THE MOST COMMON CONGENITAL

HEART DISEASES IN CHILDREN

Halszka Kamińska
Department of Pediatric Cardiology
and General Pediatrics
7 1
2 1
%
5 4+
/ 98%
6
0
1
3+ + 3 96/5 + 98%
75%
9 +
7 8
8 % 7
% 5 24/5 +
% 75%
 CHD – clinical snippets
•
Shunting L  R = HF
•
Shunting R  L = cyanosis
•
Duct-dependent  treatment in neonatal
period
•
LR shunting  surgery AFTER
pulmonary resistance drop AND [!]
BEFORE plmonary hypertension evolves
•
CHD clinical picture: shunt direction +
pulmonary blood flow quantity + grade of
systemic circulation impairement
 CHD – surgical treatment

Necessary in neonatal period:

A) Duct-dependent – PROSTAGLANDIN !!!:

• Duct-dependent pulmonary circulation
• Duct-dependent systemic circulation
• Duct-dependent mixing of blood flow

B) With critically increased pulmonary blood flow

 CHD – surgical treatment

AFTER neonatal period or even later:

A) NON-CYANOTIC
• Increased pulmonary blood flow (LR shunts)
• Left outflow obstruction
B) CYANOTIC
• Diminished pulmonary blood flow
• Increased pulmonary blood flow
CHD

CYANOTIC NON-CYANOTIC

Pulmonary blood flow TOF, AT, AP, Ebstein -

INCREASED TGA, TAC, TAPVD PDA, VSD, ASD, AVSD,

APW

NORMAL - CoA, AS + PS (not

critical)
CHD with LR
SHUNTING
 CHD with LR shunting(non-cyanotic +
increased pulmonary blood flow)

•
Shunting L  R ~ NO CYANOSIS!!!
•
Heart failure symptoms after pulmonary resistance drop (increased
shunting) ~ usually 3rd-6th week of life
•
HF = sweating, getting tired while feeding, failure to thrieve,
dyspnoea with intecostal recessions
•
Increased pulmonary blood flow  respiratory trackt infections
•
Increased pulonary blood flow  increased pulminary blood
pressure  remodelling of pulmonary arteries  IRREVERSIBLE
PULMONARY HYPERTENSION
•
SURGICAL CORRECTION – usually 3-6 month of life (or later if no
symptoms)
•
RV BP > LV BP  reversed shunting: RL  CYANOSIS
(Eisenmenger’s syndrome) – UNTREATABLE!
 CHD with LR shunting(non-cyanotic +
increased pulmonary blood flow)

•
Ventricular septal defect (VSD)
•
Atrial septal defect (ASD)
•
Atrioventricular septal defect (AVSD)
•
Aortopulmonary window (APW)
•
Persistent ductus arteriosus (PDA)
 VSD

AP
W

PDA ASD AVSD

 VSD

The most common

CHD in children
(20%)?
AP
W

PDA ASD AVSD

VSD

The most common

CHD in children
(20%)
 VSD

The most
common CHD in
Down syndrome
(35-40%)?
AP
W

PDA ASD AVSD

The most
common CHD in
Down syndrome
(35-40%)

AVSD
 VSD

Early start of
symptoms (HF) ~
usually 3rd-6th week
AP
of life?
W

PDA ASD AVSD

 VSD

Early start of
symptoms (HF) ~
usually 3rd-6th week
AP
of life
W

AVSD
 VSD

Symptoms (HF) ~
mild and usually
late (in adulthood)?
AP
W

PDA ASD AVSD

 Symptoms (HF) ~
mild and usually
late (in adulthood)?

PDA ASD
 VSD

Possible
percutaneous
treatment?
AP
W

PDA ASD AVSD

 VSD

Sometimes…

Possible
percutaneous
treatment

PDA ASD
 CHD with impaired systemic
blood flow(non-cyanotic, with
normal pulmonary blood flow)
 CHD with impaired systemic blood flow

•
If CRITICAL impairment  circulatory
collapse and death!
•

•
Aortic stenosis (AS)
•
Coarctation of the aorta (CoA)
•
Interrupted aortic arch (IAA)
•
Hypoplastic left heat syndrome (HLHS)
CHD with impaired systemic blood flow
AS

CoA
HLHS

IAA
 COARCTATION OF THE AORTA (CoA)

•
5-10% CHD
•
The most common in Turner’s
syndrome
•
CRITICAL  symptoms after
DA closure  Treatment: PGE1
+ early surgery
•
Arterial hypertension ~
popular complication
•
For re-coarctation  possible
percutaneous interventions
 CYANOTIC CHD with
DECREASED pulmonary
blood flow
 CYANOTIC CHD ~ decreased Qp

