General Considerations

Question 1

Percentage of ACHD with complex cardiovascular anatomy

Answer 1

20%

Question 2

Transition from pediatric to adult CHD program should start at what age?

Answer 2

12 years

Question 3

Complete transfer from pediatric to adult CHD program should end at what age?

Answer 3

21 years of age

Question 4

Anomalous left coronary artery from the pulmonary artery (ALCAPA)

Answer 4

Bland - White - Garland Syndrome

Question 5

Pulmonary Hypertension with cyanosis due to right to left shunting

Answer 5

Eisenmenger Syndrome

Question 6

Septal defect resulting in direct left ventricle to right atrium shunt

Answer 6

Gerbode Effect

Question 7

Double inlet left ventricle with D-looped ventricles and normally related great vessels

Answer 7

Holmes Heart

Question 8

Coronary sinus septal defect in the presence of left superior vena cava

Answer 8

Raghib Defect

Question 9

Partial anomalous pulmonary venous connections of the right lower pulmonary vein to the IVC - RA junction, often accompanied by pulmonary artery hypoplasia and aortopulmonary collateral formation

Answer 9

Scimitar Syndrome

Question 10

Series of left- sided obstructive lesions

Answer 10

Shone Syndrome

Question 11

Form of double outlet right ventricle with D - malposed, side - by - side great vessels, sub - pulmonary VSD, hypoplastic aortic arch

Answer 11

Taussig - Bing Malformation

Question 12

Early palliative procedure for transposition of the great arteries, with the inferior vena cava directed to the left atrium via homograft

Answer 12

Baffes Procedure

Question 13

“Classic”—direct end to end anastomosis of subclavian artery to pulmonary artery

Answer 13

Blalock - Taussig - (-Thomas) Shunt

Question 14

“Modified”—tube graft from subclavian artery to pulmonary artery

Answer 14

Blalock - Taussig (-Thomas) Shunt

Question 15

Atriopulmonary anastomosis for single ventricle heart disease

Answer 15

Fontan or Fontan - Kreutzer

Question 16

Closed infundibular resection for relief of pulmonary stenosis

Answer 16

Brock Procedure

Question 17

Includes the right ventricle into the pulmonary circulation, was the unique modification for tricuspid atresia

Answer 17

Fontan - Björk Modification

Question 18

“Classic”—end to end anastomosis of superior vena cava to right pulmonary artery

Answer 18

Glenn Procedure

Question 19

“bidirectional”—end to side anastomosis of superior vena cava to right pulmonary artery

Answer 19

Glenn Procedure

Question 20

Bidirectional Glenn in context of interrupted inferior vena cava with azygos continuation to the superior vena cava

Answer 20

Kawashima Procedure

Question 21

Anterior translocation of the pulmonary arteries, so that both branch pulmonary arteries run anterior to the aorta. Most commonly used as part of the arterial switch operation

Answer 21

Lecompte Maneuver

Question 22

Atrial switch operations for transposition of the great arteries, with atrial baffling using native ATRIAL tissue to redirect systemic and pulmonary venous flow

Answer 22

Senning

Question 23

Atrial switch operations for transposition of the great arteries, with atrial baffling using native PERICARDIAL tissue to redirect systemic and pulmonary venous flow

Answer 23

Mustard Procedure

Question 24

In double outlet right ventricle, posterior translocation of the aortic root towards the left ventricle, with baffling of the left ventricle to the aorta in its new position

Answer 24

Nikaidoh Procedure

Question 25

Neonatal palliative procedure for hypoplastic left heart syndrome including aortic arch reconstruction with anastomosis of the native aorta to the pulmonary artery, which becomes the “neo-aorta,” as well as atrial septectomy and a modified BT shunt

Answer 25

Norwood Procedure

Question 26

Direct anastomosis of the left pulmonary artery to the descending aorta

Answer 26

Potts Shunt

Question 27

Intra-cardiac routing of the left ventricle to the aorta, which arose from the right ventricle. Usually accompanied by a right ventricle to pulmonary artery conduit.

Answer 27

Rastelli Procedure

Question 28

Intrapulmonary baffle of the left coronary artery performed for anomalous left coronary artery from the pulmonary artery

Answer 28

Takeuchi Repair

Question 29

Direct anastomosis of the right pulmonary artery to the ascending aorta

Answer 29

Waterston Shunt

Question 30

How many percent of patients with ACHD have abnormal pulmonary tests?

Answer 30

40%

Question 31

One of the leading causes of noncardiac death in ACHD patients

Answer 31

Pneumonia

Question 32

Specific pulmonary complication in patients living with Fontan Physiology

Answer 32

Plastic Bronchitis

Question 33

Hemoptysis may occur in upto _______ of ACHD patients with Eisenmenger Syndrome

Answer 33

1/3

Question 34

Pulmonary hypertension is found in up to ____% of ACHD patients and is strongly associated with increased morbidity and mortality.

Answer 34

10%

Question 35

Highest prevalence of impaired renal function.

Answer 35

Cyanotic ACHD

Question 36

Second leading cause of non cardiovascular death in ACHD patients

Answer 36

Cancer

Question 37

Malignancy associated with Fontan Physiology

Answer 37

Hepatocellular Carcinoma

Question 38

Championed the segmental approach for description of congenital anatomy

Answer 38

Drs. Stella and Richard Van Praagh

Question 39

In the normal heart the RV is connected to the PA by a ________________.

Answer 39

Complete Muscular Conus (Infundibulum)

Question 40

In the normal heart, the LV is connected to the aorta by _____________.

Answer 40

Aortic-mitral fibrous continuity (without a complete conus)

Question 41

The heart starts to form in the __________ weeks of gestation and is nearly fully formed by _________ weeks gestation.

