Congenital heart diseases

Question 1

What are three kinds of atrial septal defects and location in the septum for each?

Answer 1

Ostium secundum (80%)-central interatrial septum
Ostium primum - low septum
Sinus venosus defect - high septum

Question 2

What is the pathophysiology of ASD

Answer 2

Blood from LA to RA, increasing right heart output- RA/RV dilation occurs with big shunts with pulmonary to systemic flow ratio > 1.5:1.0

Question 3

When do symptoms in a patient’s lifetime occur for ASD

Answer 3

After 40 - exercise intolerance, dyspnea on exertion, fatigue

Question 4

What do you see on physical exam in ASD

Answer 4

Wide, fixed splitting of S2 due to increased pulmonary flow

Question 5

What three diagnostic modalities can be used to diagnose ASD

Answer 5

1. ) TEE - best (use bubble study contrast for better results)
2. ) CXR - large pulmonary arteries
3. ) ECG - RBBB, right axis deviation, Afib/flutter

Question 6

What are five main complications of ASD

Answer 6

1. ) Pulmonary HTN - common after age 40
2. ) Eisenmanger - irreversible pulmonary HTN, causing reversed right to left shunt, resulting in heart failure and cyanosis
3. ) RVF
4. ) Afib
5. ) Stroke from paradoxal emboli - important

Question 7

What should you do for treatment of ASD

Answer 7

Only surgery if very large or if pulmonary to systemic blood flow greater than 2:1

Question 8

What is the pathophysiology of ventricular septal defect

Answer 8

Blood flow from LV into RV, but small defects are asymptomatic, only large ones can cause eisenmangers syndrome

Question 9

What are the symptoms of VSD for small shunts, large shunts without increased pulmonary resistance, and large shunts with increased pulmonary resistance

Answer 9

Small shunt - asymptomatic

Large shunt without PR - CHF, growth failure, recurrent lower respiratory infections

Large shunt with PR - SOB, dyspnea on exertion, chest pain, syncope, cyanosis

Question 10

What are the clinical exam signs of VSD and the effects of valsalva and handgrip

Answer 10

Blowing holosystolic murmur with thrill

Valsalva and handgrip decrease murmur intensity

Increased pulmonary component of S2 as pulmonary HTN, with aortic regurgitation

Question 11

What three diagnostic modalities can you use for VSD

Answer 11

ECG: Biventricular hypertrophy when PR is high**

Cxr: Pulmonary artery bigger, seen with increased cardiac silouhette (not due to increased heart size)**

Echo: Septal defect
Softer murmur = bigger VSD

Question 12

What are four complications of VSD

Answer 12

1. ) Endocarditis
2. ) Progressive aortic regurgitation
3. ) Heart failure
4. ) Pulmonary HTN/eisenmangers

Last two overlap with ASD

Question 13

When is medications indicated and surgical repair indicated for VSD

Answer 13

Medications: diuretics, afterload reductors (ACE), digoxin

When pulmonary flow to systemic flow ratio greater than 2:1, or patient has infective endocarditis (not asymptomatic patients with small defects) - same as ASD

Question 14

When is endocarditis prophylaxis indicated for patients with VSD

Answer 14

When VSD is complicated or history of endocarditis

Question 15

What is the pathophysiology of coarctation of the aorta

Answer 15

Has adult and child form, in adults you have narrowing/constriction of aorta distal to aortic arch at origin of left subclavian artery near ligamentum arteriosum, leading to increased left ventricular afterload

Question 16

What condition is coarctation of the aorta associated with in adults

Answer 16

Bicuspid aortic valve - adults

Turners syndrome - children

Question 17

What are the symptoms of coarctation of the aorta

Answer 17

Think about less blood flow to legs - hypertension in arms and hypotension in legs, more developed upper body, delayed femoral artery compared to radial artery, cold extremities

Headache (high BP above), claudication with exercise and leg fatigue (less O2 below)

Question 18

What are two diagnostic modalities used for coarctation of the aorta

Answer 18

1. ) ECG - LVH (due to increased afterload)

2. ) CXR - notching of ribs due to collateral circulation across intercostal arteries engorging - impt

Question 19

What are four complications of coarctation of the aorta

Answer 19

1. ) Severe HTN
2. ) Rupture of cerebral aneurysms
3. ) Aortic dissection
4. ) Infective endocarditis

You can remember the first three because of increased HTN around proximal aorta and everything above

Question 20

What is the standard treatment for coarctation of the aorta

Answer 20

Surgical decompression

Can also use percutaneous balloon aortoplasty in select cases

Question 21

What is the pathophysiology of patent ductus arteriosus

Answer 21

Communication between aorta and pulmonary artery that persists after birth, forming a left to right shunt

Question 22

What is the function of the ductus arteriosus in the fetus

Answer 22

To shunt blood away from non-functioning lungs, maintained by low oxygen tension and prostaglandins

Question 23

What three conditions do you see patent ductus arteriosus occur in

Answer 23

Congenital rubella syndrome, high altitude, premature births

Question 24

What are the clinical signs of patent ductus arteriosus

Answer 24

LVH secondary to left to right shunt, RVH secondary to pulmonary HTN, pulmonary HTN, eisenmanger’s

2. ) Continuous machine murmur with loud P2 due to pulmonary HTN
3. ) Wide pulse pressure and bounding peripheral pulses**
4. ) Lower extremity clubbing - toes more clubbing than fingers (differential cyanosis - think about it)

Question 25

What two diagnostic modalities can be used for patent ductus arteriosus

Answer 25

1. ) CXR - Increased pulmonary vascular markings, enlarged silouette from increased pulmonary artery size, calcifications
2. ) Echo - shows defect

Question 26

What are the indications for surgery in patent ductus arteriosus

Answer 26

If no peripheral vascular disease - surgical ligation

If pulmonary HTN or right to left shunt present - no surgery, use indomethacin (counterracts PGE)

Question 27

What are four things wrong with the heart in tetralogy of fallot

Answer 27

1. ) Stenosis of pulmonary artery
2. ) Right ventricular hypertrophy
3. ) VSD
4. ) Aorta overriding VSD

Question 28

What went wrong in embrylogy to cause TOF

Answer 28

Defect in development of infundibular septum

Question 29

What are the symptoms of tetralogy of fallot

Answer 29

Cyanosis early this time because of RIGHT TO LEFT shunt

Tet spell: Squat after exertion to increase systemic vascular resistance, helping shunt blood from RV to lungs instead of aorta.

Second line: O2, morphine, beta blocker

Question 30

What are the degree of clinical symptoms depend on for TOF

Answer 30

Amount of stenosis of right outflow tract

Question 31

What are some diagnostic modalities used for TOF

Answer 31

Echo - test of choice - can see all abnormalities

Cxr - boot shaped heart

Question 32

What is the treatment for TOF

Answer 32

Surgery within first year of life

Question 33

What are the most common causes of death in TOF

Answer 33

Sudden cardiac death and heart failure