•
CYANOSIS!
•
Tetrallogy of Fallot (TOF)
•
Pulmonary atresia (AP)
•
Tricuspid atresia (AT)
•
Ebstein’s anomaly
TETRALLOGY OF FALLOT (TOF)

•
3,5% CHD; the most common
cyanotic CHD
•
RVOTO + VSD + Ao dextraposition
+ RV hypertrophy
•
Cyanosis may not be present at the
beginning („pink” TOF)
•
CYANOTIC SPELL ~ sudden RVOT
obturation  rapid decrease of Qp
+ reversed shunt VSD
•
Treatment: Propranolol (cyanotic
spell prevention); SURGICAL
CORRECTION usually 3-6 months
OR promptly after first cyanotic
spell
 PULMONARY ATRESIA WITH VENTRICULAR SEPTAL
DEFECT (AP+VSD)
•
AP+VSD = TOF+AP
•
Usually hypoplastic MPA, LPA, RPA
•
CYANOSIS (mixed blood in Ao)
•
MAPCAs ~ major aorto-pulmonary
corlateral arteries ~ sometimes may
INCREASE Qp!
•
TREATMENT:
•

•
PGE1
•
Aorto-pulmonary anastomosis
•
(B-T)
•
Anatomical correction – only if
pulmonary arteries grow enough
(50%)
•
Patients with effective MAPACAs
colateral circulation may live without
surgery ~ BUT! MAPCAs are
UNSTABLE  new may evolve /
old may obstruct
 PULMONARY ATRESIA with INTACT VENTRICULAR
SEPTUM (AP+IVS)

•
Usually accompanied by:
•

•
RV hypoplasia
•
TV anomalies
•
Coronary arteries’
anomalies~ 50%
•
MPA usually NOT
hypoplastic
•
CYANOSIS! ~ shortly after
birth, sometimes reduced
systemic blood flow!
•
TREATMENT: PGE1 
Rashkind (mixing blood at
atrial level)  surgical
intervention (usually many
stages)
 EBSTEIN’s ANOMALY

•
Often +ASD
•
CYANOSIS ~ RL
shunting (FO/ASD) +
decreased Qp (ITV)
•
ARRHYTHMIAS!!!
(AF, NCN, WPW)
 CYANOTIC CHD with
INCREASED pulmonary
blood flow
 CYANOTIC CHD with INCREASED Qp

•
Total anomalus pulmonary venous
dreinage (PAPVD)
•
Transposition of great arteries (TGA)
•
Common aortic trunc (CAT)
 TOTAL ANOMALUS PULMONARY
VENOUS DREINAGE (TAPVD)
•
ALL VEINS to RA!
•
Patent FO necessary to survive!
•
FO ~ RL shunt = CYANOSIS
•
Early surgical correction ~ especially if obstructed pulmonary veins
 TRANSPOSITION OF GREAT
ARTERIES (TGA)
•
Usually eutrophic /
hypertrophied
neonates
•
CYANOSIS appears
after DA closure
•
TREATMENT: PGE1
+ Rashkind
•
Simple TGA ~
arterial switch in
2nd week of life
 COMMON ARTERIAL TRUNK (CAT)

•
ALWAYS VSD + CAT with common valve above it
•
Very early drop of pulmonary resistance  PH
And then there were those……
hard to qualify
 „CLINICAL CAMELEONS”

•
Depending on anatomical variant may
present as:
•

• HF (LR shunts + ↑ Qp)

• Cyanosis (↓ / ↑ Qp)
•

•
Double outlet right ventricle (DORV)
•
Tricuspid atresia (AT)
 DOUBLE OUTLET RIGHT VENTRICLE (DORV)
•
Rich morfological spectrum
•
3 clinical types ~ with symptomatology simillar to:
•

•
VSD (no RVOTO; dominant LR shunt  ↑ Qp)
•
TOF (RVOTO ~ in fact can be tricky to tell DORV vs. TOF)
•
TGA (cyanosis from blood mixing + ↑ Qp)
•
TREATMENT ACCORDING TO DOMINANT SYMPTOMS (MPA banding, B-T,
surgical correction)
 TRICUSPID ATRESIA (AT)
AT variants +AP +RVOT no
O RVOTO

Type I (normal A
arterial B C
(70%)
position) ~
70%
Type II A B C
(+ d-TGA) ~ (20%)
25% (hypoplastic
Ao)

Type III X A B
(+ l-TGA/other (LVOTO)

malformations)
~ 3-7%