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4/27/2015 IM Board Review 2015 Timir Paul, MD, PhD, MPH, FACC, FSCAI Peripheral Arterial Disease Lower Extremity Question Based on the epidemiologic studies, which of the following risk factor has the highest risk for developing lower extremity PAD 1. diabetes 2. smoking 3. hypertension 4. hypercholesterolemia 5. hyperhomocysteinemia 1 4/27/2015 Case • 60 year female with CKD (GFR 27), diabetes, uncontrolled HTN, diastolic HF, presents for routine f/u. She c/o leg numbness. • Denies any rest or exertional leg pain, fatigue • No ulcer, gangrene, discoloration or cold foot What do you do next? What do you do next? 1. Proceed with CTA or MRA 2. ABI 3. Exercise ABI 4. Get more history and thorough PE 5. Aggressive risk factor modification Screening for Asymptomatic Patient With PAD Class I indication: Age ≥50 years with a history of smoking or diabetes or Age ≥65 years 2 4/27/2015 Question • A 65 yr male with h/o HTN, HLP and former smoker c/o bilateral thigh pain on walking and relieves at rest. He noticed this stable sx for last 1 yr. Currently taking HCTZ and rosuvastatin. BP 138/70, HR 68/min. No femoral bruits. Deep tendon reflexes are symmetric and normal at knees and ankles. Pedal pulses are 1+ B/L. Resting ABI 0.92 both legs. Question • Which of the following is the most appropriate test? • A. CTA of B/L lower extremities • B. MRI of lumber spine • C. Segmental lower extremity pressures • D. Exercise ABI • E. Angiogram Patient With Intermittent Claudication • ABI is the first test to diagnose symptomatic and asymptomatic PAD (class I) • If resting ABI is normal but clinical suspicion is high exercise ABI is recommended (class I) If ABI > 1.4, TBI is indicated 3 4/27/2015 Question • A 71 yr male reports of both thighs and calves pain. ABI 0.68 in left and 0.75 in right ankle. There is no ulcer or gangrene. His sx improved slightly with exercise and intensive lifestyle modification. He also c/o dyspnea on exertion and mild b/l leg edema. He is taking ASA 325 mg daily • What is the next step 1. Start cilostazol 2. Duplex lower extremity US 3. 2 D echo 4. Start statin 5. Add plavix Contraindications to Cilostazol patients with symptoms of CHF Low EF . Pletal® (cilostazol) Package Insert. Rockville, Md: Otsuka America Pharmaceutical, Inc; 1999. PAD Clinical Presentations 4 4/27/2015 PAD ~ CAD CAD PAD • • • • • • • • • • Non cardiac pain Atypical chest pain Typical angina UA/NSTEMI STEMI No leg pain Atypical leg pain Claudication Critical limb ischemia Acute limb ischemia Clinical Presentations Asymptomatic: Without obvious symptomatic complaint (but usually with a functional impairment). This diagnosis should only be made if the patient is physically active. Exertional Pain “Atypical” leg pain: Lower extremity discomfort that is exertional but that does not consistently resolve with rest, consistently limit exercise at a reproducible distance, or meet all “Rose questionnaire” criteria. Classic claudication: Lower extremity symptoms confined to the muscles with a consistent (reproducible) onset with exercise and relief with rest. Yet it has poor sensitivity and specificity for identifying patients with PAD Clinical Presentations Rest Pain Critical limb lschemia: - Ischemic rest pain - Ulceration/ non-healing wound - Gangrene Acute limb ischemia: The five “P”s, defined by the clinical symptoms and signs that suggest potential limb jeopardy: Pain Pulselessness Pallor Paresthesias Paralysis (& polar, as a sixth “P”). 5 4/27/2015 Clinical Presentations of PAD ~15% Classic (Typical) Claudication 50% Asymptomatic ~33% Atypical Leg Pain (functionally limited) 1%‐2% Critical Limb Ischemia Diagnosis of PAD Diagnostic tools • Non-invasive Functional Studies – Ankle-Brachial Index (ABI) - Toe-Brachial Index (TBI), and Segmental Pressures – Pulse Volume Recording (PVR) • • Non-invasive imaging studies –Arterial Duplex ultrasound, MR Angiography, CT Angiography Invasive imaging – Angiography (gold standard) Interpreting the Ankle‐Brachial Index ABI 1.00–1.4 Interpretation Normal 0.91–0.99 Borderline 0.41–0.90 Mild-to-moderate disease ≤0.40 Severe disease >1.40 Noncompressible ABI is 95% sensitive and 99% specific for PAD Rooke et al, circulation 2011 Hirsch AT, et al. J Am Coll Cardiol. 2006;47:e1‐e192. Figure 6. 