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MANAGEMENT OF CONGENITAL HEART DISEASE IN INFANTS Dr Syed Raza Consultant Cardiologist

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Objectives <ul><li>Prevalence </li></ul><ul><li>Common congenital heart defects </li></ul><ul><li>Diagnostic Workup </li></ul><ul><li>Management </li></ul>

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Size Of The Problem ! <ul><li>Commonest birth defect </li></ul><ul><li>33% of all birth defects </li></ul><ul><li>In US alone 35,000 babies are born with heart defects every year. </li></ul><ul><li>Prevalence of 8 per 1000 births. </li></ul><ul><li>1 million grown up adults with heart defects </li></ul>

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Size of The Problem! <ul><li>Incidence is higher in pre-term infants. </li></ul><ul><li>Can be as high as 10-15% in infants who have a parent with CHD </li></ul><ul><li>5-10% association with abnormal karyotyping. </li></ul><ul><li>Females - more prone to have PDA and ASD </li></ul><ul><li>Males - more prone to have valvular aortic stenosis, coarctation of the aorta, TOF and Transposition of the Great Vessels </li></ul><ul><li>Maternal rubella is associated with PDA </li></ul>

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Other Associated Birth Defects (TRAVEL) <ul><li>T – Tracheoesophageal fistula </li></ul><ul><li>R – Renal anomalies </li></ul><ul><li>A – Anal atresia </li></ul><ul><li>V – Vertebral anomalies </li></ul><ul><li>E – Esophageal atresia </li></ul><ul><li>L – Limb defects </li></ul>

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How to deal with the problem? <ul><li>Family physicians must know how to screen for cardiac defects in infants. </li></ul><ul><li>Must be competent to stabilize the infant and work as a team with Paediatric Cardiologist and Cardiac surgeon. </li></ul><ul><li>Be able to provide psychosocial support to the family. </li></ul>

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CLASSIFICATION <ul><ul><li>Acyanotic - Left-to-right shunt - oxygenated to un oxygenated blood </li></ul></ul><ul><ul><li>Cyanotic - Right-to-left shunt - deoxygenated blood to oxygenated blood </li></ul></ul><ul><ul><li>Hemodynamic and Blood Flow Patterns </li></ul></ul><ul><ul><ul><li>Increased pulmonary flow </li></ul></ul></ul><ul><ul><ul><li>Decreased pulmonary flow </li></ul></ul></ul><ul><ul><ul><li>Obstruction to blood flow (out of the heart) </li></ul></ul></ul>

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Common Types of CHD <ul><li>ACYANOTIC </li></ul><ul><li>Ventricular septal defect (Commonest – Membranous type) </li></ul><ul><li>Atrial septal defect </li></ul><ul><li>Atrioventricular canal defect </li></ul><ul><li>Pulmonary stenosis, </li></ul><ul><li>Patent ductus arteriosus </li></ul><ul><li>Aortic stenosis and </li></ul><ul><li>Coarctation of the aorta </li></ul>

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Types of CHD – contd: <ul><li>CYANOTIC </li></ul><ul><li>Tetralogy of Fallot </li></ul><ul><li>Transposition of the great arteries. </li></ul>

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Heart Anatomy

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Patent Ductus Arteriosus

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Atrial Septal Defect

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Ventricular Septal Defect

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Hypoplastic Left Heart Syndrome

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Coarctation of the Aorta

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Tetralogy of Fallot

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Tetrology of Fallot

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Transposition of the Great Arteries

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Problems Associated : <ul><li>Acyanotic CHD </li></ul><ul><li>Congestive Cardiac Failure </li></ul><ul><li>Cyanotic CHD </li></ul><ul><li>Hypoxia </li></ul>

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History Taking <ul><li>Must be careful. Some of the symptoms are very subtle. </li></ul><ul><li>Obtain a detailed prenatal history including exposure to infections (cytomegalovirus, toxoplamosis, rubella or varicella), medication usage, drug and alcohol use, nutrition and exposure to radiation. </li></ul>

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Associated Risk Factors <ul><li>Diabetes </li></ul><ul><li>Family history of CHD </li></ul><ul><li>Alcohol </li></ul><ul><li>Exposure to drugs such as Phenytoin, Warfarin, Lithium, Thalidomide etc. </li></ul><ul><li>First-trimester rubella exposure (PDA) </li></ul><ul><li>Residence at high altitudes (PDA) </li></ul><ul><li>Karyotype abnormalities </li></ul>

