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Congenital Heart Disease dengan darah yang miskin oksigen.

Overview

- US: 1,000,000 adults with congenital heart disease

- ± 50% of all patients born with a congenital heart
disease needs life long, specialised care (Meberg,
Otterstadt et al)

Etiology

- Genetic
 single gene mutation
 syndromes Noonan Leopard, Ells van Creveld,
Kartagener
- environmental
 Maternal rubella(PDA, PS, ASD),
- Prevalence :
 Thalidomide and isotretionin early during
 1/1500 live births
gestation (cardiac malformation nonspesific),
 5-10% of all CHD
 Chronic maternal alcohol abuse(VSD)
 Female > Male
- Multifactorial induce Embriological Malformation
- Keywords:
- Multifactorial induce Embriological Malformation
 Acyanotic
 Plethora
 Shunt in the atrium
- Types

 Secundum defect (50-70%)

 the site of fossa ovalis
 Primum defect (15-30%)
- PAPVR = PARTIAL ANOMALOUS
 associated with other endocardial cushion
PULMONARY VENOUS RETURN
defects
- PVOD = PULMONARY VASCULAR
 Sinus Venosus (10%)
OBSTRUCTIVE DISEASE (EISENMENGER’S
 2 type (superior and inferior vena caval
SYNDROME)
type)
- ECD = ENDOCARDIAL CUSHION DEFECT  large, associated with anomalous
- PTA = persistent truncus arteriosus pulmonary venous drainage
- TA=Tricuspid atresia  Coronary sinus (rare)
- HLHS=HYPOPLASTIC LEFT HEART  associated with unroofed coronary sinus
SYNDROME - clinical manifestation
- TAVPR=TOTAL ANOMALOUS PULMONARY  Symptom
VENOUS RETURN  Most ASDs are asymptomatic  until
adult
Atrial Septal Defect
 DOE
- Atrial septal defect (ASD) adalah kelainan bawaan  Fatigue
berupa lubang atau celah pada dinding pemisah  Recurrent lower RTI
antara dua atrium jantung.  Palpitation due to atrial tachyarrythmias
 Adanya lubang di septum menyebabkan  Physical Examination
sejumlah darah di atrium kiri berpindah ke  RV heaving
atrium kanan. Akibatnya, darah kaya oksigen  Fixed splitting S2
yang seharusnya dipompa ke seluruh tubuh  Systolic murmur at upper LSB
 Middiastolic murmur at lower LSB
  Diagnostic Studies  VSD + PVD + reversed shunt >> dyspnea,
 Chest radiographs (RAH, RVH, Prominent cyanosis
PA with increased pulmonary vascular)  Baterial endocarditis
 ECG (RVH, RAH, RBBB)
 Echo (RVH, RAH, ASD)
 Cardiac Catheterization to confirm ASD,
assess pulmonary vascular resistance, and
diagnose concurrent CAD in adult
- Therapy
 Percutaneous Closure
 only for secundum (contra in others)
 adequate superior/inferior rim around
ASD
 no R-L shunting
 Surgical Closure
 Large defect (>10mm) unless pulmonary
vasculer disease
 Good prognosis:
 closure age < 25, PA pressure <40
 If >25 or PA>40, decreased survival
due to CHF, stroke, and afib

Ventricular Septal Defect

- A ventricular septal defect (VSD) is a hole in the

heart that's present at birth (congenital heart
defect). The hole is between the lower heart
chambers (right and left ventricles). It allows
oxygen-rich blood to move back into the lungs
instead of being pumped to the rest of the body.

