Constrictive Pericarditis Curable Paradoxical Diastolic Heart Failure ASE EBRC 2018

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Constrictive Pericarditis Curable Paradoxical Diastolic Heart Failure ASE EBRC 2018 May 8 th, 2018 Jae K. Oh, MD Samsung Professor of CV Diseases Director, Pericardial Disease Clinic Co-Director, Integrated CV Imaging 2018 MFMER 3712003-1 Constrictive Pericarditis Contents Clinical features and Diagnostic criteria Symptoms and signs Imaging Hemodynamics Echo Diagnosis Constriction Mimickers Effusive-constrictive Pericarditis Management Summary 2018 MFMER 3712003-2 1

Constrictive Pericarditis Symptoms/Signs & Exam Findings Right Heart Failure Ascites Edema Abdominal pain JVP elevation with Kussmaul Rapid y descent of JVP Pericardial Knock (S3) Pleural effusion 2018 MFMER 3712003-3 Constrictive Pericarditis Traditional Imaging and Hemodynamic Features Pericardial calcification (CXR and CT) Increased pericardial thickness (Echo, CT, and MRI) Hemodynamics by Cath Increased RA pressure with rapid y descent Equalization of LV/RV end-diastolic pressures :Dip and Plateau (M or W pattern) Pulmonary artery systolic pressure < 50 mmhg High ratio between RV end-diastolic and systolic pressure 2018 MFMER 3712003-4 2

Constrictive Pericarditis Pericardial Thickening or Calcification 27% had calcification on CXR Ling et al AIM 2000 2018 MFMER 3712003-5 26/ 143 (28%) Talreja, Oh et al. Circulation 2003 2018 MFMER 3712003-6 3

Constriction vs Restrictive Myocardial Disease Traditional Hemodynamic Data Comparison Equalization LV/RV Enddiastolic pressure Pulmonary artery systolic pressure (PASP) 50 mmhg RVEDP / PASP 1/3 Vaitkus and Kussmaul AHJ 1991 Talreja, Nishimura et al. JACC 2008 2018 MFMER 3712003-7 Constrictive Pericarditis (N=7) Restrictive Cardiomyopathy (N=11) Dissociation between Intracardiac and Intrathroracic Pressure Hatle et al. Circulation 1989 2018 MFMER 3712003-8 4

Constrictive Pericarditis Mitral Inflow vs Cath E E Dissociation between intrathoracic and intracardiac pressures Differential ventricular filling with respiration CP992397-39 2018 MFMER 3712003-9 Hemodynamics in Constriction Intracardiac pressure Δ < intrathoracic pressure Δ Interventricular dependence CP1051850-19 2018 MFMER 3712003-10 5

Constriction Ventricular Septal Motion Abnormality Consider constriction if there is septal motion abnormality in patients with HF and preserved EF (HFpEF) 2018 MFMER 3712003-11 Doppler was diagnostic for CP in 22 of 25 (88%) patients who were proven to have constriction at the time of operation Echo Dx of Constriction 1.Abnormal Septal Motion 2.Restrictive Mitral Inflow with Respiratory Variation > 25% Mitral Inflow Doppler Hepatic Vein Doppler 3.Hepatic Vein Diastolic Flow Reversals with Expiration Oh et al JACC 1994 2018 MFMER 3712003-12 6

Diastolic Function Assessment by Echo e velocity reflects LV relaxation Firstenberg et al: J Appl Physiol, 2001, Nagueh et al: JACC 1997, Oki et al: AJC 1997, Sohn et CP1254003-8 al:jacc 1997, Ommen et al: Circ 2000 Opdahl et al: Circulation 119:2578, 2009, and more 2018 MFMER 3712003-13 M. Garcia JACC 1996 JW Ha et al AJC 2004 2018 MFMER 3712003-14 7

Medial e = 12 cm/s Lateral e = 8 cm/s Reuss et al, EHJ Imaging June 2008 2011 MFMER slide-15 2018 MFMER 3712003-15 Echo Diagnostic Criteria Septal motion abnormality MV Flow Velocity Restrictive (E/A >1) Medial e 8 cm/s Hepatic Vein Diastolic reversal with expiration Sensitivity 87 % Specificity 91 % Welch et al Circ Imaging 2014 2018 MFMER 3712003-16 8