6 4/27/2015 Treatment of PAD Options • Exercise • Medical Therapy • Revascularization – – – – – Percutaneous Transluminal Angioplasty (PTA) Stents Atherectomy Thrombolytics Vascular surgery Goals of Therapy for Patients With PAD Limb outcomes • Improve ability to walk – Increase in peak walking distance – Improvement in quality‐of‐ life (QoL) • Prevention of progression to CLI and amputation Cardiovascular morbidity and mortality outcomes • Decrease in morbidity from non‐fatal MI and stroke • Decrease in CV mortality from fatal MI and stroke Non‐pharmacologic Therapy for Intermittent Claudication 7 4/27/2015 Supervised Exercise Rehabilitation Class I indication: Supervised exercise training program is recommended as an initial treatment modality for patients with intermittent claudication and pre‐requisite for revascularization Pharmacotherapy for Claudication • Cilostazol –Cilostazol increase walking distance to 100‐ 150% and is a class I recommendation –Cilostazol is contraindicated in HF patients • Pentoxifylline (do not answer) Pharmacologic Effects of Cilostazol • Platelets aggregation inhibitor • Antithrombotic • Vasodilation • Increase blood flow • Increase HDL (10%) • Decrease TG (15%) • Inhibition of vascular smooth muscle cell proliferation (in vitro) Drug Class Phosphodiesterase III inhibitor Dosing 50‐100 mg bid 8 4/27/2015 Medications for Patients With PAD Therapeutic Goal Drug To Reduce Ischemic Events To Improve Claudication Symptoms Aspirin/Clopidogrel Yes No Cilostazol No Yes Treatment to Improve Cardiovascular Outcomes PAD is CAD equivalent • Risk factors modification is a class I indication • The same ATP III/JNC goal for ‐ Blood pressure ‐ Cholesterol • Smoking cessation • Hba1C < 7.0 9 4/27/2015 Drug Therapy • Aspirin (75 mg to 325 mg) • Plavix is an alternative • Not both ‐ Blockers Are Effective and Not Contraindicated in PAD Radack K. Arch Intern Med. 1991;151:1769. Non Drug Therapy • Supervised exercise program and rehabilitation in all symptomatic PAD patients • Angioplasty or Surgery – If medical therapy fails – Rest pain – Poorly healing ulcer For acute limb ischemia‐ Start heparin and interventional cardiology consultation for embolectomy or arterial thrombolysis/PTA 10 4/27/2015 Asymptomatic PAD Revascularization • Class III • Revascularization (endovascular or surgical) is not indicated as prophylactic therapy in an asymptomatic patient with lower extremity PAD Renal Artery Stenosis (RAS) Diagnosis of RAS Class I indications: 1. Resistant, accelerated and malignant hypertension 2. New azotemia or worsening renal function after administration of ACEI/ARB 3. Sudden unexplained pulmonary edema 4. Unexplained atrophic kidney or discrepancy in size between 2 kidneys > 1.5 cm 5. HTN before age 30 or severe HTN after age 55 years 11 4/27/2015 Diagnostic modalities Class I Duplex ultrasound CT angiogram MRA Renal angiogram is the gold standard Indications for Revascularization Class I: Percutaneous revascularization is indicated for patients with significant RAS and recurrent unexplained heart failure or sudden unexplained pulmonary edema Class IIa Percutaneous revascularization is reasonable for patients with hemodynamically significant RAS and unstable angina Indications for Revascularization Hypertension: Preservation of renal function 12 4/27/2015 Diseases of Aorta Questions A 62 yr old male with family h/o AAA presents for routine f/u examination. He is a past smoker. BP is 138/82 with HCTZ. Which one of the following is a correct statement. 1. Screen for lipids 2. US Screen for AAA 3. US screen for AAA when he will be 65 4. Goal BP is <130/80 5. Do nothing as he is asymptomatic Questions • Which of the following are indications for infrarenal AAA repair? 1. Diameter > 5.5 cm 2. Expansion rate greater than 1 cm/yr 3. Inflammatory or infectious etiology 4. Recurrent atheroembolism 5. All of the above 13 4/27/2015 Question A 68 yr male with HTN, DLP, current smoker and family h/o of AAA. He had an US done 2 yrs ago that did not reveal AAA. When is the next US should be performed for f/u 1. 1 yr 2. 3 yrs 3. 5 yrs 4. No further US 5. Any time between 70‐75 yrs Abdominal Aortic Aneurysms (AAA) Signs and Symptoms ‐ AAA • Pain‐ abdomen, back, flank • Pulsatile abdominal mass • Physical examination lacks sensitivity (69% for <5 cm) • Livedo‐ atheroembolism – – – – – Blue or gangrenous toes Livedo reticularis Elevated serum creatinine Eosinophilia Accelerated HTN 14 4/27/2015 Screening high risk population AAA Surveillance Size (cm) 3.0‐3.4 3.5‐3.9 4‐4.9 >5 Surveillance interval 3 yrs 1 yr 6 months 3 months • If AAA 4‐5.4 US every 6‐12 months • For smaller AAA every 2‐3 yrs AAA‐ indications for surgery 1. Diameter > 5.5 cm 2. Expansion rate greater than 1 cm/yr 3. Inflammatory or infectious etiology 4. Recurrent atheroembolism 5. Symptomatic 6. Ruptured or contained rupture 15 4/27/2015 Thoracic Aortic Aneurysm Risk Factors of Thoracic AA • • • • Hypertension, tobacco, atherosclerosis Famililal Congenital‐ bicuspid aortic valve Infectious and Inflammatory – Syphilis, TA, GCA, Behcet, Ankylosing spondylosis • Connective tissue disease – EDS, Marfan, Turner’s Trauma‐ sudden deceleration Ascending Aortic Aneurysm Surveillance Aneurysm 3.5‐ 4.4 cm Annual CT or MR Aneurysm 4.5‐ 5.4 cm Semi‐annual CT or MR 16 4/27/2015 Medical Management of TAA • Control hypertension First : beta‐blockers 2nd : vasodilator Other risk factors modification Indications for Thoracic AA Surgery Ascending aortic diameter >= 5.5 cm Exceptions: ‐ Expansion rate greater than 0.5 cm/yr ‐ Infectious etiology ‐ Traumatic origin ‐ Symptomatic ‐ Bicuspid aortic valve, Marfan, Turner, Ehlers‐Danlos, familial thoracic aortic aneurysm and dissection (>4.0‐5.5 cm) ‐ Patients undergoing aortic valve repair or replacement and who have an ascending aorta or aortic root > 4.5 cm Acute Aortic Dissection 17 4/27/2015 Acute Aortic Syndromes • Aortic dissection (classic) • Intramural hematoma (IMH) • Penetrating atherosclerotic ulcer (PAU) Question A 64‐year‐old man presents with the sudden onset of tearing chest pain. On presentation, he has a heart rate of 130 beats/min with a systolic blood pressure of 80 mm Hg. A bedside TEE demonstrates the presence of a proximal aortic dissection. A pericardial effusion with partial diastolic collapse of the right ventricle is also present. Significant respiratory variation is noted across mitral and tricuspid Doppler inflows. Appropriate treatment is 1. Immediate percutaneous pericardiocentesis to relieve the tamponade, followed by surgery to replace the ascending aorta 2. Proceed immediately to the operating room 3. Emergency angiography to define coronary anatomy, followed by surgery 4. Intra‐aortic balloon pump to stabilize the hemodynamics, followed by surgery Question A 60‐year‐old hypertensive man presents with tearing back pain. MRI confirms the presence of a descending thoracic dissection originating beyond the left subclavian artery. Appropriate initial treatment includes 1. Immediate surgery to replace the descending aorta 2. Intravenous nitroprusside followed by immediate surgery 3. Intravenous nitroprusside alone; surgery for persistent pain, or for involvement of renal or mesenteric arteries 4. Intravenous beta‐blockade and nitroprusside; surgery for persistent pain, or for involvement of renal or mesenteric arteries 18 4/27/2015 Question • Which of the following has the lowest mortality? • 1. Type A dissection treated medically • 2. Type A dissection treated surgically • 3. Type B dissection treated medically • 4. Type B dissection treated surgically Questions 63 yr male admitted for symptomatic repair of 5.6 cm of AAA. He has h/o DM, dyslipidemia and family h/o MI in brother at age 58. BP is borderline controlled with ACE and HCTZ. what is the next appropriate step? 1. Proceed with surgery 2. Nuclear stress test 3. Exercise stress test 4. Titrate up BP meds 5. Transfer patient to ICU and start nitroprusside Question • 58 yr male with h/o CAD presents with severe chest pain radiating to back. BP 185/100, HR 100/min. A decrescendo murmur was heard on the precordium. What you should do next: 1. Stat surgery consult 2. EKG and troponin 3. IV nitroprusside 4. BB and IV nitroprusside 5. CT with contrast 19 4/27/2015 Question 58 yr male with h/o CAD presents with tearing chest pain radiating to back. BP 100/60 in right arm and 140/70 in left arm, HR 100/min. CXR showed widened mediastinum What you should do next: 1. EKG and troponin 2. Surgery consultation 3. BB and IV nitroprusside 4. Emergent CT with contrast 5. 3 and 4 Types of Dissection Stanford classification • Ascending aortic dissection – Type A • All others dissections – Type B Acute Aortic Dissection Clinical Presentation ‐ Chest pain radiating to back – CHF – Shock – Syncope – CVA ( focal neurologic deficits) – Paraplegia – Acute arterial occlusion – HTN, AR, pulse deficits/ differential BP 20 4/27/2015 Dissecting Ascending Aortic Aneurysm Type A Dissection • • • • • • Chest pain radiating to back Acute AR murmur and HF Widened mediastinum Blood pressure differential between arms Cardiac tamponade Evidence of thromboembolism or dissection of branch arteries (stroke, MI ) • This is an emergency • Treatment is surgery • In what other conditions you see wide mediastinum? Anthrax Acute Aortic Dissection Medical management • Pain control • Hypertensive patients – IV beta‐blocker (caution with acute AI) – or labetalol Normotensive Patients: IV beta blocker BP goal: SBP 100‐120 mm Hg Diagnostics • CT scan with contrast or MRA • TEE for critically ill patients who cannot move from a monitored setting 21 4/27/2015 Type B Aortic Dissections • Uncomplicated Type B dissections usually treated medically • Beta blockers and nitroprusside for acute dissections • Long term BB for all patients • Surgery – If pain persists ‐ If major aortic vessels (renal arteries) are involved Other Diseases of Aorta • Aortic atheroma • Penetrating aortic ulcer • Intramural Hemotoma – Crescent like thickening of aortic wall – Absence of dissection flap • Marfan syndrome – Aortic root dilatation – MVP • Takayasu’s arteritis Intramural Hemotoma • Intramural hematoma is a variant of aortic dissection • Type B is common • Risk factors, signs & symptoms and management of acute IMH are the same as for aortic dissection • No intimal flap by TEE • Relatively smooth luminal surface • False negative aortogram 22 4/27/2015 Takayasu’s Arteritis • Young adult women from Japan and other parts of Asia • Areas of focal stenosis in multiple major arteries • The mainstay of therapy is corticosteroids • Large vessels bypass and angioplasty/stenting is often required Coarctation of Aorta Coarctation of Aorta Aortopathy/Association • Aortic medial Changes • Bicuspid aortic valve • 5 fold increase in intracranial aneurysm in patient with coarctation • Often associated with Turners Syndrome • Usually occurs just distal to left subclavian artery • BP differential between upper and lower extremities • Collaterals: subclavian, axillary, internal mammary, scapular, intercostal 23 4/27/2015 Physical exam • • • • • • Delayed femoral pulses Systolic murmur LSE → con nuous Collateral murmurs 4th sound with LVH and hypertension Loud A2 if hypertension Ejection click ± systolic murmur with bicuspid aortic valve Diagnostic Imaging • CXR – Figure “3” sign – aortic knob – Rib notching from enlarged intercostal arteries • Echo – Doppler • CT, MRI / MRA • Angiogram Indications for Intervention • Peak to peak gradient > 20 mmHg • Peak to peak gradient < 20 mmHg in presence of imaging evidence of significant coarctation with radiological evidence of significant collateral flow 24 4/27/2015 Pericardial and Myocardial Diseases Question A 68‐year‐old man develops recurrent chest discomfort 5 days after an anterior myocardial infarction. The pain is sharp and radiating toward both clavicles. He is afebrile and there is no pericardial friction rub on physical exam. BP 132/70, HR 110. EKG showed diffuse ST elevations. CXR showed pleural effusion. He in on 325 mg aspirin, statin, plavix, metoprolol and lisinopril. Question The most appropriate therapy for this patient is – High dose aspirin – Indomethacin and Colchicine – Indomethacin – Corticosteroids – Nitro drip and morphine PRN 25 4/27/2015 Dressler’s Syndrome Post‐cardiac injury syndrome • Fever, pericarditis, pleuritis (typically a low grade fever and a pericardial friction rub) • Occurs in the first few days to several weeks following MI or heart surgery • Incidence: 6‐25% • Treat with high‐dose aspirin/NSAIDs Question A 45 yr female presents for evaluation of left sided pleuritic type chest pain for last 2 weeks. No relation to exertion or rest but worse when supine and got better transiently on leaning forward. The pain got better with acetaminophen. Reports that she had low grade fever for the last 1 week but no cough. She was treated for acute pericarditis 1 year ago with ibuporfen and her symptom was resolved in few days. Currently she is taking acetaminophen intermittently. Temp 100F, HR 105/min, BP 140/88. No JVD, wheezing. EKG showed sinus tachycardia without and ST changes. Question Which of the following is the most appropriate management? A. B. C. D. E. Aspirin Ibuprofen Colchicine Colchicine and aspirin Prednisone 26 4/27/2015 Common Causes – Idiopathic is the commonest – viral infections: ‐ ‐ ‐ ‐ ‐Coxsackie A and B (highly cardiotropic) are the most common viral cause of pericarditis and myocarditis Uremia Myocardial infarction (acute, post) Medications (hydralazine, procainamide, INH) Radiation Acute Pericarditis Diagnostic Clues • Chest pain‐ pleuritic; worse by lying supine, relieved by sitting and leaning forward • Pericardial friction rub • Pathognomic for pericarditis • EKG : most important clue • Troponin and CK‐MB may be elevated in myopericarditis • An extensive initial evaluation is not warranted in uncomplicated patients because of low diagnostic yield Evaluation of Acute Pericarditis • Pericarditis is a clinical and electrocardiographic diagnosis • Pericardial effusion on echo neither confirms nor excludes the diagnosis • Do not need an echo to diagnose acute pericarditis • A normal echo does not rule out pericarditis • Pericardiocentesis with subsequent pericardial fluid analysis should be reserved for cardiac