Pjb 2018 5a2f4t

Structures of the heart

Normal Heart

KLASIFIKASI • PJB ASIANOTIK – ASD – VSD – PDA

- AVSD - PS

• PJB SIANOTIK – TOF – TGA – TAPVD

- TRIKUSPID ATRESIA - TRUNKUS ARTERIOSUS - ATRESIA PULMONAL

Atrial Septal defect ( ASD ) • Insidence : + 10 % •  :  ratio = 1,5 to 2 : 1 • Anatomy :  Defect on foramen ovale : Secundum ASD  Defect at SVC and RA junction: sinus venosus ASD  Defect at ostium primum : primum ASD

ANATOMY

ASD

Atrial Septal Defect

Diagram of ASD

Clinical Features Symptoms  Most infants : asymptomatic …..undetected  The first present at age 6 to 8 weeks with a soft murmur and possibly a fixed and somewhat widely split S2  Infant with large ASD may present with poor growth, recurrent lower respiratory tract infection and heart failure

Atrial septal defect

Lungs

LV

LA

AO

PA

Systemic RV

RA

Qp > Qs

Atrial septal Defect

RA

RV

RA

LA

RV

LV

LA

LV

Atrial Septal Defect

Auscultation :1st HS N or loud widely split and fixed 2nd HS Ejection Sistolic Murmur

Atrial Septal Defect

Diagnosis Differential  Primary Atrial Septal Defect ECG : LAD  Partial Anomalous Pulmonary Vein Drainage  Pulmonary Stenosis  Innocent Murmur

Atrial Septal defect

Management Surgery : Preschool age Recent treatment: transcatheter closure using ASO (Amplatzer septal occluder)

ASD Large Shunt

Small Shunt Infants

Children/Adults

Observation Evaluation At age 5-8 yrs Cath

FR<1.5

PH (-) Heart Failure (+) PVD (-) Anti failure

Success

FR>1.5

Conservative

Heart Failure (-)

Age >1yrs W >10kg

PH (+) PVD (+) Hyperoxia

Fail

Surgical Closure

Reactive

Transcatheter closure (Secundum ASD) / Surgical Closure(other tipe of ASD)

Non reactive

Conservative

Transchateter closure of ASD

Atrial septal defect

Ventricular septal defect • Insidence  20 % of all CHD  No sex influenced • Anatomy  Subarterial defect : below pulmonary and aortic valve  Perimembranous defect: below aortic valve at pars membranous septum  Muscular defect

VSD

Ventricular Septal defect

Lungs

LV

LA

AO

PA

RA

RV

Qp > Qs

Systemic

Ventricular septal defect

RA

RV

LA

LV

RA

LA

RV

LV

Ventricular Septal Defect

• Clinical findings Day 1st after birth: murmur (-) After 2-6 weeks : murmur (+) Murmur : pansystolic grade 3/6 or higher at LSB 3 Small muscular defect: early systolic murmur Significant defect: Mid diastolic murmur at apex

Ventricular Septal Defect

Murmur: pansystolic grade 3/6 or higher at LSB 3

Small VSD

Large VSD

Ventricular Septal Defect

Cardiomegaly Apex down ward Prominence pulmonary artery segment Increased pulmonary vascular marking

Ventricular septal Defect

Diagnosis Differential   

PDA with PH Tetralogy Fallot non cyanotic Inoscent murmur

Ventricular septal defect

Management: Definitive : VSD closure  Surgery  Transcatheter closure

DSV Heart failure (+)

Heart failure (-)

Anti failure

Aortic valve prolaps Fail

Infundibular stenosis

PVD(-)

Success PAB Evaluate in 6 mths

PH Spontaneous closure

Cath

PVD(+)

Cath Reactive

Smaller

Cath

FR<1.5 FR>1.5

Nonreactive Conservative

Surgical closure/Transcatheter closure

Patent Ductus Arteriosus Anatomy Fetus: ductus arteriosus connects PA and aorta If ductus does not closs  Patent Ductus arteriosus

PDA

Patent Ductus Arteriosus

RA

RV

LA

LV

RA

LA

RV

LV

Patent Ductus Arteriosus

Lungs

LV

LA

AO

PA

Systemic RA

RV

Qp > Qs

• PDA is more common in :  Premature infants

■ BW < 1750 g : 45% ■ BW < 1200 g : 80%  Genetic abnormalities  Infants whose mother had German measles (Rubella)

• PDA in preterm haemodynamic instability co-morbidity & mortality  EARLY DIAGNOSIS

Patent Ductus Arteriosus

• Clinical findings Small defect: Symptom (-) Growth and development normal Moderate and large defect: Decreased exercise tolerant Weigh gained not good Frequent URTI

