Congenital Heart Disease

History feeding difficulties tachypnea diaphoresis syncope cyanotic episodes failure to thrive

Congenital Heart Disease

Physical Examination colour: pink, blue, gray vitals: tachypnea, tachycardia, BP symptoms suggestive of infection palpation and auscultation of precordium chest auscultation survey for organomegaly pulses in all extremities

Cyanotic Congenital Heart Disease

R to L shunts mixing lesions

Differential Dx of Infant Shock

infection (septic shock/ meningitis) bacterial: GBS, E. coli, S. aureus virus: enteroviruses, H. simplex metabolic: amino/organic acidopathies, urea cycle defect hypoxic shock: eg. RSV, C.N.S. depression heart disease: congenital or acquired

Patent Ductus Arteriosus

5-10% of CHD risks and history that of the shunt magnitude small, restrictive--asymtpomatic, risk of endocarditis moderate to large--heart failure, arterial steal

PDA

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PDA Gross Finding

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Atrial Septal DefectClinical

relate to size of shunt. If significant: symptoms dyspnoea, poor activity tolerance, growth discrepancy, frequent chest infections signs tachypnea (may be subtle) active precordium, widely split second heart sound, diastolic rumble pulmonary flow murmur

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There is an abnormal opening between the two upper chambers of the heart the right and left atria - causing an abnormal blood flow through the heart. Some children may have no symptoms and appear healthy. However, if the ASD is large, permitting a large amount of blood to pass through the right side, symptoms will be noted.

ASDCXR
Cardiomegaly Pulmonary plethora RV enlargement Prominent PA

ASD--Natural History
<3 mm defects will be closed by age 18 months these defects probably represent a patent foramen ovale rather than a true cardiac malformation 3 to 5 or 5 to 8 mm--80% of these defects will close larger defects have little chance of closing spontaneously--closure should be considered typically by mid-childhood.

Atrial Septal Defect-management and conservative or closure--depends upon the shunt outcome
significant defects dont close and shunt increases from childhood to adult years long term sequelae--RV failure, Atrial dysrhythmias sinus venosus ASD--surgical closure, with pulmonary vein baffle Secundum ASD--surgical closure or device occlusion with closure--excellent long term outcome

ASD-Device

Ventricular Septal Defect

closure of membranous region

defects of septation during development (downgrowth of ventricles) malalignment of outlet components of the septum Tetralogy or Aortic Outflow anomalies

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a hole in the ventricular septum (a dividing wall between the two lower chambers of the heart - the right and left ventricles) occurs. Because of this opening, blood from the left ventricle flows back into the right ventricle, due to higher pressure in the left ventricle. This causes an extra volume of blood to be pumped into the lungs by the right ventricle, which can create congestion in the lungs.

VSD

Outlet Membranous

Inlet Trabecular

Ventricular Septal Defect

70% to 80% --restrictive or small defect

most should become smaller or close

infant

with moderate-sized VSD

40% to 50% will decrease in size (decrease shunt) normalization elevated pulmonary artery pressures

Large, defect

non-restrictive VSD and shunt remain large, PA pressures improvement implies something else!

high
Clinical

VSD-- pulmonary flow, cardiomegaly

VSD Treatment
if

symptomatic, or signs of significant shunt therapy with digoxin and diuretics closure indications

medical surgical

patch closure, using cardio-pulmonary bypass persistent large shunt, CHF and growth failure persistent cardiomegaly by echo or

catheterization

outcome often

following surgery excellent

undertaken in infancy

Atrio-Ventricular Septal Defect

failure endocardial

of septation of the primitive heart tube at the junction of atrium and ventricle cushion defect or A-V Canal Defect

frequently

associated with Chromosomal abnormalities--Trisomy 21 (cardiac assessment of ALL Down Syndrome infants)

Atrio-Ventricular Septal Defect

3 components common AV Valve atrial septal defect (primum) ventricular septal defect (inlet, muscular) complete--all components partial--2 separate AV Valve orifices

Atrio-Ventricular Septal Defect

Natural history, clinical S/S-that of the dominant lesion dominant atrial defect--ASD Ventricular Component--VSD AV Valve regurgitation--mitral regurgitation Associations outflow tract obstruction (right or left) unbalanced ventricle sizes

ATRIO-VENTRICULAR SEPTAL DEFECT

Atrio-Ventricular Septal Defect

treatment--of dominant lesion surgical repair--patch closure, separation of AV Valves outcome--good to excellent with primary one stage repair long term status based upon competence of AV Valves repair--typically by one year or earlier