Illustrative Cases Diagnosis and Management 2018 MFMER 3712003-17 71 year old man with dyspnea 2 years after CABG Physical Examination JVP elevation Prominent S3 Peripheral edema Cath : Equalized end-diastolic pressures CT was obtained: Calcified Pericardium 2018 MFMER 3712003-18 9

71 year old man with calcified pericardium Mitral inflow E= 80 A= 20 cm/s Medial e = 5 cm/s Amyloidosis 2018 MFMER 3712003-19 Constrictive Pericarditis in the Modern Era Novel Criteria for Diagnosis in the Cardiac Cath Laboratory (Talreja, Nishimura, Oh, Holmes. Jan. 2008 JACC) Discordant change Constriction Concordant change Restrictive CM 2018 MFMER 3712003-20 10

52 year old man with NYHA 3-4 waiting for heart transplantation (had Echo, Cath, and MRI) You just took over his care in HF service. What would you do? Diastolic Reversal Flow with Expiration E = 16 cm/s 1= Pericardiectomy 2= Transplantation 3= Myocardial Bx 4=Repeat Cath Medial e = 20 cm/sec 5= A long talk with previous staff and surgeon 2018 MFMER 3712003-21 An e-mail from a junior staff 52 year old man waiting for heart transplantation (had Echo, Cath, and MRI) Diastolic Reversal Flow with Expiration E = 16 cm/s Medial e = 20 cm/sec 2018 MFMER 3712003-22 11

Explanted Heart 2018 MFMER 3712003-23 Case # 1 27 yo man with fatigue and dyspnea Sep. 2015 Flu-like symptoms, treated with inhaler Oct. 2015 Pre-syncopy and palpitation Pericardial rub Pericardial effusion on Echo Treated with Ibuprofen 2400 mg/d Colchicine 0.6 mg BID Not feeling better and CRP 60 Underwent pericardial window 2018 MFMER 3712003-24 12

27 year old man underwent a window Referred to Mayo Pericardial fluid studies were negative Not feeling better RUQ abdominal pain and fatigue U/S Enlarged gallbladder and liver Consideration of cholecystectomy 2018 MFMER 3712003-25 Interventricular Dependence and IVC Plethora Constrictive Pericarditis Insp Exp 2018 MFMER 3712003-26 13

27 yo man after pericardial window Mitral Inflow Hepatic Vein Expiratory Diastolic Flow Reversal Mitral e = 15 cm/sec 2018 MFMER 3712003-27 Miranda et al. Heat 2015 2018 MFMER 3712003-28 14

205 consecutive patients with pericardiocentesis ECP was diagnosed in 33 (16%) More frequent hemo-pericardium (33% vs 13%) Higher % of neutrophils Baseline medial mitral annulus e higher Expiratory diastolic flow reversal in HV more frequent 2 required pericardiectomy in 3.8 year follow-up Kim KH, Miranda W, Oh JK et al JACC Imaging March 2018 2018 MFMER 3712003-29 MRI DE in 2 patients with Constriction Medical RX Baseline 3 Months Circulation Oct 3 rd 2011 2018 MFMER 3712003-30 15

Transient Constriction Reversible (N=14) Persistent (N=15) Age (yrs) 54 ± 17 59 ± 16 LVEF (%) 57 ± 3 60 ± 3 E (cm/sec) 12 ± 1 11 ± 1 Steroid Rx 71 % 53 % Pericardium 3.8 ± 0.6 mm 4.0 ± 0.6 mm DE Pericardium 4.4 ± 0.4 mm 2.1 ± 0.4mm Grade 3-4/4 DE 93 % 33 % Sed rate 45 to 4 25 to 20 CRP 75 to 2 14 to 15 2011 MFMER slide-31 2018 MFMER 3712003-31 Transient Constrictive Pericarditis One week of Steroid Rx 2018 MFMER 3712003-32 16

Case #5 Heart failure with ascites and leg edema Referred for TV repair Diastolic Flow Reversals in the HV Medial 12 cm/sec Lateral 9 cm/sec 2011 MFMER slide-33 2018 MFMER 3712003-33 Hepatic Vein Doppler is a Key Constriction 1 Myocardial Disease Severe TR 2018 MFMER 3712003-34 17

