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Congenital heart disease Congenital cardiovascular defects congenital heart defects

2013 American Heart Association, Inc. All rights reserved. Unauthorized use prohibited.

Incidence
The most commonly reported incidence of congenital heart defects in the United States is between 4 and 10 per 1,000, clustering around 8 per 1,000 live births. Continental variations in birth prevalence have been reported, from 6.9 per 1000 births in Europe to 9.3 per 1000 in Asia.

Congenital Heart Defects

Commonly dibagi menjadi sianosis dan asianosis ACYANOTIC
LEFT RIGHT SHUNTS
Ventricular septal defect (30%) Patent ductus arteriosus (12%) Atrial septal defect (7%)

CYANOTIC
Tetralogy of Fallot (5%) Transposition of the great arteries (5%) Atrioventricular septal defect complete (2%)

OUTFLOW OBSTRUCTION
Pulmonary stenosis (7%) Aortic stenosis (5%) Coarctation of the aorta (5%)

Cyanotic Lesions
Tetralogy of Fallot Transposition of Great Arteries Tricuspid Atresia Pulmonary stenosis

Cyanotic - TOF
Insiden 50% dari penyakit jantung sianotik Gambaran klasik dari 4 kelainan Large VSD Overriding aorta (dekstroposisi) Subpulmonary stenosis Right ventricular hypertrophy Klinis : dispneu, sianosis diperburuk saat menangis, bisisng sistolik , bunyi S2 lemah, 50% teraba getaran, Clubbing of fingers

Penunjang : rontgen toraks = jantung seperti sepatu boot ekg = RVH

 Investigations:

Cyanotic - TOF

boot shaped heart (upturned apex due to RVH) ECG RVH

Management
Initially medical Surgical at around 6 months Cyanosed neonates require a shunt Hypercyanotic spells > 15 mins:
Sedation and pain relief with morphine IV propanolol IV fluids Bicarbonate to correct acidosis Ventilation reduced metabolic demand

USS
Showing VSD, and Doppler showing left to right shunt during systole (blue)

ToF
Hemodinamik

kadar SaO2
Atrium kanan 60% Ventrikel kanan 60% Arteri pulmonal 60%

Atrium kiri 100% Ventrikel kiri 90%

Aorta 75% Terjadi sianosis sentral Kadar SpO2 75%

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Sianosis sentral (ringan s/d berat)

di lidah, gusi, mukosa mulut JANGAN HANYA DILIHAT DI BIBIR

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Jari tabuh (clubbing fingers)

lihatlah Sudut dari bantalan kuku (nail bed)

Normal

Clubbing

Normal

Clubbing

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Cyanotic - Transposition
Transposition of the great arteries Tertukarnya posisi aorta dan arteri pulmonaris, bersatunya atrium, bersatunya aorta dan arteri pulmonaris Klinis :
Cyanosis Muncul pada 2-3 hari setelah duktus tertutup Presentation delayed if there is VSD etc Muncul bisisng sistolik

Management: - memperbesar ASD Pembedahan

USS
Showing VSD, and Doppler showing left to right shunt during systole (blue)

Cyanotic - Tricuspid Atresia

Insiden : 2-5% dari semua CHD Kelainana : atresia trikuspid, ASD, hipoplasia ventrikel kanan dan VSD Klinis : hipoksia berat, tanpa perbaikan 90% meninggal dalam 1 tahun, sianosis, Diagnosis menggunakan ekokardiografi Terapi : prosedur fontan

ACYANOTIC
LEFT RIGHT SHUNTS
Ventricular septal defect (30%) Patent ductus arteriosus (12%) Atrial septal defect (7%)

OUTFLOW OBSTRUCTION
Pulmonary stenosis (10%) Aortic stenosis (5%) Coarctation of the aorta (10-15%)

