CONGENITAL AND ACQUIRED HEART

DISEASESMohamad Afiq Bin Johari

Nur Aisyah Bt Mohamad AminNor Amirah Bt Mohd Redzwan

Congenital Heart Disease

• Failure of normal cardiac development which present at birth

• In 8 per 1000 live born infants have significant cardiac malformation

Classification

Atrial Septal Defect

• Represent 10% of all CHD• Classification :– 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

– Partial • Superior axis (negative deflection)

• 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• Stroke• Infective endocarditis

Ventricular Septal Defects

• Accounting for 30% of all cases of CHD• Defect anywhere in the ventricular septum,

perimembranous (adjacent to the tricuspid valve) or muscular (completely surrounded by muscle)

Small VSD

• Smaller than aortic valve in diameter (3mm)• Clinical features– Symptoms• asymptomatic

– Physical signs• Loud pansystolic murmur at lower left sternal edge• Quiet pulmonary 2nd sound

Investigation • Chest radiograph – Normal • ECG – Normal • Echocardiography – Anatomy of defects.

Management • Mostly close spontaneously. No murmur with

normal ECG on follow-up• Prevention of bacterial endocarditis by

maintaining good dental hygiene

Large VSD

• Defect are same size or bigger than 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

Investigation

• Chest radiography– Cardiomegaly– Enlarged pulmonary arteries– Increased pulmonary vascular markings– Pulmonary oedema

• ECG– Biventricular hypertrophy by 2 months old

• Echocardiography – Anatomy defects, pulmonary hypertension

Management

• 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

Complication

• Eisenmenger complex• Pulmonary hypertension• Congestive heart failure• Infective endocarditis

Persistent Ductus Arteriosus

• Ductus arteriosus connects the pulmonary artery to the descending aorta

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

expected date due to defect in constrictor mechanism of the duct

• In preterm infant, PDA is due to prematurity not from CHD

Clinical features

• Continuous murmur beneath the left clavicle (pressure in aorta > pulmonary)

• Pulse pressure increased, causing a collapsing or bounding pulse

• When duct is large, there will be increased pulmonary blood flow with heart failure and pulmonary hypertension

• Investigation– Chest radiography and ECG usually normal, unless

it large– Can be identified by echocardiography

• Management – Closure with coil or occlusion device introduced

via a catheter at 1 year old.

CYANOTIC HEART DISEASE

•Bluish colour due to lack of oxygen in the body• Peripheral (finger, toes)• Central (mucosae)

•Right to left shunt

Example of heart disease :•Tetralogy of Fallot – 5%•Transposition of great arteries – 5%

TETRALOGY OF FALLOT

• Most common cyanotic CHD

• Consist of 4 structural defects:– Overriding of the aorta– Ventricular septal defect (VSD)– Pulmonary stenosis– Right ventricle hypertrophy

INVESTIGATION

ECG

MANAGEMENT

• Definitive surgery at around 6-9 months of age• Infant who very cyanosed in the neonatal

period required a shunt to increase pulmonary blood flow– Placement of an artificial tube between the

subclavian artery and pulmonary artery, or– Balloon dilatation of the right ventricular outflow

tract

• Hypercyanotic spell are usually self-limiting

• If prolonged:– Sedation and pain relief is needed (morphine is

excellent)– IV propanolol (or α adenoreceptor agonist) – IV volume administration– Bicarbonate to correct acidosis– Muscle paralysis and artificial ventilation in order to

reduce metabolic oxygen demand

TRANSPOSITION OF THE GREAT ARTERIES

Aorta arises from the right ventricles and the pulmonary artery arises from the left ventricle

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

Mixing can occur at the atrial (ASD/paten foramen ovale/VSD/PDA) level

SYMPTOMS

• Neonatal cyanosis• Cyanosis is less severe if more mixing of blood

occur from associated anomalies

PHYSICAL SIGNS

Cyanosis is always present

Second heart sound is often loud and single No murmur

INVESTIGATIONCHEST RADIOGRAPHY

Classic finding of a narrow upper mediastinum with an ‘Egg on side” appearance of the cardiac shadow

ECG

This is usually normal

MANAGEMENT

• In the sick cyanosed neonate, the key is to improve mixing of blood

• Maintaining the patency of the ductus arteriosus with a prostaglandin is mandatory

• A balloon atrial septostomy may be life-saving procedure which may need to performed in 20% of those with transposition of the great arteries

OUTFLOW OBSTRUCTION IN THE WELL CHILD

AORTIC STENOSIS• Aortic valve leaflets are partly fused together

giving restrictive exit from the left ventricle• Often associated with mitral valve stenosis

and coarctation of the aorta.

