B P9 C83 Catheter-Based Treatment of Congenital Heart Disease in Adults

Question 1

Congenital valvular pulmonary stenosis accounts for ____% of all congenital heart disease.

Answer 1

5-10%

Question 2

Before pulmonary valvuloplasty is performed, a complete right heart catheterization should be performed, followed by _____ to profile the right ventricular outflow tract (RVOT)

Answer 2

Right ventricular (RV) angiography

Question 3

Angio- graphic measurements of the pulmonary annulus allows for the selection of the appropriately sized balloon, which is approximately _____% of the measured pulmonary annulus

Answer 3

120%

Question 4

After dilation of the pulmonary valve, repeat angiography should be performed to rule out _____

Pulmonary regurgitation is best assessed on _____.

Answer 4

Vascular injury

Post-procedure echocardiography

Question 5

The most common complication of pulmonary valvuloplasty is _____.

Answer 5

Pulmonary regurgitation (<10% with 2+ or greater pulmonary regurgitation), which is usually well tolerated

Question 6

There are currently two available valve systems approved by the Food and Drug Administration (FDA) for transcatheter pulmonary valve replacement (TPVR):

Answer 6

Melody Transcatheter Pulmonary Valve (Medtronic, Inc., Minneapolis)

SAPIEN XT Pulmonic Valve (Edwards Lifesciences, Irvine, CA)

Question 7

The Melody valve is composed of a valved segment of _____.

Answer 7

Bovine internal jugular vein that is fixed with glutaraldehyde and then sutured to a platinum-iridium stent frame

Question 8

_____ is routinely performed prior to valve deployment to prevent stent fractures of the Melody valve frame.

Answer 8

RVOT stenting

Question 9

There are currently two sizes of Melody valves available: _____ mm, with the delivery system available in three sizes: 18, 20, and 22 mm

Answer 9

20 and 22mm

Question 10

The Edwards SAPIEN transcatheter heart valve (Irvine, CA) was originally designed for placement in the _____ position, but was found to have high success rates when placed in the pulmonary valve position,with the first successful pulmonary implantation in 2006.

Answer 10

Aortic

Question 11

This device is composed of bovine pericardium of three equal-sized leaflets that are hand-sewn to a cobalt chromium balloon-expandable stent with a polyethylene terephthalate fabric cuff.

Answer 11

The Edwards SAPIEN transcatheter heart valve (Irvine, CA)

Question 12

Additionally,the _____ has a new outer polyethylene terephthalate skirt which decreases the incidence of paravalvular leak.

Answer 12

SAPIEN S3

Question 13

The SAPIEN valve comes in larger diameters of _____mm. These larger valve sizes allow for per- cutaneous intervention in patients with large native RVOTs, transannular patches, and large RV to PA conduits.

Answer 13

23, 26, and 29 mm

Question 14

Valve selection is influenced by:

Answer 14

(1) Patient cohort (conduit versus transannular patch)
(2) Ease of use of the delivery system (stiffness and lack of flexibility of the delivery system for the Sapien)
(3) Operator experience/preference

Question 15

Long-term complications of pulmonary valve systems include:

Answer 15

Stent frame fracture (Melody valve)
Valve dysfunction
Endocarditis

Question 16

There are currently no stents approved by the FDA for use in pulmonary arteries; however, _____ have been used and have shown good radial strength, low profiles, and achievable diameters.

Answer 16

(1) Palmaz Genesis stents (Cordis, Milpitas, CA)
(2) EV3 family of stents (Covidien/ Medtronic, Minneapolis, MN)

Question 17

Although CoA can present as an isolated lesion, it is also commonly found in genetic syndromes including:

Answer 17

Turner and Williams syndrome

Question 18

The most common cardiovascular malformation associated with CoA is _____.

Answer 18

Bicuspid Aortic Valve (BAV)

Question 19

_____ is now considered a mainstay in the management of adults with native CoA and recoarctation.

Answer 19

Transcatheter approach with stenting of the aorta

Question 20

The covered _____ stent is approved by the FDA for use in coarctation, but other stents are often used off-label.The diameter of the implanted balloon should not be larger than that of the surrounding aorta or _____ times the narrowest dimension.

Answer 20

Cheatham pulmonary (CP) stent (NuMED, Inc., Hopkinton, NY)

3.5x

Question 21

_____ are the third most common form of congenital heart disease.

Answer 21

Atrial septal defects (ASDs)

Question 22

If the septal rim is deficient (<___ mm in contiguous zones), stable positioning will be more difficult to achieve and at times may not be possible.

