The short view of pulmonary artery (PA) branching and continuous-wave Doppler signal through the area of abnormal color flow in Figure 17.15 shows which of the following?

1. ASD
   
2. VSD
   
3. PDA
   
4. Coarctation of aorta

A 26-year-old patient is referred to you for a murmur. A transthoracic echocardiogram (TTE) is performed which fails to show any valvular pathology or a shunt lesion. The TTE is remarkable for dilatation of right atrium and right ventricle. What would the next step be?

1. Reassure the patient; no further test is needed
   
2. Cardiac catheterization
   
3. Transesophageal echocardiogram (TEE)
   
4. Cardiac magnetic resonance imaging (MRI)

A sinus venosus ASD is commonly associated with which of the following?

1. Cleft mitral valve
   
2. Partial anomalous pulmonary venous drainage
   
3. Persistent left superior vena cava (SVC)
   
4. Goose-neck deformity

In the patient mentioned in Question 17.16, a TEE was performed which showed a sinus venosus ASD with a partial anomalous venous return of the right upper pulmonary vein. What is the next step in management?

1. Cardiac catheterization to evaluate Qp/Qs
   
2. Cardiac MRI
   
3. Cardiac computed tomography (CT)
   
4. Consultation with a CT surgeon
   
5. Referral for percutaneous closure of the ASD

Secundum ASDs make up what proportion of ASDs?

1. 90%
   
2. 25%
   
3. 80%
   
4. 75%

A primum ASD is associated commonly with which of the following?

1. Left axis deviation on surface electrocardiogram (ECG)
   
2. Cleft of the anterior mitral leaflet
   
3. Left axis deviation and cleft mitral leaflet
   
4. Persistent left SVC

A patient with hypertension is diagnosed with coarctation of the aorta. What is the most common associated finding one should look for?

1. Pulmonary valve stenosis
   
2. Bicuspid aortic valve
   
3. Cleft mitral valve
   
4. Ebstein’s anomaly

A patient is diagnosed with a PDA. The peak systolic gradient across the defect is 105 mmHg. No other abnormality is noted on the echocardiogram. The BP recorded at the time of the echocardiogram is 130/77 mmHg. Based on the data given, what does the patient have?

1. Suprasystemic RV pressure
   
2. Normal RV pressure.
   
3. Systemic RV pressure
   
4. Cannot tell

Which of the following does TOF not include?

1. Overriding of the aorta
   
2. RV hypertrophy
   
3. PS
   
4. VSD
   
5. ASD

Which of the following is an anomaly that should be sought out in a patient with TOF being referred for surgical repair?

1. Anomalous origin of left anterior descending (LAD) artery from right coronary artery (RCA)
   
2. Anomalous RCA from LAD
   
3. Anomalous left circumflex artery from RCA
   
4. None of the above

A patient with a history of repaired TOF is referred to you for complaints of fatigue and lack of exercise tolerance. Her echocardiogram shows moderate RV enlargement with mild hypokinesis. What is the probable cause of her right-sided enlargement?

1. Severe tricuspid regurgitation (TR)
   
2. Severe pulmonary valve regurgitation
   
3. Severe mitral regurgitation
   
4. Severe aortic regurgitation

What is the treatment of choice for the correction of dextro (D)-transposition of great arteries (TGA)?

1. Senning procedure
   
2. Mustard procedure
   
3. Jatene arterial switch
   
4. Rastelli procedure

What is the position of the aorta in D-TGA?

1. Central
   
2. Anterior and rightward
   
3. Anterior and leftward
   
4. Posterior

What is the position of the aorta in levo (L)-TGA?

1. Anterior and rightward
   
2. Anterior and to the left
   
3. Central
   
4. Posterior

In what percentage of patients with L-TGA is complete heart block seen?

1. 90%
   
2. 30%
   
3. 50%
   
4. 10%

Ebstein’s anomaly can be seen in up to what proportion of patients with L-TGA?

