MANAGEMENT OF CONGENITAL HEART DISEASE IN INFANTS

Dr Syed RazaConsultant Cardiologist

Objectives

• Prevalence• Common congenital heart defects• Diagnostic Workup• Management

Size Of The Problem !

• Commonest birth defect• 33% of all birth defects• In US alone 35,000 babies are born with heart

defects every year.• Prevalence of 8 per 1000 births. • 1 million grown up adults with heart defects

Size of The Problem!

• Incidence is higher in pre-term infants. • Can be as high as 10-15% in infants who

have a parent with CHD• 5-10% association with abnormal

karyotyping.• Females - more prone to have PDA and ASD• Males - more prone to have valvular aortic

stenosis, coarctation of the aorta, TOF and Transposition of the Great Vessels

• Maternal rubella is associated with PDA

Other Associated Birth Defects (TRAVEL)

• T – Tracheoesophageal fistula • R – Renal anomalies • A – Anal atresia • V – Vertebral anomalies• E – Esophageal atresia• L – Limb defects

How to deal with the problem?

• Family physicians must know how to screen for cardiac defects in infants.

• Must be competent to stabilize the infant and work as a team with Paediatric Cardiologist and Cardiac surgeon.

• Be able to provide psychosocial support to the family.

CLASSIFICATION

• – Acyanotic - Left-to-right shunt - oxygenated to un

oxygenated blood

– Cyanotic - Right-to-left shunt - deoxygenated blood to oxygenated blood

– Hemodynamic and Blood Flow Patterns • Increased pulmonary flow• Decreased pulmonary flow• Obstruction to blood flow (out of the heart)

Common Types of CHD

ACYANOTICVentricular septal defect (Commonest –

Membranous type)Atrial septal defect Atrioventricular canal defect Pulmonary stenosis,Patent ductus arteriosus Aortic stenosis andCoarctation of the aorta

Types of CHD – contd:

CYANOTIC

Tetralogy of Fallot Transposition of the great arteries.

Heart Anatomy

Patent Ductus Arteriosus

Atrial Septal Defect

Ventricular Septal Defect

Hypoplastic Left Heart Syndrome

Coarctation of the Aorta

Tetralogy of Fallot

Tetrology of Fallot

Transposition of the Great Arteries

Problems Associated :

• Acyanotic CHD Congestive Cardiac Failure

• Cyanotic CHD Hypoxia

History Taking

• Must be careful. Some of the symptoms are very subtle.

• 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.

Associated Risk Factors

• Diabetes • Family history of CHD • Alcohol• Exposure to drugs such as Phenytoin,

Warfarin, Lithium, Thalidomide etc.• First-trimester rubella exposure (PDA)• Residence at high altitudes (PDA)• Karyotype abnormalities

How to suspect CHD?

• Feeding problems• Fatigue and excessive sweating• Severe growth impairment• Recurrent chest infection

CLINICAL SIGNS

• Signs of poor growth• Tachypnea• Sub costal recession• Tachycardia• Cyanosis• Peri orbital oedema• Murmur

MURMUR

• The presence or absence of a heart murmur is unreliable

• Murmur at birth that disappears before baby’s discharge from hospital – likely PDA

• Murmur is not present in many severe forms of CHD, such as tricuspid atresia, Coarctation of the aorta and Transposition of the great vessels.

• The intensity of the murmur is unrelated to the severity of the abnormality.

Pulmonary vs Cardiac Cyanosis

Suspicion of cyanosis should be confirmed by pulse oximetry.

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.

Heart Failure Signs

Presence of third heart sound

Lung crepitations

Oedema – late sign (Peri-orbital before leg)

Hepatomegaly

Ascitis

INVESTIGATIONS

• ECG- a normal ECG does not exclude the presence of CHD if index of suspicion is high.

• Chest radiographs - cardiomegaly and increased pulmonary vascular markings.

A serious underlying heart defect is unlikely if ECG and CXR are normal.