tamponade, suspected purulent, tuberculous or neoplastic pericarditis 27 4/27/2015 Acute Pericarditis Treatment – NSAIDs (Indomethacin 75‐225 mg daily, Ibuprofen 400‐800 mg tid‐qid) for 1 month – Colchicine 0.6 mg bid for 3 months – ASA 650‐800 mg q 6‐8 hrs (2 to 4 g/day) preferred in pericarditis with MI – Corticosteroids are symptomatically effective, but preferably avoided. Use for short term only in refractory cases of relapsing pericarditis ESC Guidelines for Management of Pericardial Diseases • First episode of Acute Pericarditis: NSAIDs Class I NSAIDs + Colchicine Class IIa Eur Heart J 2005 Recurrent /Relapsing idiopathic Pericarditis – Colchicine (0.6 mg BID) should be started with 1st episode of recurrent pericarditis (6 months) – NSAIDs for 1 month – Prednisone are occasionally effective – Steroid dependency requiring gradual tapering over 3‐12 months – Pericardiectomy may be helpful in drug refractory recurrent pericarditis 28 4/27/2015 ESC Guidelines for Management of Pericardial Diseases • Recurrent Pericarditis: NSAIDs + Colchicine Class I Pericardial Effusion Pericardial Tamponade Etiology • Includes all causes of pericarditis • Hypothyroidism 29 4/27/2015 Pericardial Effusion Benign Tamponade • Normal JVP • No Pulsus • Elevated JVP • Pulsus paradoxus • Prominent X descent Signs and Symptoms of Tamponade Signs ECG • Electrical alternans • Diffuse low voltage • S tachycardia • S. Tachycardia • Hypotension or narrow PP • Decrease urine output • ± pericardial rub • Elevated JVP • Prominent x descent • Attenuated y descent • Pulsus paradoxus Pulsus alternans Severe Cardiomyopathy Tamponade 2‐D Echo/doppler findings • • • • • Pericardial effusion Diastolic collapse of RV and RA Dilated IVC (IVC plethora, 97% sensitivity, 99% PPV) Abnormal ventricular septal motion Increased respiratory variation of the mitral and tricuspid flow velocities • Reduced chamber size 30 4/27/2015 Pericardiocentesis • Do For tamponade or If need to make a diagnosis • Don’t Do If there is any suggestion of aortic dissection Question • A 67 yr male s/p CABG 5 years ago. He presents with increasing DOE for the last 4‐5 months. Exam showed JVP 10 cm, pedal edema and hepatomegaly. • EKG and CXR‐ unremarkable Question • Most likely diagnosis 1. Tamponade 2. Restrictive cardiomyopathy 3. Constrictive pericarditis 4. Ischemic cardiomyopathy 5. Effusive constrictive pericarditis 31 4/27/2015 Question • A 54 yr male presents for evaluation of fatigue. She also reports of intermittent LE edema for the last 4 months. She had past medical h/o breast cancer 10 yrs ago, treated with surgery, radiation and chemo. Has h/o hypertension, but no diabetes. • Physical exam shows HR 75, BP 110/70. JVP distends with inspiration. Carotid pulses are full and brisk. Heart sounds revealed S4. • Labs • BUN/Cr: 18/0.8, albumin 4.5, Hb 14.0 • ECG ‐ unremarkable Question • Echo showed normal RV and LV systolic function with EF 60%. A small LV cavity with normal wall thickness, diastolic dysfunction without respiratory variation. Valves are normal. • What is the diagnosis • 1. Hypertrophic cardiomyopathy • 2. Restrictive CMP • 3. Constrictive pericarditis • 4. Hypertensive CMP • 5. Chemo induced CMP Constrictive Pericarditis Etiologies 32 4/27/2015 Presenting Symptoms • CHF (fatigue, DOE) • • • • • Chest pain GI symptoms Tamponade Arrhythmia Liver disease 67% 8% 6% 5% 4% 4% Presenting Signs • Mostly RV failure symptoms Elevated JVP 88% Peripheral edema Hepatomegaly 64% 53% Pericardial Knock 47% Ascites 37% Kussmaul’s 21% Pulsus Paradoxus 19% Constrictive Pericarditis Diagnostic Tests • • • • • • Chest X‐ray Computed tomography Echocardiography Magnetic Resonance Imaging Cardiac Catheterization Surgical Exploration 33 4/27/2015 CXR and CT Pericardial calcification Pericardial Thickening >2 mm Thickness MRI is the most sensitive to measure pericardial thickness Normal pericardial thickening does not exclude constrictive pericarditis (pericardial thickening may be normal in 20% of cases) Prominent Y descent in RAP Constrictive Pericarditis Treatment of Constrictive Pericarditis • Cautious diuretics • Acute /transient Effusive Constrictive Pericarditis can be treated with aggressive anti‐inflammatory • Pericardiectomy is the most effective treatment for chronic constriction • Surgical mortality from pericardiectomy is approximately 6‐19% 34 4/27/2015 Summary Constrictive Pericarditis • • • • • • Respiratory Variation in diastolic filling Increased ventricular interdependence Ventricular discordance Hepatic vein flow reversals with expiration BNP is not markedly elevated LA is not markedly elevated Myocardial Diseases Restrictive Cardiomyopathy