CONGENITAL AND ACQUIRED

HEART DISEASES

FikrialiHidayahHafizah

Emira

Changes in the circulation from fetus to newborn

Congenital Heart Disease

• Heart defects are among the most common birth defects and are the leading cause of birth defect-related deaths• Approximately 8 people in 1000 are born with a congenital heart

defect• Many defects do not need treatment, but some complex congenital

heart defects require medication or surgery

Causes

• Unknown (90%)• Genetic• Trisomies 13, 18, 21 & turner’s syndrome

• Environmental • Maternal infection (rubella), • Drugs (alcohol, warfarin)• Maternal illness (DM, phenylketonuria, and SLE)

Classification

Acyanotic Cyanotic•Asymptomatic at birth•Cyanosis is late feature•Left to right shunt

•Bluish colour due to lack of oxygen in the body• Peripheral• Central

•Right to left shunt

•Atrial septal defects (ASD) – 7%•Ventricular septal defects(VSD) – 30%•Patent ductus arteriosus(PDA) – 12%

•Tetralogy of Fallot – 5%•Transposition of great arteries – 5%•Atrioventricular septal defect

Atrial Septal Defect (ASD)

• Secundum (80%)• Defect in the center of the atrial septum involving foramen ovale

• Partial AVSD (20%)• Inter-atrial communication between the bottom end of the atrial septum

and the atrioventricular valves• Abnormal AV valves, with left AV valve which has three leaflets and tend

to leak (regurgitation valve)

Clinical features

• Symptoms• Asymptomatic (commonly)• Recurrent chest infections / wheeze• arrhythmias

• Physical signs• Ejection systolic murmur (upper left sternal edge)• Fixed and widely split 2nd heart sound• Partial AVSD, pansystolic murmur from AV regurgitation

Investigation • Chest radiograph• Cardiomegaly• Enlarged pulmonary arteries• Increased pulmonary vascular markings

• ECG• Secundum

• Partial right bundle branch block, right axis deviation• Due to R ventricular enlargement

• Partial • Superior axis (negative deflection)• Due to defect of the heart where the AV node is.• Displace the conduction to the ventricle superiorly.

• Echocardiography

Management

• Secundum• Cardiac catheterization with insertion of an occlusion device

• Partial • Surgical correction at 3-5 years old to prevent right heart failure

and arrhythmias in later life

Complication

• Congestive heart failure• Arrhythmias• Pulmonary hypertension• Cyanosis dt Eisenmenger syndrome• Stroke

VSD (30%)

• Defect anywhere in the ventricular septum, perimembranous (adjacent to the tricuspid valve) or muscular• Classification: i) Small VSD ii) Large VSD

Small VSD

• Smaller than aortic valve (3mm)• Asymptomatic (common)• Physical signs: i) Loud pansystolic murmur at lower left sternal edge ii) Quiet pulmonary 2nd sound• Investigations: i) Chest radiography- Normal

ii) ECG – Normaliii) Echocardiography - Demonstrates the precise anatomy of defect

Management of small VSD

• Mostly close spontaneously. No murmur with normal ECG on follow-up

• Prevention of bacterial endocarditis by maintaining good dental hygiene

Large VSD

• > diameter of aortic valve• Clinical features– Symptoms• Heart failure with breathlessness and failure to thrive after 1 week• Recurrent chest infections

– Physical signs• Tachypnea, tachycardia and enlarged liver from heart failure• Soft pansystolic murmur / no murmur• Apical mid-diastolic murmur• Loud pulmonary 2nd sound

Management of large VSD

• Drug therapy with diuretics combined with captopril• Surgery was performed at 3- 6 months old in order to :– Manage heart failure and failure to thrive– Prevent permanent lung damage from pulmonary hypertension and

high blood pressure

PERSISTENT DUCTUS ARTERIOSUS (PDA)

• Ductus arteriosus connects the pulmonary artery to the descending aorta.

• In term infants, it normally closes shortly after birth.• In PDA, it has failed to close by 1 month after the expected

date of delivery due to a defect in the constrictor mechanism of the duct.

• In the preterm infant, the presence of PDA is not from CHD but due to prematurity.

CLINICAL FEATURES

Continuous murmur beneath the left clavicle. Continuous because the pulmonary artery pressure < aorta throughout the

cardiac cycle.

Increased pulse pressure causing a collapsing or bounding pulse

When the duct is large there will be increased pulmonary blood flow with heart failure & pulmonary hypertension

INVESTIGATIONS

Chest Radiograph- Normal or enlarged heart with increased pulmonary vasculature.

