TRP Written Exam 2021

Booklet 1 - Answers Q1a. PH

1. PAH: 1.1 idiopathic; 1.2 heritable; 1.3 drug/toxin- induced; associated with 1.4.2. HIV or 1.4.3. portal hypertension 2. Left Heart Disease: 2.1 DCM, 2.2 restrictive CM; 2.3 severe AI or MR 3. Lung Disease or Hypoxia: 3.2 interstitial lung disease 4. Obstruction: 4.1 CTEPH; 4.2.5 Parasites 5. Unclear or Multifactorial Mechanisms: 5.1 sickle cell disease; 5.2 sarcoidosis

Q1b. PVR

PVR = mean PAP - mean PCWP Cardiac Output

= Wood units (or x 80 dynes-sec-cm-5)

Normal < 1.25 units or < 100 dynes-sec-cm-5 Q1c. PAH Treatments

1. Prostanoids (IV, inhaled, oral) 2. Prostacyclin agonists (e.g. selexipag) 3. Endothelin receptor antagonists 4. PDE5 inhibitors 5. DHP CCB in patients who have positive vasodilator testing 6. Soluble guanylate cyclase stimulator (e.g. riociguat) Q2.

Aspirin: irreversible inhibition of platelet cyclo- oxygenase activity leading to decreased thromboxane A2 production Carvedilol: B1, B2 and a1 antagonist with anti- inflammatory properties Fondaparinux: activation of ATIII to (indirectly) and selectively inhibit Factor Xa

Q2.

Clopidogrel and ticagrelor are both P2Y12 adenosine receptor antagonists.

Clopidogrel is a pro-drug that requires metabolism to active drug via cyp2C19; ticagrelor is active drug.

Clopidogrel is irreversible; ticagrelor is reversible. Q3. Mechanical MVR

Warfarin for an INR target of 3.0. ASA 75-100 mg daily only if antiplatelet therapy is indicated (vascular dz). Change in 2020 guideline

In patients with mechanical valves and appropriately anticoagulated with warfarin define the approximate annual risk of a) Thromboembolism 1-4% b) Major bleeding 1-2% Q3. Pre-op

Ok for OR; no need for further testing

Hold warfarin; when INR < 2, start LMWH; hold LMWH > 12 hours pre-op (Refer to anticoagulation clinic gets half marks)

Endocarditis prophylaxis required: amoxicillin 2 g PO 1 hour pre-op Q4.

Hibernating myocardium: non-contractile myocardium due to chronic ischemia, which will recover function with revascularization.

Stunned myocardium: non-contractile myocardium immediately post revascularization, which will recover function with time. Q5.

Sampling Site Oxygen Saturation High SVC 67 Low SVC 67 High RA 78 Mid RA 92 Low RA 81 Hi IVC 71 Low IVC 71 RV Inflow 90 Mid RV 87 RV Outflow 88 PA 88 Aorta 98 Pulmonary 98 Q5.

Sampling Site Oxygen Saturation High SVC 67 Low SVC 67 High RA 78 Mid RA 92 Low RA 81 Hi IVC 71 Low IVC 71 RV Inflow 90 Mid RV 87 RV Outflow 88 PA 88 Aorta 98 Pulmonary vein 98 Q5.

Secundum ASD

Qp/Qs: calculate Mixed Venous O2 (Flamm)

MV = (3SVC + IVC) / 4 = [3(67)+1(71)] / 4 = 68

Qp = Ao – MV = 98-68 = 30 = 3:1 = closure Qs PV – PA 98-88 10 Q6.

1. MV 2 leaflets, TV 3 leaflets 2. Septal attachment of a leaflet of the TV (no septal attachment of MV) 3. MV 2 pap muscles/TV >= 3 pap muscles 4. No continuity of TV & PV due to the infundibular septum/ MV & AV continuous 5. Higher insertion of the MV on the ventricular septum (more atrial insertion) 6. TV is associated with anatomic RV/MV is associated with anatomic LV Q7.

1. Bicuspid AV 2. Marfan Syndrome (fibrillin-1) 3. Vascular EDS (collagen III) 4. Loeys-Dietz (TGF-B receptor for most) 5. Familial Thoracic 6. Aneurysm-Osteoarthritis Syndrome 7. Turner Syndrome Q8.