Causes Myocardial Noninfiltrative diseases ‐idiopathic ‐familiar ‐scleroderma Infiltrative – Amyloidosis – Sarcoidosis – Hemochromatosis – Glycogen storage diseases Endomyocardial – – – – – – endomyocardial fibrosis hypereosinophilic synd carcinoid anthracycline toxicity Radiation metastatic cancer 35 4/27/2015 Restrictive Cardiomyopathy signs and symptoms • • • • • • • Edema Elevated JVP Dyspnea Rapid X and Y descents Ascites Hepatomegaly Low output symptoms Atrial arrhythmias are frequent Right sided heart failure is more severe than left HF Cause specific Echo findings Restrictive cardiomyopathy • Amyloid heart disease – Thickened myocardium/septum (refractile on Echo) – Pericardial effusion – Thickened regurgitant valves Endomyocardial fibrosis Apical thrombus without underlying apical akinesis Mitral regurgitation Amyloid vs LVH ECG: Amyloid low voltage Treatment Restrictive Cardiomyopathy • Treatment directed to underlying disease (e.g. amyloidosis) • Diuretics for symptomatic relief • Pacemaker for conduction abnormalities • B‐blockers should be avoided because elevated HR frequently maintains cardiac output • Avoid digoxin and CCB in amyloid because they bind to amyloid protein and increase risk of toxicity 36 4/27/2015 Treatment Restrictive Cardiomyopathy • Heart transplantation is the only proven therapy for severe restrictive CMP • Corticosteroids and cytotoxic drugs are appropriate during early stages of eosinophilic endocarditis • Endomyocardial fibrosis can be surgically resected and the mitral valve can be replaced Constriction vs Restriction • Both have similar presentations and clinical examination • It is important to differentiate restrictive cardiomyopathy from constrictive pericarditis • Treatment is different • Rarely, restrictive CMP and constrictive pericarditis coexist , particularly in radiation induced heart disease Dip and Plateau Is this diagnostic for constriction? Abrupt cessation of diastolic filling 37 4/27/2015 Constriction vs Restriction Restrictive CMP Constriction Similar Hemodynamics High/Equalization of diastolic pressures Dip and Plateau/Square Root Constriction vs Restriction Hemodynamic Criteria Criteria • • • • • • Constriction LVEDP‐ RVEDP RVSP RVEDP/RVSP Ventricular Elevated filling P Dip and Plateau < 5 <50 >0.3 Discordance + + Restriction >5 >50 <0.3 Concordance + + Wood P. AJC, 1961; Yu et al. Circ 1953 Constriction vs Restriction Constriction Restriction • Atrium not markedly dilated markedly dilated • Pul HTN • BNP mild > mild and common not markedly elevated markedly elevated 38 4/27/2015 LV‐RV Pressure Relationship Ventricular Interdependence Restrictive CMP Constriction Think “ Constriction” • When there is heart failure (esp RHF) out of proportion to myocardial or valve findings • When there is a h/o prior chest radiation: breast cancer, lung cancer, hodgkins • When there is a h/o prior cardiac surgery • When there is h/o TB, RA and LFTs abnormalities 39 4/27/2015 Take home message Constriction vs Restriction • Differentiate restriction by – Enlarged atrium, moderate pulmonary HTN (PA pressure > 50), moderately elevated BNP compared to constriction – Interventricular concordance in restriction and discordance in constriction Hypertrophic Cardiomyopathy Question An asymptomatic 20 yr male wants to play soccer. Routine P/E reveals systolic murmur and brisk carotid upstroke. Echo showed 17 mm thick interventricular septum. Which one was is true? 1. Can play soccer 2. Can do bowling 3. Start BB 4. Repeat echo in 6 months 5. Genetic screen for first degree relatives 40 4/27/2015 Questions 35 yr female has progressive dyspnea and angina for the last 4 months. There is a 2/6 late peaking systolic murmur that increases in intensity with valsalva. Echo showed EF 65%, SAM, septum 2.3 cm, gradient 30 mm Hg What is the next best step? 1. Beta blocker 2. Septal ablation 3. Surgical myomectomy 4. ICD 5. Nothing as gradient is low Questions 34 yr female with h/o syncope presents with dyspnea. Echo showed LVH with a gradient of 25 mm Hg. Exercise stress by modified Bruce protocol showed no EKG changes. Baseline BP was 128/76 and at 5 min of exercise BP was 110/74. What is the next best step 1. Beta blocker 2. Pacemaker 3. BIV pacemaker 4. ICD 5. Myomectomy Hypertrophic Cardiomyopathy • Autosomal dominant (50%) • Echo screening of all first‐degree relatives of patients with HCM • Syncope, sudden cardiac death (after vigorous exercise) • Chest pain, dyspnea, arrhythmias • Exam – Bifid pulse – – – – Usually no radiation to carotids/carotid ‐brisk Early systolic murmur increases with standing and valsalva Murmur decreases with handshake and sitting Risk