DIAGNOSIS • Medical history : asphyxia,preterm,congenital rubella, chromosomal aberrations

• Physical examination : bounding pulse, wide blood pressure, hyperactive precordium, present of murmur ed by CXR, ECG Confirmed by Echocardiography

Patent Ductus Arteriosus

Auscultation : continuosus murmur at upper LSB 2

Chest X-Ray

Large PDA: Prominence of the left atrium, left ventricle, ascending aorta, Pulmonary vascular marking 

ECG • • • •

Small PDA : normal Moderate PDA : LVH Large PDA : BVH PDA with PVOD : RVH

Patent Ductus Arteriosus

Diagnosis Differential AP-window Arterio-venous fistulae Management premature: ibuprofen PDA closure : surgery transcatheter closure

MANAGEMENT • Medical treatment : prostaglandin synthesis inhibitor Preterm neonates : usefull Aterm neonates : useless

• Transcatheter closure : mostly choice treatment • Surgical closure : Infant < 5 kg with large PDA Preterm neonates : medical treatment unsuccessful or contraindicated

PDA IN PRETERM NEONATES • Special problem : haemodynamic instability • Treatment should be started as soon as PDA suspected  Once a significant shunt is present increased pulmonary blood flow  damage to premature lungs

• PDA can be closed with prostaglandin synthesis inhibitors

TRANSCATHETER CLOSURE 

*Transcatheter occlusion is effective with a high rate of complete occlusion *Complication rare

Tetralogy Fallot Incidence 5-8% from all CHD Anatomy Cause: Left-anterior deviation of infundibular septum Sindroma consist of 4 items:  VSD  pulmonary stenosis  aortic over-riding  RVH

Tetralogy Fallot

Central cyanosis

Central cyanosis

Pathophysiology • Cyanosis is a bluish discoloration of the skin and mucous membranes resulting from an increased concentration of reduced hemoglobin • Clinical cyanosis occurs when the amount of reduced hemoglobin in the cutaneous vein may result 5 g/100ml • The critical level of reduced hemoglobin in the cutaneous vein may result from either desaturation of arterial blood or increased extraction of oxygen by peripheral tissue

Cardiac causes of cyanosis • Inadequate pulmonary blood flow (severe cyanosis) – Tricuspid atresia – Pulmonary atresia – Tetralogy of Fallot

• Independent pulmonary and systemic circulation (severe cyanosis) – Tranpose great artery

• Mixing (moderate cyanosis) – Truncus arteriosus

Diagnosis Clinically : cyanosis Single 2nd HS, ejection systolic murmur

X Ray : Boot Shaped ECG: RAD, RVH

Tetralogy Fallot

Single 2nd HS, ejection systolic murmur

CXR : Boot-shaped Concave pulmonary segment Apex upturned Decreased pulmonary blood flow

Tetralogy Fallot

ECG : RAD, RVH Echocardiography : to confirm diagnosis

Tetralogy Fallot

• Diagnosis Differential  Pulmonary Atresia  Double outlet right ventricle and pulmonary stenosis  Transposisi of great arteri and pulmonary stenosis

Management  Paliative treatment: Blalock-Taussig shunt  Definitive: total correction

• clinically • ECG

Tetralogy of Fallot

< 1 yr

• CXR • echo

> 1 yr

spell (+) propranolol

spell (-) age 1 yr

failed

cath

succeed

BTS evaluation

cath

BTS/ PDA Stent

small PA

total correction

good sized PA

Transposition of Great Artery Insidence 5% of CHD Anatomy Abnormality of formation of trunkal septum that cause aorta arising from RV and PA arising from LV

Transposition of Great artery

Transposition of Great artery

Hemodynamic normal “series”

Hemodynamic of TGA “parallel”

Transposition of Great artery

• Clinical aspects More frequent in male Birth weight usually normal normal or bigger Cyanotic vary from mild to severe Auscultation : single 2nd HS and loud Murmur vary from silent to pansystolic murmur or continuous murmur

Transposition of Great artery

• Diagnosis Clinically : Suspicious if neonates presents with cyanotic with birth weight normal or bigger Murmur (-) Single 2nd HS and loud

Transposition of Great artery

Murmur (-) Single 2nd HS and loud

Transposition of Great artery

ECG : RAD RVH Echocardiography : to confirm diagnosis Cardiac catheterization: usually is not needed

Transposition of Great artery

Diagnosis Differential  trunkus arteriosus  trikuspid atresia  pulmonary atresia Management

Surgery: arterial switch Paliative : Blalock-Taussig shunt

Transposition of Great artery

TERIMA KASIH