67 year old man with severe AS came for AVR Case #6 Low Flow Low Gradient AS LVOT TVI = 21cm SV= (1.9) 2 x 0.785 x 21 = 60 cc LVOT D = 1.9 cm AVA = 60 / 76 = 0.79 cm 2 MG 26 mmhg 2018 MFMER 3712003-35 67 year old man with heart failure and LFLG AS Mitral Annulus Tissue Doppler E = 100 cm/s Medial e = 9 cm/s Lateral e = 6 cm/s 2018 MFMER 3712003-36 18

4/23/2018 67 year old man with AS and Constriction Hepatic Vein Doppler c/w constriction Radiation Heart Disease Circulation CV Imaging 2015 2018 MFMER 3712003-37 Constriction with Atrial Fibrillation Medial e = 11 cm/s Lateral e = 7 cm/s 2011 MFMER slide-38 2018 MFMER 3712003-38 19

Constriction with A. Fibrillation After Cardioversion 2011 MFMER slide-39 2018 MFMER 3712003-39 Case #2 A 27 yo woman with dyspnea Marked Septal Motion Abnormality 2018 MFMER 3712003-40 20

4/23/2018 A 27 yo woman with dyspnea Constrictive Pericarditis? Mitral Inflow E = 10 cm/sec 1. E is increased 2. Septal motion abnormality 3. Mitral inflow variation 2018 MFMER 3712003-41 A 27 yo woman with dyspnea Pulsus Paradoxus with Asthma Mitral Inflow IVC E = 10 cm/sec HV 2018 MFMER 3712003-42 21

Constrictive vs COPD/Asthma SVC Flow Velocities COPD Constriction Boonyaratavej S, et al. J Am Coll Cardiol 1998 Dec; 32 : 2043-8 CP983059-17 2018 MFMER 3712003-43 Pericardiectomy at Mayo Clinic The first cardiac surgery in 1936 2018 MFMER 3712003-44 22

Pericardiectomy at Mayo Clinic The first cardiac surgery in 1936 2018 MFMER 3712003-45 Pericardiectomy Courtesy of W. Edwards, MD Courtesy of H. Schaff, MD 2018 MFMER 3712003-46 23

Pericardiectomy for Constrictive Pericarditis at Mayo Clinic (n=1,066) 60 Total patients 50 40 30 20 10 0 1940 1950 1960 1970 1980 1990 2000 2010 Year of surgery Murashita, Schaff, Greason et al Ann Thorac Surg 2017 2018 MFMER 3712003-47 The establishment of the diagnosis of CP has a three-fold importance 1. It affords the explanation of a group of symptoms and signs 2. It obviates confusion with other conditions 3. Expert thoracic surgery may now lead to cure what was once a hopeless disease P.D. White The Lancet 1935 2018 MFMER 3712003-48 24

Constriction vs Restriction Take Home Points Dx of Constriction should be based on hemodynamics Restrictive mitral inflow with or without respiratory variation Preserved or increased medial mitral e Respiratory variation of septal motion Hepatic vein expiratory diastolic flow reversal Under-diagnosed and mis-diagnosed 2018 MFMER 3712003-49 Pericardial Diseases Team Department of CV Diseases Fellows (Old and Current) Cardiology Nandan Anavekar, MD Raul Espinosa, MD Sharonne Hayes, MD Garvan Kane, MD Allen Luis, MD Rowlen Melduni, MD William Miranda, MD Jae Oh, MD Cardiac Surgery Rheumatology Kevin Greason, MD Eric Matteson, MD Hartzell Schaff, MD Kevin Moder, MD Josheph Dearani, MD John Stulak, MD Rocky Daily, MD Cardiac Pathology Imaging Eric Williamson, MD Phillip Young, MD James Glockner, MD - Joseph Maleszewski, MD CV Echo Lab JW Ha, MD Michel Senni, MD Gabriella Veress, MD Lieng Ling, MD Smonporn Boonyaratavej, MD Terrence Welch, MD F. Syed, MD, KH Kim,MD Dali Feng, MD J. Haley, MD D. Talreja, MD C. Tei, MD WM Soo, MD JH Yang, MD J. Dal-Bianco, MD G. Achyraya, MD 2018 MFMER 3712003-50 25

Questions & Discussion 2018 MFMER 3712003-51 26