Aneurysm: Contour abnormality in both systole and diastole True: Full thickness infarcted myocardium forms wall (3 layers) Wide neck of mouth of aneursym Low risk for rupture Pseudo: Contained perforation of the heart (by ) Narrow neck of mouth of aneurysm High risk of rupture Q9.

Friederich’s ataxia: HCM, conduction abnormality

Tuberous sclerosis: rhabdomyoma, cardiomyopathy

Hereditary hemorrhagic telangiectasia: pulmonary AV fistula, high output heart failure Q10.

1. LQTS1: alpha subunit of the IKS potassium channel (loss of function); broad-based T wave; exertion, especially swimming

2. LQTS2: alpha subunit of the IKR potassium channel (loss of function); bifid T wave; loud noise

3. LQTS3: Sodium channel (gain of function); long ST segment; sleep Q11.

Class I recommendations for surgery for NVE (ACC/AHA 2020)

1. Valve dysfunction with heart failure; 2. Left sided caused by S aureus, fungal or other highly resistant organisms; 3. Evidence of heart block, annular or aortic abscess, sinus or destructive penetrating lesions 4. Evidence of and persistent infection after a prolonged period (>5 days) of appropriate antibiotic therapy, as indicated by presence of persistent fever or bacteremia.

6. (Recurrent emboli is 2a so would be wrong answer to this question). Q 12. SGLT -2

• a) Inhibition of Sodium-glucose co-transporter 2 in the proximal collecting tubule to prevent reabsorption of glucose. • b) Dapagliflozin vs placebo in patients with HFrEF with or without diabetes. Dapagliflozin significantly reduced the risk of worsening heart failure or death from cardiovascular causes in both diabetics and non-diabetics. Q13. Brugada Syndrome Type 1 ECG: coved ST segments, with ST-segment elevation >2 mm, followed by a negative T-wave Type 2 ECG: J-point elevation >2 mm, gradual ST segment descent, +/- biphasic T wave (saddleback appearance) Provocative agents: procainamide, flecainide, ajmaline Type 3 ECG: no relevance Q14. Hypertriglyceridemia

1. Genetics 2. DM 3. Alcohol 4. Hypothyroidism 5. CRF/nephrotic syndrome 6. Oral contraceptive pill/strogen 7. Steroids 8. Beta blocker 9. Diuretics 10. Obesity Q15.

RA 15 mean RV 45/5/15 PA 45/30/37 PCWP 15/20/17 PA sat 61% Ao sat 95% CO 4 L/min Transplant

Pulmonary Vascular Resistance (PVR) = TPG/cardiac output = 20/4 = 5 Wood units Trans-Pulmonary Gradient (TPG) = mean PAP - mean PCWP = 37-17 = 20 Diastolic Pulmonary vascular Pressure Gradient (DPG) = diastolic PAP – mean PCWP = 13 (better if < 7) Pretransplant desire a PVR < 3 and a TPG < 14

Patient is not currently an acceptable transplant candidate Q16.

Resistant HTN: on at least 3 anti-hypertensives, including a diuretic.

In what conditions is the 4th Korotkoff sound is recommended as the diastolic BP? Pregnancy Severe AI Can’t hear the 5th Q17.

Updated 2018 Q17.

Modified WHO classification of maternal cardiovascular risk: principles

Risk class and risk of pregnancy by medical condition I No detectable increased risk of maternal mortality and no/mild increase in morbidity. II Small increased risk of maternal mortality or moderate increase in morbidity. III Significantly increased risk of maternal mortality or severe morbidity. Expert counselling required. If pregnancy is decided upon, intensive specialist cardiac and obstetric monitoring needed throughout pregnancy, childbirth, and the puerperium. IV Extremely high risk of maternal mortality or severe morbidity; pregnancy contraindicated. If pregnancy occurs termination should be discussed. If pregnancy continues, care as for class III.

Modified from Thorne et al. WHO Class IV Pulmonary arterial hypertension of any cause Severe systemic ventricular dysfunction (LVEF <30%, NYHA III–IV) Previous peripartum cardiomyopathy with any residual impairment of left ventricular function Severe mitral stenosis, severe symptomatic aortic stenosis Native severe coarctation Marfan syndrome with aorta dilated > 45 mm Aortic dilatation >50 mm in aortic disease associated with bicuspid valve, Turners ASI>25 mm/m2 BSA • Pregnancy not recommended (extras) – Vascular ED – Fontan with any complication Q18.