Answer 22

<5mm

Question 23

In contrast to the ASO, the _____ does not have a waist and is therefore not self-centering. Its primary benefit is that it can be placed in a small central defect and also covers numerous satellite defects

Answer 23

Cribriform Device

Question 24

The _____ has been approved by the FDA for PFO closure in the context of cryptogenic stroke

Answer 24

Amplatzer PFO occluder

Question 25

The ASO device is filled with interwoven _____ to facilitate platelet aggregation and endothelialization.

Answer 25

Dacron polyester fibers

Question 26

Device sizing for the ASO should be based on the stop flow method of balloon sizing. In order to decrease the risk of device erosion, over inflation of the balloon should be avoided (not >___ the static echocardiographic dimension)

Answer 26

1.5x

Question 27

Defects with a deficient _____ rim (<5 mm) are considered higher risk for an erosion.

Answer 27

Retroaortic Rim

Question 28

The _____ clinical trial (evaluating PFO closure with the Amplazter PFO Occluder) showed that among adults with a history of a cryptogenic ischemic stroke, closure of a PFO was associated with a lower rate of recurrent ischemic strokes than medical therapy alone during extended follow-up.

Answer 28

RESPECT Trial

Question 29

GORE redesigned its septal occluder system which is now marketed as the _____ .The new device consists of a five-wire nitinol frame, which adds radial strength and improves structural integrity, and it is covered with the same expanded polytetrafluoroethylene (ePTFE) membrane as the original Helex device

Answer 29

GORE Cardioform Septal Occluder (GSO) (W.L. Gore and Associates, Flagstaff,AZ)

Question 30

Owing to its non–self-centering design, the GSO can only close defects up to __ mm in diameter.

Answer 30

18 mm

Question 31

The principal modification from the original GSO is its anatomically adaptable “______” which expands to conform to the ASD size and shape, allowing different devices to treat a range of ASD diameters

Answer 31

“intra‐disc occluder”

Question 32

Available sizes for GORE devices:

Answer 32

The available sizes are 27, 32, 37, 44, and 48 mm and are designed to treat defects ranging from 8 to 35 mm

Question 33

The Gore _____ clinical study was a prospective single arm registry that evaluated the safety and efficacy of the GCA device. The study showed excellent technical success with 100% closure rate and low adverse event rates

Answer 33

ASSURED clinical study

Question 34

The _____ study determined safety and efficacy of PFO closure with the GORE CARDIO- FORM Septal Occluder or GORE HELEX Septal Occluder plus antiplatelet medical management compared to antiplatelet medical management alone in patients with a PFO and history of cryptogenic stroke. The risk of subsequent ischemic stroke was lower among those assigned to PFO closure combined with antiplatelet therapy than those assigned to anti- platelet therapy alone

Answer 34

REDUCE study

Question 35

The ____ ASD is a congenital abnormality that is caused by a deficiency of the common wall between the superior vena cava (SVC) and the right sided pulmonary veins

Answer 35

Superior sinus venosus ASD

Question 36

_____ are the most common congenital heart defects and can range in size from tiny pinholes to near absence of the septum

Answer 36

Ventricular septal defects (VSDs)

Question 37

VSDs can be an isolated finding or associated with other complex congenital heart diseases, primarily conotruncal defects (e.g.,_____).

Answer 37

ToF DORV ToGA

Question 38

Catheter-based device closure of Perimembranous VSD remains controversial because of the associated risk of _____.

Answer 38

Heart block

Question 39

Inlet VSDs are not amenable to transcatheter techniques because _____.

Answer 39

There is no circumferential tissue for secured placement of device

Question 40

The _____ PDA. occluder device is made of a nitinol wire mesh packed with Dacron polyester fabric to facilitate platelet aggregation and endothelialization.

Answer 40

ADO-I

Question 41

The second-generation ADO-II has symmetric retention skirts, which allow it to be placed in an antegrade or a retrograde fashion. The ADO-II is not packed with polyester fibers because the _____ than in ADO-I.

Answer 41

Nitinol wire weave is tighter

Question 42

In patients with long, tubular ducts, a _____ may be the optimal occlusion device.

Answer 42

Vascular plug

Question 43

The AVP-II has a wide assortment of sizes (_____ mm).The AVP-IV has fewer available sizes (_____ mm) and is slightly longer than AVP-II, but it offers an even lower profile, to easily navigate tortuous anatomy.

Answer 43

AVP-II: 3 to 22mm AVP-IV: 4 to 8 mm

Question 45

The ____ has a single nitinol wire coil, which can be wound in a funnel shape when it is advanced from the catheter. (PDA)

Answer 45

Nit-Occlud device (PFM Medical, Carlsbad, CA)

Question 46

Device closure has become the first choice for secundum defect closure, when feasible, based on the morphology (includes stretched diameter

Answer 46

Retroartic (except ito sa rims) - 2mm minimum IVC (most important) SVC Posterior rim AV rim