Session 10 Lecture Cardiac Tumors, Cardiomyopathies, Single Ventricles, and CCTGA

Fetal Cardiac Tumors, require an urgent referral to what care to watch for what compromise ?

Require an urgent referral to perinatal care to watch for hemodynamic compromise

Common types of tumors include, name 4

1. Rhabdomyoma

2. Teratoma

3. Fibroma

4. Myxoma

Incidence and Types of Fetal Cardiac Tumors

Rhabdomyoma % ?

Pericardial Teratoma % ?

Fibroma % ?

Hemangioma % ?

Myxomas % ?

Lipomas % ?

Malignancies % ?

Rhabdomyoma 70-80%

Pericardial Teratoma 10-15%

Fibroma 10-15%

Hemangioma <5%

Myxomas <1%

Lipomas <1%

Malignancies <1%

Fetal Cardiac Tumors, Presentation, name 2

Pericardial effusion, Arrhythmias

Fetal Cardiac Tumors

Gestational age at diagnosis ranges when?

• Gestational age at diagnosis ranges from 21 to 38 weeks

Fetal Cardiac Tumors,

Usually they become apparent at what trimester?

Usually they become apparent at the third trimester

Fetal Cardiac Tumors

They may grow up to when and then they what?

They may grow up to 32 weeks and then regress

what is the most common Fetal Cardiac Tumors?

Rhabdomyoma

Rhabdomyoma, has a high association with what? Can you descibe how this tumor can be and where would it be found?

High association with tuberous sclerosis

May be a single tumor or multiple tumors in ventricles or IVS

Rhabdomyoma

can you decribe the obstruction it has?

what are the abnormalities it has?

what can this cause to the fetal?

Atrioventricular valve regurgitation and intracavitary obstruction

Arrhythmias and conduction abnormalities

May cause fetal myocardial dysfunction and hydrops

Teratoma, its a what tumor attached to where?

where is it found?

A single tumor attached to the base •

found in the pericardial reflection at the junction of SVC, RA, AsAo

Teratoma

what chamber does it impinges on? and they cause what of the what (name 3 things)

Impinges on the right atrium and they cause compression of the SVC, great arteries and cardiac chambers

Teratoma, always accompanies by what? what are with the tumor

Always accompanies by pericardial effusion

Multicystic areas within the tumor

Teratoma

what are the hemodynamic consequences in utero, what do they cause and what are they associated with?

They have hemodynamic consequences in utero and grow rapidly

May cause tamponade and are associated with hydrops up to ~80%

(Intervention in utero is necessary)

Fibroma, is a what tumors?

Single Intracardiac tumors

Fibroma, are found within where and are found within the what wall?

They are surrounded by what?

Do Fibromas regress?

Found within the ventricular septum and are found within the left or right ventricular wall

They are surrounded by myocardium

Fibromas do not regress

Fibroma

May influence the what system, cause what obstruction? and name 4 others things

May influence the conduction system, cause an outflow track obstruction, CHF, AV regurgitation or sudden death from an arrhythmia

(May need premature delivery and urgent surgery)

Myxoma, arises from the what?

Most frequently in what chamber (?%) and what other chamber (?%)

Attached to the what by a what?

Arises from the endocardium •

Most frequently in left atrium (75%) and right atrium (25%) •

Attached to the fossa ovalis by a pedicle

Myxoma, may cause what insufficiency?

Postnatally they are associated with what?

May cause what and require surgical resection?

May cause AV valve insufficiency

Postnatally they are associated with emboli

May cause CHF and require surgical resection

Other Rare Fetal Cardiac Tumors

1. Hamartomas •

2. Lipomas •

3. Malignant tumors: •

4. Rhabdomyosarcomas •

5. Fibrosarcomas •

6. Lymphosarcomas •

7. Giant cell sarcomas •

8. Fibromyxosarcomas •

9. Leiomyosarcomas •

10. Neurogenic sarcomas •

11. Undifferentiated sarcomas

Echo Interrogation;

Detailed evaluation of what (name 4)

Description of (name 4)

Look for obstruction of what (name 2)

Assess what output?

