1. Hemodynamic Conference Eckhard Alt, M.D. Holger Salazar, M.D. Robert Smith, M.D., M.Sc. Tulane University School of Medicine Cardiac Cath Conference December 23, 2003

2. Outline Right Heart Catheterization Overview Review of Waveform Analysis Practice Case Case Presentation with RHC Results Discussion of Differential Diagnosis Review of Echocardiographic Findings and Follow up Discussion

3. Right Heart Catheterization Measures Central Venous Pressure/Right Atrial Pressure Measures RV Pressures and PA Pressures Gives Indirect Measure of Left Atrial Pressure (PCWP) Avoids Septal Puncture Estimates Cardiac Output Quantifies Oxygen Utilization Useful in Diagnosis of Shock Etiology Useful for Peri-Operative Volume Management

4. Pressure Waveforms

12. Practice Case

13. RA

14. RV

15. PA

16. PCW

17. Diagnosis?

20. M5

21. M12

22. Diagnosis Non-Ischemic Cardiomyopathy

23. Case Presentation CC is a 19 yo AAM with no significant PMHx who presented with a 2 year history of progressive abdominal distention. Pt. reported that the abdominal distention had particularly worsened during the six months prior to presentation and he presented to the medicine clinic at the insistence of his family. He reported that he was active in sports and denied LE edema, SOB, PND, and orthopnea. In fact, he reported that, aside from his worsening abdominal distention, he generally felt well. He was admitted from the clinic for workup of his abdominal distention.

24. PMHx: None Medications: None Family History: No family h/o heart disease Social History: Denies EtOH, Tobacco, Drugs. One lifetime sexual partner

25. Physical Exam 123/72 62 16 97.2 Comfortable, NAD JVD present at 9 cm, + hepatojugular reflux nlS1S2, 2/6 HSM  apex Decreased breath sounds at bilateral bases Abd distended with + fluid wave. Liver was palpable 3 cm below the costal margin and the spleen tip was palpable No LE edema

26. Labs Na 134 K + 3.9 Cl - 100 HCO3 - 27 BUN 13 Cr 0.9 Glucose 89 Ca 8.9 LDH 118 AST 37 ALT 11 AP 75 TP 7.9 Alb 3.0 TB 1.8 CK 21 CKMB 0.4 Troponin <0.05 TSH 3.17

27. Labs (cont) WBC 12.2 Hgb 12.2 Hct 36.6 Plt 190 MCV 90 Neutrophils 70% Lymphocytes 22% Basophils 0% Eosinophils 1% Monocytes 7% INR 1.4 PTT 35.6 Blood Cultures Drawn

28. Ascites Fluid Clear and Yellow WBC’s 21 RBC’s 453 Albumin 2.6 TP 4.8 LDH 74 Glucose 104 Cholesterol 20 Gram Stain and cultures sent Cytology sent

29. ECG

30. CC CC

35. During this admission, a TTE was performed and showed a large pericardial effusion without evidence of tamponade (the study has been lost). Blood cultures were negative for bacterial infection and fluid cultures were smear negative and culture negative for AFB, fungus and bacteria Clinically, he looked well and was discharged by the primary service for outpatient workup. He failed to keep his appointments and presented to the ER with SOB approx. 1 month after discharge. During this second admission, workup included echocardiography, left and right heart cath. The echocardiographic findings will be discussed at the end of the case.

36. C5

37. C8

38. C2

39. RA

40. RV

41. PA

42. PCW

43. RV/LV

44. Differential Diagnosis Constrictive Pericarditis Restrictive Cardiomyopathy

45. Etiologies of Constrictive Pericarditis Common Causes - Idiopathic -Infection Bacterial: TB Fungal: Histoplasmosis, Coccidiomycosis Viral: Coxsackie Parasitic: Amebiasis, Echinococcus -Drugs -Neoplastic Lymphoma, Melanoma, Primary Mesothelioma, Breast & Lung cancer -Following Cardiac Surgery -Connective Tissue Disease RA, SLE, Scleroderma, Dermatomyositis -Trauma -Renal Failure -Radiation -AICD/Pacer placement Uncommon causes -Sarcoidosis -Post MI -Asbestosis -Amyloidosis -Drug Induced Lupus -Acute Rheumatic Fever Rare Causes -Actinomycosis -Asbestosis -Whipples Disease -Lassa Fever -Sclerotherapy of Esophageal Varices

46. Restrictive Cardiomyopathy Primary RCM - Loeffler’s cardiomyopathy -Idiopathic RCM -Endomyocardial Fibrosis Secondary RCM Infiltrative Noninfiltrative - Sarcoidosis -Fabry’s Disease -Amyloidosis -Hemochromatosis -Post Radiation -Glycogen Storage Therapy Disease -Gaucher’s Disease -Scleroderma -Hurler’s Disease -Pseudoxanthoma Elasticum -Storage Disease

47. Echocardiographic Presentation Holger Salazar, M.D.

49. Chene3-23

50. Chene3-8

51. Chene3-9

52. Chene3-3

53. Chene3-13

54. Chene3-12

55. Chene3-preop,continuing 14

56. Chene3-14

57. Chene3-preop, continuing 5

58. Chene3-preop, continuing 9

59. Chene3-11

60. Chene3-5

61. Chene3-20

62. Chene3-preop, continuing 1

63. Chene3-preop, continuing 4

64. Diagnosis Constrictive Pericarditis

65. Follow Up Pericardial biopsy (done during pericardectomy) showed dense fibrous tissue with focal dystrophic calcification and mesothelial hyperplasia The pericardium was densely calcified and adherent Epicardial biopsy showed dense fibrous tissue without evidence of active inflammation or malignancy Pericardial fluid was bloody and contained atypical mesothelial cells Pericardial fluid was smear and culture negative for AFB Pericardial fluid was smear and culture negative for bacteria and fungi Serum ANA was negative PPD was negative HIV was negative

66. Follow Up (cont) The underlying etiology remains unclear The patient has developed refractory atrial fibrillation with RVR Anticoagulation has been complicated by a lower GI bleed He failed to improve after pericardectomy, and has recently been referred to transplant clinic