 X-ray:
 Cardiomegaly of varying degrees
 Pulmonary Vascular marking increase
 Echocardiography
 Physical examination
 RV heaving
 Wide splitting S2, accentuated P2
 Harsh holosystolic murmur at LSB
- Prevalence
 Small defect >> louder murmur >> great
 Most common CHD 15-20%
turbulence
- Types of VSD:
 Systolic thrill at LSB
 Perimembraneous (70%)
 Middiastolic rumble at apex
 Inlet (5-8%)
 If PVD, murmur diminishes and cyanosis
 Outlet (infundibular) (5-7%)
may be present
 Muscular defect (5-20%) marginal, central, or
 Diagnostic Studies
apical
 Chest radiographs (LAH, LVH, RVH,
- Keywords:
Prominent PA with increased pulmonary
 Acyanotic
vascular)
 Plethora
 ECG (LAH, LVH, RAH)
 Shunt in the ventricle
 Echo (LAH, LVH, RAH, VSD)
- Clinical manifestations
 Cardiac Catheterization (02 sat in RV >>
 Symptom RA)
 If small >> VSDs remain asymptomatic  Treatment
 If large >> CHF (tachypnea, FD, FTT,
frequent lower RTI)
  By age 2 at least 50% small and moderate
VSDs undergo partial or complete
spontaneous closure
 Surgical is recommended in first few
months for CHF or PVD children, if no
PVD, can be corrected later
Patent Ductus Arteriosus
- Patent ductus arteriosus (PDA) adalah kondisi ketika
pembuluh darah yang menghubungkan aorta dan arteri
paru tetap terbuka setelah bayi lahir. PDA merupakan
jenis kelainan jantung bawaan yang biasanya dialami
oleh bayi prematur.
 Selama di dalam rahim, bayi belum
membutuhkan paru-paru untuk bernapas
karena sudah mendapatkan oksigen dari ari-ari
(plasenta). Oleh sebab itu, sebagian besar
darah yang akan menuju paru-paru dialihkan
ke seluruh tubuh melalui ductus arteriosus.
 Ductus arteriosus adalah pembuluh darah
yang menghubungkan aorta (pembuluh yang
mengalirkan darah kaya oksigen dari jantung
ke seluruh tubuh) dan arteri pulmonal
(pembuluh yang mengalirkan darah dari
jantung ke paru-paru).
- Prevalence:
 5-10% of all CHD, Female:Male 1:3
- Keywords:
 Acyanotic
 Plethora
 Shunt between PA and Descending Aorta
- Clinical manifestations
 History: asymptomatic if the ductus is small,
may cause lower respiratory infection,
atelektasis, CHF
 Physical Examination: tachycardia,
Continuous murmur at left infraclavicular area,
maybe cyanosis only in the lower half of body,
systolic thrill at ULSB
 ECG: LVH (small-mod PDA), LVH+RVH
(large PDA)
 X-ray: cardiomegaly varying degrees,
pulmonary vasc marking increased
 Echocardiography
- Treatment
 Spontaneous closure during 1st month (rare)
 In the absence of other CHD >> should be
occluded (surgical or ligation)
 For neonatus and premature with CHF >>
prostaglandin synthesis inhibitor
(indomethacin)
Tetralogy of Fallot
- Tetralogy of Fallot adalah salah satu penyakit jantung
bawaan pada bayi baru lahir. Penyakit jantung ini
terdiri dari empat jenis kelainan struktur pada jantung
bayi.
 Tetralogy of Fallot menyebabkan darah yang
dialirkan ke seluruh tubuh bayi tidak mengandung
cukup oksigen
 Tetralogy of Fallot (ToF) terjadi ketika bayi
masih di dalam kandungan, tepatnya ketika
jantungnya sedang berkembang
 Kelainan struktur jantung pada ToF
menyebabkan darah yang kaya oksigen
bercampur dengan darah yang kekurangan
oksigen. Akibatnya, darah yang mengalir ke
seluruh tubuh tidak mengandung oksigen yang
cukup dan bayi pun kekurangan oksigen.
 Berikut ini adalah empat kelainan jantung yang
terjadi pada ToF:
 Ventricular septal defect (VSD), yaitu
lubang di dinding yang memisahkan
ventrikel kanan dan kiri
 Pulmonary valve stenosis, yaitu
penyempitan pada katup pembuluh darah
yang ke paru-paru (katup pulmonal)
sehingga darah dari jantung yang ke paru-
paru berkurang
 Posisi aorta tidak normal, yakni bergeser
ke kanan mengikuti VSD yang terbentuk
(Dextroposition of Aorta)
 Right ventricular hypertrophy atau
penebalan pada otot ventrikel kanan
jantung akibat jantung bekerja terlalu
keras untuk memompa darah
- Clinical manifestations
 History: cyanosis at birth/shortly thereafter,
Dyspneu,Squatting, hipoxic spells develop
later
 Physical Examination:
 Cyanosis, tachypnea, clubbing
 ESM at the upper, S2 single
 ECG: RAD (cyanotic TOF), RVH, RAH
 X-ray: oligemi, heart size N/<, Boot shaped
heart
  is a palliative surgical procedure to treat
patients with cyanotic heart diseases
characterized by decreased pulmonary artery
flow.
 The BT shunt aims to supply the pulmonary
artery with blood flow sufficient to relieve
cyanosis without inducing pulmonary over-
circulation. The classic BT shunt procedure
was performed through a lateral thoracotomy,
dividing the subclavian artery and
anastomosing it to the pulmonary artery in an
end-to-side manner. The original technique has
been modified extensively and has evolved to
the mBTT shunt, which utilizes an
interposition polytetrafluoroethylene (PTFE)
graft to establish a systemic-pulmonary shunt
without sacrificing the subclavian artery or any
of the brachiocephalic tributaries

DENTAL PROCEDURE

- Elimination of focal infection before correction of

CHD
- In highest risk population, prophylaxis of IE should
be considered before dental procedure