Investigations : contd

• Echocardiogram - primary diagnostic test.• Phonocardiogram - heart sounds are recorded and

displayed as a diagram• MRI - used to evaluate heart structure, size or blood

flow

• Lab tests - Hb and Hct (polycythemia), • ESR (rheumatic fever, Kawasaki disease,

myocarditis), • ABG (presence of a right to left shunt), • Clotting times (PT, PTT) and platelet count, • Na, K, digoxin level

DIAGNOSIS

• Studies suggest that 30 to 60 percent of congenital heart defects can be detected prenatally by high resolution four-chamber transvaginal echocardiogram.

• Foetal echocardiography with outflow-tract views can be particularly helpful in detecting anomalies of the great arteries.

• SCAN USUALLY PERFORMED AT 18-22 WEEKS

Cardiac Catheterization

• Can be either diagnostic or interventional– Pressures in the heart can be measured– CO can be evaluated– Blood samples can be obtained and tested (O2

sat)– Contrast can be injected to study blood flow,

vessels and chambers– Balloon angioplasty can be performed to

stretch stenosed areas or blockages in vessels– Can study electrical activity of the heart

MRI

• Provides structural details when echocardiogram images are not optimum.

• Magnetic resonance imaging provides excellent anatomic evaluation particularly if repair is contemplated.

• Yields even more information than angiography/catheterization.

TREATMENT AIMS

• 1) improving cardiac function

• 2) removing accumulated fluid and sodium

• 3)decrease cardiac demands

• 4) improve tissue O2

• 5) Improve nutrition and hydration

• 6)Prevention of infection

• 7) Patient and family support

TREATMENT OPTIONS

• MEDICATION• CLOSURE DEVICE THERAPY• SURGERY• IMPROVING GENERAL WELL BEING

Heart Failure Management

• Diuretics - Lasix, spironalactone, thiazides (may need K replacement)

• Improve contractility – Digoxin. • Decrease afterload - hydralazine

(vasodilator), nifedipine (Ca channel blocker), nipride (vasodilator), or captopril (ACE inhibitor)

Heart Failure Management: Cont’d

Improve oxygenation - administer O2 as needed by hood, mask or nasal prongs.

Adequate nutrition is also important may need six to eight small meals daily rather than 3 large meals, may need tube feedings

Important Points about Care of the Child with a Heart Defect

• Parents taking a child with a heart defect home should have discharge planning which includes name/number of the specialist and all emergency numbers.

• Instruction in what to do if child becomes cyanotic, CPR training.

• Should not allow a child with a cyanotic heart defect or severe aortic stenosis to cry for extended periods of time.

CLOSURE DEVICE : AMPLATZ

CLOSURE DEVICE

BALOON VALVULOPLASTY

BALOON VALVULOPLASTY

SURGERY

Nutrition Requirements

• Adequate nutrition is extremely important in infants with CHD

• Infants who are unable to gain sufficient weight with breast feeding, supplementation may be provided:

Options include a formula with a high caloric density, nocturnal enteral feeding or continuous 24-hour feeding with a nasogastric or duodenal tube

Nutrition Requirements: Contd.

• Don’t usually have to restrict sodium intake (regulates water balance)

• Need vitamin supplements and maybe an iron supplement

Prophylaxis Against Bacterial Endocarditis: ACC/AHA Guidelines

• 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.

Infective Endocarditis Prophylaxis

• 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)

Prophylaxis Not Recommended

• 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)

VACCINATION

Pneumococcal vaccine is recommended at two years of age

Influenza vaccine should be given yearly beginning at age six months.

MMR at 12 months instead of 15

Psychological Issues

• The diagnosis of CHD is cause for much distress amongst family members

• Parents experience shock, denial, guilt, anger, despair or confusion on learning that their infant has a cardiac defect.

• Greater stress if requires surgical intervention.

Effect of High Demand of Child’s Medical Treatment and Care

• Parents and care givers forced to give up jobs and careers

• Easy fatigability• Distress and irritability• No leisure time• Loss of resources• Depression

Handling Psychological Stress

• 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

• Consultation with a mental health professional may help to cope with the challenge.

General Physician’s Role

Important link between family and specialist Physician should provide correct information

and clear any doubts and misconceptions.Provide reassurance and maintain positive

attitude.Educate the family .Provide moral supportArrange for support group and psychological

help.

THANK YOU

ANY QUESTIONS ?