ECG- Usually normal

Echocardiograph- Diagnostic investigation.

Management of PDA

Closure with coil or occlusion device introduced via a catheter at 1 year old.

CYANOTIC HEART DISEASES

TETRALOGY OF FALLOT

TETRALOGY OF FALLOT (TOF)

• Most common cyanotic CHD

• Consist of 4 cardinal anatomical features A large VSD Overriding of the aorta Subpulmonary stenosis causing right ventricular tract obstruction Right ventricular hypertrophy

CLINICAL FEATURES

SYM

PTO

MS

Presence of murmur in the first 2 month of life

Severe cyanosis Depends on the severity of the subpulmonary stenosis

Hypercyanotic spells

Rapid increase in cyanosis, irritability / inconsolable crying, breathlessness

and pallor

May lead to myocardial infarction, cerebrovascular accident or death if

not treated earlier

Squatting on exercise

CLINICAL FEATURES

SIG

NSClubbing of the fingers and toes will develop in

older children

A loud harsh ejection systolic murmur at the left sternal edge from

day 1 of life

With increasing right ventricular outflow tract

obstruction, the murmur will shorten and cyanosis will

increase

INVESTIGATIONS

Chest Radiograph- Boot-shaped heart, pulmonary artery bay and decreased pulmonary vascular markings.

ECG - Normal at birth. Right ventricular hypertrophy when older.

Echocardiograph- Demonstrate the cardinal features.

MANAGEMENT

• Initial management : medical palliation with IV PGE infusion.• Corrective surgery at 6 months or single stage repair at 1 -2

years closing VSD & artificial patch to relieve RV outflow obstruction.

• Modified Blalock Taussig shunt• After surgical repair : Need life-long follow up for late

ventricular dysfunction.

MANAGEMENT (HYPERCYANOTIC SPELL)

• Knee-chest/squatting position.• Administer 100% oxygen.• IV morphine• IV propranolol• IV crystalloid or colloid• IV sodium bicarbonate• Heavy sedation, intubation & mechanical ventilation• IV phenylephrine• Emergency Blalock Taussig shunt

TRANSPOSITION OF THE GREAT ARTERIS

TRANSPOSITION OF THE GREAT ARTERIES (TGA)

• The aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle.

• The deoxygenated blood returning to the right side of the heart is being pumped out to the body while the well-oxygenated blood returning from the lungs enters the left side of the heart and is pumped back to the lungs.

• Incompatible with life unless there are VSD, ASD & PDA present.

CLINICAL FEATURES

SYMPTOM

Neonatal cyanosis- Usually on day 2 of life when ductal closure leads to a marked reduction in mixing desaturated & saturated blood.- Cyanosis will be less severe if there is more mixing of blood from associated abnormalities.

CLINICAL FEATURES

SIGNS

Cyanosis

No murmur but systolic murmur from increased flow or stenosis

within the left ventricular (pulmonary) outflow tract

Second heart sound is often loud & single

INVESTIGATIONS

Chest Radiograph- Narrow upper mediastinum with an ‘egg on side’ appearance.

ECG - Rarely help, usually normal.

Echocardiograph- Demonstrate abnormal arterial connection.

MANAGEMENTSimple TGA (intact ventricular septum)

- IV PGE infusion promotes intercirculatory mixing at PDA.- Early balloon atrial septostomy (BAS) if restrictive interatrial communication.

- Surgical repair of choice : Arterial swicth operation 2 – 4 weeks age.

TGA with VSD- Does not usually require intervention during early neonatal period.

- Elective one-stage arterial switch operation + VSD closure < 3 months age.

TGA with VSD and PS- Blalock Taussig shunt during infancy followed by Rastelli repair at 4 – 6 years of

age.

RHEUMATIC FEVER(RF) & RHEUMATIC HEART DISEASE(RHD)

RHEUMATIC FEVER(RF)

RHEUMATIC HEART DISEASE(RHD)

Is an acute, immunologically-mediated, multi-system inflammatory that follows an episode of GAS after interval of few weeks (<1 month)

Is the most serious complication of acute RF & end result of carditis, which affects 30-45% of patients with acute RF.

