Mock Royal College Written Exam

Pediatric Cardiology TRP 2021

8 questions

Total time: 60 minutes

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1. 15-year old boy with systemic hypertension presents for screening. Echocardiogram shows aortopathy.

a. What are FOUR possible differential diagnoses associated with aortopathy in a child, aside from Marfan’s.

Other: Idiopathic ascending aorta dilation, other autosomal dominant aortopathies (ACTA2 mutation), Turner syndrome, TOF and other CHD, trauma, severe hypertension, atherosclerosis, drugs

b. What are FIVE non-cardiac clinical features of Marfans syndrome?

1. Ectopia Lentis

2. Pectus Excavatum or carinatum of moderate severity

3. Pes Planus

4. Scoliosis (>20 degrees)

5. Spondylolisthesis

Other: Positive wrist or thumb sign, joint hypermobility, armspan-height ratio >1,05m diminished extension elbows (<170 degrees), high arched palate, spontaneous pneumothorax, apical blebs, striae atrophicae, recurrent/incisional hernia, fhx of FBN1 mutation or meets diagnosis for Marfan

c. What is ONE gene mutation typically associated with Marfans syndrome?

1. FBN1

d. List FOUR other cardiac manifestations for Marfans syndrome, aside from aortopathy.

1. Mitral valve prolapse/regurgitation

2. Calcification of the mitral valve <40 years

3. Dilation of pulmonary artery

4. Dilation or dissection of ascending/descending aorta (<50 years old)

Other (not part of diagnostic criteria): TV prolapse, LV dysfunction, arrhythmias, coronary artery aneurysms

1. Ehlers-Danlos Syndrome (vascular type IV)

2. Bicuspid aortic valve

3. Takayasu Arteritis

4. Unrepaired coarctation of the aorta

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e. List THREE indications for intervention for aortopathy in Marfan’s syndrome in an older teenager.

1. Aortic root or ascending aorta dilation >44-50mm

2.Symptomatic (pain consistent with rupture, compress adjacent organs esp trachea, esophagus, left mainstem bronchus, AI with ascending aortic aneurysm)

3.Growth of >0.5cm/y

Other: aortic dissection; root/ascending aorta is >44-50mm with fhx of dissection <50mm, severe AI or MR requiring surgery, undergoing aortic valve repair/replacement; progressive aortic dilation or AI advancement if pregnant

Reference: 2010 AHA/ACC guideline for the diagnosis and management of patients with Thoracic Aortic Disease

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2. 14-year old healthy teenager presents with 14 days of fever, headache, and new murmur. You suspect that this patient

has infective endocarditis.

a. What FOUR other features may you find on her physical examination?

Other: intracranial hemorrhage, conjunctival hemorrhages, glomerulonephritis, roth spots, mycotic aneurysms

b. List THREE major criteria for the diagnosis of infective endocarditis.

1. Persistently positive blood cultures (Viridans streptococcus (Strep Bovis, HACEK group, s. aureus, community acquired enterococci). At least two positive blood cultures >12h

apart (or 3 or majority of 4 at least 1h apart)

2. Echo evidence: positive for IE (oscillating intracardiac mass in path of regurgitant jet or on implanted material, abscess, or new partial dehiscence of prosthetic valve) or complicated IE (paravalvar abscess)

3. New valvar regurgitation (worsening, changing or pre-existing murmur not sufficient)

c. What are FIVE indications for surgical intervention?

1. Heart block

2. Abscess

3. Valve dysfunction causing heart failure

4. Recurrent emboli and persistent vegetations despite appropriate antibiotic therapy

5. Large mobile vegetation

Other: Resistant organism (S. aureus, fungi), persistent infection, relapsing prosthetic valve endocarditis, Reference: 2014 AHA/ACC guideline for the management of patients with valvular Heart Disease: Executive Summary

1. Fever >38C

2. Major arterial emboli

3. Janeway Lesions

4. Osler Nodes

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3. Two-week old infant presents to ER with tachypnea, lethargy, decreased oral intake and poor urine output. Saturation

93%. The infant has poor femoral pulses on examination.

a. List FOUR differentials aside from aortic coarctation

Other: Tachycardia induced cardiomyopathy

b. List THREE associated echo findings that may be present if the patient also has wide spaced nipples and a webbed

neck.

1. LV systolic dysfunction

2. Blunted abdominal aortic Doppler

3. Bicuspid aortic valve

c. The patient is found to have coarctation of the aorta. What are THREE long term complications that can occur after

repair.

1. Berry aneurysms

2. Persistent systemic hypertension

3. Re-coarctation

d. List THREE clinical or investigation findings for a teenager with coarctation that is less likely to be present in a

neonate.

1. Systemic hypertension in right arm

2. Rib notching on chest x-ray

3. LVH on ECG

1. Critical aortic stenosis

2. Dilated cardiomyopathy

3. Myocarditis

4. ALCAPA

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4. Six-month old female is diagnosed with missed Kawasaki Disease. History of 14 days of fever and conjunctivitis 4 weeks

prior. She is found to have giant coronary artery aneurysms in all three main coronaries

a. What is the definition for a giant coronary artery aneurysm?

1. ≥8 mm in diameter or with a Z score ≥10

Note: do not “resolve,” “regress,” or “remodel.” They rarely rupture and virtually always contain thrombi (the oldest of which may calcify) that can become occlusive.

b. List FIVE criteria for the diagnosis of incomplete KD.

