and tricuspid valves
• Anterior defects are away from bundle of His so safe to
suture
• 3mm to 3cm
• >1cm defects- large
• Blood shunted from left to right
• Overfilling of rt heart and pulmonary hypertension
VSD
 Clinical features
• Small- asymptomatic
• Large-
• Exertional dyspnoea
• Pulmonary hypertention
• Cardiac failure
Signs
Loud pansystolic murmur3-4 left intercostal space
Thrill
Rales
Growth retardation
 Investigations
• X Ray- small defect- normal
• Big defects- ventricular enlargement
• ECG-ventricular hypertrophy
• 2DEcho- diagnostic
• Cardiac catheterization access the lt to rt shunt
 Treatment
• Surgery- closure of defect by midline sternotomy
• Defect closed with dacron prosthetic material
 Cyanotic congenital heart diseases
• Pt becomes cyanosed soon after birth
 Cyanosis
• Cyanosis in children happens when there is clinically apparent amount
of desaturated hemoglobin

• Usually requires 3-4 grams/ dL of reduced hemoglobin

• This usually correspond to oxygen saturation of 70-80%

• Therefore mild desaturation may clinically be missed

 General causes of Cyanosis

Cyanosis

Pulmonary Cardiac Others

Intrapulmonary Intracardiac
Airway disease
shunting shunting
 Mechanisms of Cardiac Cyanosis

Right to left Recirculation

Mixing
shunt

TOF Single ventricle TGA

 Cyanotic Heart Disease
Tetralogy of
Fallot

Transposition
Of Great Arteries

Truncus
Arteriosus

Tricuspid
Atresia

Total Anomalous
Pulmonary venous Return
 Fallots tetralogy
• Combination of 4 abnormalities- tetralogy
• Stenson 1671
• Understood by Fallots- 1888
• Constitutes 50% of all cyanotic heart diseases
 Tetralogy of Fallot (TOF)
1. Pulmonary
stenosis
(obstruction of
outflow tract of
rt ventricle)
2. VSD
3. Overriding aorta
4. RV hypertrophy
 Pathology
• Ventricular obstruction- infundibular or valvular
• Rt ventricular obstruction increases the rt ventricular systolic pressure
• Equal to the left ventricular pressure
• VSD is large 2-3cm
• Rt Ventricular venous blood shunted to aorta– cyanosis
• Rt ventricular pressure never exceeds lt ventricular pressure—cardiac
enlargement and failure not seen
• Pulmonary flow decreased –severe anoxia
• 1/3 rd pt are cyanotic at birth and do not survive infancy if not operated
 Clinical features
• All pts symptomatic
• Dyspnoea on exertion
• After walking short distance pt squats- pathognomic
• Signs
• Varying degree of cyanosis
• Clubbing
• Heart normal size
• Systolic murmur 3-4 ICS left side with thrill
• Second pulmonic sound decreased, aortic sound increased
 Investgations
• Chest Xray- non specific
• ECHO- diagnostic
• Cardiac catheterization-
– rt to lt shunt
– Decreased blood flow in lungs
– Equal pressure in ventricles
• Selective angiocardigraphy-
– status of pulmonary arteries and aorta
 Treatment
• Life saving anastomotic procedure
• Shunt procedures of ill babies <4months
• Blalock shunt- left subclavian with left pulmonary artery
• Waterson- ascending aorta with rt pulmonary artery
• Aorto pulmonary anastomosis with goretex graft
• Potts- descending aorta with lt pulmonary artery
Surgical Palliation
 Surgical Management -corrective
• VSD closure
– transatrial / trans ventricular access if possible
– Patch closure of VSD
• Relief of RV outflow obstruction
– Infundibular area widened with annular patch
 Late complications
• Conduction abnormalities
– RBBB
– Left anterior hemi-block
– Atrial flutter and fibrillation
– Ventricular tacharrhythmias
Transposition of Great Areries (TGA)
• Aorta originating
from the right
ventricle, and
pulmonary artery
originating from the
left ventricle
• Accounts for 5-7% of
all congenital heart
disease
• Incompatible with life unless communication with pulmonary
and systemic circulation
• Patent ductus arteriosus
• ASD
• VSD
• Only if above three than baby survives
 Clinical features
• Infants deeply cyanotic at birth
• Anoxic spells of unconciousness
• Cardiac failure
• Dypnoea on exertion
• Child survives more than 2yrs –clubbing, polycythemia
 TGA .. Acute Management
• PGE-1 with no supplemental O2
Maintain ductus arteriosus patency, this will increase the effective
pulmonary blood flow, and thence increase the left atrial pressure,
therefore inhance the left to right shunt at the atrial level
• Balloon atrial septostomy
Life saving procedure in the presence of inadequate atrial septal defect
 Corrective operation
• Total corrective transposition done within 1st two yrs of life
• Switching over the aorta and pulmonary artery and
transposition of abnormal coronary arteries