Basic understanding about

Congenital Heart Disease:

a pathophysiology and classification

review

winda azwani, MD
 Objectives

• Understand several common type of Structural lesion

CHD
• Understand classification of CHD
• Understand pathophysiology CHD Pathophysiology

• Understand sign & symptom

Clinical picture
 • Ventricular • Tetralogy of Fallot:

Congenital Septal
Defect 30-35%
•
5-7 %
Pulmonary valve

heart disease
stenosis: 5-7 %
• Atrial Septal Defect: • Aortic valve
6-8 % stenosis: 4-7 %

(CHD) • Patent Ductus

Ateriosus: 6-8 %
• D-Transposition of
great arteries: 3-5 %
• Coarctation of
Aorta: 5-7 %
epidemiologY

CHD becomes our

national health problem
Pathophysiology & classification
Pathophysiology Classification

Pulmonary blood flow Increased PBF

Decreased PBF

Cyanosis Cyanotic

Acyanotic

Ductus arteriosus dependent Non-ductal dependent

Ductal -dependent

Obstructive lesion Left-sided heart

Right-sided heart
 Clinical Manifestation

Agarwal N, Agarwal M, Joshi R. Neonatal and pediatric Cardiac Emergencies – When to Start Prostaglandin in Neonate. 2015. 64-72p
 How to understand CHD classification?
Cyanosis Obstructive lesion
Cyanotic Acyanotic Right-sided Left-sided obstruction
Tetralogy of Fallot Ventricular septal defect obstruction
(VSD)
Tricuspid Atresia Atrial septal defect (ASD) Pulmonary stenosis Aortic stenosis
Pulmonary atresia Patent ductus arteriosus
(PDA) Tetralogy of Fallot Coarctation of aorta

Pulmonary atresia Interrupted aortic arch

 Let’s make it simple…

Left heart
Mitral valve
Right heart Aortic valve
Aorta, aortic arch
Pulmonary veins
Tricuspid valve Systemic Circulation
Pulmonary valve
Pulmonary artery
Systemic veins
Pulmonary Blood Flow

https://www.stanfordchildrens.org/content-public/topic/images/64/125864.gif
Coarctation of aorta

Type Pathophysiology Clinical finding

• Discrete • Reduced • Fast breathings,
• Long segment systemic blood sweating while
flow feeding, not
• Duct-dependent eating well,
if severe being fussy a lot
of the time.

https://stanfordhealthcare.org/medical-c
Coarctation of Aorta

• 8-10% of CHD

• Narrowing of distal part of

the aortic arch

• Lower half of the body 

duct dependent

• Symptoms will develop as

DA start to close

The Royal Children’s Hospital Melbourne

Tomar M. J Intensive & Crit Care. 2016;2(2):12
Critical Aortic Stenosis

• 6% of CHD

• Ductal Closure  LV
maintain systemic blood
flow

• Acute increase afterload

• LV function compromise
+ myocardial
dysfunction 
congestive HF + shock

The Royal Children’s Hospital Melbourne

Tomar M. J Intensive & Crit Care. 2016;2(2):12
Interrupted Aortic Arch

• Major pathology : failure

development in a portion of the
aortic arch

• No direct connection between

ascending and descending aorta

• Lower half of the body  duct

dependent

The Royal Children’s Hospital Melbourne

Yun SW. Congenital heart disease in the newborn requiring early intervention. Korean J Pediatr. 2011;54(5):183-91
Hypoplastic Left Heart Syndrome (HLHS)

• 2-3% of all CHD

• Left side of the heart is

unable to support systemic
circulation

• Pulmonary circulation 
patent foramen ovale

• Systemic circulation 
dependent to ductus
arteriosus

Yun SW. Congenital heart disease in the newborn requiring early intervention. Korean J Pediatr. 2011;54(5):183-91
Right Heart Obstruction
Critical Pulmonary Stenosis

• 10% of all CD

• Well tolerated in fetal

circulation

• After birth  PVR falls

• Pulmonic circulation
maintained by ductus
arteriosus

The Royal Children’s Hospital Melbourne

Ren X, Cannistra LB. Pulmonic Stenosis: Medscape; 2017
 Pulmonary atresia with ventricular septal defect (PA-VSD)

• ToF type pulmonary atresia

• Natural history depends on

pulmonary blood supply

• ductal-dependent

The Royal Children’s Hospital Melbourne

Yun SW. Congenital heart disease in the newborn requiring early intervention. Korean J Pediatr. 2011;54(5):183-91.
Ebstein’s Anomaly

• Apical displacement of septal tricuspid leaflet

• Atrialization of RV  Small RV functionally

• RV can’t generate sufficient pressure to open

pulmonary valve

The Royal Children’s Hospital Melbourne

https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/graphicsummaries/ebgs
 Lack of Intercirculatory Mixing

Transposition of the Great Artery (TGA)

• Atrioventricular concordance,
Ventriculoatrterial discordance

• TGA without VSD/ Intact Ventricular

Septum (IVS)
• TGA-VSD

• limited intercirculatory mixing

progressive cyanosis within the first
few hours of life

• Mixing  foramen ovale, ductus

arteriosus. Ventricular septal defect

The Royal Children’s Hospital Melbourne

Yun SW. Congenital heart disease in the newborn requiring early intervention. Korean J Pediatr. 2011;54(5):183-91.
Take Home Message

• Pathophysiology of CHD determines clinical manifestation

• Several type of CHD make it simple!
• First step : start with the normal heart 
• Left vs Right heart lesion would help us to understand the
pathophysiology of CHD
Terima Kasih
Semoga
bermanfaat………

Correspondance:
azwani.winda.md@gmail.com