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Invasive Cardiology Board Review > Adult Congenital Heart Disease > Flashcards

Adult Congenital Heart Disease Flashcards

1
Q

What is the typical presentation of ASDs in adults?

A

Shortness of breath or palpitations, often discovered incidentally

ASDs can also be found during workup for stroke due to paradoxical embolization.

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2
Q

What is a ‘positive’ step-up in oxygen saturation at the atrial level?

A

≥7% (absolute difference in mean saturation values)

A ‘positive’ step-up at ventricular or pulmonary arterial levels must be ≥5%.

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3
Q

What method is used to calculate pulmonary blood flow assuming a specific oxygen consumption?

A

The Fick method

Assumes oxygen consumption of the lungs to be 125 mL/min/m².

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4
Q

Which systemic venous saturation is usually higher, IVC or SVC?

A

IVC saturation is typically higher than SVC saturation

This is because the kidney receives 25% of the cardiac output and consumes less oxygen.

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5
Q

What formula is used to estimate mixed venous saturation?

A

The Flamm formula

This formula is used in situations where direct measurements are not available.

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6
Q

When is ASD closure indicated according to recent guidelines?

A

Evidence of RA or RV enlargement, paradoxical embolism, or platypnea–orthodeoxia

Closure may also be considered for net left-to-right shunting with pulmonary arterial pressure <2/3 systemic pressure.

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7
Q

What is the significance of a QP:QS ratio of >1.5:1?

A

It has traditionally been used to define a shunt that should be closed

This definition may not be the best for adults with long-standing right ventricular volume overload.

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8
Q

What is a common complication associated with pulmonary arteriovenous malformations?

A

Stroke, brain abscess, life-threatening hemoptysis or hemothorax, and hypoxemic respiratory failure

Pulmonary arteriovenous malformations can cause serious complications and are often congenital.

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9
Q

What should be considered if dilation of the coronary sinus is observed?

A

The possibility of a persistent left-sided vena cava

This is typically normal physiology with no clinical manifestations.

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10
Q

What are the signs and symptoms supporting a diagnosis of PDA?

A

Dyspnea on exertion, widened pulse pressure, left subclavicular murmur

The murmur is classically described as a continuous ‘machinery’ murmur.

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11
Q

What is Eisenmenger’s syndrome?

A

A condition where pulmonary arterial pressure exceeds systemic pressure due to chronic left-to-right shunting

It leads to a reversal of shunt flow and consequent cyanosis.

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12
Q

What is the recommended follow-up for patients with small PDA and no left heart volume overload?

A

Clinical follow-up every 3 to 5 years

Endocarditis prophylaxis is not recommended for asymptomatic patients with unrepaired PDA.

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13
Q

What is the ideal timing for surgical correction of defects resulting in significant pulmonary overcirculation?

A

Before 2 years of life for VSDs and before 6 months for AV canal defects and conotruncal abnormalities

Early correction is critical to prevent irreversible pulmonary vascular disease.

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14
Q

What is the prognosis for patients with Eisenmenger’s syndrome compared to those with idiopathic pulmonary arterial hypertension?

A

77% survival vs. 35% survival at 3 years, untreated

Despite a better prognosis, it remains a serious condition.

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15
Q

What is the gold standard of treatment for pulmonary arteriovenous malformations?

A

Embolotherapy

This is the preferred method when possible to manage these malformations.

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16
Q

What does the Fick method calculate in the context of cardiac shunts?

A

Pulmonary blood flow, systemic blood flow, and shunt flow

It is essential for determining the hemodynamics of congenital heart defects.

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17
Q

What is heart-lung transplant used for?

A

End-stage Eisenmenger’s syndrome treatment

Eisenmenger’s syndrome is a complication of congenital heart disease that leads to pulmonary vascular disease.

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18
Q

What is the risk of late surgical correction with an arterial switch procedure after pulmonary vascular disease has developed?

A

Significant mortality risk

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19
Q

Does pulmonary artery banding alter the disease course in pulmonary vascular disease?

A

No

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20
Q

How does administration of 100% oxygen affect systemic oxygen saturation in patients with right-to-left shunting?

A

Will not significantly improve

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21
Q

What has long-term oxygen therapy in Eisenmenger’s syndrome been shown to improve?

A

Nothing (symptoms, functional capacity, or survival)

Sandoval J, et al. Am J Respir Crit Care Med 2001;164(9):1682–1687.

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22
Q

What is the most common congenital anomaly of the tricuspid valve?

A

Ebstein anomaly

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23
Q

In Ebstein anomaly, which leaflet of the tricuspid valve arises from the normal position?

A

Anterior leaflet

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24
Q

What characterizes the right ventricle in Ebstein anomaly?

A

Divided into two chambers: atrialized RV and functional RV of variable size

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25
Q

What is a common communication present in Ebstein anomaly?

A

Atrial level communication (PFO or ASD)

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26
Q

What mechanisms promote atrial level right-to-left shunting in Ebstein anomaly?

A
  • Right ventricular outflow tract obstruction
  • Poor RV filling
  • Tricuspid valve regurgitation
  • RV failure
  • Increased pulmonary vascular resistance
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27
Q

How do patients with right-to-left shunting in Ebstein anomaly experience systemic venous congestion compared to those with an intact septum?

A

Do not easily develop systemic venous congestion

28
Q

What is the management approach for asymptomatic patients with Ebstein anomaly?

A

Periodic monitoring only

29
Q

What may patients with exertional cyanosis secondary to atrial level shunting benefit from?

A

Percutaneous device closure

30
Q

What does temporary balloon occlusion of a defect in Ebstein anomaly lead to?

A

Elevation of RA pressure and a drop in cardiac output

31
Q

What are common surgical interventions for severe symptoms in Ebstein anomaly?