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How to suspect CHD? <ul><li>Feeding problems </li></ul><ul><li>Fatigue and excessive sweating </li></ul><ul><li>Severe growth impairment </li></ul><ul><li>Recurrent chest infection </li></ul>

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CLINICAL SIGNS <ul><li>Signs of poor growth </li></ul><ul><li>Tachypnea </li></ul><ul><li>Sub costal recession </li></ul><ul><li>Tachycardia </li></ul><ul><li>Cyanosis </li></ul><ul><li>Peri orbital oedema </li></ul><ul><li>Murmur </li></ul>

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MURMUR <ul><li>The presence or absence of a heart murmur is unreliable </li></ul><ul><li>Murmur at birth that disappears before baby’s discharge from hospital – likely PDA </li></ul><ul><li>Murmur is not present in many severe forms of CHD, such as tricuspid atresia, Coarctation of the aorta and Transposition of the great vessels. </li></ul><ul><li>The intensity of the murmur is unrelated to the severity of the abnormality. </li></ul>

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Pulmonary vs Cardiac Cyanosis <ul><li>Suspicion of cyanosis should be confirmed by pulse oximetry . </li></ul><ul><li>Pulmonary disorders - administration of 100 percent oxygen usually increases the arterial oxygen saturation to at least 95 percent. Cyanotic CHD - oxygen saturation will only increase to 80 to 85 percent. </li></ul>

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Heart Failure Signs <ul><ul><li>Presence of third heart sound </li></ul></ul><ul><ul><li>Lung crepitations </li></ul></ul><ul><ul><li>Oedema – late sign (Peri-orbital before leg) </li></ul></ul><ul><ul><li>Hepatomegaly </li></ul></ul><ul><ul><li>Ascitis </li></ul></ul>

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INVESTIGATIONS <ul><li>ECG - a normal ECG does not exclude the presence of CHD if index of suspicion is high. </li></ul><ul><li>Chest radiographs - cardiomegaly and increased pulmonary vascular markings. </li></ul><ul><li>A serious underlying heart defect is unlikely if ECG and CXR are normal. </li></ul>

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Investigations : contd <ul><li>Echocardiogram - primary diagnostic test. </li></ul><ul><li>Phonocardiogram - heart sounds are recorded and displayed as a diagram </li></ul><ul><li>MRI - used to evaluate heart structure, size or blood flow </li></ul><ul><li>Lab tests - Hb and Hct (polycythemia), </li></ul><ul><li>ESR (rheumatic fever, Kawasaki disease, myocarditis), </li></ul><ul><li>ABG ( presence of a right to left shunt), </li></ul><ul><li>Clotting times (PT, PTT) and platelet count, </li></ul><ul><li>Na, K, digoxin level </li></ul>

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DIAGNOSIS <ul><li>Studies suggest that 30 to 60 percent of congenital heart defects can be detected prenatally by high resolution four-chamber transvaginal echocardiogram. </li></ul><ul><li>Foetal echocardiography with outflow-tract views can be particularly helpful in detecting anomalies of the great arteries . </li></ul><ul><li>SCAN USUALLY PERFORMED AT 18-22 WEEKS </li></ul>

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Cardiac Catheterization <ul><li>Can be either diagnostic or interventional </li></ul><ul><ul><li>Pressures in the heart can be measured </li></ul></ul><ul><ul><li>CO can be evaluated </li></ul></ul><ul><ul><li>Blood samples can be obtained and tested (O2 sat) </li></ul></ul><ul><ul><li>Contrast can be injected to study blood flow, vessels and chambers </li></ul></ul><ul><ul><li>Balloon angioplasty can be performed to stretch stenosed areas or blockages in vessels </li></ul></ul><ul><ul><li>Can study electrical activity of the heart </li></ul></ul>

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MRI <ul><li>Provides structural details when echocardiogram images are not optimum. </li></ul><ul><li>Magnetic resonance imaging provides excellent anatomic evaluation particularly if repair is contemplated. </li></ul><ul><li>Yields even more information than angiography/catheterization. </li></ul>

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TREATMENT AIMS <ul><li>1) improving cardiac function </li></ul><ul><li>2) removing accumulated fluid and sodium </li></ul><ul><li>3)decrease cardiac demands </li></ul><ul><li>4) improve tissue O2 </li></ul><ul><li>5) Improve nutrition and hydration </li></ul><ul><li>6)Prevention of infection </li></ul><ul><li>7) Patient and family support </li></ul>

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TREATMENT OPTIONS <ul><li>MEDICATION </li></ul><ul><li>CLOSURE DEVICE THERAPY </li></ul><ul><li>SURGERY </li></ul><ul><li>IMPROVING GENERAL WELL BEING </li></ul>