Acyanotic - VSDs
Most common congenital heart defect, 2/1000 births Usually in the membranous part of the septum Classified by size:
Small asymptomatic which normally close spontaneously. May have a thrill, pansystolic murmur at the LLSE Large same/bigger than aortic valve. Present with breathlessness, HF, failure to thrive, difficulty feeding, recurrent chest infections after 1 week of age

Jenis VSD
SVC Ascending aorta Main pulmonal artery

Right pulmonal artery Inter atrial septum Pulmonal valve Lower right pulmonal vein

Upper right pulmonal vein

VSD sub arterial doubly committed (SADC) atau VSD supracristal

Left ventricle

VSD perimembran inlet

Tricuspid valve

VSD perimembran outlet (KASUS TERBANYAK) VSD mid muskularis

Inter ventricular septum

IVC

VSD muskularis posterior

Descending aorta

VSD muskularis apikal VSD muskularis anterior

Manifestasi klinis VSD

Tanda gagal jantung kongestif : - Intoleransi aktifitas fisik, sulit menyusu - Gagal tumbuh - Takikardia - Takipneu - Diaforesis

Laki-laki, 9 bulan, BB 5,2 kg

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Auskultasi VSD
murmur

ULSB

Pan sistolik

Pan sistolik

LLSB

Apikal
Pan sistolik Carey Coombs

Murmur Hollow (Pan) sistolik di LLSB (akibat turbulensi darah di VSD) Murmur Pan sistolik di ULSB (akibat turbulensi darah mencapai pulmonal) Suara P2 mengeras (A2 < P2) & klik ejeksi di ULSB Murmur Carey Coombs (Mid diastolic apical murmur) di Apikal

Eka Gunawijaya

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Perjalanan alamiah VSD

VSD kecil : - 75 80% menutup spontan umur < 2 tahun, sub arterial doubly committed (SADC)
VSD sedang : - jarang menutup spontan - sudah bisa menyebabkan gagal tumbuh - gagal jantung kongestif setelah umur 6 bulan VSD besar : prosedur penutupan VSD mesti dilakukan umur < 1 tahun, karena : - tidak berespon terhadap obat-obatan - gagal jantung & PHT bisa muncul awal (umur 3 bulan)
Eka Gunawijaya 24

kecuali VSD

Penanganan
Medis : Obat anti gagal jantung - Diuretik kombinasi (furosemid + spironolakton) - Preparat kalium (bila hanya memakai furosemid) - ACE inhibitor (kaptopril) - Obat inotropik (dopamin, dobutamin, digoksin) Asupan tinggi kalori Pembatasan aktifitas bila PHT Kebersihan gigi dan profilaksis Infective endocarditis (IE) Tindakan untuk VSD : Prosedur bedah paliatif : Pulmonal artery banding (PA banding) Prosedur penutupan VSD --- PENANGANAN AKHIR

Eka Gunawijaya

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Penanganan akhir
Penutupan VSD 2 pilihan :
Surgical VSD closure melalui prosedur Open heart
bila BB mencapai 6 kg, atau mengerjakan prosedur bedah paliatif terlebih dahulu (PA banding)

Prosedur non bedah

Transcatheter VSD closure memakai AMVO device, saat BB kg, tetapi bukan untuk VSD SADC mencapai 6

Eka Gunawijaya

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Patent Ductus Arteriosus

Berkisar 5 - 10% dari total PJB Rasio Perempuan : Laki = 3 : 1 Risiko terjadi PDA pada bayi baru lahir : - BBLR - Prematur - Asfiksia - RDS (Respiratory distress syndrome) - TTNb (Transient tachypneu of newborn) - Ibunya terinfeksi Rubella saat hamil

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Fisiologi DA & Patofisiologi PDA

Ductus arteriosus (DA) berguna pada masa janin Prostaglandin E2 (PGE2) dari plasenta akan menghilang setelah lahir menyebabkan : - DA menutup spontan 3 jam setelah lahir - DA menutup sempurna 3 hari pada bayi aterm, 3 minggu pada prematur Bila DA tidak menutup pada usia 3 minggu PDA tidak akan pernah menutup selamanya ! Bayi berat badan lahir rendah (BBLR) : < 2000 g : 36% dengan PDA 2000-2400 g : 12% dengan PDA
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Auskultasi PDA