of SCD in HCM ~ 1% annually Pulsus bisferiens? Aortic regurgitation 41 4/27/2015 EKG and Echo findings of HOCM • Q waves that mimic MI • Deep T inversions in precordial leads in apical HOCM • Echocardiography Systolic anterior motion of MV (SAM) Asymmetrical septal hypertrophy MR HOCM vs AS HOCM AS • • • • • • • • • • • • Pulsus parvus et tardus Usually present Single sustained Present May be present Murmur • • • • • Decrease murmur Decrease mummur Decrease murmur Increase or no change Increase Carotid pulse‐ Bifid pulse Carotid radiation – none Apex beat‐ tripple ripple Ejection sound‐ none AR‐ none Murmur – – – – – Valsalva‐ ↑ Squatting to standing‐ ↑ Hand gripping‐ ↓ Amyl nitrate‐ ↑ Post PVC ↑ Hemodynamics • Aortic pressure tracing: Spike and dome pattern‐ a rapid rise and fall followed by a plateau Post –PVC HOCM – Decreased pulse‐pressure AS ‐ Increased pulse ‐pressure 42 4/27/2015 AS vs HOCM Post PVC beat AS HOCM Differentiating features: 1. Aortic Upstroke 2. Pulse pressure 3. Contour‐ Spike and dome HOCM vs Athlete’s Heart Echo HOCM • LV diastolic cavity (LVEDD) < 45 mm Athlete’s Heart • LV diastolic cavity > 55 mm • Marked LA enlargement • No LA enlargement • SAM and MR usually present • Absent HOCM‐ High risk of SCD Major RF • Prior cardiac arrest or • Sustained Ventricular Tachycardia/V fib • Family h/o SCD Minor RF • Recent unexplained syncope (>=2 episodes in yr) • NSVT • Blunted or hypotensive BP response during exercise • Massive ventricular wall thickness >3 cm • • • • Age < 30 yrs at diagnosis Familial form Significant outflow tract gradient > 50 Malignant genotype 43 4/27/2015 Who needs ICD • Risk factors 0 1 2+ Prior SCD Sustained VT Recommendation Reassurance Individualize ICD (recommend) ICD ICD Drug Treatment • • • • • Beta –blockers Calcium channel blockers Disopyramide Avoid digoxin, ACEI and other vasodilators Avoid diuretics Non drug therapy • Septal ablation or surgery for symptomatic patients despite maximal medical therapy • Myomectomy (gold standard) • ICD needed for patients at high risk of SCD – VT or NSVT on holter – Family h/o sudden cardiac death 44 4/27/2015 Indications for Surgery or Septal Ablation • Angina, dyspnea and/or syncope resulting in significant impairment in quality of life • Symptoms persists despite appropriate medical therapy • LVOT obstruction (>50 mm Hg) HOCM • Endocarditis prophylaxis ? No Discussion 45 4/27/2015 Myocarditis Clinical Presentations • Acute myocarditis • Fulminant myocarditis – Dramatic acute heart failure with cardiogenic shock in the absence of other etiologies – Hemodynamic compromise, rapid onset of sx (usually within 2 weeks) – Need high dose vasopressor or even a VAD • Giant cell myocarditis – Heart failure with a progressive, relentless, downhill course – Biopsy showed multinucleated giant cells without granulomas – Ventricular arrhythmias are common • Chronic active myocarditis Diagnostic tests – Echo – Cardiac MRI • Delayed enhancement on T1‐weighted images • Endomyocardial Biopsy – In fulminant and giant cell myocarditis (Class I) – Otherwise EMB is a Class IIb 46 4/27/2015 Therapeutic Approaches • Medical Treatment – – – – Conventional / supportive treatment Specific treatment/ interferon Immuno‐suppressants Immuno‐modulating therapy • Mechanical Circulatory Support – IABP – Ventricular assist device – Indicated in Fulminant Myocarditis • Heart Transplantation Take Home Message • Myocardial biopsy is not routinely indicated in patients with suspected myocarditis • In general, there should be low threshold for biopsy: ‐ Suspicion of giant cell myocarditis ‐ Rapidly deteriorating myocarditis ‐ Fulminant myocarditis • Patients with fulminant myocarditis have an excellent long term prognosis • Patients with giant cell myocarditis have an extremely poor prognosis with median survival 6 months Take Home Message • Immunosuppressive therapy has not been shown to improve outcomes and should be reserved for patients with giant cell myocarditis or clinical deterioration despite standard therapy • Steroids is indicated in hypersensitivity myocarditis • Temporary mechanical support/devices may be life‐saving especially in fulminant myocarditis 47 4/27/2015 Question • A 45 yr female with HTN and CKD presents with dyspnea and decreased urine output. She was given IV diuretics and her sx improved. However, her renal function deteriorates with BUN 80 and Cr 5.1. On hospital day 4 her BP 85/45, HR 120. No JVD. Heart sound diminished. TTE showed right atrial collapse, moderate sized effusion and respiratory variation across the mitral valve, IVC decrease with respiration. Question Next step of management 1. Urgent pericardiocentesis 2. IV hydration 3. Immediate dialysis 4. Continue diuresis 5. Repeat TTE in next day Question • A 35 yr male presented with low grade fever, cough and pleuritic chest pain. ECG showed diffuse ST elevation. A TTE showed large pericardial effusion. He was treated with ibuprofen. He returns a month later for f/u. He denies any chest pain but he notes B/L ankle swelling. ECG normal. A repeat TTE shows resolved pericardial effusion but new findings consistent with constriction. 