1. 100%
   
2. 90%
   
3. 80%
   
4. 25%

The classic Blalock–Taussig shunt is a connection between which of the following?

1. Ascending aorta to PA
   
2. Subclavian artery to PA
   
3. Descending aorta to left PA
   
4. SVC to right PA

What is a Rastelli repair?

1. Closure of VSD and placement of right ventricle–PA conduit
   
2. Balloon atrial septostomy
   
3. Repair of VSD
   
4. Repair of ASD

The Fontan operation is used to repair which of the following defects?

1. Hypoplastic left heart syndrome
   
2. Triscuspid or mitral atresia
   
3. Hypoplastic right heart syndrome
   
4. Double inlet single ventricle
   
5. None of the above
   
6. All of the above

Right aortic arch is associated with which of the following?

1. TOF
   
2. Truncus arteriosus
   
3. Pulmonary atresia
   
4. None of the above
   
5. All of the above

A patient with a history of a Fontan operation presents with complaints of edema. On examination he has ascites, and his serum chemistry is significant for a low albumin level. What does this patient have?

1. Celiac disease
   
2. Pericardial constriction
   
3. Renal failure
   
4. Protein-losing enteropathy

Which of the following is/are a current class I indication regarding surgery for anomalous coronary arteries?

1. An anomalous left main coronary artery coursing between the aorta and PA
   
2. Ischemia caused by coronary compression (when coursing between the great arteries or has an intramural course)
   
3. An anomalous RCA between aorta and PA causing ischemia
   
4. All of the above
   
5. None of the above

Which of the following is not a class I indication regarding congenital heart disease in an adult >40 years of age?

1. Atrial level shunts with right ventricle enlargement and without pulmonary arterial hypertension (PAH) are recommended closure to prevent right ventricle failure, improve exercise capacity, and decrease atrial arrhythmia
   
2. Intervention is recommended for coarctation with obstruction for palliation of hypertension
   
3. Complex congenital heart disease with de novo presentation should receive comprehensive care at an adult congenital heart disease (ACHD) center with multidisciplinary input
   
4. In adults with a new presentation of a simple shunt or valve lesion and no hemodynamic compromise, evaluation by a general cardiologist in consultation with an ACHD cardiologist is reasonable
   
5. Patients with newly diagnosed coronary artery anomalies should be evaluated by an ACHD team with expertise in imaging, coronary artery disease management, intervention, and surgical revascularization

Regarding hypertension and coarctation of aorta, which of the following is the true statement?

1. The prevalence of hypertension is higher with later repair
   
2. The prevalence of hypertension is lower regardless of age of repair
   
3. The prevalence of hypertension is similar to people with no coarctation
   
4. Hypertension is not a problem after repair

Regarding the use of angiotensin-converting-enzyme (ACE) inhibitors in coarctation of the aorta, which of the following is the true statement?

1. ACE inhibitors have no known side effect in patients with coarctation of aorta
   
2. ACE inhibitors have been reported to precipitate acute renal failure in the setting of coarctation
   
3. Neither A nor B
   
4. Both A and B

Regarding screening for diabetes in ACHD, which of the following is the true statement?

1. Screening for diabetes mellitus should be undertaken in patients >40 years, body mass index >25 kg/m² with or without risk factors
   
2. Appropriate screening can include fasting glucose, hemoglobin A1C, or 2-h 75 g oral glucose tolerance test
   
3. If tests are normal, repeat testing at 3-year intervals
   
4. All of the above
   
5. None of the above

Regarding arrhythmias in ACHD, the following are true except:

1. Wolff–Parkinson–White syndrome and accessory-pathway-mediated tachycardia are associated with Ebstein anomaly
   
2. The most common arrhythmia facing older adults with congenital heart disease is intra-atrial reentrant tachycardia (IART)
   
3. The highest incidence of IART is seen in those who have undergone Mustard or Senning repair for D-TGA or the Fontan procedure for single-ventricle physiology
   
4. In the older adults, ventricular tachycardia (VT) is seen in patients with repaired TOF
   
5. All of the above
   
6. None of the above

Which of the following are true regarding pulmonary disease in ACHD?