RHEUMATIC FEVER

PATHOGENESISBody produce antibody against GAS that cause pharyngitis

These antibody cross react with human tissues because of antigenic similarity between strep components & human CT (MOLECULAR MIMICRY)

Immunological mediated inflammation & damage to human tissues (heart, joint, brain & CT)

After latent period 1-3 weeks, damage to heart valves, joints, subcutaneous tissue & basal ganglia of brain.

Most patient with RF has elevated 1/ more ASOT

WHY NOT ALL PATIENTS THAT HAVE GAS THROAT INFECTION WILL HAVE

RF?

1) MICROORGANISM VARIABLE• Only certain strain produce

immunologically active antigen

2) HOST VARIABLE• Some of us produce large

amount of antibody after infection

CLINICAL FEATURES• Latent period : 1-3 weeks between the onset & previous

pharyngeal infection.• Symptoms are grouping together as no single laboratory/

symptoms can suggest ARF.• Modified Jones Criteria:

MODIFIED JONES CRITERIA:• Initial episodes of ARF:-2 major-1 major + 2 minor-plus evidence of previous GAS infection

• Recurrent attack of ARF:-2 major-1 major + 2 minor-3 minor-plus evidence of previous GAS infection

MAJOR MINOR

SPACE

S-SUBCUTANEOUS NODULEP-PANCARDITISA-ARTHRITISC-CHOREA (sydeham chorea)E-ERYTHEMA MARGINATUM

LEAF

L-LEUCOCYTOSISE-ESR (elevated)A-ArthralgiaF-Fever

*raised CRP & ASOT*pallor, anorexia, LOW

INVESTIGATIONS

1. Throat swab = for throat culture group A beta hemolytic2. ASOT = elevated > 200 Todd units3. FBC = anemia & leukocytosis4. ESR & CRP = elevated5. CXR & ECG = carditis features6. Echocardiogram = delineate valve involvement

MANAGEMENT

1) Bed rest2) Anti-strep therapy:• IV/oral penicillin• Oral erythromycin if allergic to penicillin3) Anti-inflammatory therapy• Mild/no carditis = oral aspirin for 2-4 weeks, tapering over 4 weeks• Pericarditis/ moderate to severe carditis = oral prednisolone for 2-4 weeks, taper with

addition of aspirin as above.4) Anti failure medications • Diuretics, ACE inhibitors, digoxin

AIM TO SUPPRESS INFLAMMATORY RESPONSE to minimize cardiac damage, provide symptomatic relieve & eradicate pharyngeal strep infection

SECONDARY PROPHYLAXIS

-As secondary prevention of ARF & development of CRF1) IM Bencathine Penicillin (3-4 weeks)2) Oral penicillin3) Oral erythromycin (if allergies to penicillin)

-Until age 21/ 5 years after last ARF attack which was longer-Lifelong for patient with carditis & vulvular involvement.

COMPLICATION

CHRONIC RHEUMATIC HEART DISEASE

RHEUMATIC HEART DISEASE

Irreversible deformity of 1/more cardiac valves

Left sided of heart > right sided

Could be due to stenosis / regurgitation

Lead to cardiac failure

Predispose to infective endocarditis

Valve commonly involve=• Mitral valve (65-70%)• Aortic valve (25%)• Tricuspid valve (10%)• Pulmonary valve

COMPLICATIONS

• ARRHYTHMIAS• HEART FAILURE

• INFECTIVE ENDOCARDITIS

FEATURE RHEUMATIC FEVER RHEUMATIC HEART DISEASE

ONSET Acute Chronic

AGE Children Adults

PATHOLOGY SPACE Valvular disease(mitral stenosis/mitral

regurgitation)ASCHOFF BODIES Pathognomonic Not seen

DIAGNOSIS John’s criteria Not applicable

INFECTIVE ENDOCARDITIS(IE)

DEFINITION

• Infection due to cardiac valves/mural surface of endocardium, resulting in mass

formation of thrombotic debris & organism name vegetations.

• All children of any age group with congenital heart disease at risk of IE.

