Congenital heart diseases

CONGENITAL
HEART DISEASES
BY
M. Sandeep kumar MSc(N)
SVIMS
TIRUPATI

∗ Congenital cardiovascular defects, also known as congenital heart
defects, are structural problems that arise from abnormal formation of
the heart or major blood vessels. Defects range in severity from tiny
pinholes between chambers that may resolve spontaneously to major
malformations that can require multiple surgical procedures before
school age and may result in death in utero, in infancy, or in childhood.
AMERICAN HEART ASSOCIATION
INTRODUCTION

Congenital heart disease is a problem with the
heart's structure and function that is present
at birth.
DEFINITION

 Approximately 5 to 8 per 1000 live births .
 About 2 or 3 in 1000 infants will be symptomatic during the first year
of life with significant heart diseases that will require treatment.
 CHD is the major cause of death in the first year of life.
 There are more than 35 well recognized cardiac defects the most
common heart anomaly is VSD.
INCIDENCE

 Maternal Infections:- rubella, herpes simplex.
 Maternal Medication:- thalidomide, phenytoin
 Gestational diabetes
 Phenylketonuria
 Alcohol consumption
 Exposure to environmental toxins and infections
 Family history of cardiac defects
 Associated with chromosomal abnormalities.
ETIOLOGY

Congenital heart defects have been divided into two categories based
on cyanosis cyanotic and Acyanotic.
Based on hemodynamic chracteristics(blood flow pattern within the
heart)
• Increased pulmonary blood flow
• Decreased pulmonary blood flow
• Obstruction to blood flow
• Mixed blood flow
Classification,,,,,,

Atrial septal defect is an opening in the atrial septum
permitting free communication of blood
between the atria (left to right).
Seen in 10% of all CHD.
ASD

∗ Three major types
∗ Ostium secundum
∗ Most common
∗ In the middle of the septum in the region of the foramen ovale
∗ Ostium primum
∗ Low position
∗ Form of AV septal defect
∗ Sinus venosus
∗ Least common
∗ Positioed high in the atrial septum
∗ Frequently associated with PAPVR
ASD

∗ ASD remains asymptomatic in most of the infants and young children.
∗ Older children may have recurrent chest infection, breathlessness and bulging of
the chest due to enlargement of right ventricle.
∗ Another important feature is growth failure, which may be the only manifestation
in some children.
∗ The typical murmur is ejection systolic, soft, and best heard over upper left
sternal border (usually the second space).
CLINICAL MANIFESTATIONS

∗ X-ray :- shows right atrial and ventricular enlargement,
increased pulmonary vascularity, enlarged pulmonary artery
and rather small left ventricle and aorta
∗ ECG reveals RVH and right axis deviation.
∗ Echo
∗ Cardiac catheterization.
DIAGNOSIS……..

∗ MEDICAL:- CCF and arrhythmias should be managed
medically. Antibiotic prophylaxis during dental procedures is
necessary.
∗ SURGICAL:- The closure of defect by open-heart surgery gives
gratifying results. It is best done in childhood. Operation
performed electively between ages 1 and 3 years
Treatment

∗ NON SURGICAL:-
Amplatzer septal clouser.
∗ PROGNOSIS:- MORTALITY < 1%.
TREATMENT

∗ VSD is the most common Acyanotic congenital heart disease.
It accounts for 25%.
∗ Defects can occur in both the membranous portion of the
septum (most common) and the muscular portion.
VSD

∗ Three major types
∗ Small, hemodynamically insignificant
∗ Between 80% and 85% of all VSDS
∗ < 3 mm in diameter
∗ All close spontanously
∗ 50% by 2 years
∗ 90% by 6 years
∗ 10% during school years
∗ Muscular close sooner than membranous
Types

∗ Moderate VSDs
∗ 3-5 mm in diameter
∗ Least common group of children (3-5%)
∗ Without evidence of CHF or pulmonary hypertension, may be
followed until spontaneous closure occurs.
∗ VSD may be associated with PS, TGA, PDA, ASD, COA.
Types ….,

∗ Large VSDs with normal PVR
∗ 6-10 mm in diameter
∗ Usually requires surgery, otherwise…
∗ Will develop CHF and FTT by age 3-6 months
Types ….,

∗ The size of the left-to-right shunt depends on two determinants,
namely the size of the VSD (largely) and the pulmonary vascular
resistance (PVR) in relation to systemic vascular resistance.
∗ Right ventricular hypertrophy
∗ Right ventrical also enlarges
Hemodynamics/Pathophysiology
….