1. Clinicians should have a low threshold for assessing patents with ACHD for PAH, with echocardiogram and hemodynamic cardiac catheterization
   
2. Patients with Eisenmenger’s should be followed up closely by an ACHD specialist
   
3. Treatment of PAH in the setting of ACHD with pulmonary vasodilator drugs can be useful and may lead to functional improvement
   
4. It is reasonable to consider serial evaluations of lung function in all adults with CHD
   
5. All of the above

Which of the following are true regarding recommendations for liver disease in ACHD?

1. Serial evaluation of liver function should be performed in all patients with a previous history of Fontan palliation
   
2. Patients with palliation for CHD prior to 1992 should be screened for hepatitis C
   
3. Gallstones necessitating cholecystectomy are seen with increasing frequency in ACHD patients
   
4. All of the above
   
5. None of the above

Which of the following are not true regarding renal disease in ACHD?

1. Renal function should be routinely assessed in all patients with moderate to complex CHD
   
2. In the presence of renal dysfunction, all effort must be made to minimize additional renal injury
   
3. Novel biomarkers such as urinary interleukin 18 and neutrophil gelatinase-associated lipocalin may be predictive of acute kidney injury
   
4. Renal dysfunction is not a poor prognostic marker in patients with ACHD

Which of the following is not a class I recommendation regarding diagnostic testing in an ACHD patient?

1. Echocardiography in the ACHD patient should be interpreted by physicians with expertise in both acquired and congenital heart disease
   
2. Echocardiography reporting should include both anatomic diagnoses and quantitative assessment of chambers, valves, and great vessels in a format accessible to physicians caring for ACHD patients
   
3. Diagnostic cardiac catheterization in the older adult with CHD should use a team approach that includes interventionalists skilled in the evaluation of the physiology associated with CHD, as well as being skilled in selective coronary angiography.
   
4. Sixty-four detector row or higher computed tomography angiography can be useful in lieu of invasive coronary angiography to exclude important coronary artery disease when the pretest probability is low to intermediate.

TOF may be associated with which of the following genetic defects?

1. Point mutation in major histocompatibility complex gene
   
2. 22q11 deletion
   
3. 9–22 translocation
   
4. None of the above

In a patient who had complete repair of TOF, which of the following sequelae may exist?

1. PR or pulmonary stenosis
   
2. Branch PA stenosis
   
3. VSD
   
4. Aortic regurgitation
   
5. All of the above.

In a 19-year-old patient with severe asymptomatic PR post TOF repair, what would be the best monitoring strategy in addition to symptoms and heart failure?

1. Serial TTE with monitoring of right ventricle diameter
   
2. Annual cardiac CT scan for right ventricle volume and ejection fraction (EF)
   
3. Annual cardiac MRI for right ventricle volume and EF
   
4. Clinical follow-up alone

What are the indications for redo surgery in a TOF patient with prior repair?

1. Severe symptomatic PR or severe PR with RV dysfunction
   
2. 
   

   Valvular or subvalvular PS with a gradient of >50 mmHg or RVOT obstruction with RV dysfunction

   
   
3. Residual VSD with a shunt ratio of >1.5
   
4. Severe aortic regurgitation with LVEF <50%
   
5. All of the above
   
6. None of the above.

The electrical abnormalities post TOF repair include which of the following?

1. VT and sudden death
   
2. Atrial flutter or fibrillation
   
3. Right bundle branch block
   
4. AV block
   
5. All of the above
   
6. None of the above

Which of the following are predictors of sudden death post TOF repair?

1. Severe PR
   
2. RV dilation or dysfunction
   
3. QRS duration of >180 ms
   
4. Nonsustained VT
   
5. Syncope
   
6. All of the above