Congenital Heart Disease

Adult congenital heart disease

What does ACHD stand for?

1. Physiological factors

2. Original anatomic diagnosis

3. Types of surgical repair and potential complications

What factors should be used to assess ACHD?

1. Hemodynamically significant intracardiac shunts

2. Hypoxemia

3. Pulmonary hypertension

4. End-organ dysfunction

5. Exercise limitation

6. Associated arrhythmias

What physiological factors are used to assess ACHD severity?

CHD with left-to-right shunts

What is the most common CHD?

1. Volume overload

2. CHF

3. Pulmonary hypertension

4. Endocarditis

What is the clinical significant of CHD with left-to-right shunt?

1. Close spontaneously

2. Closed with a device

3. Surgical closure when clinically significant

How can CHD with left-to-right shunts be fixed?

1. Type

2. Size

3. Number

4. Location

5. Chamber size

6. Shunt size

7. PA pressure

What should be evaluated (echo goals) when scanning a CHD with left-to-right shunt?

Atrial septal defect (ASD)

A hole or opening in the septum between the atria.

1. Increased pulmonary blood flow

2. Right heart chamber enlargement (volume overload)

3. Exercise intolerance

4. Pulmonary hypertension

ASDs may cause ___.

Ostium secundum

What is the most common type of ASD?

Within the fossa ovalis

Where is an ostium secundum ASD located?

Ostium primum

Second most common type of ASD.

Ostium primum ASD

Located in the apical region near the AV valves.

Type of AVSD.

Apical region

Where is an ostrium primum ASD located?

MV cleft

Ostium primum ASDs are associated with ___.

Sinus venosus

ASD associated with partial anomalus pulmonary venous connections.

Cavoatrial junction

Where are sinus venosus ASDs located?

Coronary sinus

ASD caused by unroofing of the coronary sinus, resulting in communication with the floor of the LA.

Very ratere.

Persistent left SVC

Coronary sinus ASDs are associated with ___.

Patent foramen ovale (PFO)

Separation between the septum primum and septum secundum.

Channel or tunnel-like.

Should close after birth, but some don’t.

Embolic event

Patent foramen ovale can be the site responsible for an ___.

TEE

What modality is best for visualization of a patent foramen ovale (PFO)?

Atrial septal aneurysm (ASA)

Redundancy or saccular deformity of the IAS with increased mobility.

Risk factor for embolic events.

PFO

Atrial septal aneurysms are associated with ___.

1. > 10 mm septal plane

2. > 15 mm total excursion

Excursion of IAS (atrial septal aneurysm):

Bubble study

What is another name for a saline contrast study?

Saline contrast study

What study can be used to evaluate for ASD or PFO?

Ventricular septal defect (VSD)

Hole between the ventricles.

May be isolated or in conjunction with other defects.

1. Increased pulmonary blood flow

2. Left heart chamber enlargement

3. CHF

4. Exercise intolerance

5. Endocarditis

6. Pulmonary hypertension

What can VSDs cause?

1. Muscular

2. Perimembranous

3. Inlet VSD

4. Outlet VSD

5. Malaligned

What are the types of VSDs?

Muscular VSD

Completely surrounded by septal myocardium.

Single or multiple.

Vary in size.

Large ones may require device closure or surgery.

Apex or moderator band

Muscular VSDs are most commonly found in the region of the ___.

Perimembranous

VSD in the membranous portion of the IVS (near AV).

Septal leaflet of TV

Perimembranous VSDs are usually partially or completely closed by the ___.

Inlet VSD

Located in the inlet portion of the ventricular septum within the confines of the attachments of the TV apparatus.

Surgically

Inlet VSDs can only be fixed ___.

Cleft anterior MV leaflet

Inlet VSDs are associated with ___.

Outlet VSD

Located in the ventricular outflow tract beneath the semilunar valves.

1. Supracristal

2. Doubly committed subarterial

What is another name for outlet VSD?

Prolapse of the AV into the defect causing Al.

Outlet VSDs are associated with ___.

Truncus arteriosus

Outlet VSDs present in the ___.

Malaligned VSD

Malalignment and deficiency of ventricular septal myocardium.

Malaligned VSD

Which VSD is most associated with other anomalies (TOF, DORV)?

Atrioventricular septal defect (AVSD)

Caused by a defect in the AV septation that results in abnormalities of the IAV, IVS, and AV valves.

Requires surgical repair.

1. AV canal defect

2. Endocardial cushion defect

What is another name of an AVSD?

1. Partial

2. Intermediate

3. Complete

What are the types of AVSD?

Left-to-right

Shunting is usually ___.

Left

Pressure is normally higher in the ___ side of the heart.

1. Pulmonary HTN

2. Impairment of RV compliance

These can cause shunting to be right-to-left or bidirectional.

1. Diastolic flattening of IVS

2. Dilated RA, RV, and/or PA

Signs of potentially significant left-to-right shunting.

Patent ductus arteriosus (PDA)

Failure of spontaneous closure of the hole between the PA and Ao after birth that results in persistent patency.

Ductus arteriosus

Area that connects the PA and Ao during fetal circulation.

1. Indomethacin

2. Percutaneous device

3. Surgery

Treatment for a patent ductus arteriosus.

Obstructive lesions

May be found along the outflow tracts of the RV and LV.

Associated with pressure-overload of the ventricle, which can lead to hypertrophy and fibrosis.

1. At the level of the semilunar valves.

2. Below the valve within the ventricle.

3. Above the valve within the great arteries.

Where are obstructive lesions found?

1. Valvular aortic stenosis

2. Aortic coarctation

3. Subvalvular aortic stenosis

4. Valvular pulmonic stenosis

5. Double-chamber RV

6. Supravalvular and peripheral pulmonic stenosis

What are the types of obstructive lesions?

Systolic murmur

Clinical presentation of obstructive lesions.

Bicuspid AV stenosis

What is the most common CHD.