SIGNS

• Small volume, slow rising pulse• Carotid thrill(always)• Ejection systolic murmur at the upper right

sternal edge and radiate to the neck• Delayed and soft aortic second sound• Apical ejection click

•isolated enlargement of the ascending aorta (white arrow). •left ventricle is enlarged (yellow arrow)•The descending aorta is not enlarged (green arrow).

•Tall R wave in V6 indicate left ventricular hypertrophy

MANAGEMENT

• Balloon dilatation• Eventually they will require aortic valve

replacement

PULMONARY STENOSIS

• Pulmonary valve leaflet are partly fused together giving restrictive exit from the right ventricle.

SIGNS

• Ejection systolic murmur best heard at the left sternal edge, thrill may be present

• Ejection click best heard at the upper left sternal edge

• If severe, there will be prominent right ventricular impulse(heave).

•It can be normal or post stenotic dilatation of the pulmonary artery.

•In ecg there will be upright T wave in V1 that indicate right ventricular hypertrophy

MANAGEMENT

• Most children are asymptomatic• When the pressure gradient across the

pulmonary valve become markedly increased (> 64 mmHg) , intervention will be required

• Trans-catheter balloon dilatation will be done

ADULT TYPE COARCTATION OF THE AORTA

• Not duct dependent• Gradually become severe over many years

FEATURES

• Asymptomatic• Systemic hypertension in the right arm• Ejection systolic murmur at the upper sternal

edge• Collaterals heard with continuous murmur at

the back• Radio-femoral delay

•The red arrows point to rib notching caused by the dilated intercostal arteries.•blue arrow to the actual coarctation•green arrow to the post-stenotic dilation of the descending aorta.

•Deep S wave in V2 and tall R wave in V6 and upright T wave indicates left ventricular hypertrophy

MANAGEMENT

• Stent may be inserted at cardiac catheter when the condition become severe.

• Sometime surgical repair will be needed.

Rheumatic feverInfective endocarditis

Heart failure

Acquired heart diseases

Rheumatic fever

Acute rheumatic fever• Is an acute immunologically mediated, multisystem

inflammatory that follows an episode of group A streptococcal pharyngitis (GAS) after an interval of a few weeks.

Rheumatic heart disease (RHD)• The most serious complications of acute rheumatic fever

and is the end results of carditis, which affects 30% to 45% of patients with acute rheumatic fever.

Rheumatic fever

• Outbreaks of streptococcal pharyngitis are followed by cases of rheumatic fever.

• Acute rheumatic fever remains an important preventable cause of cardiac disease .

• 50% , if there is a past history of rheumatic fever ( thus secondary prophylaxis is needed )

• Peak incidence 5 to 15 years ; more common females• A family history of rheumatic fever and lower socio

economic status are additional factors.

Epidemiology

Body produce antibody against group A streptococcal (GAS) that cause pharyngitis.

- Hyaluronic acid in GAS capsule = connective tissue of the joints Ab attack the joints causes arthritis-M-protein in GAS cell wall = mycardium Ab produced against GAS attack heart causes carditis

Pathophysiology

• Latent period : 1 to 3 weeks between the onset and previous pharyngeal infection.• Symptoms are grouping together as no

single laboratory or symptoms can suggest ARF• Jones criteria :

Clinical features

Jones criteria

Making the diagnosis :Initial episodes of ARF : 2 major or 1 major + 2 minor criteriaPlus evidence of previous group A streptococcal infection

Recurrent attack of ARF :2 major or 1 major + 2 minor or 3 minorPlus evidence of previous group A streptococcal infection