1. Fever with 7 days with no other explanation

2. Five days of fever with 2 or 3 compatible clinical criteria (Bilateral conjunctival injection without exudate; erythema/cracking of lips, strawberry tongue, erythema or oral and pharyngeal mucosa; rash (maculopapular, diffuse erythroderma, or erythema multiforme-like); erythema and edema of hands and feet in acute phase or peeling

in subacute phase; cervical unilateral lymphadenopathy (1.5cm))

3. CRP 3.0mg/dL and/or ESR 40mm/h

4. Three or more other laboratory findings (anemia for age, platelets 450,000 after 7th day of fever, albumin 3g/dL, increased ALT, WBC 15,000/m3, urine 10WBC/hpf)

5.Positive echo [z-score of LAD or RCA 2.5, CA aneurysm or 3 other echo findings (dec LV function, MR, pericardial effusion, or LAD or RCA z-score 2 to 2.5)]

f. What are THREE classes of medication used to treat those with giant coronary artery aneurysms?

1. ACE inhibitor

2. Beta blocker

3. Statin

Other: Thromboprophylaxis

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5. 12 year old boy presents with work of breathing, and exercise intolerance. Chest x-ray shows bilateral atrial dilation. No

history of pericarditis. Echocardiogram confirms biatrial enlargement and normal ventricular systolic function.

a. List FOUR typical cath findings consistent with this diagnosis.

1. Prominent y-descent, normal respiratory variation

2. Square root sign

3. RVSp >55mmHg

4. RVEDp/RVSp <1/3

Other: LVED-RVED >5mmHg, RV-LV interdependence absent, lack of variation in early PCW-LV gradient

b. List THREE medical management plans.

1. Loop diuretics (carefully)

2. Beta-blockers (carefully, may need higher HR for CO due to fixed SV)

3. Anticoagulation

Other: Rhythm control, possible ACEI (debatable)

c. Patient failed medical management and was placed on transplant list. What are FOUR absolute contraindications

for cardiac transplantation.

1. Active infection

2. Ongoing malignancy

3. Medical non-compliance

4. Drug abuse

Other: Non-reactive PVR, presence of non-cardiac conditions that shorten life expectancy, recent or unresolved pulmonary infarction, psychiatric illness that will interfere significantly with compliance, systemic disease likely to limit post-transplant survival, active peptic ulcer disease with recent GI bleed

d. Name ONE reason why patients with CHD are more likely to have rejection post-transplant than those with

cardiomyopathy.

1. Sensitized recipients with panel reactive antibody (PRA) in 27% of the population – cellular/humeral rejection

Note: Reason for high PRA is due to many blood transfusions, previously implanted cryopreserved tissue valves, conduits, previous transplants, VADS/ECMO. PRA >10% is shown to have inferior outcomes compared to non-sensitized patients.

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6. Two-month old with tetralogy of Fallot presents to clinic with saturations of 60%.

a. What are FIVE immediate management steps you would like to take.

1. Knees to chest

2. Calm baby

3. Oxygen

4. Beta-blocker

5. IV fluids

Other: Phenylephrine

b. Baby weighs 2.9kg and has recurrent desaturation episodes on propranolol. What are TWO possible interventions

that could be undertaken in this infant.

1. Full surgical repair

2. Interim repair with RVOT stent or shunt

c. What are THREE long term complications in repaired TOF?

1. Arrhythmia

2. Pulmonary valve insufficiency

3. Sudden Death

Other: Recurrent PS

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7. Four-year old girl presents to the emergency department with an earache. In the triage she is found to have a heart rate

of 40 bpm, atrial rate of 92bpm, and AV-dissociation.

a. List FIVE possible etiologies.

1. Congenital CHB from maternal Lupus, Sjogren’s, maternal connective tissue disorder

2. Fetal myocarditis

3. CCTGA

4. Left atrial isomerism

5. Medication induced: amiodarone, beta blocker, Ca-channel blocker, antiarrhythmic, digoxin, procainamide

Other: Infiltrative (amyloidosis, hemochromatosis, sarcoidosis, tumours), rheumatologic (SLE, rheumatoid arthritis, scleroderma, ankylosing spondylitis), infectious (rheumatic fever, toxoplasmosis, viral myocarditis, bacterial endocarditis, trypanosomiasis, syphilis, lyme disease)

b. You suspect a congenital etiology. What are FIVE indications for pacemaker insertion?

1.Symptomatic bradycardia

2.Ventricular dysfunction or low cardiac output

3.Wide QRS escape

4.Complex ventricular ectopy

5.Under 1 year if they have ventricular rate <55bpm or <70bpm in congenital heart disease

Other: Ventricular rate <50bpm if >1 year old, pause of 2-3 times cycle length, symptomatic chronotropic incompetence, 2B: congenital complete AV block without above features

Reference: 2008 ACC/AHA Guidelines for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices: Executive Summary

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8. Four-hour old term baby with no prenatal care has features of Trisomy 21. ECG shows left axis deviation.

a. What are FOUR possible causes of the LAD in baby?

1. AVSD

2. Tricuspid atresia

3. Pulmonary atresia with intact ventricular septum

4. Mechanical shift (eg large right pleural effusion, congenital diaphragmatic hernia)

b. What is the ONE most likely congenital heart lesion in this situation?

1. AVSD

c. What are the SIX anatomic features associated with this lesion?

1. AV-valve leaflets at the same level at cardiac crux

2. Absent AV septum

3. Unwedged aortic valve (pushed anterior and superior)

4. Cleft AV-valve pointing to ventricular septum

5. Clockwise rotation of LV papillary muscles

6. Gooseneck elongated LVOT (inlet/outlet disproportion)

d. What are the FOUR mechanisms for LV outflow tract obstruction?

1. Gooseneck

2. Subaortic fibromuscular ridge

3. Septal hypertrophy

4. Abnormal LAVV chordal attachments

Other: Abnormal orientation of papillary muscles