2nd ICS

Upper left sternal border (ULSB)

Apical (Apex)

- Aliran PDA sepanjang sistolik & diastolik Murmur kontinyu (Machinery murmur) terdengar di ULSB - Banyak darah mengalir dari LA ke LV katup Mitral membuka keras saat diastolik murmur Carey Coombs (Mid diastolic apical murmur, atau Apical diastolic rumble) terdengar di daerah apikal (apeks )

USS
Showing VSD, and Doppler showing left to right shunt during systole (blue)

Perjalanan alamiah PDA

Sering terjadi gagal jantung kongestif :
- PDA kecil : asimtomatis pada bayi, tetapi tetap berisiko PHT saat remaja atau dewasa - PDA sedang : gagal jantung saat kanak2, berisiko PHT - PDA besar : gagal jantung semasa bayi, amat berisiko PHT

Pulmonary hypertension (PHT) persisten menimbulkan sindrom Eisenmengers tampak sebagai Different Cyanosis

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patomekanisme Different

cyanosis
Tidak ada sianosis di ujung jari-jari tangan

PHT sindrom Eisenmengers PDA yg semula Left to Right shunt, berubah menjadi Right to Left shunt muncul klinis Different cyanosis
tidak sianosis ke tangan kanan tidak sianosis ke kepala tidak sianosis ke tangan kiri
Vena kava superior Aorta ascenden Arteri pulmonal kiri Vena pulmonal kiri

Arteri pulmonal kanan

LA RA

RV LV

tetapi sianosis di ujung jari-jari kaki

Vena kava inferior Aorta descenden

sianosis ke kaki kanan & kiri

Penanganan
Medis (sama seperti pada VSD) :
Obat anti gagal jantung Asupan tinggi kalori Pembatasan aktifitas bila PHT Kebersihan gigi dan profilaksis endokarditis

Penutupan PDA :
Prosedur bedah : ligasi PDA (sejak neonatus) Prosedur non bedah : transcatheter PDA occlusion dengan ADO device (saat BB mencapai 6 kg)

Acyanotic Atrial Septal Defect

Two types: Secundum ASD (80%)
Defect in centre of atrial septum involving foramen ovale# Foramen ovale closes when baby takes first breath due to increased left atrial pressure

Partial atrioventricular septal defect

Associated with Downs syndrome

Clinical features
Commonly asymptomatic Recurrent chest infections/wheeze HF Arrhythmias in adulthood

 Pulmonary stenosis partial fusion of pulmonary valve leaflets Clinical features: Mostly asymptomatic Ejection systolic murmur at upper left sternal edge Soft/absent S2 Prolonged RV impulse with delayed valve closure in severe cases

Acyanotic - PS

Investigations:
ECG RVH

Management
Transcatheter balloon dilatation

 Aortic stenosis partial fusion of aortic valve leaflets (1-3 leaflets) Clinical features: Asymptomatic Chest pain, syncope, reduced exercise tolerance if severe Low volume, slow rising pulses Carotid thrill Ejection systolic murmur at R sternal edge neck

Acyanotic - AS

Investigations:
ECG LVH post-stenotic dilation of aorta may be seen

Management
Transcatheter balloon valvotomy when presssue gradient >64 mmHg

 Coarctation of the aorta constriction in the diameter of the aorta, most usually after the site of the ductus arteriosus Clinical features: Can present as neonatal collapse HF Weak/absent femoral pulses In adults: raised BP (particularly R arm), radiofemoral delay, ejection systolic murmur Management: Stenting Surgical repair

Acyanotic - Coarctation

USS
Showing VSD, and Doppler showing left to right shunt during systole (blue)