CONGENITAL HEART DISEASE Dr Siti Aisyah Abd Majid Family Medicine Trainee, PPUKM
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- Slide 1
- CONGENITAL HEART DISEASE Dr Siti Aisyah Abd Majid Family
Medicine Trainee, PPUKM
- Slide 2
- Objectives Recognise the various presentations and causes of
congenital heart conditions at birth, during infancy and childhood
period Manage and follow-up congenital heart conditions in children
Refer appropriately children with congenital heart diseases to the
Paediatrician
- Slide 3
- OVERVIEW Congenital heart disease (CHD) the most common
congenital disorder in newborns (6-21 per 1000 live births). In
preterm infants 2-3 times greater than term infants One of the
leading causes of perinatal & infant death from congenital
malformations. (UK Northern Congenital Abnormality Survey)
- Slide 4
- Anatomy of the heart 4 chambers o RA, RV o LA, LV 4 valves o
Mitral / bicuspid o Tricuspid o Aortic o Pulmonary
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- Anatomy of the heart 5 great vessels o SVC, IVC o Pulm artery o
Pulmonary vein o Aorta Deoxygenated blood o From SVC + IVC o RA, RV
o Pulm artery lungs Oxygenated blood o From pulm vein o LA, LV o
Aorta systemic circulation
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- Fetal circulation In utero, placenta acts as the lungs,
therefore less blood passes into the actual fetal lungs. 2
structures within a fetal heart that allow this "bypass o Patent
ductus arteriosus o Foramen ovale
- Slide 7
- Fetal Circulation PFO PFO is a hole between the two atriums. It
allows mixing of blood between the two right and left atrium. PDA
allows mixing between the pulmonary artery and the aorta as it is a
passageway between these two major vessels.
- Slide 8
- Fetal Circulation This change in the pressures allows more
blood to flow into the lungs. The changes in pressure cause the PDA
and PFO to eventually close. The final closure usually takes
several days. The pressure in the lungs of a fetus is higher than
that in the body. This increased pressure encourages the right to
left shunt. After a baby is born the pressure in the lungs
decreases as the vessels in the lungs begin to relax.
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- AHA Classification of CHD Septal defects ASDVSD Obstructive
defects ASPSCOA Cyanotic defects TOFTGATricuspid atresiaPulmonary
atresiaTruncus arteriosusTAPVCHLHS
- Slide 11
- ASD Opening in the atrial septum permitting free communication
of blood between the atria. 10% of all CHD Hemodynamics: o Lt Rt
shunt o Increased blood flow into RA enlarged RA + RV o Pulm
HPT
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- VSD opening in the ventricular septum, which allows free
communication between the Rt & Lt ventricles. 20 to 25% of all
CHD Hemodynamics: o Lt Rt shunt o Increased blood flow into RV
enlarged RV o Pulm HPT
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- Aortic Stenosis (AS)
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- 5 percent of all CHD. Narrowing of the aortic valve. Depending
on the severity of the stenosis, the symptoms at birth can vary
from none to decreased blood flow and decreased oxygenation to the
systemic circulation
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- Pulmonary Stenosis (PS) 5-8% of all CHD. Narrowing of the
pulmonary valve. Hemodynamics: RV pressure hypertrophy RV failure.
RV pressures maybe > systemic pressure. Post-stenotic dilation
of main PA. W/intact septum & severe stenosis R-L shunt through
PFO cyanosis. Cyanosis is indicative of Critical PS.
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- Coarctation of Aorta (COA) an obstruction to the outflow from
the left ventricle at or near the aortic valve that causes a
systolic pressure gradient of more than 10mmHg. 7% of CHD. 3 Types
Valvular Most common. Subvalvular(subaortic) involves the left
outflow tract. Supravalvular involves the ascending aorta is the
least common. Hemodynamics: Obstruction of left ventricular outflow
pressure hypertrophy of the LV.
