penyakit jantung katup'13

SIRKULASI JANIN

Dr. Sevina Marisya, Mked(ped), SpA

Fetal circulation* Differs from adult circulation in several ways* Almost all differences are attributable to the fundamental defference in the site of gas exchange Adult: lungs Fetus: placenta

Course of Fetal CirculationThere are 4 shunts in fetal circulation:• Placenta• Ductus venosus• Foramen ovale• Ductus Arteriosus

Some important aspects of fetal circulation:1. The placenta receives the largest amount of

combined ventricular output(55%) and has the lowest vascular resistance in the fetus

2. SVC drains the upper part of the body, IVC drains the lower part of the body and placenta. O2 saturation in the IVC(70%) is higher than in the SVC(40%)

3. Most of SVC blood goes to the RV. One third of the IVC blood is directed by the crista dividens to the LA through the foramen ovale, the remaining two third enters the RV and PA.

4. Less oxygenated blood in the PA flows through the widely open ductus arteriosus to the descending aorta and then to the placenta for oxygenation.

Changes in Circulation after Birth

The primary change in circulation

after birth is a shift of blood flow

for gas exchange from the placenta to the lungs.

The placental circulation disappears, and the pulmonary circulation is established.

1. Interruption of the umbilical cord result in the following: a. Systemic vascular resistance >> as a result of the removal of the very low resistance placenta b. Closure of the ductus venosus as a result of lack of blood return from the placenta2. Lung expansion results in the following: a. Pulmonary vasc resistance <<, pulmonary blood flow >> and fall in PA pressure b. Functional closure of foramen ovale as a result of increased pressure in the LA c. Closure of patent ductus arteriosus (PDA) as a result of increased arterial oxygen saturation.

Thank you

PENYAKIT JANTUNG BAWAAN

Structures of the heart

Normal Heart

• Penyakit jantung bawaan (PJB):– Non-sianotik terbesar

• Defek Septum Ventrikel (DSV) : 30%• Defek Septum Atrium (DSA)• Duktus Arteriosus Persisten (DAP)• Stenosis Pulmonal

– Sianotik• TOF (Tetralogi of Fallot)• Atresia Pulmonal• TGA (Tranposisi Great Artery)• Single Ventrikel

Defek Septum Ventrikel• Insiden

30 % dari PJB

• Anatomi Defek subarteri : di bawah katup aorta dan pulmonal Defek Perimembran: below aortic valve at pars membranous septum Defek Muskular

• Menurut besarnya diklasifikasikan:– DSV kecil : < 5 mm2/m 2 luas permukaan

tubuh– DSV sedang : 5-10 mm2/m 2 luas

permukaan tubuh– DSV besar : defek lebih dari ½ diameter

aorta atau > 10 mm2/m 2 luas permukaan tubuh

VSD

Ventricular Septal Defect

LA LV

RV RA

PA AO

Systemic

Lungs

Qp > Qs

Ventricular Septal defect

RA

RV

RA LALA

RV LVLV

Ventricular septal defect

Defek Septum Ventrikel

Defek Septum Ventrikel

• 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

Small VSD

Large VSD

Ventricular Septal Defect

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

Ventricular Septal Defect

KardiomegaliKonus pulmonalis menonjolVaskularisasi paru meningkatApex down ward

Defek septum Ventrikel

Diagnosis Differential

PDA with PH Tetralogy Fallot non cyanotic Inoscent murmur

Defek septum ventrikel

Management:

Definitive : VSD closure Surgery Transcatheter closure

Gagal Jantung : Digoksin 0.01 mg/kg/hari dibagi 2 dosisInfeksi Sal. Nafas: antibiotik

DSV

Heart failure (+) Heart failure (-)

Anti failure

Fail Success

PAB

Evaluate in 6 mths

Surgical closure/Transcatheter closure

Aortic valve prolaps

Infundibular stenosis

PH SmallerSpontaneousclosure

Cath

PVD(-) PVD(+) Cath

Cath

Reactive Non-reactive

Conservative

FR>1.5FR<1.5

Ventricular septal defect

VSD before occlusion

Ventricular septal defect

VSD during deploying the device

Ventricular septal defect

VSD after occludedusing ASO

Defek Septum Atrium (DSA)

