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Congenital Heart Disease > General Considerations > Flashcards

General Considerations Flashcards

1
Q

Percentage of ACHD with complex cardiovascular anatomy

A

20%

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2
Q

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

A

12 years

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3
Q

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

A

21 years of age

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4
Q

Anomalous left coronary artery from the pulmonary artery (ALCAPA)

A

Bland - White - Garland Syndrome

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5
Q

Pulmonary Hypertension with cyanosis due to right to left shunting

A

Eisenmenger Syndrome

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6
Q

Septal defect resulting in direct left ventricle to right atrium shunt

A

Gerbode Effect

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7
Q

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

A

Holmes Heart

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8
Q

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

A

Raghib Defect

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9
Q

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

A

Scimitar Syndrome

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10
Q

Series of left- sided obstructive lesions

A

Shone Syndrome

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11
Q

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

A

Taussig - Bing Malformation

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12
Q

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

A

Baffes Procedure

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13
Q

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

A

Blalock - Taussig - (-Thomas) Shunt

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14
Q

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

A

Blalock - Taussig (-Thomas) Shunt

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15
Q

Atriopulmonary anastomosis for single ventricle heart disease

A

Fontan or Fontan - Kreutzer

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16
Q

Closed infundibular resection for relief of pulmonary stenosis

A

Brock Procedure

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17
Q

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

A

Fontan - Björk Modification

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18
Q

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

A

Glenn Procedure

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19
Q

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

A

Glenn Procedure

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20
Q

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

A

Kawashima Procedure

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21
Q

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

A

Lecompte Maneuver

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22
Q

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

A

Senning

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23
Q

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

A

Mustard Procedure

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24
Q

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

A

Nikaidoh Procedure

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25
Q

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

A

Norwood Procedure

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26
Q

Direct anastomosis of the left pulmonary artery to the descending aorta

A

Potts Shunt

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27
Q

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.

A

Rastelli Procedure

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28
Q

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

A

Takeuchi Repair

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29
Q

Direct anastomosis of the right pulmonary artery to the ascending aorta

A

Waterston Shunt

30
Q

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

A

40%

31
Q

One of the leading causes of noncardiac death in ACHD patients

A

Pneumonia

32
Q

Specific pulmonary complication in patients living with Fontan Physiology

A

Plastic Bronchitis

33
Q

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

A

1/3

34
Q

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

A

10%

35
Q

Highest prevalence of impaired renal function.

A

Cyanotic ACHD

36
Q

Second leading cause of non cardiovascular death in ACHD patients

A

Cancer

37
Q

Malignancy associated with Fontan Physiology

A

Hepatocellular Carcinoma

38
Q

Championed the segmental approach for description of congenital anatomy

A

Drs. Stella and Richard Van Praagh

39
Q

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

A

Complete Muscular Conus (Infundibulum)

40
Q

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

A

Aortic-mitral fibrous continuity (without a complete conus)

41
Q

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

A

3rd, 8th weeks

42
Q

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.

A

5th to 6th weeks

43
Q

Epidemiology of ACHD

A

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.

44
Q

The most common aneuploidy and is usually caused by trisomy 21.

It is also the most common chromosome abnormality associated with CHD.

A

Down Syndrome

45
Q

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

A

AV Canal

46
Q

Bile duct paucity, butterfly vertebrae, renal defects

A

Alagille Syndrome (JAG 1, Notch2)

47
Q

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

A

CHARGE (CHD7)

48
Q

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

A

22q11.2DS (TBX1)

49
Q

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

A

Ellis - van Creveld Syndrome (EVC, EVC2)

50
Q

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

A

Holt - Oram Syndrome (TBX5)

51
Q

Common Atrium

A

Ellis - van Creveld Syndrome (EVC, EVC2)

52
Q

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

A

Kabuki Syndrome (KMT2D, KDM6A)

53
Q

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

A

Noonan Syndrome (PTPN 11)

54
Q

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

A

VACTERL Association (unknown gene)

55
Q

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

A

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

56
Q

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

A

asymptomatic nonsustained ventricular tachycardia (VT)

57
Q

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

A

Inducible sustained VT

58
Q

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

A

D-loop TGA

59
Q

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

A

L-loop TGA

60
Q

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

A

2%

61
Q

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

A

Interatrial re-entrant tachycardia (IART)

62
Q

What are the preferred antiarrhythmic in patients with complex CHD?

A

Sotalol and dofetilide

63
Q

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

A

Atrial Fibrillation

64
Q

Most common cause of cardiac death in adults with CHD.

Accounts for upto 40% of ACHD Mortality

A

Heart Failure

65
Q

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

A

Sudden Death

66
Q

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

A

50%

67
Q

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.

A

70%

68
Q

Criteria for Shunt Vascularity in Chest Radiograph

A

(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)

69
Q

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

A

1.5:1, 2.5:1

70
Q

The atrial situs follows the abdominal situs in ____________ of cases

A

70% to 80%

71
Q

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

A

abdominal and atrial right isomerism (two morphologic right atria)

72
Q

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.

A

Left atrial isomerism (two morphologic left atria)

Congenital Heart Disease (3 decks)

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