 If septal defect is small, there may be no symptoms at all.
 The disease is detected incidentally during a routine clinical examination.
 Large defect causes recurrent chest infections, congestive cardiac failure,
failure to thrive, exertion dyspnoea, bacterial endocarditis, pulmonary
vascular obstructive disease.
 In symptomatic patients, heart is moderately or greatly enlarged (usually
biventricular).
 The characteristic holosystolic murmur heared best at left sternal boarder.
Clinical Features

∗ X-ray chest:- is usually normal. Minimal cardiomegaly and slight
increase in pulmonary vascularity may be noticed in all defects.
∗ ECG in small defects is usually normal but may show left ventricular
hypertrophy.
∗ The 2-dimensional echocardiogram reveals volume overload of the left
ventricle and left atrium, and the position and size of the septal defect
∗ Cardiac catheterization and selective angiocardiography are of much
help in locating the site of the shunt.
DIAGNOSIS

∗ small VSDs (60-90%) undergo spontaneous closure by 3
years. The moderate VSDs close in only 10% cases whereas
large VSDs only infrequently close spontaneously.
Nevertheless, reduction in size may occur often in small and
moderate VSDs and occasionally in large VSDs.
VSD

∗ MEDICAL:- Antibiotic prophylaxis for endocarditis is indicated.
∗ SURGICAL:- palliative ( pulmonary artery banding), complete repair
small defects are sutured.
∗ NON SURGICAL:- device closer during cardiac catheterization ( Dacron
patch).
∗ PROGNOSIS:- membranous low mortality < 2%, muscular carry high
mortality.
TREATMENT

Faillure of fetal ductus arteiosus t close within the first week of life. This
will allow blood to pump left to right.
∗ Persistence of normal fetal vessel joining the pulmonary artery to the aorta
∗ Closes spontaneously in normal term infants at 3-5 days of age
∗ Epi facts
∗ Accounts for about 10% of all cases of CHD
∗ Higher incidence of PDA in infants born at high altitudes (> 10,000 feet)
∗ More common in females
Patent ductus arteriosus

∗ Asymptomatic or signs of CHF
∗ Machinery like murmur.
∗ Bonding pulse
∗ Bacterial endocarditis.
Clinical manifestations….,

∗ Treatment consists of surgical correction when the
PDA is large except in patients with pulmonary
vascular obstructive disease
∗ In preterm infants indomethacin is used (80-90%
success in infants > 1200 grams)
Treatment………..,

∗ In a preterm it can be closed medically using
indomethacin.
∗ In a term baby if still open at 3 months of age then
coil closure by cardiac cauterization is the method of
choice.
PDA…..,////

∗ Medical:- prostaglandin ( indomethacin)
∗ Surgical:- ligation of PDA through thorocotomy.
∗ Nonsurgical:- coils to occlude the PDA are placed in the
catheterization.
∗ Prognosis:- mortality < 1%.
TREATMENT;

Localized narrowing near the insertion of ductus arteriosus,
which results in increased
pressure proximal to the defect
(head and upper extremities)
and decreased pressure distal to the obstruction
(body and lower extremities).
COARCTATION OF THE AORTA

∗More common in males
∗Almost always juxtaductal
∗85% of children with CoA have a bicuspid
aortic valve.
Coarctation of the Aorta

