CYANOTIC HEART DEFECTS

DISCLOSURE

Relevant relationships with commercial

entities – none

Potential for conflicts of interest within this

presentation – none

Steps taken to review and mitigate potential

bias – N/A

END-OF-WEEK OBJECTIVES

This lecture is designed to meet the following end-of-week learning objective:

1. Describe the pathophysiology and consequences of cyanotic heart defects

Part 1 – Approach to cyanosis

Part 2 – Cyanotic Heart Defects

MODULE OBJECTIVES

By the end of this module, you should be

able to:

1. Describe the normal cardiac anatomy and physiology

2. Identify the pathophysiology, and consequences of cyanotic heart defects with key examples

3. Name common cyanotic heart defects

NORMAL HEART

Right Atrium

Right Ventricle

Left Atrium

Left Ventricle

Aorta

Pulmonary Artery

to lungs

from lungs

CYANOTIC HEART DEFECTS

Mixing Separation R to L Shunt

RA

RV

PV

Ao PA

SVC IVC

PA Ao

LA

LV

RA

RV

PV

PA Ao

LA

LV

SVC IVC

TETRALOGY OF FALLOT (TOF)

• 10% of CHD

• Anatomy

– Large VSD

– Pulmonary stenosis

– Overriding aorta

– RVH

• Pathophysiology

– RL shunt via VSD

Boot-shaped Heart

TETRALOGY OF FALLOT (TOF)

Patch closure of VSD

Enlarge RV outflow tract

TRANSPOSITION OF THE GREAT ARTERIES (TGA)

• 5% of CHD

• Anatomy

– Aorta from right ventricle, pulmonary artery from left ventricle

• Pathophysiology

– Separation of pulmonary and systemic circulation

Egg on a string

TRANSPOSITION OF THE GREAT ARTERIES (TGA)

Balloon Atrial Septostomy Arterial Switch Procedure

TRUNCUS ARTERIOSUS (PTA)

• <1% of CHD

• Anatomy

– Common arterial trunk

• Pathophysiology

– Mixing

– pulmonary blood flow

– Coronary ischemia

Cardiomegaly

Increased

pulmonary

vasculature

RV-PA conduit

VSD closure

TRUNCUS ARTERIOSUS (PTA)

EISENMENGER SYNDROME

• Anatomy

– Large septal defect

• Pathophysiology

– Initial LR shunt

– Pulmonary hypertension

– RL shunt

PVR

Dark lung fields

CYANOTIC HEART DEFECTS

Mixing Truncus arteriosus

Tricuspid atresia

TAPVC

Separation TGA

R to L Shunt TOF

PS/PA, Ebstein’s

Eisenmenger

RA

RV

PV

Ao PA

SVC IVC

PA Ao

LA

LV

RA

RV

PV

PA Ao

LA

LV

SVC IVC

CYANOTIC HEART DEFECTS

5 T’s:

1. Truncus arteriosus

2. Transposition of the great arteries (TGA)

3. Tricuspid atresia

4. Tetralogy of Fallot (TOF)

5. Total Anomalous Pulmonary Venous Connection (TAPVC)

2 P’s:

• Pulmonary stenosis

• Pulmonary atresia

2 E’s:

• Ebstein’s anomaly of the tricuspid valve

• Eisenmenger syndrome

MODULE OBJECTIVES

By now, you should be able to:

1. Describe the normal cardiac anatomy and physiology

2. Identify the pathophysiology, and consequences of cyanotic heart defects with key examples

3. Name common cyanotic heart defects