48 4/27/2015 Question What is the next step 1. Obtain cardiac MRI to better assess the pericardium 2. Start a course of colchicine 3. Obtain RHC and LHC 4. Obtain TEE and surgical consultation 5. Reassure the patient and f/u in 3 months for worsening symptoms Question • A 72 yr female with no cardiac disease or malignancy presents to clinic with fatigue, lower extremity swelling and SOB. On exam she is pale, lethargic with dry skin. BP 95/60 and HR 60. 3+ edema in LE. ECG showed low voltage with electrical alternans. Echo showed large pericardial effusion. Question What is the next step of management 1. Pericardiocentesis 2. Start IV diuresis 3. Check TSH 4. Right and left heart cath 5. Cardiac MRI to assess pericardium and r/o infiltrative diseases. 49 4/27/2015 Question • A 65 yr male with cardiomyopathy; EF of 25‐ 30%, presents for follow‐up. BP 144/ 88. He is taking carvedilol 6.25 bid, lisinopril 10 mg daily, rosuvastatin, asa and lasix. He is doing well and currently asymptomatic. But he still feels fatigue and exercise tolerance is not adequate. He wants to improve his exercise tolerance. What is the next best recommendation. Question • 1. Add spironolactone • 2. Start outpatient continuous dobutamine infusion • 3. Enroll in exercise training program • 4. Add digoxin • 5. Increase the dose of lisinopril to adequately control BP. Question • A 30 yr female presented to urgent care with 3 days of fever and SOB. She was diagnosed with viral infection and was sent home. Now she presents for routing follow up 3 months later. On exam she appears healthy and without symptoms. BP 100/60, HR 70, RR 12. No JVD but apical displacement noted. Echo showed EF 35% with dilated heart, no other abnormality. 50 4/27/2015 Which is next best recommendation • • • • • 1. Start ACEI, BB, diuretics 2. Repeat echo in 6 months and f/u sx 3. Start ACEI and BB 4. Start ACEI, BB and steroids 5. She is well compensated, no need any meds Question • Which of the following conditions is not associated with elevated right ventricular diastolic pressures with a deep and plateau pattern. • 1. Acute RV infarction • 2. Massive PE • 3. Cardiac tamponade • 4. Restrictive cardiomyopathy • 5. Constrictive pericarditis Question Which abnormal LV filling pattern is least likely to benefit from BB therapy? 1. Impaired relaxation 2. Grade II diastolic dysfunction 3. Restrictive filling pattern 4. None of the above 5. 2 and 3 51 4/27/2015 Question • 70 yr old male presents with new exertional dyspnea, orthopnea and leg edema. No CP. No h/o HTN, DM or smoking. BP is 110/65, HR 62. Elevated JVP, lungs clear, distant heart sound with quite precordium. EKG showed low voltage QRS. CXR‐ normal size heart, pulmonary congestion. Echo showed normal EF, normal cavity size with moderately concentric LVH, a small pericardial effusion. Coronary angiography showed mild diffuse non‐obstructive CAD. Which of the following is the next best step 1. Start coreg 3.125 bid and lisinopril 2.5 daily 2. Start aspirin, statin, low dose bb and ACE 3. order serum and urine immunoelectrophoresis 4. If symptoms persists bring back to cath lab for ergonovine challenge to look for spasm 5. Start low dose coreg, lisinopril and aldosterone Question • 81 yr female presents to ER with severe SOB at rest. H/o long standing HTN. Recent Echo showed EF 60%, LVH and pseudonormal pattern of diastolic filling • BP 182/ 62 • HR 106, irregularly irregular • Respiration 30 per min • Cr 1.5, trop < 0.01, ECG: Afib • CXR: pulmonary congestion 52 4/27/2015 What is the next most appropriate step in management • • • • • 1. control BP 2. diuresis 3. control HR 4. TEE with DC cardioversion 5. all of the above Questions Summary Tamponade • Physical findings – sinus tachycardia – elevated jugular venous pressure – pulsus paradoxus – Hypotension • Electrocardiogram – sinus tachycardia – low voltage – electrical alternans • Echocardiographic – a large effusion with a swinging heart – RA collapse; early RV diastolic collapse – reciprocal changes in right and left ventricular volumes with respiration – respiratory flow variation across the AV valves is the most specific finding – IVC plethora is very sensitive 53