1.Thorat swab, ASOT2.FBC : anemia, leukocytosis 3.ESR and CRP : elevated4.CXR and ECG : carditis features5.Echocardiogram : delineate valve

involvement ( commonly mitral valve )

Investigations

• Aim to suppress inflammatory response so as to minimize cardiac damage, provide symptomatic relieve and eradicate pharyngeal streptococcal

infection. 1. Bed rest : restrict activity until acute phase reactans return to normal 2. Anti-streptococcal therapy :

• IV c penicillin 50000/kg/dose 6H or oral penicillin • V 250mg 6H (30kg), 500mg 6H (>30kg) for 10 day• oral erythromycin for 10 days if allergic to penicillin.

Management

3. Anti-inflammatory therapy :• mild/no carditis : Oral aspirin 80-100 mg/kg/day in

4 doses for 2-4 weeks, tapering over 4 weeks• pericarditis or moderate to severe carditis : Oral

prednisolone 2 mg /kg/day in divided doses for 2 to 4 weeks, taper with addition of aspirin as above.

4. Anti-failure medications :- diuretics, AGE inhibitors, digoxin

Secondary prophylaxis• Aim as secondary prevention of ARF and development CRF• 1. IM Bencathine Penicillin 0.6 mega unit (<30kg) or 1.2 mega unit

(>30kg) every 3-4 weeks• 2. Oral penicillin V 250mg twice daily• 3. Oral erythromycin 250mg twice daily if allergy to penicillin• Until age 21 years or 5 years after last attack ARF whichever was

longer• Lifelong for patient with carditis and valvular involvement

• Chronic rheumatic heart disease:- Recurrent bouts of rheumatic fever with carditis resulting scarring

and fibrosis of valve (mital commonly) - incompetent valve - VR

Complications

Infective endocarditis

• Serious infection characterized by colonization or invasion of the heart valves or the mural endocardium by a microbe

• This lead to the formation of vegetations composed of thrombotic debris and organisms, often associated with destruction of the underlying cardiac tissues.

• Depending on the severity of infection, IE is subdivided into 2 clinical forms:

• 1. Acute bacterial endocarditis (ABE)• 2. Subacute bacterial endocarditis (SABE)

Infective endocarditis

Acute bacterial endocarditis ( ABE)

Subacute bacterial endocarditis ( SABE)

• Infection of previously normal heart valves

• By a highly virulence organism • Produces necrotizing,

ulcerative destructive lesions• Difficult to cure with

antibiotics and usually required surgery

• Infection of deformed valves• By a lower virulence organism• Cause insidious infections. Thus, less

destructive• Cures are often produce with

antibiotics

• An uncommon condition but has a high morbidity and mortality if untreated

• Increased incidence now likely related to:– Increased survival of CHD patients– Aggressive management of ICU patients

• 17% of cases are in children with no previously known heart disease

• 43% of pediatric cases are in postoperative patients• Most common underlying conditions: Tetralogy of Fallot,

VSD, Aortic stenosis

Epidemiology

1. Streptococcus Viridans (50%): often after dental procedures

2. Staphylococcus aureus: CVP related3. Group D streptococcus (enterococci): GI surgery

related4. Streptococcus bovis, HACEK5. Not identified in 10% of cases

Pathogens

• The valves have no dedicated blood supply, so defensive immune mechanisms (such as WBC) cannot directly reach the valves via the bloodstream.

• If an organism attaches to a valve surface and forms a vegetation, the host immune response is blunted.

• The lack of blood supply also has implications on treatment, since drugs also have difficulty reaching the infected valve.

• Normally, blood flows smoothly through these valves.• If they damaged - from RHD, for example - the risk of

bacteria attachment is increased.

Pathophysiology

• Bacterial implants on the valves :1. The circulating bacteria are lodged on previously damaged valves by RHD and CHD.

Once microorganisms establish themselves on the surface of the vegetation, platelet aggregation and fibrin deposition accelerate at the site.

As the bacteria multiply, they are covered by ever-thickening layers of platelets and thrombin, which protect them from neutrophils and other host defenses.

