Congenital

Question 1

Timing for medical, surgical, and interventional therapy of unrepaired complete AV canal defects with Eisenmenger physiology

Answer 1

Complete AV canal defects have both atrial and ventricular septal defects. Characterized by a primum ASD that is contiguous with a posterior VSD and a common AV valve.

Surgical: Heart and lung transplantation or lung transplantation with intracardiac repair are treatment options in Eisenmenger patients. Transplantation should be reserved for severely symptomatic patients, since overall survival with medical management is usually quite good even in patients with severe pulmonary arterial disease

Question 2

Williams Syndrome

Answer 2

Childlike facies, peripheral pulmonary stenosis, hypercalcemia, familial dyslipidemias

Mutation in the elastin gene

Cardiac associations : supravalvular stenosis, coarctation of the thoracic or abdominal aorta and renal artery stenosis

Question 3

Holt-Oram Syndrome

Answer 3

Heart and hand syndrome
Autosomal dominant mutation in TBX5
- Conduction system abnormalities
- Association with Secundum ASD

Question 4

Secundum ASD

Answer 4

• Absence of tissue in the fossa ovalis
• RA and RV enlargement
• RV heave, Ejection murmur in the PA, Fixed split 2nd sound, may get tricuspid diastolic flow rumble

Question 5

Pulse oximetry with ASD

Answer 5

Measure at rest and exercise to evaluate unrepaired ASD to determine direction and magnitude of shunt.

Question 6

Closure of ASD

Answer 6

RA and RV Enlargement w symptoms (Class 1) without symptoms (Class 2)
- As long as no evidence of pulmonary vascular disease. (PASP <50% systemic, PVR <1/3 SVR; no R to L shunt on pulse ox)
- Sinus or Primum ASD needs surgical closure

Question 7

Sinus Venosus ASD

Answer 7

• Posterior to the fossa ovalis
• Usually associated with anomalous right upper pulmonary vein
• Right sided enlargement

Question 8

RV volume overload and no ASD think?

Answer 8

Anomalous pulmonary veins. Get MRI or TEE to identify the shunt.

Question 9

Primum ASD (Partial A-V canal defect)

Answer 9

Inferior portion of the septum
- Mitral valve is usually cleft
- Often associated with Down’s Syndrome

Question 10

Noonan’s Syndrome

Answer 10

Short stature, webbed neck, wide spaced eyes
- Autosomal Dominant
- Associated with pulmonary stenosis

Question 11

Turner Syndrome

Answer 11

Web neck, wide spaced eyes, low set ears
- Association with coarctation of the aorta

Question 12

Ebstein’s Anomaly

Answer 12

Inferior displacement of the tricuspid valve
- 50% have secundum ASD or PFO
- 25% have accessory pathway

Question 13

Differential Cyanosis

Answer 13

• Pink fingers and blue toes seen with PDA

Question 14

Erythrocytosis with Eisenmenger’s

Answer 14

Inf Hb <= 20 or Hct <= 65, no phlebotomy

Question 15

Pulmonary valve replacement in TOF

Answer 15

• Mild or greater RV or LV dysfunction
• Severe RV dilation: RVEDVi >=160 ml/m2; RVESVi >= 80 ml/m2
• RVEDV >= 2x LVEDV
• RVSP >= 2/3 systemic pressure
• Progressive decrease in exercise capacity

Question 16

Tetralogy of Fallot

Answer 16

• Pulmonic stenosis
• VSD
• Stenotic RVOT
• Associated with DiGeorge Syndrome

Question 17

DiGeorge Syndrome

Answer 17

• Truncus arteriosus/ conotruncal defects/ TOF, hypoparathyroidism, Thymus dysfunction, Cleft palate, small low-set ears, short width of eye openings, hooded eyes, long fase
• 22q 11 deletion
• Autosomal dominant
• Associated with TOF

Question 18

Glenn Shunt

Answer 18

SVC to R Pulmonary artery
Bidirectional Glenn: SVC into both PAs

Question 19

Blalock-Taussig Shunt

Answer 19

Subclavian vein connected to pulmonary artery.

Question 20

Work up for neurological deficiency in patient with shunt

Answer 20

Scan head and rule out iron deficiency

Question 21

Transposition D vs L

Answer 21

D-loop: RV on R
L-loop: RV on L

Aorta follows the loop
d-TGA - aorta anterior and to the R
L-TGA - aorta anterior and to the L

D-transposition after Mustard: think RV failure and tricuspid regurgitation
L-transposition: Monitor for heart block, systemic AV regurgitation (TR) and heart failure

Question 22

Marfan Syndrome

Answer 22

FBN1 mutation
- Connective tissue protein fibrillin-1
- Aortic root dilation, lens dislocation, wrist/ thumb sign

Question 23

Complete AV septal defects

Answer 23

• Cleft in the anterior leaflet of the left AV valve
• Lateral rotation fo LV papillary muscles
• Attachment of the left and right AV valves at the same level at the cardiac crux
• Due to the absence of the AV septum, the LV inflow is shortened and the LV outflow is elongated (“goose-neck deformity”) creating a ratio of LV inlet to LV outlet <1.
• Due to the presence of a common AV valve, the aortic valve is no longer “wedged” between the tricuspid and mitral valves and is pushed anteriorly (“sprung”)

Question 24

Most common acquired characteristic from a sub pulmonary (supracristal) VSD?

Answer 24

Aortic insufficiency

Question 25

Most common VSD associated with coarctation

Answer 25

Perimembranous

Question 26

PDA Association

Answer 26

• Maternal Rubella
• Basically an AV fistula so wide pulse pressure
• Dynamic LV
• Machinery murmur
• Close with L sided enlargement (PA systolic pressure <50% systemic & PVR < 1/3 systemic)

Question 27

Pulmonary Stenosis Grade

Answer 27

Mild = Peak gdt < 36 mmHg (peak vel <3 m/sec)
Moderate = Peak gdt 36-64 mmHg (peak vel 3-4 m/sec)
Severe = Peak gdt 64mmHg (peak vel >4 m/sec), mean >35mm Hg

• RV failure late (>50 years) unless associated lesions

Question 28

Pulmonary Stenosis treatment

Answer 28

Balloon valvulotomy with moderate or severe PS and otherwise unexplained symptoms of HF, cyanosis from interatrial R to L shunt, &/or ex intolerance

Moderate or severe PS & otherwise unexplained symptoms of HF, cyanosis, &/or ex intolerance who are ineligible for, or who have failed balloon valvuloplasty, surgical repair is recommended

Question 29

Other imaging with coarctation

Answer 29

• Image entire aorta
• Image head to look for brain aneurysms