Defects of the Right Heart Review

40%

1. Most common cause is congenital malformation of cusps

2. Most common in pediatric patients

PS

What is commonly demonstrated with PS due to obstruction of the outflow?

RVH

Valvular (pulmonary) stenosis is commonly associated with?

1. Maternal rubella

2. Clubbing of fingers

3. Turner syndrome

4. Noonan syndrome

5. Polycythemia vera

What has a strong association with PS caused by dysplastic valve?

Noonan Syndrome

Infundibular stenosis is associated with?

TOF

Is PS associated with a VSD or ASD?

VSD

Mild pulmonic stroke volume (PSV)

0.9-3 m/s

Moderate pulmonic stroke volume (PSV)

3-4 m/s

Severe pulmonic stroke volume (PSV)

> 4 m/s

Severe PS has a PPG of?

64 mmHg

Absence of the pulmonary valve opening

Pulmonary atresia

In pulmonary atresia, the PA is supplied with blood by the?

Ductus arteriosus

If there is no VSD with pulmonary atresia, what must be patent?

PFO or small ASD needs to be patent for blood to get from the right to left heart

In pulmonary atresia, flow from PDA backfills the?

MPA (flow reversal)

Pulmonary atresia with VSD and overriding aorta is referred to as?

Pseudotruncus

What procedure is used to correct pulmonary atresia?

Fontan (IVC connected by conduit to RPA)

1. Absence of the opening of the TV

2. Lack of communication (blood flow) between RA and RV

3. Hypoplastic RV and dilated RA

4. VSD commonly associated finding

Tricuspid atresia

What must be present in cases of tricuspid atresia?

PFO to allow flow to move from RA to LA

1. Congenital malformation

2. Tricuspid leaflets more inferior and toward the apex

3. Small RV

4. Atrialized portion of RV causes very large RA

5. Causes severe TR that originates closer to the apex than normal

Ebstein anomaly

What is the best view to demonstrate Ebstein anomaly?

Apical 4

Symptoms of Ebstein anomaly include?

1. Dyspnea

2. Cyanosis

3. Right heart failure

4. Hepatomegaly

5. Ascites

Ebstein anomaly is usually associated with?

Secundum ASD (shunt flow right to left due to increased right heart pressures)

30% of cases of Ebstein anomaly have associated?

Wolff-Parkinson white syndrome

Normal shunt flow with Eisenmenger syndrome is?

Left to right

Chronic shunt flow with Eisenmenger syndrome is?

Left to right but can lead to increased right heart pressures (RV pressures continue to increase to eventually exceed LV pressures and shunt flow reverses right to left)

Eisenmenger syndrome has RVSP of?

> 120 mmHg (greater than systolic pressure)

Eisenmenger syndrome will lead to?

Cyanosis and right heart failure

Blood drains from left arm directly into coronary sinus through brachiocephalic vein

Persistent left SVC

The primary finding in persistent left SVC is?

Significantly dilated coronary sinus

1. Direct communication between ascending aorta and MPA

2. Causes diastolic flow reversal in descending thoracic aorta

3. Presents with continuous murmur

Aortopulmonary window

Symptoms of aortopulmonary window include?

1. Respiratory infection

2. Tachypnea

3. Tachycardia

Aortopulmonary window leads to?

CHF and Pulmonary HTN

Shunting with an aortopulmonary window is left to right unless?

Eisenmenger syndrome is present

Blood continues to be shunted through the ductus but from the descending aorta to the PA

PDA

PDA is associated with?

Premature birth

What murmur does PDA demonstrate?

Continuous (toward the transducer during systole and diastole)

PDA causes?

1. LV volume overload

2. Dilated hyperkinetic LV

3. Dilated LA

What type of flow may you see with PDA in the descending thoracic aorta?

Diastolic flow reversal

Does PDA cause increased or decreased LV preload?

Increased

Which syndrome is most likely to occur with PDA?

Eisenmenger syndrome