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- Tetralogy of Fallot (TOF)
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- TOF 1.Stenosis of the pulmonary artery 2.Interventricular
communication 3.Deviation of the origin of the aorta to the right
4.Hypertrophy, almost always concentric in type, of the right
ventricle. Failure of obliteration of the foramen ovale may
occasionally be added in a wholly accessory manner Fallot,
tienne-Louis-Arthur. Contribution to the pathologic anatomy of
morbus caeruleus (cardiac cyanosis). Marseilles Med. 1888;
25:418-20.
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- Transposition of Great Arteries (TGA)
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- TGA 5% of all CHD The aorta is connected to the right ventricle
(rather than the left), so instead of pumping blood to the lungs it
pumps it back to the body. On the left side of the heart the
pulmonary artery is connected to the left ventricle which pumps the
blood that returns from the lungs back to the lungs. There are two
separate circuits at work. One handles and recirculates the
unoxygenated blood from and to the body; the other handles and
recirculates the oxygenated blood from and to the lungs. These
babies need the PFO and PDA to remain open so there is mixing of
oxygenated blood with unoxygenated blood.
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- Tricuspid Atresia
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- Atresia = blocked Right atrium is unable to allow blood flow
into the right ventricle because the tricuspid valve is blocked.
Since the right ventricle has not been working, it becomes smaller
in size and underdeveloped. This defect may be seen with a single
ventricle, which means instead of a left and right ventricle there
is just one large ventricle. The survival of an infant with
tricuspid atresia is dependent on communication between the right
and left atriums via an ASD, VSD or PFO.
- Slide 24
- Pulmonary Atresia 5 days, particularly if it is accompanied
with rash, lymphadenopathy, conjunctivitis & distal extremity
changes. The majjor complication coronary artery aneurysm
- Slide 43
- Family History Infant risk of CHD with affected parent (AVSD or
TOF) 4% Infant risk of CHD with an affected sibling 2% Burn J,
Brennan P, Little J, et al. Recurrence risks in offspring of adults
with major heart defects: results from first cohort of British
collaborative study. Lancet 1998; 351:311 Other heart diseases that
have familial predisposition: o Family dilated cardiomyopathy o
Hypertrophic cardiomyopathy o Familial causes of sudden cardiac
death (long QT syndrome)
- Slide 44
- Physical findings GENERAL T achycardia o Causes: arrhythmia
(SVT, VT), heart failure, Lt to Rt shunt disease o ECG is helpful
in evaluating cause of tachycardia o Tachyarrhythmia - Require
urgent referral, risk for potentially life-threatening cardiac
disease HPT o Need to rule out COA
- Slide 45
- Failure to thrive o Due to inadequate food intake, increased
metabolic demands o Suggestive of cardiac cause if child easily
tired while feeding, have Sx of respi distress, murmurs+ Poor
perfusion due to low cardiac output o CRT > 3 secs o Cool
extremities o Decreased peripheral pulses o May lead to shock /
impending shock Febrile as mentioned
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- RESPIRATORY ABNORMALITIES o Wheezing primary pulmonary disease,
cardiac disease a/w elevated Lt venticular end-diastolic pressure,
pulm HPT o Tachypnoea due to: a)Increased pulmonary blood flow /
pulm venous congestion b) Elevated Lt ventricular end-diastolic
pressure o Rales heart failure, pulmonary overcirculation o Stridor
due to airway obstruction, caused by congenital vascular anomaly eg
vascular ring
- Slide 47
- CVS FINDINGS THAT WARRANT REFERRAL TO CARDIOLOGIST
FINDINGSNATUREDIAGNOSIS Loud murmur, grade 3 or higher ** intensity
less than Garde 3 usually innocent / functional murmurs Harsh
murmurESM @ upper Lt sternal border Pulmonary stenosis ESM @ upper
Rt sernal border Aortic stenosis Continuous murmurPDA Pansystolic
murmurPSM @ Lt sternal borderVSD
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- FINDINGSNATUREDIAGNOSIS Diastolic murmur Loud or single S2Loud
S2Pulmonary HPT Widely split S2ASD Gallop rhythmConstant 3 rd heart
soundCardiomyopathy, AV incompetent valves, L-R shunt disease
Friction rubSandpaper-like soundsPericarditis
- Slide 49
- Innocent / functional murmurs o Majority murmurs in
infants/children o Due to turbulence across the branch pulmonary
