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CONGENITAL HEART DISEASE Dr Siti Aisyah Abd Majid Family Medicine Trainee, PPUKM

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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

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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)

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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

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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.

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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

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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.

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Pulmonary Atresia 5 days, particularly if it is accompanied with rash, lymphadenopathy, conjunctivitis & distal extremity changes. The majjor complication coronary artery aneurysm

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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)

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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

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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

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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

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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

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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

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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

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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

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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)

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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

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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

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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