Adult Congenital

Question 1

Phlebotomy in Eisenmengers? When for (2)

Answer 1

Not unless evidence of:

1. Mod to Severe hyperviscosity symptoms
2. Pre-operatively for Hct >65%

Question 2

Cleft MV is associated with?

Part of “complete endocardial cushion defect? (4)

Answer 2

Primum ASD & inlet VSD & widened tricuspid commissure. NOTE: Partial endocardial cushion defect has (3) ASD, Cleft MV and widened TV commissure.

Question 3

4 elements of complete endocardial cushion defect? Partial? (3)

Answer 3

Complete: Primum ASD, cleft MV, inlet VSD & TV widened commissure.
Partial: ASD, cleft MV and TV widened commissure

Question 4

Ebstein’s (4) elements & association?

Answer 4

TV dysplastic and adherent to RV
 PFO or secundum ASD (50%)
 atrialized RV
 Pulmonic atresia or PS
 Associated with WPW

Question 5

Pulmonic stenosis: PA may be dilated on x-ray but pulmonary vascular will be?

Answer 5

Past the PV the blood flow is LESS so pulmonary vascular marking will be less

Question 6

Isolated valvular PS treatment for:
asymptomatic peak >/= 60mmHg
symptomatic >/= 50mmHg

Answer 6

Valvuloplasty preferred over surgery

Question 7

Atrial septal aneurysm (ASA) definition?

Answer 7

> 15mm excursion on echo

Question 8

Coarctation of aorta associated with?

Answer 8

Berry aneurysm in 10%

Question 9

Anomalous LM from the right cusp? Which is bad? Anterior or posterior to PA?

Answer 9

Anterior is benign.

Between PA AND Ao is bad.

Question 10

LM from PA - left to right shunt?

Answer 10

no

Question 11

ALCAPA?

Answer 11

Anomalous origin of the LM from the PA (ALCAPA)

Question 12

ALCAPA post repair monitoring?

Answer 12

Current guidelines recommend noninvasive testing for ischemia every 3-5 years after repair of ALCAPA

Question 13

Unique ECGs (3) related to congenital disease.
Ebstein’s
ASD
AV septal devects

Answer 13

Ebstein’s = WPW. A few patients with a secundum ASD have notching of the QRS (Crochetage). Patients with AV septal defects often have bifasicular block (left anterior fasicular block and RBBB)

Question 14

Initial Marfan’s imaging?

Answer 14

Initial and 6 months

Question 15

Serial Marfan’s imaging?

Answer 15

Yearly imaging is recommended for patients with Marfan syndrome if stability of the aortic diameter is documented. I
4.5 cm or greater, or if the aortic diameter shows significant growth from baseline, more frequent imaging should be considered.

Question 16

Initial Loeys -Dietz imaging?

Answer 16

Loeys-Dietz syndrome or a confirmed genetic mutation known to predispose to aortic aneurysms and aortic dissections (TGFBR1, TGFBR2, FBN1, ACTA2, or MYH11)
complete aortic imaging at initial diagnosis and 6 months thereafter to establish stability

Question 17

Serial Imaging Loeys-Dietz?

Answer 17

yearly MRI

cerebrovascular & pelvis

Question 18

Imaging Turner Syndrome?

Answer 18

imaging of the heart and aorta for evidence of bicuspid aortic valve, coarctation of the aorta, or dilatation of the ascending thoracic aorta.

Normal then repeat imaging should be performed ev- ery 5 to 10 years

Question 19

VO2 max in HOCM versus athletic heart?

Answer 19

A distinguishing feature during cardiopulmonary exercise testing is that conditioned athletes with physiologic hypertrophy can achieve maximum oxygen uptakes > 45 mL/kg/min (>110% predicted) whereas those with HCM typically cannot.

Question 20

HTN in pregnancy? What to use?

“Beta HC Mom”

Answer 20

Beta blockers (particularly labetalol)
hydralazine, and hydrochlorothiazide.
CCBs methydopa
Not effective in preventing preeclampsia.

Question 21

Risk of CAD by how much in DM?

Answer 21

approximately 2x

Question 22

Hormones for for CAD prevention– Class I or III?

Answer 22

Class III recommendation to starting or continuing estrogen plus progestin therapy for primary or secondary prevention of cardiovascular disease

Question 23

Post CABG enzymes elevation for MI? How high?

Answer 23

5x

Question 24

Noncardiac surgery – 6 risk factors?

Answer 24

high-risk type of surgery
 history of ischemic heart disease
 history of congestive heart failure (e.g., the S3   gallop described in the vignette for this question),    history of cerebrovascular disease
 preoperative treatment with insulin
preoperative serum creatinine > 2 mg/dL.

Question 25

Enlargement?

Answer 25

ASD the structure enlargements RA, RV and PA only. The LA, LV and aorta are normal.

VSD, the RA remains normal as does the aorta. Everything else is involved in the shunt pathway, so the RV, PA, LA and LV all enlarge.

Partial anomalous PV return affects the same structures as an ASD.

PDA - the PA, LA, LV and aorta proximal to the shunt all enlarge as they are in the shunt pathway. +’pulmonary hypertension or pulmonary valve regurgitation, the RV may also enlarge on occasion. Otherwise the RV and RA are normal