Congenital Heart Disease

Introduction
• A congenital heart disease is a problem with the structure of the heart,
present at birth and the most common type of birth defect.
• The defects can involve the walls of the heart, the valves of the heart,
and the arteries and veins near the heart.
• Defects can disrupt the normal flow of blood through the heart.
• They occur due to abnormal forming of cardiovascular system mostly
during the 6th to 8th weeks of gestational age.
Introduction cont..

• It may be diagnosed before birth, right after birth, during childhood, or

not until adulthood.
•It is possible to have a defect and no symptoms at all.
• In adults, if symptoms are present, they may include: shortness of
breath and limited ability to exercise.
Epidemiology
• CHD is the most frequently occurring congenital disorder, responsible
for 28% of all congenital birth defects. The birth prevalence of CHD is
reported to be 8-12/1000 live births.
• Considering a rate of 9/1000, about 1.35 million babies are born with
CHD each year globally.
• In Nepal, prevalence of CHD is 7 per 1000 (Shrestha et. al., 2023)
Types

1) Acyanotic heart diseases and

2) Cyanotic heart diseases.
Cyanotic Heart Disease

• Tetralogy of Fallot
•Transposition of the great arteries
•Tricuspid atresia
•Pulmonary atresia
•Persistent truncus arteriosus
•Total anomalous pulmonary venous return
Acyanotic Heart Disease

•Ventricular septal defect

•Atrial septal defect
•Patent ductus arteriosus
•Atrioventricular septal defect
Obstructive Heart Disease

•Pulmonic stenosis
•Aortic stenosis
•Coarctation of aorta
Causes

Maternal causes:
• Infection: febrile illness in first trimester
• Disease condition (diabetes, rubella, systemic lupus erythematous)
• Smoking, Alcohol intake
• Maternal intake of teratogenic agents (eg, lithium, anticonvulsants,
NSAIDs, cotrimoxazole, thalidomide, retinoic acid etc.)
Causes cont..

Prenatal exposure and the risk of congenital heart disease:

• Gestational diabetes: (transposition, atrioventricular septal defects,
hypoplastic left heart, cardiomyopathy, PDA)
•Rubella: (PDA, peripheral pulmonary stenosis, VSD)
•Lupus: (complete heart block)
•Phenylketonuria: (VSD, ToF, PDA, single ventricle)
Causes cont..

•Fetal causes: Chromosomal Abnormality, syndromic condition.

•Genetic Cause: Parents with CHD
• Environmental Cause: Exposure with environmental pollution,
exposure with organic solvents, herbicides, pesticides, ionizing
radiation.
Clinical Features

•Slow feeding •Tachycardia

•Breathelesness •Tachypnea
•Irritability •Cardiac murmur
•Pallor and sweating •Cardiomegaly
•Failure to gain weight •Shock
•Cyanosis
Diagnosis

• History taking
•Physical examination (auscultation of heart sound)
•Chest X-ray
•Electrocardiogram ( ECG)
•Echocardiogram or transesophageal echocardiogram (TEE)
•Cardiac catheterization
•MRI
A. Acyanotic Heart Disease
Atrial Septal Defect (ASD)

• Abnormal opening between the atria, allowing blood from the higher-
pressure left atrium to flow into the lower pressure right atrium.
• It account for 5 to 10% of all CHD.
Types:

•Ostium primum (ASD 1)- opening at the lower end of septum; may be
associated with mitral valve abnormalities.
•Ostium secundum (fossa ovalis) (ASD 2)- opening near center of atrial
septum, in the position of foramen ovale. These defects are amenable to
closure in the catheterization laboratory. It is the most common type of ASD.
•Sinus venosus defect- opening near junction of superior vena cava and right
atrium; may be associated with partial anomalous pulmonary venous
connection.
•Coronary sinus ASD- It occurs when there is a defect in the wall between
the coronary sinus and the left atrium.
Pathophysiology

Increased left atrial pressure (Oxygenated blood)

Left to right shunt
Burden on right side of the heart
Increased pulmonary blood flow
Pulmonary stenosis/ pulmonary hypertension
Right ventricular hypertrophy
Increased right atrial pressure
Right to left shunt
Cyanosis
Clinical manifestations

•Patient may be asymptomatic

•May develop congestive heart failure
•Heart murmur present: larger the shunt, the more louder the pulmonary
and tricuspid murmurs.
• Easy fatigability, recurrent respiratory infections, or exertional
dyspnea.
•Poor weight gain
•Swelling of legs, feet or abdomen
Findings

• The enlarged right ventricle results in a parasternal impulse.

