Congenital Heart Disease Non-cyanotic B

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    Congenital Heart Disease

    (Non-Cyanotic)IVANNA PUTERI .S (030.04.112)

    JANE UTAMI SETIAWAN (030.04.113)JAROT WAHYU ARDHI (030.04.115)

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    The Hearts Blood Flow

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    Prevalence

    0.5-0.8% of live births 2-3 in 1000 newborn infants

    symptomatic with heart disease in the first

    year of life Diagnosis during : 1 week of age in 40-

    50%, 1 month of age in 50-60% of

    patient Congenital heart disease remains the

    leading cause of death in children with

    congenital malformations.

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    Etiology

    Unknown Genetic factor

    Maternal conditions and teratogenicagents

    Congenital rubella syndrome

    Maternal ingestion of drugs

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    Classification of Non-CyanoticCongenital Heart Lessions

    a. Increased volume loadatrial septal defect (ASD)

    ventricular septal defect (VSD)AV septal defect (AV canal)

    patent ductus arteriosus (PDA)

    b. Increased pressure loadcoartation of the aorta (COA)

    atrial stenosis (AS)

    mitral stenosis (MS)

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    Atrial Septal Defects

    3 Type of ASD :1.Ostium Secundum ASD

    2.Sinus Venosus ASD3.Ostium Primum ASD

    In adult occurs commonly infemales

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    Ostium Secundum Defect

    Most common form ofASD

    Occur at fossa ovalis Stucturally normal AV

    valves

    Single or multiple Openings 2 cm or

    larger in diameter

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    Sinus Venosus Atrial Septal

    Defect Occur in the upper part ofthe atrial septum in closerelation to the entry of

    the superior vena cava Sometimes the superiorvena cava straddles thedefect, some systemicvenosus blood enters the

    left atrium The clinical manifestation

    similar to secundum ASD

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    Ostium Primum(Atrioventricular Septal Defect)

    Occur in the lowerportion of the atrial

    septum and over liesthe mitral andtricuspid valves

    Markedly abnormal

    AV valves

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    Clinical manifestation

    Asymtomatic until third decade In younger children : failure to thrive

    In older children : varying degrees of exerciseintolerance Right ventricular diastolic volume increase Ejection time prolonged throughout all phases of

    respiration Systolic ejection murmur is heard Easy fatigability Reccurent pneumonia

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    Diagnose Anamnesa Physical examination

    Chest rontgenogram ECG Echocardiogram

    Treatment Surgical to do elective closure Palliation with pulmonary arterial banding

    Prognose good if the closure is carried out Worse if heart failure and atrial fibrilation occur

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    Ventricular Septal Defect

    the most common of all congenitalcardiac defects

    25% of all congenital heart disease,or about 2 in every 1000 live births

    Most defect occur in membranoustypes

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    Ventricular Septal Defect

    Ventricular septal defect is an abnormal openingin the wall between the two ventricles

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    Clinical manifestation

    According to the size of thedefect and pulmonaryblood flow and pressure :

    Small VSD left to right shunt, normal

    pulmonary arterial pressure

    Asymptomatic

    Characteristic : loud, rash,blowing holosystolicmurmur, frequentlyaccompinied by a thrill

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    Large VSDDyspnea

    Feeding difficulties

    Poor growth

    Recurrent pulmonary

    infection and cardiacheart failure in earlyinfancy

    Holocystolic murmurgenerally harsh thansmall VSD

    Profuse perspiration

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    Diagnose Chest X ray Electrocardiogram

    Complications

    Infective endocarditis

    Arrhytmia Heart failure

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    Therapy

    Small VSDProtection and care of endocarditis

    Large VSDSurgerry

    PrognoseSpontaneous closure 30-80% of cases within

    the first and two years of life

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    AV Septal Defect (AV Canal)

    Ostium primum defect is in the lower portion ofatrial septum and overlies the mitral andtricuspid valve.

    Contagious atrial & VSD with markedly abnormalAV valve. Common with down syndrom & may occur with

    PS.

    Asymptomatic. Exercise intolerance. Recurrent pneumonia.