Sequelae of Longstanding Shunts

Ventricular volume load Pulmonary hypertension Pulmonary Vascular Disease Eisenmengers Syndrome

Pulmonary Hypertension
Elevated pulmonary artery pressure high flow high resistance large defect (direct transmission) Pulmonary Vascular Disease (PVOD) primary rare disease reactive processsecondary to persistant, significant shunt lesions more prevalent and early in Trisomy 21

Eisenmengers Syndrome
End point of progressive PVOD Muscularization, thrombosis, obliteration of lung vessels increased vascular resistance, decreased shunt shunt reversal--cyanosis, polycythemia paradoxical emboli, brain abscess pulmonary hemorrhage

Cyanotic and Pulmonary obstructive flow lesions

cyanotic disease presents in infancy less severe lesions pulmonary obstruction presents as murmur may present following closure of arterial duct critical obstruction to pulmonary blood flow separation parallel circulation group of heterogeneous relatively uncommon lesions 3 most common abnormalities

May range from mild valve stenosis to critical obstruction to complete Atresia

Transposition of the Great Arteries

big boys!

Prevalence 3-4 per 10,000 live births isolation and parallel systemic and vascular circulation, with systemic venous blood returning to the systemic arteries. Recirculate the de-oxygenated blood

TGA
Diagnosis, based upon presentation of a cyanotic infant cxr-increased pulmonary blood flow management prostaglandin balloon septostomy arterial switch repair outcome dependent upon surgical repair arterial switch

Tetralogy of Fallot-coeur en sabot

 establish

Tetralogy of Fallottherapy and outcome

pulmonary blood flow Taussig shunt

critically blue infant--prostaglandin

palliation-Blalock

connects aorta to pulmonary artery

Transposition of the Great Arteries

Coarctation of the Aorta

Pathognmonic finding--absent femoral pulses presentation based upon severity critical obstruction--hemodynamic collapse following ductal closure moderate to severe--chest pain, dyspnoea hypertension, abnormal pulses, aortic click, murmur

Transposition of the Great Arteries

outcome--early residual

normal LV function implies good long term potential abnormalities include pulmonary artery stenosis outcome of the re-implanted coronaries

unknown--the

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the aorta and pulmonary artery start as a single blood vessel, which eventually divides and becomes two separate arteries. Truncus arteriosus occurs when the single great vessel fails to separate completely, leaving a connection between the aorta and pulmonary artery.

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the aorta is narrowed or constricted, obstructing blood flow to the lower part of the body and increasing blood pressure above the constriction. Usually there are no symptoms at birth, but they can develop as early as the first week after birth. If severe symptoms of high blood pressure and congestive heart failure develop, and surgery may be considered.

PEMERIKSAAN PENUNJANG DASAR

Pemeriksaan rutin bila dijumpai kecurigaan adanya PJB pada keluhan utama, anamnesa dan pemeriksaan fisik EKG Foto polos dada Laboratorium sederhana :

Anemia, infeksi,polisitemia dan kedaruratan (analisa gula darah, elektrolit, gula darah)

FOTO POLOS DADA

Perlu diperhatikan :
1. 2. 3.

Letak dan posisi jantung terhadap organ lain Ukuran dan bentuk jantung Vaskularisasi paru

Bisa untuk menentukan diagnosa/diferensial diagnosanya !

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Jantung normal, foto posteroanterior. Pada tepi kiri jantung, dari arah sefalokaudal terdapat aorta (A), arteri pulmonalis (P), apendiks atrium kiri (AAKI), dan ventrikel kiri (VKI). Sepanjang tepi kanan jantung terdapat vena kava superior (VKS) dan antrium kanan (AKA). Ventrikel kanan (VKA) tidak ikut membatasi tepi jantung.

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Jantung normal, foto lateral. Di sebelah anterior tampak ventrikel kanan (VKA), dan arteri pulmonalis (P). Di sebelah posterior, tepi jantung dibentuk oleh atrium kiri (AKI), dan ventrikel kiri (VKI). Atrium kiri terletak langsung menghadap esofagus.

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Foto dada pasien situs inversus totalis. Tampak apeks mengarah ke kanan, sedangkan gambaran udara dalam lambung tampak di bagian kanan atas rongga abdomen.

Heart Failure--Effects of volume overload

maintainence of cardiac output with large systemic to pulmonary shunt elevated sympathetic stimulation attenuated heart rate and stroke volume responses to stressors (exercise) abnormalities in myocardial O2 demand/perfusion loss of peripheral perfusion (steal) and development of a catabolic state