• The risk is highest when there is-turbulent jet of blood as with VSD, COA & PDA.-prosthetic material has inserted at surgery.• It subdivided into 2 clinical forms:-Acute bacterial endocarditis-Subacute bacterial endocarditis

FEATURES ABE SABE

MOST COMMON ORGANISM

STAPH AUREUS STAPH VIRIDANS

VIRULENCE OF ORGANISMS

HIGHLY VIRULENT LESS VIRULENT

DURATION < 6 WEEKS > 6 WEEKS

PREVIOUS CONDITIONS OF VALVES

NORMAL, HEALTHY VALVE USUALLY PREVIOUSLY DAMAGE

LESION ON VALVES INVASIVE, DESTRUCTIVE,SUPPURATIVE

USUALLY NOT INVASIVE & SUPPURATIVE

CLINICAL FEATURES FEATURE OF ACUTE SYSTEMINC INFECTION

SPLENOMEGALY, CLUBBING, PETECHIAE

TREATMENT DIFFICULT TO CURE WITH ANTIBIOTIC & USUALLY REQUIRED SURGERY

CURES OFTEN WITH ANTIBIOTIC

PREDISPOSING FACTOR

2) UNDERLYING HEART DISEASE• RHD

• Congenital heart disease

3) IMPAIRED HOST DEFENSE• Lymphoma• leukaemia

1) BACTERIMIA, SEPTICEMIA & PYAEMIA

• Periodontal infection• Infection of GIT

• IVDU

PATHOGENESIS• Valve has no dedicate blood supply.• Defense mechanism (WBC) can’t reach valve via bloodstream.• If organism attach to valve surface & form vegetation, host

immune response blunted.• Lack of blood supply implication of treatment as drug don’t

reach valve.• Normally blood flows smoothly through these valves.• If they damaged (RHD), increase risk of bacteria attachment.

BACTERIAL IMPLANTS ON VALVES:

CIRCULATING BACTERIA LODGED ON PREVIOUSLY DAMAGED VALVES

ALTERED B.FLOWHEMODYNAMIC STRESS ON VALVESDAMAGE TO ENDOTHELIUM FORMATION OF PLATELET THROMBIGET INFECTED FROM CIRCULATING BACTERIA

NON-BACTERIAL THROMBOTIC ENDOCARDITIS-dt endothelial abnormalities & hypercoagulability states followed by bacterial contamination

MICROORGANISM ESTABLISH THEMSELVES ON THE SURFACE OF VEGETATION, PLATELET AGGREGATION & FIBRIN DEPOSITION ACCELERATE AT THE SITE

BACTERIAL MULTIPLYCOVERED BY EVER-THICKENING LAYERS OF PLATELETS & THROMBIN, WHICH PROTECT THEM FROM NEUTROPHILS & OTHER HOST DEFENSE

CLINICAL FEATURES

1) SEPTIC SIGNS = fever, rigors, night sweats, malaise, LOW, anemia, splenomegaly,clubbing

2) CARDIAC LESION = new murmur/change of murmur, abscess at aortic root causes prolong PR interval & complete AV block, HF

3) EMBOLIC PHENOMENON = causes abscess to relevant organ4) IMMUNE COMPLEX DEPOSITION = • Hematuria in GN & ARF• Roth spot & conjunctival hemorrhage• Splinter hemorrhage : Osler’s nodes, Janeway lesion• Skin : petechiae

MODIFIED DUKE CRITERIA

MAJOR CRITERIA MINOR CRITERIA

• Blood culture positive-typically from 2 seperated blood cultures:o Viridans strepo Strep boviso HACEK groupo Staph aureuso Community acquired enterococci• Evidence of endocardial involvement on

echocardiogram

• Predisposing heart condition :-prior surgery, indweling catheter• Fever >38C• Vascular phenomena-major arterial emboli-septic pulm infarcts-mycotic aneurysm-intracranial hemorrhage-conjunctival hemorrhage-Janeway’s lesion• Immunologic phenomena-glomerulonephritis-Osler’s nodes-Roth’s spots-+ve RF

MAKING THE DIAGNOSIS

• DEFINITE : 2 major/ 1 major + 3 minor / 5 minor• POSSIBLE : 1 major + 1 minor / 3 minor• REJECTED-firm alternative diagnosis-resolution of IE after 4 or few days of antimicrobial therapy-no pathological evidence of IE at surgery or autopsy-not meet criteria possible IE

INVESTIGATION

1) Blood culture2) ESR/CRP = inflammation3) FBC =leukocytosis4) Echocardiography5) UFEME = hematuria secondary to GN6) CXR = multiple cavitation in septic emboli or increase cardiothoracic

ratio

MANAGEMENT

1) Bed rest2) Antibiotic therapy• EMPERICAL = IV penicillin (4 weeks)

+ IV/IM gentamycin ( 2 weeks)• VIRIDANS = vancomycin + gentamycin3) Surgery for removal of infected prosthetics material4) Prophylaxis = dental treatment & surgery aw bacteremia