Cardiotoxicity of anthracyclines: cardiomyopathy/LV systolic dysfunction

To limit this cardiotoxicity: 1. Use another drug 2. Limit the dose/use continuous infusions 3. Avoid concomitant use of trastuzumab (anti-HER2, Herceptin) 4. Add dexrazoxane (if 300mg/m2 of doxorubicin is used) 5. Close monitoring 6. Avoid/treat other cardiac stresses (e.g. HTN) 7. Early treatment/prophylactic treatment with ACE/BB/?statin Q19.

A continuous murmur is heard through S2.

Continuous murmurs result when there is a continuous pressure gradient during systole and diastole.

They can be classified as 1) aorto pulmonary connections; 2) arterio-venous; 3) abnormal arterial flow; and 4) abnormal venous flow

Causes:

1. PDA 9. Venous hum 2. A-P window 10. Mammary souffle 3. Aortic-RV/RA/LA fistula 11. ALCAPA 4. BT shunt, Potts, 12. Periphl pulm stenosis Waterston 13. Pulmonary AV fistula 5. Coronary AV fistula 14. Severe T of F, truncus 6. Coarctation arteriosus, tricuspid atresia, TAVR 7. COTA collaterals 15. Lutembacher Syndrome 8. Bronchial collaterals Q20. 1. Beta blockers 2. Sotalol 3. Amiodarone 4. Steroids 5. Colchicine 6. Magnesium 7. (Bi)-atrial pacing 8. Omega 3 fatty acids/Vit A and E 9. Botulinum toxin into fat 10.Statins probably not! Q21.

Pulmonary vein 1 (100% saturated) IVC 2 (renal shunt) SVC 3 Coronary Sinus 4 (heart extracts maximally at rest) Q22. Ebstein’s Anomaly

Apical displacement of the TV leading to atrialization of the RV

ECG findings 1. WPW (may have multiple pathways) 2. Right atrial enlargement (Himalyan p waves) 3. First degree AV block 4. Atypical RBBB 5. T wave inversion V1-V4 and inferior leads

Cyanotic with exercise? Shunt right to left with exercise across either ASD or PFO Q23.

Absolute contraindications to lytics (STEMI ACC AHA 2013) 1. Any prior ICH 2. Known structural cerebral vascular lesion (e.g., arteriovenous malformation) 3. Known malignant intracranial neoplasm (primary or metastatic) 4. Ischemic stroke within 3 mo, EXCEPT acute ischemic stroke within 4.5 h 5. Suspected aortic dissection 6. Active bleeding or bleeding diathesis (excluding menses) 7. Significant closed-head or facial trauma within 3 mo 8. Intracranial or intraspinal surgery within 2 mo 9. Severe uncontrolled hypertension (unresponsive to emergency therapy) 10.For streptokinase, prior treatment within the previous 6 mo Q24.

Allopurinol: inhibits xanthine oxidase, a main source of reactive oxygen species—less oxygen wastage, and less endothelial injury.

Ranolazine: inhibits the late sodium current (INa), which decreases calcium overload and improves diastolic function.

Trimetazidine: stimulates myocardial glucose consumption by inhibiting fatty acid metabolism.

Nicorandil: nitrate-like moiety plus a moiety that opens mitochondrial ATP-sensitive potassium channels (mimicking ischemic preconditioning and dilating resistance vessels).

Ivabradine: slows heart rate by inhibiting If SA node pacemaker current. Q25.

HAS-BLED: 0-1 points= 1%, 5 points= 12.5%

Hypertension Abnormal renal or liver function Stroke Bleeding history or predisposition Labile INR Elderly (age > 65) Drugs/alcohol concomitantly ATRIA

0.83% (0-3 points), 2.41% (4 points), 5.32% (5 points), 17% (10 points)

Anemia (3) Severe renal disease (3) Age >= 75 (2) Prior hemorrhage (1) Hypertension (1)

HEMORR HAGES 2

Hepatic or renal disease Ethanol abuse Malignancy Older (age > 75) Reduced platelet count or function Rebleeding risk Hypertension (uncontrolled) Anemia Genetic factors Excessive fall risk Stroke Others

ORBIT

ABC

Age Biomarkers (GDF-15, hs-troponin T, Hb) Clinical (prior history of bleeding) Q26. ICD (ACC/AHA)

