1. Cath Conference August 6, 2008 Priya Pillutla, M.D. Kimble Poon, M.D.

2. History 34 y/o M, no PMH 34 y/o M, no PMH 2 months prior to admission - URI 2 months prior to admission - URI URI resolved but +SOB, LE edema URI resolved but +SOB, LE edema OVMC – Dx’d with pericarditis and R heart failure OVMC – Dx’d with pericarditis and R heart failure NSAIDs: no improvement NSAIDs: no improvement Repeat TTE: thickened pericardium Repeat TTE: thickened pericardium Transferred here for further management Transferred here for further management Priya Pillutla, M.D.

3. Still complains of SOB and LE edema Still complains of SOB and LE edema No fevers or constitutional symptoms No fevers or constitutional symptoms Meds – Ibuprofen Meds – Ibuprofen NKDA NKDA Social - +tobacco Social - +tobacco Priya Pillutla, M.D.

4. Physical Exam BP 130/84, HR 80, RR 14, sat 100% RA Morbidly obese JVP 15 cm Normal carotid upstrokes RRR nl s1/s2. +S3 +pericardial knock Lungs clear Lower extremity edema Priya Pillutla, M.D.

5. Electrocardiogram

6. Transthoracic Echocardiography Priya Pillutla, M.D.

7. Echocardiographic evidence for pericardial constriction

8. Thickened pericardium and tram-tracking Ventricular interdependence Septal bounce Respiratory variation of inflow velocities Normal or elevated mitral annulus motion Resolution after therapy

9. Thickened pericardium and tram-tracking Pericardial thickness >3mm is abnormal but not sensitive or specific for constriction Tram-tracking: during diastole, the parietal pericardium and visceral pericardium are straight and fixed This is in contrast to normal pericardial movement and cardiac tamponade

11. Tram-tracking in pericardial constriction

13. Absence of tram-tracking in a patient with cardiac tamponade During diastole, the visceral pericardium expands outward as the ventricle fills

14. Absence of tram-tracking in a patient with no pericardial disease

15. Ventricular interdependence During inspiration, the RV is preferentially filled at the expense of the LV

17. During expiration, the LV fills at the expense of the RV

18. Septal bounce 2D manifestation of ventricular inter- dependence

20. Respiratory variation of inflow velocities MV variation >25% TV variation >40%

21. Peak 99 cm/s Trough 57 cm/s Difference 42 cm/s % variation 42/57 = 74%

22. Peak 80 cm/s Trough 38 cm/s Difference 42 cm/s % variation 42/38= 110%

23. Normal or elevated mitral annulus motion Because the lateral motion of the ventricle is constricted, motion along the basal to apical axis is exaggerated E’ > 7 is consistent with constriction

24. E’ = 17

25. Resolution after therapy Variation disappears after definitive therapy

26. Peak 110 cm/s Trough 105 cm/s Difference 5 cm/s % variation 5/105 = 5%

27. Peak 60 cm/s Trough 50 cm/s Difference 10 cm/s % variation 10/50 = 20%

28. Presence of effusion

29. Diagnosis Effusive-pericardial constriction

30. Right heart catheterization Priya Pillutla, M.D.

39. Summary Pericardial effusion Pericardial effusion Thickened pericardium Thickened pericardium Severely restricted cardiac motion Severely restricted cardiac motion Steep x and y descent on RA pressure waveform Steep x and y descent on RA pressure waveform Near equalization of diastolic pressures in all chambers Near equalization of diastolic pressures in all chambers Findings consistent with effusive-constrictive pericaditis Priya Pillutla, M.D.

40. Management Pericardiectomy was performed Pericardiectomy was performed Large effusion Large effusion Pericardial thickening especially adjacent to right ventricle Pericardial thickening especially adjacent to right ventricle Difficult dissection Difficult dissection Visceral pericardium removed up to the phrenic nerve laterally and the diaphragm inferiorly Visceral pericardium removed up to the phrenic nerve laterally and the diaphragm inferiorly Intraoperative TEE showed improved diastolic filling Intraoperative TEE showed improved diastolic filling Priya Pillutla, M.D.

41. At discharge: At discharge: Resolution of shortness of breath and edema Resolution of shortness of breath and edema Pericardial biopsy - nonspecific inflammation, thickening of the pericardium Pericardial biopsy - nonspecific inflammation, thickening of the pericardium Effusion – micro, chemistry negative Effusion – micro, chemistry negative Priya Pillutla, M.D.

