Congenital Heart

Disease

Habibie Arifianto, MD, FIHA

Department of Cardiology & Vascular Medicine

Faculty of Medicine, Sebelas Maret University
UNS Hospital
Dr. Moewardi General Hospital
What is Congenital Heart Disease?

 is abnormality in
cardiocirculatory structure or
function that is present on
birth, even if it is discovered
much later

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 8

Congenital Heart Disease

 Reported birth prevalence of CHD varies widely

among studies worldwide.

 The estimate of 8 per 1,000 live births is generally

accepted as the best approximation

 A worldwide annual birth rate around 150 million

births 1.35 million live births with CHD every year

Van der Linde,D. J Am Coll Cardiol 2011;58;2241-7

Prevalence of CHD Subtype

Van der Linde,D. J Am Coll Cardiol 2011;58;2241-7

Fahed AC et.al. Circ Res. 2013;112:707-720
Multifactorial CHD

 20% of CHD cases known to be associated with

genetic syndromes(chromosomal anomalies,
Mendelian syndromes, nonsyndromal single
gene disorders) and teratogens

 Down syndrome and velocardiofacial syndrome

are the most common

 80% unknown
Blue GM. MJA 2012;197:155-159
Blue GM. MJA 2012;197:155-159
 Fetal and Perinatal Circulation

Changes in Circulation After Birth

Shift of blood flow for gas exchange from
placenta to the lungs
1. Interuption of the umbilical cord
• Increase of SVR
• Closure of ductus venosus
2. Lung expansion
• Reduction of PVR
• Functional closure of PFO
• Closure of DA
Cardiac Chambers Pressure
Classification of CHD

adapted from: Rilantono, L R. 5 Rahasia Penyakit Kardiovaskular. FKUI. 2012

Plethora Vs Oligemia

Vs
adapted from: Rilantono, L R. 5 Rahasia Penyakit Kardiovaskular. FKUI. 2012
adapted from: Rilantono, L R. 5 Rahasia Penyakit Kardiovaskular. FKUI. 2012
SHUNT LESION
NORMAL CARDIAC CIRCULATION
SHUNT LESION CIRCULATION
ATRIAL SEPTAL DEFECT
Natural History
Chief of Complain

 Progressive shortness of breath with exertion

Preload-reduced LV + volume overload in pulmonary
circulation

 Atrial Arrythmia
RA dilatation, stretching conduction system

 Stroke/systemic ischemic event

Paradoxical embolization thrombus through defect

 Cyanosis, unresponsive with O2 suplemental

ASD + Pulmonary Hypertension —> systemic blood flow cross
through ASD mixing with pulmonary venous —> LA, LV
Signs & Symptoms

 Signs
 Physical Examination: Wide fixed splitting S2, pansystolic murmur @ tricuspid
area
 ECG: Complete RBBB

 Symptoms
 Remain asymptomatic until Increasing of Pulmonary Vascular Resistance
(PH)
Treatment
Ventricle Spetal Defect
 Most common form of CHD, 20%
human cardiac malformation

 Perimembranous type, 80% of all

VSDs
Type of VSD
VSD circulation
Natural History
Signs & Symptom

 Sign:
 Physical Examination: Holosystolic murmur @ lower sternal border

 Symptom
 Remain asymptomatic until Increasing of Pulmonary Vascular Resistance
(PH)
 Large VSD  Heart Failure secondary to lung overflow
Patent Ductus Arteriosus

 The Ductus Arteriousus

usually close
spontaneously (72 hours
of birth), through the
contraction of an
arteriolar smooth muscle
— signaled by the rise in
postnatal systemic oxygen
level

 Common in preterm
infants
Pathophysiology

 most L-R shunt (aorta to MPA) — systole and diastole — diastolic“runoff” —> impaired
coronary and splanchnic perfusion
 Large PDA — LVEDP increase — pulmonary congestion
Natural History
Differential Cyanosis
Treatment
OBSTRUCTIVE LESSION
Congenital Obstructive Lesion
Branch PA stenosis

supravalvar stenosis

valvar stenosis

Subvalvar stenosis
CoArctation Aorta

Aortic stenosis

Subaortic stenosis
Complex Congenital Malformation
Tetralogy of Fallot

Malalignment of Infundibular
septum to anterior-cephalad

Narrowing of RV Outflow Overriding aorta

(pulmonal stemosis)

Subaortic VSD

RV Hypertrophy as a result
Of hemodynamic complication
Natural History
Treatment
Thank You