∗ High blood pressure and bounding pulse in the arms.
∗ Week or absent femoral pulses and cool lower extremities with lower blood pressure.
∗ There are signs CHF.
∗ Hypotension.
∗ Mechanical; ventilation and inotropic support are often necessary before surgery.
∗ Older children may experience dizziness, headache, fainting, and epistaxis resulting
from hypertension.
∗ Child are at risk for hypertension, ruptured aorta, aortic aneurysm and stroke.
Clinical manifestations…..,

∗ Medical management consists in tackling CCF and
hypertension, dilatation of the associated PDA with a constant
infusion of prostaglandin E1 in critical situations, and
restriction of strenuous exercise. Antibiotic prophylaxis
against bacterial endocarditis is desirable.
Treatment

∗ The best age for surgery is 3 to 5 years,
∗ Surgical repair is the treatment of choice for infants younger than 6 months of age with long segment stenosis or
complex anatomy.
∗ Resection and end to end anastomosis.
∗ Enlargement of constricted area with prosthetic graft (left subclavian vein).
∗ Thorocotomy is required.
∗ Postoperative hypertension should be treated with iv and oral anti- hypertensive.
∗ In recurrence percutanous baloon angioplasty technique is effective.
∗ using a dacron graft or, preferably, subclavian flap. With the latter, chances of development of recoarctation in
later life are considerably reduced.
Surgical treatment………,

∗ Balloon angioplasty
∗ Stents may be placed in aorta to maintain patency
∗ Motality is less than 5% in isolated COA..
∗ risk is increased in complex defects.
Non surgical………,

∗ Narrowing or stricture of the aortic valve , causing resistant to blood flow in the
left ventricle, decreased cardiac output, left ventricular hypertrophy and
pulmonary vascular congestion.
∗ The prominent consequences of aortic stenosis is the hypertrophy of left
ventricular wall.
∗ LVH results in MI Or scarring of papillary muscles of the left ventricle causes
mitral insufficiency.
Aortic stenosis ?

∗ Depending on the site of obstruction to the outflow of blood
from the left ventricle in relation to the aortic valve,
congenital aortic stenosis may be divided into: valvular, sub
valvular (sub aortic), and supra valvular. Valvular stenosis
accounts for 75% of the cases of aortic stenosis. Subvalvular
stenosis is of three types: discrete membranous, fibro-
muscular and idiopathic hypertrophic.
Classification…,

1. Obstruction tends to be progressive.
2. Sudden episodes of myocardial ischemia or low cardiac
output, can result in death.
3. Surgical repair rarely result in normal valve.
Valvular stenosis/////

∗ A stricture in the aortic outflow tract causes resistance to
ejection of blood from the left ventricle.
∗ The extra workload on the left ventricle causes hypertrophy.
∗ If left ventricular failure develops left atrial pressure
increases; this causes increased pressure in the pulmonary
veins, which results in pulmonary edema.
PATHOPHYSIOLOGY…..,

∗ newborn with AS demonstrates decreased cardiac output, faint pulses,
hypotension, tachycardia, and poor feeding.
∗ children shows signs of exercise intolerance, chest pain, dizziness when standing
for a long time.
∗ systolic ejection murmur may or may not present
∗ Patient at risk of Bacterial endocarditis, coronary insufficiency and ventricular
disfunction.
Clinical manifestations;

∗ The patient should have close follow-up.
∗ He should be discouraged from overexertion, i.E. Competitive sports,
athletics and strenuous exercise.
∗ The patient who gets valve replacement has got to be on
anticoagulants.
∗ Secondly, neither the prosthetic nor the homograft valve lasts
indefinitely. The results of surgery in discrete membranous
subvalvular aortic stenosis are better than in valvular
TREATMENT ,,,,,,,,,,

∗ Surgery in the form of aortic valvotomy and aortic valve replacement
is indicated in the presence of significant manifestations or a large
resting gradient of 60 to 80 mm hg.
• Unfortunately, surgery in the form
Of valvotomy may be complicated
by aortic regurgitation which is worse
than the stenosis.
TREATMENT

