A Second Opinion ALSHARQIA ECHO CLUB September 2013 © Copyright 2013, Saudi Aramco. All rights reserved.

2 Doc: Would you mind seeing John for a quick second opinion? Referred to cardiology clinic March Known case of cor Pulmonale and heart failure. Needed wheelchair assistance, extremely breathless.

3 Medications Bumetanide 1mg BD, Metolazone 5mg, Eplerenone 50mg Ramipril 10mg, Bisoprolol 10mg Warfarin Isosorbide 60mg, Atorvastatin 40 mg, Ezetimibe 10m Metformin 850mg TID, Lantus Insulin 60units BD, Novorapid 40, 10, 10 daily Omeprazole 40mg, Allopurinol 100mg daily, Tamusulin MR 400mics daily, Spirivia inhaler daily Amitriptyline 10mg QHS, Quinine So4 300mg 18 Medications 5 Volumes of OPD Files

4 Background 48 yr old former builder, retired 1987 because of back problems. Gradually worsening dyspnoea/oedema since :Tachycardia 122/min, BP 170/116 mmHG. ECG: atrial tachycardia 2:1. Echo reported as showing an enlarged RV, LA enlarged at 6.1 cms.

Coronary Angiography: LV function – overall mildly reduced, especially the anterior wall. Coronary arteries- essentially normal. Lung perfusion scan reported as normal. Pulmonary function tests reported as hyperventilation. DC Cardioversion back to Sinus Rhythm. Patient much improved after cardioversion.

6 December 1998 Admitted with increasing dyspnoea for 1 month. PO2 8.9KPa ? PE. Lung perfusion scan reported as small perfusion defects in the bases not matched with the ventilation scans – intermediate to high probability for PE. Warfarin started initially for 3 months but later advised life long anticoagulation.

Readmitted with AF and central chest pain. Reverted to sinus rhythm post Cardioversion. Several Cardioversions 2000 and March 2001 admitted with heart failure – oedema. Discharged on ASA, Warfarin, Bumetanide 3mg am and 2 mg pm, Spironolactone 25mg, Metolazone 2.5 mg alt days, Ramipril 5mg, Digoxin 125 daily, Amiodarone 200mg, Valsartan 80mg BD, Colchicine for gout, Quinine So4 300mg, Omeprazole 20mgBD and Sandoz K.

8 Follow UP 2001 Heart failure clinic : now Permanent AF (3 prior Cardioversions) same medications, follow up heart failure nurse. Now diabetic. Admitted with dyspnoea and renal impairment May 2001 and Metolazone stopped. Readmitted that month with oedema, dyspnoea and pleuritic chest pain. Developed A Flutter with 1:1 conduction and was cardioverted to sinus rhythm.

September presented to A&E with broad complex tachycardia. CCU narrow complex tachycardia 230/min with haemodynamic compromise - Cardioversion. Repeat coronary angiography. LV function reported as impaired, possibly early cardiomyopathy. Normal coronary arteries. EPS: no evidence of an accessory pathway but echo beats were induced consistent with AV nodal re-entrant tachycardia. Atrial flutter was also induced. Both were different to initial presentation.

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15 Background Continued Patient readmitted with narrow complex tachycardia and cardioverted and put back on Amiodarone. AV Nodal Ablation and permanent pacing carried out Nov Followed up – initially clinical improvement with management of arrhythmia OPD assessment now much worse with evidence of CCF, felt to be mainly R heart failure (echo showed PA pressure > 50mmHg) pacemaker follow up – permanent AF ARB added to ACE, consideration of trial of Sildenafil

Follow up with repeat echos – patient remains extremely short of breath, on home oxygen. Referred to chest physician for opinion. July 2007: chest physician noted O2 sats 99 – 100% on room air and FEV1 of 2.7. CXR large heart and pulmonary congestion. Hg 19.8 g/dl and haematocrit of 52%. Followed up cardiology and respiratory medicine: presumed cor pulmonale secondary to PEs but extreme breathlessness remained a puzzle. Various inhalers and oxygen, heavy doses of diuretics. Heart Failure Clinic.

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18 What more can be done at this stage? Thought Process At least moderate pulmonary hypertension. Possibility of CTEPH. What about chronic RV pacing? Work UP High Resolution CT. PFT & 6 minute walk test. Right and left heart catheterization. Review Echo and discuss possible CRT

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20 Pulmonary Hypertension Work Up 6 min walk – refused PFTs High Resolution CT Referral for assessment of PHT and possible disease targeted therapy

21 High Resolution CT Cardiomegaly, no pulmonary emboli but mosaic attenuation

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25 Tricuspid Regurgitant Velocity

26 TAPSE Tricuspid Annular Plane Systolic Excursion

27 Right & Left Cardiac Catheterization Haemodynamics Aorta 161/79 mmHg LV 175 mmHg LVEDP 10 mmHg RA 20/10 mmHg RV 39 mmhg RVEDP 2 mmHG PA 43/10 mmHG mean 23 mmHG Pulmonary Vascular Resistance 0.69 Wood Units Systemic Vascular Resistance Wood Units Saturations Aortic Sat: 98% LV Sat: 97% SVC Sat: 65% IVC Sat: 84% High RA 98% Mid Ra 97% Low Ra 86% RV 90% PA 90% Systemic Flow: 4.67 l/min Pulmonary Flow l/min QP/QS: 4.04

28 TOE

29 TOE

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31 Why did so many echoes miss the ASD? RV RA LA Intra Atrial Septum LV

32 CTPA

33 CTPA

34 CTPA

35 OSTIUM SECUNDUM ASD Diagnosis

36 Success or Failure ? First seen Cardiology 12 years earlier Multiple cardioversions Seven Cardiologists Several other..ologists 13 Echocardiograms at 2 different hospitals Three cardiac catheterizations EPS AV Nodal Ablation Permanent Pacemaker Labelled as Pulmonary Emboli/Cor Pulmonale Chronic Cardiac Cripple Home Oxygen Therapy

37 Take Home Messages Echo Technologists Think out of the box. Dont follow the herd! Ask yourself why the right side of the heart is dilated. Think ASD. Inject saline. Cardiologists Dont follow the herd! Think out of the box. Always seek a firm diagnosis. Beware known case of. Always look before you burn!

38 ASD Closure ASD Closure under GA mm Amplatzer Device Well tolerated

39 Follow UP Felt better. Less breathless. No longer episodes of cyanosis. Functional capacity remained limited. 10 months later presented with minor stroke. Good recovery.

40 Repeat TEE Post-Stroke

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45 Large amount of thrombus on LA side of Amplatzer device. Managed with intensification of anticoagulation and addition of Clopidogrel.