A
  • Tricuspid valve repair or replacement
  • Shunt closure
  • Ventricular plication
  • Atrial reduction
  • Antiarrhythmic surgery
32
Q

What recent evidence suggests can improve the prognosis of surgical management in Ebstein anomaly?

A

Aggressive preoperative treatment

33
Q

What characterizes Tetralogy of Fallot (TOF)?

A
  • Pulmonic stenosis
  • VSD
  • Right ventricular hypertrophy
  • Overriding aortic valve
34
Q

What was historically done prior to performing a complete repair of TOF?

A

Palliative Blalock–Taussig shunts were employed

35
Q

What is a common late complication after surgical repair of TOF?

A

Pulmonary insufficiency

36
Q

What is a frequent complication encountered in adults with TOF, especially those with pulmonary atresia?

A

Formation of aortopulmonary collaterals

37
Q

What is the typical origin of coronary artery fistulae?

A

Persistence of sinusoids from early embryogenesis

38
Q

What percentage of coronary fistulae originate from the RCA?

A

55%

39
Q

What can coronary fistulae lead to besides being small and usually asymptomatic?

A

Myocardial ischemia distal to the fistula

40
Q

What is transposition of the great arteries characterized by?

A

Two separate circulations requiring a shunt for infantile survival

41
Q

What is a common complication after the Sening and Mustard procedures for transposition of the great arteries?

A

Failure of the systemic (right) ventricle

42
Q

What is Scimitar syndrome characterized by?

A
  • Hypoplasia of the right lung
  • Anomalous right pulmonary venous drainage to the IVC
  • Aortopulmonary collateral flow
43
Q

What is the most common congenital coronary anomaly?

A

Origin of the LCx from the RCA

44
Q

What is the peak-to-peak pressure gradient mentioned in the text?

A

51 mm Hg

45
Q

What factors contribute to the pathophysiology of hypertension in patients with coarctation of the aorta?

A
  • Activation of the renin–angiotensin–aldosterone system
  • Decreased renal blood flow
46
Q

What is the survival rate of patients with unoperated coarctation of the aorta?

A

35 years

47
Q

What is a common indication for balloon angioplasty and/or stent placement in coarctation patients?

A

Gradient of 20 mm Hg or more

48
Q

What is depicted in Figure A32-18?

A

A right anterior oblique (RAO) angiographic image of an anomalous left main coronary artery originating from the right coronary cusp

49
Q

What is the association of the anomalous left main coronary artery?

A

Increased risk of sudden death in children and young adults

50
Q

What is the peak-to-peak pressure gradient threshold for valvuloplasty in asymptomatic patients with a doming pulmonic valve?

A

> 40 mm Hg

51
Q

What is the recommended management for asymptomatic patients with a peak-to-peak gradient <30 mm Hg?

A

Safe observation

52
Q

What are the surgical indications for patients with valvular pulmonic stenosis?

A
  • Right ventricular systolic pressure >80 mm Hg
  • Dysplastic valve morphology
  • Severe pulmonic stenosis with severe pulmonary regurgitation
  • Associated hypoplastic pulmonary anulus
53
Q

What is the so-called suicide RV?

A

Stenosis at the level of the valve is relieved, causing dynamic obstruction along the infundibulum when right ventricular pressures exceed 100 mm Hg

54
Q

What is the estimated death rate during pulmonary balloon valvuloplasty?

A

0.24%

55
Q

What are some acute complications of pulmonary balloon valvuloplasty?

A
  • Ventricular ectopy
  • AV node block
  • Pulmonary regurgitation
56
Q

What anatomical and functional abnormalities are associated with the heart after repair of Tetralogy of Fallot (TOF)?

A
  • Pulmonary valve insufficiency
  • Conduction delay (right bundle-branch block)
  • Right ventricular dilation
  • Increased pulmonary insufficiency
  • Ventricular dysfunction
57
Q

What is the median lifespan of a bioprosthetic valve?

A

10 years

58
Q

What are the indications for valve replacement in patients with repaired TOF?

A
  • Symptoms of exercise intolerance or heart failure
  • QRS duration >180 ms
  • QRS prolongation >3.5 ms/year
  • Sustained arrhythmia
  • Right ventricular end-systolic volume >85 mL/m2
  • Right ventricular end-diastolic volume >170 mL/m2
59
Q

What characterizes single ventricle physiology?

A

One functional ventricle must generate sufficient cardiac output for both systemic and pulmonary circulations

60
Q

What is the purpose of a Glenn procedure?

A

To route superior venous circulation to the pulmonary arterial system

61
Q

What are venovenous collaterals and their risk?

A

Collaterals that feed blood to pulmonary veins, bypassing the lungs, and carry a risk for paradoxical embolization

62
Q

What is the recommended technique for evaluating subaortic stenosis?

A

Echocardiography, particularly transesophageal

63
Q

What is the peak instantaneous gradient that indicates surgical intervention for subaortic stenosis?

A

50 mm Hg

64
Q

Who should receive prophylaxis for subacute bacterial endocarditis (SBE)?

A
  • Patients with implanted prosthetic materials
  • History of infective endocarditis
  • Unrepaired cyanotic congenital heart disease
  • Repaired congenital heart disease with residual defects
  • Completely repaired congenital heart disease with prosthetic material for 6 months following the procedure
65
Q

What are the indications for intervention in pulmonary artery stenosis?

A
  • Right ventricular systolic pressure elevation (at least 50% of systemic pressure)
  • Translesion gradient of at least 30 mm Hg
  • Flow discrepancy to the distal pulmonary vascular bed
  • At least 50% luminal narrowing

Invasive Cardiology Board Review (42 decks)

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