1. ICD therapy is indicated in patients who are survivors of cardiac arrest due to VF or hemodynamically unstable sustained VT after evaluation to define the cause of the event and to exclude any completely reversible causes. (Level of Evidence: A) (16,319–324) 2. ICD therapy is indicated in patients with and spontaneous sustained VT, whether hemodynamically stable or unstable. (Level of Evidence: B) (16,319–324) 3. ICD therapy is indicated in patients with syncope of undetermined origin with clinically relevant, hemodynamically significant sustained VT or VF induced at electrophysiological study. (Level of Evidence: B) (16,322) 4. ICD therapy is indicated in patients with LVEF less than or equal to 35% due to prior MI who are at least 40 days post-MI and are in NYHA functional Class II or III. (Level of Evidence: A) (16,333) 5. ICD therapy is indicated in patients with nonischemic DCM who have an LVEF less than or equal to 35% and who are in NYHA functional Class II or III. (Level of Evidence: B) (16,333,369,379) 6. ICD therapy is indicated in patients with LV dysfunction due to prior MI who are at least 40 days post-MI, have an LVEF less than or equal to 30%, and are in NYHA functional Class I. (Level of Evidence: A) (16,332) 7. ICD therapy is indicated in patients with nonsustained VT due to prior MI, LVEF less than or equal to 40%, and inducible VF or sustained VT at electrophysiological study. (Level of Evidence: B) (16,327,329) Q27. Stroke Risk in AF

Non-Valvular AF: AF in the absence of moderate to severe MS or a mechanical heart valve (ACC AHA AF 2019, CCS 2020)

Guidelines Father Daughter

CCS (2020) OAC (I) Nothing

ACC/AHA (2019) OAC or ASA or nothing OAC or ASA or nothing (IIb) (IIb)

ESC (2020) OAC (IIa) Nothing (III) Q28.

Dabigatran: DTI, RE-LY, 80%

Rivaroxaban: aXa, ROCKET-AF, 33% unchanged, 33% as metabolite

Apixaban: aXa, ARISTOTLE, AVERROES, 25%

Edoxaban: aXa, ENGAGE-AF TIMI 48, 35-50% Q29.

ACC CRT Class I Indications NYHA II, III or ambulatory IV on guideline- directed medical therapy LVEF <= 35% Sinus rhythm LBBB QRSd >= 150 ms Q30.

Severe Primary MR Severe Secondary MR

ERO >= 0.4 cm2 ERO>= 0.4 cm2

RV >= 60 mL RV >= 60 mL

Q31. AS 2020 ACC/AHA Valve guidelines, table 13 Q34. AS a) III b) IIb c) I d) IIa e) I f) I Red = change in 2020 guideline vs 2017 Q32. SCAD

1. FMD 2. Connective tissue disease (Marfan, Ehler Danlos, etc.) 3. Pregnancy 4. Vasculitis 5. Hormone treatment 6. Coronary spasm Q32. SCAD Classification

Type I: Evident arterial wall stain (pathognomonic of SCAD)

Type II: Diffuse stenosis of varying severity

Type III: Mimics atherosclerosis Q33. FFR

Fractional Flow Reserve = Pd/Pa

In the cath lab under maximal hyperemia using IV or IC adenosine

FFR > 0.8 defer (FAME), FFR <= 0.8 intervene (FAME 2), Q34. PAD

ABI = highest systolic pressure (DP vs PT) in foot highest systolic pressure (R vs L) in arms

<= 0.9 Abnormal 0.91-0.99 Borderline 1.0-1.4 Normal >1.4 Non-compressible Q35. Neprilysin Inhibition

Neprilysin is a neutral endopeptidase (NEP) that degrades several endogenous vasoactives peptides, including ANP, BNP, bradykinin, substance P, adrenomedullin, endothelin 1 and angiotensin II. Cont’d

Good: more ANP, BNP, bradykinin

Bad: more angiotensin II (hence ARB); more bradykinin and substance P (therefore more angioedema, much more when combined with ACE-I) Q36. Driving

Condition Private Commercial

CCS 2 No restriction No restriction

NYHA 3 HF, EF 38% No restriction Disqualified

One unexplained syncope 1 week 12 months Q36. Flying

1. PaO2 < 70 mm Hg at sea level 2. >= CCS III angina 3. >= NYHA III heart failure 4. Cyanotic congenital heart disease 5. Pulmonary hypertension/RV failure Q37. HCM

a) Prior cardiac arrest, VF or sustained VT b) OAC in all; amiodarone and disopyramide preferred Q38. RCRI