42. Effusive-Constrictive Pericarditis First characterized by Hancock in 1971 First characterized by Hancock in 1971 Constriction caused by visceral pericardium in presence of tense pericardial effusion Constriction caused by visceral pericardium in presence of tense pericardial effusion Usually diagnosed after pericardiocentesis for tamponade Usually diagnosed after pericardiocentesis for tamponade Elevated RAP despite normal intrapericardial pressure Elevated RAP despite normal intrapericardial pressure In this case, mixed findings during RHC suggested diagnosis In this case, mixed findings during RHC suggested diagnosis Priya Pillutla, M.D.

43. NEJM, 2004

44. Priya Pillutla, M.D. NEJM, 2004

45. From Guide to Hemodynamic Data in the Coronary Care Unit (Sharkey) TamponadeConstrictionEff-Const Mean RAP 10-25 mmHg 10-30 mmHg RA waveform X>Y Y may be 0 X = Y or X < Y X = Y or X < Y RA/PCWPEqualEqualEqual PASP Normal/sl. ↑ 30-45 mmHg KussmaulAbsent 1/3 of cases Rare PulsusYes Pre-tap Priya Pillutla, M.D.

46. N = 15 (largest series to date) Priya Pillutla, M.D.

47. Diagnostic criteria: Diagnostic criteria: Tamponade that evolved into constriction (failure of RAP to fall by at least 50% or less than 10 mmHg) after reduction of intrapericardial pressure to 0 Tamponade that evolved into constriction (failure of RAP to fall by at least 50% or less than 10 mmHg) after reduction of intrapericardial pressure to 0 Methods Methods Complete pressure measurements obtained prior to and following pericardiocentesis (all chambers, IPP, femoral pulsus) Complete pressure measurements obtained prior to and following pericardiocentesis (all chambers, IPP, femoral pulsus) Pericardial fluid sent for chemistry, cyto, micro, AFB Pericardial fluid sent for chemistry, cyto, micro, AFB Priya Pillutla, M.D.

48. Treatment varied Treatment varied NSAIDs NSAIDs Avoided steroids Avoided steroids Pericardiectomy for constriction and severe/persistent heart failure Pericardiectomy for constriction and severe/persistent heart failure If milder heart failure, medical therapy to allow possible spontaneous resolution If milder heart failure, medical therapy to allow possible spontaneous resolution F/U – every 3 months for a year (if pericardiectomy) then q3-5 years F/U – every 3 months for a year (if pericardiectomy) then q3-5 years Priya Pillutla, M.D.

49. Results 15 patients met criteria (~1200 consecutive patients with pericarditis; prevalence 1.3%) 15 patients met criteria (~1200 consecutive patients with pericarditis; prevalence 1.3%) All had signs of R heart failure All had signs of R heart failure 2/3 had pulsus paradoxus 2/3 had pulsus paradoxus Effusions predominantly serosanguinous Effusions predominantly serosanguinous Priya Pillutla, M.D.

50. Management Inflammatory symptoms – NSAIDs Inflammatory symptoms – NSAIDs All patients – pericardiocentesis (13/15 had improvement) All patients – pericardiocentesis (13/15 had improvement) 7/14 had pericardiectomy for persistent R heart failure 7/14 had pericardiectomy for persistent R heart failure 4 idiopathic, 1 radiation, 1 TB, 1 postsurgical 4 idiopathic, 1 radiation, 1 TB, 1 postsurgical Nonspecific inflammation of the pericardium Nonspecific inflammation of the pericardium Priya Pillutla, M.D.

51. No pericardiectomy (n=8) No pericardiectomy (n=8) 3 spontaneous resolution 3 spontaneous resolution 4 neoplasm; 1 radiation pericarditis with LV dysfunction 4 neoplasm; 1 radiation pericarditis with LV dysfunction After complete workup of all patients: After complete workup of all patients: Idiopathic (7) Idiopathic (7) Neoplasm (4) Neoplasm (4) Radiation pericarditis (2) Radiation pericarditis (2) Postsurgical (1) Postsurgical (1) TB (1) TB (1) Other case series – bacterial infections, fungal Other case series – bacterial infections, fungal Priya Pillutla, M.D.

52. Importance of correct diagnosis: Importance of correct diagnosis: Visceral pericardium needs to be removed Visceral pericardium needs to be removed Dissection can be difficult/hazardous Dissection can be difficult/hazardous Can resolve spontaneously ~ can watch and wait if heart failure symptoms not severe Can resolve spontaneously ~ can watch and wait if heart failure symptoms not severe Priya Pillutla, M.D.

53. Video