arteries that are not fully developed because of the relatively
small amount of pulmonary blood flow in utero o Typically disapears
by 6 months of age increase in size of the branch pulmonary
arteries reduction in turbulence Characters: o a/w quiet precordium
o a/w normal S2 o Crescendo-decrescendo pattern o Intensity <
Grade 3 o Asymptomatic child
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- MANAGING CHILDREN WITH CHD IN PRIMARY CARE Developmental issues
Feeding & growing Medications Immunization Infective
endocarditis prophylaxis
- Slide 51
- Developmental issues Causes: o Chronic disease o Genetic causes
o On cardiopulmonary bypass Denver II test screen for cognitive
& behavior in 4 developmental domains 1/3 of children with CHD
who required surgical or catheter intervention have GM, FM and
social skills delay. (Weinberg et al, 2001) School-aged children
with hx of newborn cardiac surgery for complex CHD are at increased
risk of inattention and hyperactivity (Shillingford et al,
2008)
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- Feeding & Growing Infants with complex CHD often experience
poor nutritional status due to poor feeding (Jadcherla et al, 2009)
Parents complaints: Longer to feed, poor appetites, refuse to eat
It is important to plot the weight & length/height at every
visit Breastfeeding: should be encouraged as long as there is
weight gain May consider to fortify with formula milk to make it
24-30 calories/oz Enteral feeding: can be initiated through
nasogastric tubes in infants who fail to gain weight despite being
fed with fortified formula
- Slide 53
- Red flag: o Growth below 5 th centile o Weight become
disproportionate to their height o Rate of weight gain is < 25%
than the expected findings
- Slide 54
- Medications To ensure the child take medications before and
after the surgery Should inquire the use of over-the-counter
medication and check the interaction with current cardiac
medications Monitor if there are signs of renal insufficiency or
drug toxicity to notify the cardiologist Adjustment of drug doses
because of wt gain also should be done in consultation with
paediatric cardiologist
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- Immunization Immunization record should be reviewed DiGeorge
syndrome o Caused by deletion of 22q11.2 o Result in
immunocompromise o Associated cardiac defects - TOF, interruptud
aortic arch, truncus arteriosus, pulmonary atresia o Immunology
consultation is needed before the administration of live
vaccines
- Slide 57
- Spleen provides primary immunologic defense by being a
reservoir for T lymphocytes that initiate the production of IgM
(first Ab produced in infection) Asplenic pt should take
prophylactic antibiotics & should receive all their scheduled
immunizations (Price et al, 2007) AAP recommends that prophylactic
antibiotics be discontinuedat 5 years of age, provided the child
has not had pneumococcal infx & has received pneumococcal
immunizations. (AAP Committee on Infectious Disease, 2000)
- Slide 58
- IE Prophylaxis American Heart Association (AHA) recommends
maintaining oral health & hygiene with regular brushing,
flossing and dental visit since age 1 year old Prophylactic
antibiotic prior to dental procedure are limited to children with:
o Artificial heart valves o Prev Hx of IE o Serious CHD o Cyanotic
heart disease
- Slide 59
- APPROPRIATE REFERRAL URGENT o In pt with or at imminent risk
for hemodynamic compromise & death o Cardiogenic shock, central
cyanosis, tachyarrhythmias, syncope
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- TIMELY o In pt with a suspected cardiac condition that may
progress & has the potential to result in hemodynamic
compromise, significant morbidity & become life threatenning
over a period of weeks / months o Referral should not be more than
a week o Eg: VSD, PDA, TOF
- Slide 61
- ROUTINE o Pt with potential cardiac lesions that is unlikely to
proceed to hemodynamic compromise and can over a matter of weeks o
Eg: asymptomatic patient with a loud non-innocent murmur.
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- Summary Children with CHD can present a challenge to primary
care practitioners May require more focused attention on regular
visits for health maintenance & prevention of illness Timing of
referral is based upon the risk the underlying suspected cardiac
disease may progress & causing hemodynamic compromise,
morbidity & mortality
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- THANK YOU