• Insiden : + 10 % : ratio = 1,5 to 2 : 1• Anatomi :

DSA Sekundum: Defect on foramen ovale DSA Sinus venosus: Defect at SVC and RA junction DSA primum: Defect at ostium primum

ASD

Atrial Septal Defect

Atrial Septal Defect

Diagram of ASD

LA LV

RV RA

PA AO

Systemic

Lungs

Qp > Qs

Atrial septal defect

RA

RV

LA

LV

RA

RV

LA

LV

Atrial septal Defect

Klinis- Asymptomatic- Auskultasi: - Bunyi jantung I normal atau

mengeras - Bising ejeksi sistolik di daerah

pulmonum - Bising diastolik daerah

trikuspid

Defek Septum Atrium

Atrial Septal Defect

Auscultation :1st HS N or loud

widely split and fixed 2nd HS

Ejection Sistolic Murmur

ECG : RBBB right ventricular hypertrophy

Atrial Septal Defect

Pembesaran atrium kananKonus pulmonum menonjolVaskularisasi paru meningkat sesuai besarnya pirau

Atrial Septal DefectChest X-Ray

Defek Septum Atrium

Diagnosis Differential

Primary Atrial Septal DefectECG : LAD

Partial Anomalous Pulmonary Vein Drainage

Pulmonary Stenosis Innocent Murmur

Defek Septum Atrium

ManagementSurgery : Preschool age 4-5 thnRecent treatment: transcatheter closure using ASO (Amplatzer septal occluder)

ASD

Small Shunt Large Shunt

Observation

EvaluationAt age 5-8 yrs

Cath

FR<1.5 FR>1.5

Conservative

Infants Children/Adults

Heart Failure (-)

Heart Failure (+)

Age >1yrsW >10kg

Transcatheter closure (Secundum ASD) /Surgical Closure(others)

Conservative

Anti failure

FailSuccess

PH (-) PH (+)

PVD (-)

PVD (+)

Hyperoxia

Reac-tive

Nonreactive

SurgicalClosure

Atrial septal defect

Atrial septal defect

ASD before occlusion

During balloon sizing

Atrial septal defect

Atrial septal defect

ASD after occluded using ASO

PENYAKIT JANTUNG BAWAAN SIANOTIK

Tetralogy Fallot

Insiden5-8% dari PJBAnatomiCause: Left-anterior deviation of infundibular septumSindroma consist of 4 items:

VSD pulmonal stenosis aortic over-riding RVH

Tetralogy Fallot

Tetralogy Fallot

Hemodynamic acyanotic Hemodynamic cyanotic

Tetralogy Fallot

• Diagnosis

Klinis:- sianosis PS- jari tabuh setelah 6 bln- sianotik spell : sesak mendadak, nafas cepat dalam, lemas, kejang, koma- Squatting (sering jongkok)- Single 2nd HS, ejection systolic murmur

Tetralogy Fallot

Single 2nd HS, ejection systolic murmur

Tetralogi Fallot

Foto thoraks: - Boot-shaped- Apeks jantung

terangkat- Konus

pulmonalis cekung

- Vaskularisasi paru berkurang

Tetralogy Fallot

Tetralogy Fallot

ECG : RADEchocardiography : to confirm diagnosis

Tetralogy Fallot

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

• Management Sianotic spell:

- knee-chest position- O2 sungkup 5-8l/i- Morfin sulfat 0.1-0.2 mg/kg/subkutan- Sodium bikarbonat 1 mEq/kg/iv

- Propanolol 0.1 mg/kg/iv cegah dehidrasi dan rumatan propanolol

Bedah:- Paliative treatment: Blalock-Taussig

shunt - Definitive: total correction

Tetralogy of Fallot

< 1 yr > 1 yr

spell (+) spell (-)propranolol

failed succeed

BTS

total correction

cath

small PA good sized PA

• clinically• ECG

• CXR• echo

age 1 yr

cath BTS/

PDA Stent

evaluation

Tetralogy Fallot

Tetralogy Fallot