Assess of what other things (name 5)

assess for fetal what name 2

serial assessment to evalute what growth?

1. Detailed evaluation of atria, ventricles, septum and myocardium •

2. Description of number, location, tissue characteristic and size •

3. Look for obstruction of ventricular inflow or outflow and chamber compression •

4. Assess cardiac output •

5. Assess of venous flows, extracardiac Dopplers (DV, UA/UV) •

6. Assess for fetal arrhythmias and conduction •

7. Serial assessment to evaluate tumor growth

Cardiomyopathy, Refers to the disease process affecting what name 4

Refers to the disease process affecting cardiac muscles, chamber size, thickness and function

is Cardiomyopathy rare or common?

Extremely rare

what are the 2 main category types of Cardiomyopathy

Dilated

Hypertrophic

Dilated Cardiomyopathy

what happens to all found chambers?

usually has what?

what happens with the wall contraction?

what is a common finding and may lead to what

associated with what name 4

All four chambers dilate •

Usually has inflow regurgitation •

Decreased wall contraction •

Pericardial effusion is a common finding • May lead to hydrops •

Associated with maternal infections, autoantibodies, parvovirus, and complete heart block

Hypertrophic Cardiomyopathy - what is it?

also explain whe chamber size and function

what obstruction can occur

what is common

may be cause by what name 3

can it resolve ?

can lead to what name 3

Concentric hypertrophy of both ventricles •

Small ventricular chamber size with normal function •

Left ventricular outflow track obstruction can occur •

Pericardial effusion is common •

May be caused by diabetes, storage diseases, and Noonan Syndrome •

Can resolve or lead to heart failure, hydrops, and in utero death

What is Ectopia Cordis?

The heart is located outside of the chest cavity

Ectopia Cordis, what number are common with which sex? and associated with what?

The heart is located outside of the chest cavity

• 2/3rds are male •

Associated with trisomy 18

Ectopia Cordis, Described as the what?

Described as the pentalogy of Cantrell

Ectopia Cordis

(loaction??) - what two defect found where?

Deficiency of the what?

what defect

and congenital what malformation - name 4

Midline supraumbilical abdominal wall defect •

Lower sternum defect •

Deficiency of the anterior diaphragm •

Pericardial defect •

And congenital heart malformation (tetralogy of Fallot, DORV, VSD or LV diverticulum

Univentricular Heart, Aka know as what?

can describe the apparence of this and is this a rare or common condition?

Aka single ventricle •

3 chambers – two atria and a single ventricle •

Rare condition

Univentricular heart

any what disease where what empty into what?

describe 2 things it may have and what is it called ?

Any CHD where two inflow valves empty into one ventricle •

May have a second small ventricle •

May have common valve •

Called a “Y” type circulation

Univentricular Heart • Associations name 3

heterotaxy syndrome •

coarct of the aorta •

Transposition of the great arteries

Congenitally Corrected Transposition of the Great Arteries

Previously called what?

resu;ts from what?

describe the concordance ?

Previously called L-TGA •

Results from abnormal looping of the bulbus cordis •

Atrial ventricular discordance and ventricular arterial discordance

CCTGA, is know as what? (why is it not bad hint?)

describe the order in the heart?

Essential to identify morphologically correct or incorrect chambers?

Identify what two things?

where is the Aorta located?

Two wrongs make a right! •

Left atrium -> RV->aorta •

Essential to identify morphologically correct chambers!

• Identify moderator band! Look branch Pas! •

Aorta is located anterior and to the left of the pulmonary artery

CCTGA • Echo Findings:

what view wil show ventricular what? and what band?

Describe the Tricuspid valve ?

what cardio postion in what % of the cases

what view shows vessels running what and look for what?

4 chamber will show ventricular inversion • Moderator band •

Inferior placed tricuspid valve • Possible TR

• Mesocardia in 25% of cases

• 5 chamber may show vessels running parallel • Look for PA branches

CCTGA • Common associations: name 5

VSDs (70%) •

Ebstein Anomaly (30%) •

LVOT obstruction (40%) •

Dextrocardia (17%) •

Complete Heart Block (17%)