Congenital Heart Defects:

Part II- duct dependent CHDs.

Jadwiga A. Moll Prof., M.D., Ph.D.

Department of Cardiology
Polish Mother’s Memorial Hospital, Research Institute

Krzysztof W. Michalak M.D., Ph.D.

Department of Cardiology
Polish Mother’s Memorial Hospital, Research Institute
 Duct dependent CHDs.

Atr PA
Pulmonary
hypopl. RV

Duct dependent CHDs:

HLHS
Systemic
SA, CoA, IAA

Blood mixing/
TGA
parallel
Transposition of the great arteries.
Transposition of the great arteries.
 Transposition of the great arteries.

TGA – circulation scheme

Transposition of the great arteries.
 Transposition of the great arteries.
Ventriculoarterial discordance
Ao, PA & commissural alignment Transposition of the great arteries.

PA
Coronary arteries Transposition of the great arteries.
Transposition of the great arteries.
Hypoplastic left heart syndrome.
Hypoplastic left heart syndrome.
Sat O2:
60-70%
Sat O2:
96-100%
RA AS LA
8/2 D 10/2

RV
100/0-8

Sat O2:
80-90%
Hypoplastic left heart syndrome.
 Hypoplastic left heart syndrome.

3rd year – Fontan operation

8th day of life – Norwood operation

5-6 month of age – bidirectional Glenn procedure

Hypoplastic left heart syndrome.
Hypoplastic left heart syndrome.
 Critical aortic stenosis

AGE:
• NEWBORN
• NEONATE
• CHILD
• ADULT
Associated defects:
• LV, MV,HP
MANAGEMENT. TREATMENT: Treatment:
Conservative treatment • CATHETER INTERVENTIONS
ECHO: Catheter interventions • OPERATIONS
Cardiac surgery: • PHARMACOTHERAPY
- Valve morphology - autograft
CLINICAL EVALUATION:
General examination - LV-Ao gradient - homograft
ECG - LV function - Bioprosthetic valve
Critical lab tests: - Mechanical valve
- LV,IVS morphology; AI
ABB,BNP
- AoAsc
 Critical aortic stenosis
AORTIC STENOSIS.

CRITICAL AORTIC NEWBORN/NEONATALAO AORTIC STENOSIS IN

STENOSIS CHILDREN.
RTIC STENOSIS.
(DUCTUS DEPENDENT)

Balloon valvuloplasty in first >50mmHg Gradient increase with time

hours of life Balloon valvuloplasty(BVA)

<50mmHg
Mitral defect
Watchful waiting. In the future Treatment:
balloon valvuloplasty,
HP • BVA
valvotomy, pulmonary
autograft • valvotomy
• Bioprosthetic valve
• Mechanical valve
• TAVI
CRITICAL (NEWBORN) AORTIC STENOSIS:

• Severe aortic stenosis in newborn lead to heart failure (left ventricle

decompensation.
• Systemic flow is dependent on PDA patency – ductal dependent defect
• Stenosis is present in prenatal development and may coexist with fibroelastosis of
the endocardium.
• Critical fetal aortic stenosis may lead to left ventricle and mitral valve hypoplasia
(hypoplastic left heart syndrome)
• The treatment of choice is percutaneous balloon valvuloplasty in the first hours
after birth.
• Pharmacotherapy consist of alprostadil in continuous intra venous flow (Prostin
VR, 0,01-0,05ug/kg/min) – to the cath procedure – and inotropic, diuretics before
and after procedure.
Critical aortic stenosis
Critical aortic stenosis
Interrupted aortic arch IAA
Interrupted aortic arch IAA
 Critical pulmonary stenosis

➢CRITICAL / SEVERE VALVULAR

STENOSIS
➢DUCTAL DEPENDENT PULMONARY
FLOW
➢ECHOCARDIOGRAPHIC
MEASUREMENT OF PULMONARY
PEAK GRADIENT (it is not crucial for
qualification – „low flow stenosis”)
 Pulmonary valve stenosis

Indications for interventional treatment:

1. Critical stenosis in newborn.

2. Pressure gradient RV-PA over 50mmHg (ECHO Doppler

examination).

3. Pressure gradient RV-TP under 50mmHg as a result of coexisting

RV failure, TRV insufficiency, inflow from PDA to the PA.