Answer 41

3rd, 8th weeks

Question 42

Cardiac neural crest cells migrate into the developing heart in the __________ weeks and are essential for septation of the outflow, formation of the semilunar valves, and patterning of the aortic arches.

Answer 42

5th to 6th weeks

Question 43

Epidemiology of ACHD

Answer 43

Genetic or environmental cause can be identified in up to 30% of CHD cases. Single-gene disorders are found in 3% to 5% Gross chromosomal anomalies/aneuploidy in 8% to 10%, and Pathogenic copy number variants in up to 25% of CHD cases. Environmental causes are identifiable in 2% of CHD cases. The remainder of CHD is presumed to be multifactorial.

Question 44

The most common aneuploidy and is usually caused by trisomy 21. It is also the most common chromosome abnormality associated with CHD.

Answer 44

Down Syndrome

Question 45

Fifty percent of children born with Down syn-drome have CHD, most commonly defects in the ______________.

Answer 45

AV Canal

Question 46

Bile duct paucity, butterfly vertebrae, renal defects

Answer 46

Alagille Syndrome (JAG 1, Notch2)

Question 47

Coloboma, choanal atresia, genital hypoplasia, ear anomalies, hearing loss, developmental delay, growth retardation, intellectual disability

Answer 47

CHARGE (CHD7)

Question 48

Cleft palate, bifid uvula, velopharyngeal insufficiency, microcephaly, hypocalcemia, immune deficit, psychiatric disorder, learning disability

Answer 48

22q11.2DS (TBX1)

Question 49

Skeletal dysplasia, short limbs, polydactyly, short ribs, dysplastic nails, respiratory insufficiency

Answer 49

Ellis - van Creveld Syndrome (EVC, EVC2)

Question 50

Absent, hypoplastic, or triphalangeal thumbs; phocomelia; defects of radius; limb defects more prominent on left

Answer 50

Holt - Oram Syndrome (TBX5)

Question 51

Common Atrium

Answer 51

Ellis - van Creveld Syndrome (EVC, EVC2)

Question 52

Growth deficiency, wide palpebral fissures, large protuberant ears, fetal finger pads, intellectual disability, clinodactyly

Answer 52

Kabuki Syndrome (KMT2D, KDM6A)

Question 53

Short stature, hypertelorism, down-slanting palpebral fissures, ptosis, low posterior hairline, pectus deformity, bleeding disorder, chylothorax, cryptorchidism

Answer 53

Noonan Syndrome (PTPN 11)

Question 54

Vertebral anomalies, anal atresia, tracheoesophageal fistula, renal anomalies, radial dysplasia, thumb hypoplasia, single umbilical artery

Answer 54

VACTERL Association (unknown gene)

Question 55

Unusual facies, thick lips, strabismus, stellate iris pattern, intellectual disability

Answer 55

Williams - Beuren (7q11/23 deletion or ELN)

Question 56

In patients with TOF, ____________________ recorded on Holter monitoring predicts clinical VT.

Answer 56

asymptomatic nonsustained ventricular tachycardia (VT)

Question 57

________________ is a risk factor for clinical VT and sudden death in patients with repaired TOF

Answer 57

Inducible sustained VT

Question 58

Sinus node dysfunction is commonly encountered in patients with_____________ who have undergone a Mustard or Senning operation

Answer 58

D-loop TGA

Question 59

Heart block is common in patients with___________ as the AV node is superiorly and anteriorly displaced.

Answer 59

L-loop TGA

Question 60

The incidence of complete heart block in L-loop TGA is ______ % per year.

Answer 60

2%

Question 61

the most common tachyarrhythmia in CHD, accounting for 62% of atrial arrhythmias.

Answer 61

Interatrial re-entrant tachycardia (IART)

Question 62

What are the preferred antiarrhythmic in patients with complex CHD?

Answer 62

Sotalol and dofetilide

Question 63

the most common atrial arrhythmia in adults with CHD older than age 50

Answer 63

Atrial Fibrillation

Question 64

Most common cause of cardiac death in adults with CHD. Accounts for upto 40% of ACHD Mortality

Answer 64

Heart Failure

Question 65

Second most common cause of cardiac death in adults with CHD. Accounts for approximately 20% of death in patients with CHD, occuring at a rate of ~ 0.1% per patient year

Answer 65

Sudden Death

Question 66

Ascending aortic dilatation occurs in at least _______ % of patients with BAV

Answer 66

50%

Question 67

Low-risk patients can begin a training program at approximately _____ % maximal predicted heart rate at least three times per week (total-ing 150 minutes/week) and increase intensity or duration over time.

Answer 67

70%

Question 68

Criteria for Shunt Vascularity in Chest Radiograph

Answer 68

(1) uniformly distributed vascular markings with absence of the normal lower lobe vascular predominance; (2) a right descending pulmonary artery diameter that exceeds 17  mm; and (3) a pulmonary artery branch that is larger than its accompanying bronchus (best noted in the right parahilar area)

Question 69

Prominent vascularity is apparent only if the pulmonary-to-systemic flow ratio is greater than _____ to 1. As a rule, overt cardiac enlargement implies a shunt greater than ____ to 1

Answer 69

1.5:1, 2.5:1

Question 70

The atrial situs follows the abdominal situs in ____________ of cases

Answer 70

70% to 80%

Question 71

When both the aorta and inferior vena cava are on the same side of the spine, there is usually __________________.

Answer 71

abdominal and atrial right isomerism (two morphologic right atria)

Question 72

Suspected when the intrahepatic inferior vena cava is interrupted, with the presence of azygos continuation in the paravertebral gutter, on either the left or right side.

Answer 72

Left atrial isomerism (two morphologic left atria)