∗ Narrowing at the entrance to the pulmonary artery.
resistants' to blood flow causes right ventricular hypertrophy
and decreased pulmonary blood flow. Pulmonary atresia is
the extreme form of pulmonary stenosis in tat there is total
fusion of the commissures and no blood flow to the lungs.
The right ventricle may be hypoplastic.
Pulmonic stenosis,,,,,,,,,,

∗ Right ventricular hypertrophy
∗ Right atrial pressure will increases and this may result in
reopening of foramen ovale. Shunting of unoxygenated blood
to left atrium and systematic cyanosis.
∗ If pulmonic stenosis is severe CHF occurs, systamatic
venous engorgement is noted.
PATHOPHYSIOLOGY

∗ Child may be asymptomatic; some have mild cyanosis or
CHF.
∗ Progressive narrowing causes increased symptoms.
∗ Newborn's with severe narrowing will be cyanotic
∗ Risk of bacterial endocarditis
∗ Cardiomegaly.
Clinical manifestations

∗ Trans ventricular (closed) valvotomy.
∗ Pulmonary valvotomy with cardio bypass.
∗ Balloon angioplasty to dilate pulmonic valve.
∗ Prognosis:- lower than 1%
Treatment

∗ Most common cyanotic heart disease.
∗ The four abnormalities include:
∗ Pulmonary stenosis
∗ RVH
∗ VSD
∗ Overriding aorta
TETRALOGY OF FALLOT

∗ Cyanotic at birth
∗ Systolic murmur
∗ Acute episodes of cyanosis, and hypoxia, called blue spells or
tat spells.
∗ Anoxic spells occur usually during crying or after feeding.
∗ Risk of emboli, seizures and loss of conscousness or sudden
death following an anoxic spell

∗ SURGERY CAN DONE AT FIRST YEAR OF LIFE.
∗ Palliative:- Modified blalock taussing shunt operation.
∗ Complete repair:- clouser of VSD, resection infundibular stenosis
with placement of pericardial patch.
 prognosis:- mortality <3%.
Heart block and CHF are postoperatively
Occur.
Treatment ……..

∗ The term denotes congenital absence of tricuspid
valve, resulting in absence of any outlet from the
right atrium to right ventricle. The entire systemic
venous return, therefore, enters the left heart by
means of the foramen ovale or an ASD and through
VSD to the right ventricle and out to the lungs.
Tricuspid atresia

∗ Cyanosis is usually seen in newborn period.
∗ There may be tachycardia and dyspnoea
∗ Older children have signs of chronic hypoxemia with
clubbing
Clincal manifestations;

∗ Neonates whose pulmonary blood flow depended on PDA for a
contineous infusion of prostaglndin E1 is started till surgical
intervention.
∗ If ASD is small atrial septostomy.
∗ If increased pulmonary blood
flow- pulmonary artery banding.
TREATMENT

∗ The pulmonary artery leaves the left ventricle and the aorta
exists from the right ventricle with no communication
between the systematic and pulmonary circulation.
TGA

∗ Associated PDA must be present to permit blood to
systamatic circulation.
∗ Most common associated defect is Patent foramen ovale
PATHOPHYSIOLOGY

∗ Administration of prostaglandin E1.
∗ atrial septostomy.
∗ Atrial switch procedure performed
in first week of life.
TREATMENT

∗Senning procedure.
∗ Prognosis:- mortality <2%
TREATMENT

∗ In this uncommon cyanotic congenital heart disease, pulmonary
veins fail to join left atrium and, instead, are connected
anomalously so that total pulmonary venous blood reaches right
atrium. It may be of supra cardiac, cardiac, infra cardiac or
mixed type. Infra cardiac TAPVR is always obstructive. Non
obstructive type is more frequent. In both types, there is a
mixing of oxygenated and deoxygenated blood before or at the
level of the right atrium.
TAPVC……..