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Heart Failure Management <ul><ul><ul><li>Diuretics - Lasix, spironalactone, thiazides (may need K replacement) </li></ul></ul></ul><ul><ul><ul><li>Improve contractility – Digoxin. </li></ul></ul></ul><ul><ul><ul><li>Decrease afterload - hydralazine (vasodilator), nifedipine (Ca channel blocker), nipride (vasodilator), or captopril (ACE inhibitor) </li></ul></ul></ul>

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Heart Failure Management: Cont’d <ul><ul><ul><li>Improve oxygenation - administer O2 as needed by hood, mask or nasal prongs. </li></ul></ul></ul><ul><ul><li>Adequate nutrition is also important may need six to eight small meals daily rather than 3 large meals, may need tube feedings </li></ul></ul>

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Important Points about Care of the Child with a Heart Defect <ul><li>Parents taking a child with a heart defect home should have discharge planning which includes name/number of the specialist and all emergency numbers. </li></ul><ul><li>Instruction in what to do if child becomes cyanotic, CPR training. </li></ul><ul><li>Should not allow a child with a cyanotic heart defect or severe aortic stenosis to cry for extended periods of time. </li></ul>

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CLOSURE DEVICE : AMPLATZ

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CLOSURE DEVICE

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BALOON VALVULOPLASTY

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BALOON VALVULOPLASTY

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SURGERY

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Nutrition Requirements <ul><li>Adequate nutrition is extremely important in infants with CHD </li></ul><ul><li>Infants who are unable to gain sufficient weight with breast feeding, supplementation may be provided: </li></ul><ul><li>Options include a formula with a high caloric density, nocturnal enteral feeding or continuous 24-hour feeding with a nasogastric or duodenal tube </li></ul>

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Nutrition Requirements: Contd. <ul><li>Don’t usually have to restrict sodium intake (regulates water balance) </li></ul><ul><li>Need vitamin supplements and maybe an iron supplement </li></ul>

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Prophylaxis Against Bacterial Endocarditis: ACC/AHA Guidelines <ul><li>Patients who have complex cyanotic congenital heart disease (e.g., single-ventricle states, transposition of the great arteries, tetralogy of Fallot). (Level of Evidence: C) • Patients with surgically constructed systemic pulmonary shunts or conduits . (Level of Evidence: C) • Patients with congenital cardiac valve malformations , particularly those with bicuspid aortic valves. </li></ul>

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Infective Endocarditis Prophylaxis <ul><li>• Completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure. (Level of Evidence: B) </li></ul>

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Prophylaxis Not Recommended <ul><li>• Patients with isolated secundum atrial septal defect. (Level of Evidence: C) • Patients 6 or more months after successful surgical or percutaneous repair of atrial septal defect, ventricular septal defect, or patent ductus arteriosus. (Level of Evidence: C) </li></ul>

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VACCINATION <ul><li>Pneumococcal vaccine is recommended at two years of age </li></ul><ul><li>Influenza vaccine should be given yearly beginning at age six months. </li></ul><ul><li>MMR at 12 months instead of 15 </li></ul>

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Psychological Issues <ul><li>The diagnosis of CHD is cause for much distress amongst family members </li></ul><ul><li>Parents experience shock, denial, guilt, anger, despair or confusion on learning that their infant has a cardiac defect. </li></ul><ul><li>Greater stress if requires surgical intervention. </li></ul>

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Effect of High Demand of Child’s Medical Treatment and Care <ul><li>Parents and care givers forced to give up jobs and careers </li></ul><ul><li>Easy fatigability </li></ul><ul><li>Distress and irritability </li></ul><ul><li>No leisure time </li></ul><ul><li>Loss of resources </li></ul><ul><li>Depression </li></ul>

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Handling Psychological Stress <ul><li>Reinforcement of the positive, normal attributes of the child helps the family see the child as an individual with many of the same needs as healthy children </li></ul><ul><li>Consultation with a mental health professional may help to cope with the challenge. </li></ul>

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General Physician’s Role <ul><li>Important link between family and specialist </li></ul><ul><li>Physician should provide correct information and clear any doubts and misconceptions. </li></ul><ul><li>Provide reassurance and maintain positive attitude. </li></ul><ul><li>Educate the family . </li></ul><ul><li>Provide moral support </li></ul><ul><li>Arrange for support group and psychological help. </li></ul>

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<ul><li>THANK </li></ul><ul><li>YOU </li></ul><ul><li>ANY </li></ul><ul><li>QUESTIONS ? </li></ul>