• Septic signs: fever, rigors, night sweats, malaise, LOW, anemia, spenomegaly, clubbing

• Cardiac lesion: new murmur or change of murmur, abscess at aortic root causes prolong PR interval and complete AV block, heart failure

• Embolic phenomenon: causes abscess to relevant organ• Immune complex deposition: vasculitis affect any vessel

1. Hematuria in GN and acute renal failure2. Roth spot and conjuctival hemorrhage3. Splinter hemorrhage, Osler’s nodes, Janeway lesion4. Skin: petechiae

Clinical features

Major criteria Minor criteria

Blood culture positive : typical mo from 2 separated blood cultures-viridans streptococci-streptococcus bovis-HACEK group-staphylococcus aureus-community acquired enterococci

Evidence of endocardial involvement on echocardiogram

Predisposing heart condition : prior surgery, indwelling catheter

Fever : temperature > 38 degree celciusVascular phenomena :-major arterial emboli-septic pulmonary infarcts-mycotic aneurysm-intracranial haemorrhage-conjunctival haemorrhage-Janeway’s lesionsImmunologic phenomena :-gomerulonephritis -Osler’s nodes-Roth’s spots-Positive rheumatoid factorMicrobiological evidence :-postive blood culture not meeting major criterion

Modified Duke criteria

• Definite: 2 major/ 1 major+3 minor/ 5 minor• Possible: 1 major+1 minor/ 3 minor• Rejected:

-firm alternative diagnosis-resolution of symptoms with antibiotic therapy

<4days-no pathological evidence of IE at surgery or autopsy-not meet criteria possible IE

Making the diagnosis

1. Blood culture2. ESR/CRP: elevated due to inflammatory condition3. FBC: raised TWBC due to infection4. Echocardiography: vegetation5. Urine FEME: hematuria secondary to GN6. CXR: multiple cavitation in septic emboli or increase

cardiothoracic ratio

Investigations

1. Bed rest2. Antibiotic therapy:

-Empirical: IV penicillin G 200000 U/kg/day in 4-6 divided doses x4weeks +IV/IM gentamycin 3mg/kg/day in 3 divided doses x2weeks

-Viridans: vancomycin + gentamycin

3. Surgery for removal of infected prosthetics material

4. Prophylaxis : dental treatment and surgery associated with bacteremia.

Management

Heart failure

• Often called congestive heart failure (CHF) is a common, usually progressive condition with a poor prognosis

• It is syndrome in which heart is unable to provide the output required to meet the metabolic demands of the body(systolic failure) and/or inability to receive blood in to the ventricular cavities at low pressure during diastole (diastolic failure).

Heart failure

• There are two primary causes for heart failure. 1. The first, called “overcirculation failure,” occurs when

blood mixes inside the heart due to a congenital heart defect.

2. The second, called “pump failure,” occurs when the heart muscle becomes damaged and no longer contracts normally.

Causes in children or infancy

• Signs of heart failure 1. Resting tachycardia and

tachypnea2. Poor peripheral pulse

and perfusion3. Wheeze and subcostal

recession4. Hyperactive precordium

and precordial bulge5. Hepatomegaly6. Distended neck vein

• Varies with age :• Symptoms in infancy

1. Poor feeding2. Fast breathing3. Failure to thrive4. Diaphoresis with feeding5. Recurrent chest infection

• Symptoms in older children1. Shortness of breath2. Easy fatigibility3. edema

Clinical features

• General management- propped-up position- oxygen supplementation- Keep warm and gentle handling

- Fluid restriction to ¾ normal maintanence (if not dehydrated or shock)- Optomize caloric intake: low threshold for nasogastric tube feeding and consider continuos overnight infusion feeds- Correct anemia, electrolyte imbalance and teat concomitant chest infection

Management

• Anti-failure medication 1. Frusemide2. Spironolactone3. Captopril4. Digoxin5. IV inotrophics agent

• Specific management 1. Find the definitive cause2. Specific treatment for the cause

• Specific management 1. Find the definitive cause2. Specific treatment for the cause

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