Intrathoracic, intraabdominal, supra-inguinal vascular

History of ischemic heart disease History of CHF History of CVD Pre-op tx with insulin Pre-op creatinine > 177

Order BNP/NTproBNP

Q39. Amyloidosis

AA amyloid: treatment of underlying condition AL amyloid: chemotherapy TTR amyloid (wild type and mutant): supportive and liver transplant, respectively; tafamidis ok

AA most common, heart rarely involved (<5%)

Low voltages on ECG: AL (>50%) Q40. Physical Exam Q40. Physical Exam a) atrial contraction, atrial relaxation, closure of tricuspid valve, pulling down of the floor of the right atrium, venous return, TV opening, diastasis b) elevated JVP, flying M or W, Kussmaul; elevated JVP, loss of Y; elevated JVP, loss of X’, Kussmaul; absent A, dominant Y c) single S2 on inspiration, splitting on expiration; LBBB, AS, RV pacing, R-sided accessory pathway d) loud S1, opening snap, pre-systolic accentuation; amyl would make Austin Flint immediately softer, true MS louder after several seconds; Q40. Physical Exam e) ASD, severe AI, severe LV systolic dysfunction, LVH/RVH f) PI due to pulmonary HTN g) : severe AI and mixed AS + AI h) dicrotic : cardiogenic , hypovemia, sepsis, tamponade, post AVR BOOKLET 2 - Answers Q1. HCM

Brockenbrough-Braunwald sign: an increase in the obstructive gradient with a decrease in pulse pressure

Beta myosin and/or myosin binding protein; autosomal dominant Q2. Q2. a) Short run (17 beats) of AT, rate 170 bpm, with intermittent aberrancy with establishment of symptom-rhythm correlation b) BB/non-DHP CCB, continue venlafaxine, EP referral if fails medical management (echo optional). Q3. Q3.

a) DDDRO b) Rate-responsive A pace, V pace Q4a.

AT at 150, 4:3 Wenckebach, normal QRS axis Q4b.

AF, intermittent failure to sense, physiologic non- capture; pseudofusion Q5. Q5.

HCM with obstruction

Venodilation (arterial dilation), decreased preload (decreased afterload), smaller LV cavity + compensatory increased contractility (smaller LVEDV), more obstruction, increased gradient and decreased aortic pressure Q6.

AFL with slow/controlled ventricular response PVCs Run of NSVT Episode of CHB No symptoms reported Suggest cardiology referral

Q7. Q7.

Yes; for diagnosis (intermediate pre-test probability, interpretable rest ECG—RBBB ok) as well as prognosis BP response, persistence of ST depressions in recovery Clinically and electrically positive with features of high risk, including limiting angina, widespread and deep ST depressions with a DTS = -19 (4 - 5(3) - 4(2)) Q8. Q8.

Atrial (probably sinus) rhythm at 60 bpm, ventricular at 200 bpm; two unsuccessful attempts at burst anti-tachycardia pacing; one successful shock terminating VT, followed by A-pace, V-pace Discharge home as patient is feeling well (no need to admit); start/increase beta blocker (reasonable); reassess heart structure (echo, reasonable); f/u cardiology as outpatient Q9. Q9.

Ectopic low septal atrial tachycardia

Permanent form of junctional reciprocating tachycardia (PJRT)

Atypical AVNRT Q10. Q10.

Severely elevated RA pressures Pandiastolic gradient between RA and RV

Tricuspid stenosis Q11.

Sinus exit block Type 2. Q12.

Calculate the ERO in patient with severe MR. PISA radius 1.8 cm MR max velocity 514.6 cm/s Aliasing velocity 29.8 cm/s

(2x pie x 1.8 squared x 29.9) / 514.6 = 1.18 cm2 Q13.

Slow VT (see the fusion beats) Q14.

Small, reversible distal anterior/apical defect. SSS 4 SDS 4. Q15.

There is a medium-sized, severe, reversible perfusion defect involving the inferolateral wall of the left ventricle. Q16.

Wenchebach Usual treatment of vasovagal syncope (this is incidental/normal finding) Q. 17 – associated physical exam finding Q 17 – answer = pulsus alternans Q 18 – associated physical exam findings Q 18 - Coarctation • Systolic murmur (crescendo- decrescendo) • S4 • Diminished femoral • radio-femoral delay • Loud S2