•The ejection systolic murmur originates from the pulmonary valve
because of the increased blood flow.
•Echocardiogram shows increased size of the right ventricle
Complications
A small proportion of patients might develop pulmonary hypertension,
by the second or third decade.
•ASD closure is recommended to prevent complications of atrial
arrhythmias and heart failure in late adulthood.
Treatment:
• Small defects (<8 mm) can be observed. Spontaneous closure is well
recognized in small defects that are diagnosed in infancy or early
childhood.
• Most fossa ovalis defects with good margins can be closed
percutaneously in the catheterization laboratory with occlusive
devices.
• Other requires surgical closure. Closure is recommended before school
entry to prevent late complications.
Treatment cont..
Surgical treatment
• Surgical patch closure (pricardial patch or dacron patch) is done for
moderate to large defects.
• Open repair with cardiopulmonary bypass is usually performed before
school age.
• Sinus venosus defect requires patch placement, so the anomalous right
pulmonary venous return is directed to the left atrium with a baffle.
• ASD 1 type may require mitral valve repair or rarely, replacement of
the mitral valve.
Treatment cont..
Non-surgical treatment
• ASD 2 closure with a device during cardiac catheterization is
becoming common place and can be done as an outpatient procedure.
• The Amplatzer Septal Occluder is most commonly used. Smaller
defects that have a rim around them for attachment of the device can
be closed with a device; large irregular defects without a rim require
surgical closure.
•Patient should receive low dose of aspirin for 6 months
Prognosis:
• Operative mortality is very low (<1%)

Complications
•Right sided heart failure
•Pulmonary hypertension
•Eisenmenger syndrome
2. Ventricular Septal Defect (VSD)

• VSD is the most common congenital cardiac lesion identified at birth

accounting for one-quarter of all CHD.
• VSD is a communication between the two ventricles; 90% are located
in the membranous part of the ventricular septum with variable
extension into the muscular septum. Others are located in the muscular
septum and can be multiple.
Cont..
• Frequently associated with other defects, such as pulmonary stenosis,
transposition of the great vessels, patent ductus arteriosus, atrial
defects and coarctation of the aorta.
• Many VSD 20-60% will close spontaneously during the first year of
life in children having small or moderate defects. Here is also left to
right shunt.
Clinical manifestations

•Asymptomatic around 6-10 weeks of age.

•A sign of congestive heart failure is common.
• Palpitation, dyspnea on exertion and frequent chest infection are the
main symptoms in older children.
•Wide pulse pressure.
• Loud pansystolic murmur heart beat at the left sternal border (heard
throughout systole).
• Patients are at risk for bacterial endocarditis and pulmonary vascular
obstructive disease.
Treatment

Medical management:
• Medical management consists in control of congestive cardiac failure,
treatment of repeated chest infections and prevention and treatment of
anemia and infective endocarditis.
•Diuretics is given to control congestive cardiac failure
• Digoxin can be used to strengthen the heart muscle, so it’s able to
pump more efficiently
• Antibiotics may be given to prevent bacterial endocarditis and other
infection such as chest infections.
Nonsurgical treatment (catheter closure):

•Device closure during cardiac catheterization is being performed in

some centers under investigational protocols.
•It is best suited for muscular defects in relatively older children (>8 -10
kg). There is a device designed for perimembranous defects as well.
Nonsurgical treatment (catheter closure):

•Operative treatment consists in closure of VSD with the use of a patch.

The operation is performed through the right atrium. The operation can
be done as early as a few months after birth if congestive failure cannot
be controlled with medical management.
•With evidence of pulmonary hypertension, the operation should be
performed as early as possible.
•Sick infants with pneumonia who require mechanical ventilation,
surgery is considered after initial control of the infection.
Surgical treatment of VSD:

• Complete repair- (procedure of choice)- small defects are repaired

with sutures.
• Large defects usually require that a knitted Dacron patch be sewn over
the opening.
• Cardiopulmonary bypass is used for both procedures. The approach
for the repair is generally through the right atrium and the tricuspid
valve.
• Post operative complications include residual VSD and conduction
disturbances.
• Palliative- Pulmonary artery banding may be done in infants with
multiple muscular VSDs or complex anatomy.
Prognosis:
Risks depend on the location of defect, the number of defects, and the
presence of other associated cardiac defects.
•Single membranous defects are associated with low mortality (<2%);
multiple muscular defects can carry a higher risk.
•Children respond well to surgery and experience substantial catch-up
growth.

Complications: Tachyarrhythmias and right bundle branch block are

possible complications.
References
•Ghai OP.Essential Pediatrics.9th edition.New Delhi.2019:
•Gupte Suraj.The short textbook of pediatrics.13th edition.New
Delhi.2020.
•Wong D.L., Essential of Pediatric Nursing, 5th edition, Mosby ,1997.
•Hockenberry MJ. Wilson D. Rodgers CC. Wong’s essentials of
Pediatric Nursing. 2nd Ed. Thomas Press India Ltd: Elsevier; 2018
•Dutta DC. (2019) Textbook of Obstetrics .9 th edition; Jaypee Brothers
medical Publisher Ltd, New Delhi
•Subedi D.(2020) Textbook of Midwifery Nursing Part-III.1 st
edition;Akshav Publiation , Kathmandu.
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