∗ Manifestations include 3 patterns.
∗ First: Severe tachypnea, cyanosis and moribund state in neonates with severe
obstruction.
∗ Second: CCF (without cyanosis) early in life with gallop rhythm and murmurs along the
left sternal border, pulmonary hypertension when obstruction is only slight or moderate.
∗ Third: Absent or mild cyanosis in infancy; there is absolute mixing of pulmonary venous
blood with a large left to right shunt.
∗ Cyanosis become worse with pulmonary vein obstruction once obstruction occurs child
condition usually deteriorates rapidly. Without intervention cardiac failure will progress
to death.
CLINICAL MANIFESTATIONS….

∗ Corrective repair is performed in early infancy.
∗ common pulmonary vein is anastomosed to the back of the
left atrium, the ASD is closed and the anomalous
pulmonary venous connection is ligated.
∗ The cardiac type is easily corrected.
∗ Infradiaphramatic type carries highest morbidity.
∗ prognosis:- mortality < 10%
Treatment ……..

∗ Underdevelopment of the left side of the heart, resulting
in a hypoplastic left ventricle and aortic atresia. Most
blood from the left atrium flows across the patent
foramen ovale to the right atrium to the right ventricle
and out the pulmonary artery. The descending aorta
receives blood from the patent ductus arteriosus
supplying systemic blood flow.
Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart
Syndrome

∗ Congestive cardiac failure develops fairly early, particularly in
subjects with aortic atresia in whom it may occur as early as in
the first week of life.
∗ In aortic involvement, cyanosis may be differential but it is
usually generalized.
∗ PDA closes then progressive deterioration leading to death.
∗ The condition is fatal in the first month of life without
intervention.

∗ Require mechnical ventilation
∗ Prostaglandin E1 is needed to maintain ductal patency.
∗ Multi stage approach is used.
∗ Norwood at birth – anastomosis of pulmonary artery to aorta &
creation large asd.
∗ Glenn shunt procedure or hemi fonton operation (3 to 6 months)
∗ Modified fonton procedure
Treatment

∗ Heart transplantation is the another option for these infants.
Include the shortage of newborn organ donation, risks of
rejection, long term problems with chronic
immunosuppression and infection.
Treatment

∗ Incomplete fusion of the endocardial cushions. Consists of
low ASD that is continuous with a high VSD and cleft of the
mitral and tricuspid valves. Which create central
atriovetricular valve that allows blood to flow between all
four chambers of the heart.
∗ It is most common in down syndrome babies.
∗ They account for 4% OF ALL CHD.
Atrioventricular canal defect

∗ Moderate to severe CHF
∗ There may be a mild cynosis that increases with crying.
∗ Incomplete AVSD maybe indistinguishable from ASD - usually
asymptomatic.
∗ Recurrent pulmonary infections.
∗ Failure to thrive.
∗ Exercise intolerance, easy fatigability.
∗ Late cyanosis from pulmonary vascular disease w/ R to L shunt.

Atrioventricular Septal Defect
Complete Form
∗ Low primum ASD continuous
with a posterior VSD.
∗ Cleft in both septal leaflets of
TV/MV.
∗ Results in a large L to R
shunt at both levels.
∗ TR/MR, Pulm HTN w/
increase in PVR.
Incomplete Form
∗ Any one of the components
may be present.
∗ Most common is primum
ASD, cleft in the MV & small
VSD.
∗ Hemodynamics are
dependent on the lesions.

TREATMENT…..,
∗ Surgery is always required.
∗ Treat congestive symptoms.
∗ Pulmonary banding maybe required in premature infants or infants <
5 kg.
∗ Correction is done during infancy to avoid irreversible pulmonary
vascular disease. By patch closure and reconstruction.
∗ Mortality low w/incomplete 1-2% & as high as 5% with complete
AVSD.