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    Diagnose :1. Chest radiograph

    2. EEG

    3. Echocardiogram4. Cardiac Catheterization &

    Angiocardiography

    5. Selective Right Ventriculography

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    Theraphy :Surgically

    Prognose :

    Depent on : 1. Magnitude of L-R Shunt

    2. Pulmonary vasc.Resistance

    3. Severity of AV valve

    insuficiency

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    Patent Ductus Arteriosus (PDA)

    The ductus arteriosus is a blood vesselthat is present in all babies before they

    are born While in the womb, the lungs of the fetus

    cannot provide oxygen to the fetus's blood

    the fetus receives its oxygen from itsmother, through the umbilical cord

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    connects the pulmonary artery to the

    aorta, allowing blood to bypass the lungs Normally the ductus arteriosus closes on

    its own within a few days of birth as the

    lungs begin to strengthen and provide thebody with the oxygen it requires

    Ligamentum Arteriosus

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    Clinical Manifestations

    Congestive heart failure rapid breathing

    tiring more easily This condition also varies depending on

    how wide the Ductus Arteriosus opened

    A small opening may not produce any symptomsA larger opening may produce a heart murmur

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    Diagnose Chest X ray Electrocardiogram

    Treatment

    Surgical or catheter closureThe surgery in small PDA is done to prevent late complicationsThe surgery in large PDA is done to treat heart failure or prevent

    the development of pulmonary vascular disease

    Prognose

    Small PDA may live a normal spant with few or nocardiac symptoms Large PDA may cause congestive heart failure

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    Mitral Stenosis (MS)

    Symptom appear within the 1st2 year oflife.

    Underdeveloped. Cyanosis & pallor. Heart enlargement (RV & LA, Dilatation &

    Hiperthropy)

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    Diagnose: 1. ECG RVH

    2. Rontgen3. Echocardiogram

    4. Cardiac Catheterization

    5. Angicardigraphy

    Theraphy :1. Surgical

    2. Transcatheter BaloonValvuloplasty

    Prognose :Poor

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    Coarctation Of The Aorta

    98 % occur just bellow the origin of the leftsubclavian artery at the origin of the ductusarteriosus ( juxtaductal coarctation )

    C.A may be a feature of Turner Syndrome (XO) The term Shone Syndrome refers to multiple

    levels of left heart obstructive disease, including

    mitral stenosis, bicuspid aortic valve with orwithout aortic stenosis, tubular hypoplasia of theaortic isthmus, and coarctation of the aorta

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    Clinical Manifestation

    Weakness or pain ( or both ) in the legs afterexercise, but in many instances, even patientswith severe coarctation is asymptomatic

    The classic sign of C.A is a disparity inpulsation and blood pressure in the arms andlegs

    The femoral, popliteal, posterior tibial, anddorsalis pedis pulses are weak ( or absent inup to 40% of patients ), in contrast to thebounding pulses of the arms and carotidvessels

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    This differential in blood pressure iscommon in patients with coarctation whoare older than 1 year

    The pathognomonic murmur of C.A isheard in the left axilla and the left back orthe left infrascapular area

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    DIAGNOSIS

    EchocardiogramNeonates and young infants display

    rightor biventricular hypertrophy

    Echocardiography (two-dimensional)

    Color Doppler

    Pulsed and continuous wave Doppler

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    Prognosis and Complications

    Severe neurologic damage or even deathmay rarely occur from associated

    cerebrovascular disease The common serious complications are :

    1. Premature coronary artery disease

    2. Heart failure3. Hypertensive encephalopathy

    4. Intracranial hemorrhage

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    Treatment

    Infusion of prostaglandin E1Anticongestive

    The procedure of choice for isolatedjuxtaductal coarctation of aorta iscontroversial

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    Postcoarctectomy Syndrome* Acute hypertension

    * Abdominal pain

    * Anorexia, Nausea, Vomiting

    * Leukocytosis* Intestinal hemorrhage

    * Bowel necrosis

    * Small bowel obstruction Relief is usually obtained with antihypertensivedrugs and intestinal decompression

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