1. Help the family adjust to the disorder
2. Educate family about the disorder
3. Help families manage the illness at home
4. Prepare child and family for invasive procedure
5. Provide postoperative care
6. Plan for discharge and home care.
Nursing care of child with congenital
heart diseases

1. Symptoms and therapeutic care
2. Nutrition
3. Immunization
4. IQ
5. Post oerative complications like )cerebral palsy, epilepsy, mental
retardation, delay in language and speech)
Help families manage the illness at home

 Preoperative preparation including reducing anxiety, improve patient
cooperation, enhances recovery.
 Tour of intensive care unit
 Preoperative classes
 Familiarity with equipment
 Recovery topics after catheterization include lying still toprevent bleeding,
advancing diet, controlling pain and monitoring.
 After surgery nurse reviews like ambulation, coughing, deep breathing, dreinking
and eating and cpoing stratagies to divert mind from pain.
Prepare child and family for invasive
procedure

Cardiac
∗Congestive heart failure:- digoxin, diuretics.
∗Low cardiac output:- IV inotropes
∗Dysrhythmias:- identification, drug treatment, possible passing and cardio version.
∗Tamponed:- prompt removal of pericardial fluid by pericardiocentesis.
Respiratory
∗Atelectasis:- chest physiotherapy, coughing, deep breathing exercises and ambulation.
∗Pulmonary edema:- diuretics
∗Pleural effusion:- diuretics, possible chest tube drainage
∗Pneumothorax:- possible chest tube drainage.
Postoperative care

Neurological
∗Seizures:- assessment, antiepiletic drugs
∗Stroke:- assessment and treatment.
Infectious diseases
∗Infections :- antibiotics
Hematologic
∗Anemia:- iron supplimentation, possible transfusion.
∗Postoerative bleeding:- intially clotting factors, blood products may need repeat
surgery to locate and ligate souce of bleeding.
Postoperative care

∗ Vitals and BP should recorded until stable.
∗ Monitor cardiac rhythms
∗ At least hourly auscultation of lungs
∗ Warmer during hypothermia
∗ Intra-arterial BP monitoring.(heparinization to prevent clotting).
∗ Monitoring of intra arterial lines, intra cardiac lines carefully.
∗ Follow aseptic precautions while handling with lines
∗ Assess bleeding time before removing lines.
Observe vitals………

∗ Ventilator care
∗ Suction carefully to prevent vagal stimulation
∗ after extubation provide humidified oxygen through mask
or hood.
∗ Deep breathing exercises hourly
∗ Chest tube care
∗ Drainage from chest tubes immediately after surgery bright
Maintain respiratory status

∗ I/O chart should be maintained
∗ Intake include flush, dilutions,
∗ Output includes urine, drainage from chest, nasogastric tubes and blood drawn
for analysis.
∗ Observe for renal failure.
∗ Fluids are restricted during the immediate postoperative period to prevent
hypervolemia.
∗ To monitor fluid retention the child is weighed daily.
∗ NBM for 1st
24 hgours
Monitor fluids

∗ Rest should be provided to decrease workload on heart and promote
healing.
∗ Progressive schedule of ambulation and activity is planned based on
postoperative condition.
∗ Ambulation is intiated early usually 2nd
post operative day. When tubes are
removed.
∗ Activity may progress by sitting side of bed, and dangling the legs to
standing up and sitting in a chair.
∗ Monitoring of vitals.
Provide rest and progressive activity

∗ Patients need analgesics for pain control and patient controled analgesics.
∗ NSAIDS as ketorolac may used .
∗ paralyzing agent may used for agitated and hemodynamically unstable.
∗ Pain can be controlled by oral drugs ibu-profen, codein and acetominophen.
∗ Positioning
∗ Post operative depression and anxiety.
∗ Emotional support to parents
Provide comfort and emotional support

∗ The family will need both verbal and written instructions on
 Medications,
 Nutition
 Activity
 Wound care (including dressing)
 Bacterial endocardities prophylaxis.
 Folloe up oppointments with cardiologist
 When to practitionaire ; signs and symptoms of postoertive problems
 Review of cardiac defects and surgical repair.
Plan for discharge and home care

Congenital heart diseases

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