Presentation on theme: "Constrictive Pericarditis"— Presentation transcript:
1Constrictive Pericarditis Heiko J. Schmitt, M.D., Ph.D.HJS
2Outline Case presentation Pericardial anatomy HJSCase presentationPericardial anatomyClinical presentation and examCT, MRI, and echocardiographic findingsHemodynamicsOutcome after pericardectomy
3Case Presentation - History HJS67 year old man presents with a 2 months history of SOB, non-productive cough and b/l swelling of his lower extremity.occasional wheezing and more SOB after mealssymptoms started after a hunting tripno constitutional symptomsno lung disease or heart disease, occupational exposure, allergies, smoking historyHistory is remarkable for GERD and a remote pneumoniaNEJM 2004, Vol 351,
4Case Presentation - Exam HJSBecause of worsening symptoms admissionPatient now reported orthopneaafibrile, BP 150/86, HR 108, RR 28expiratory wheezes over both lungsno M/R/G, distant heart sounds2+ pitting leg edema b/lJVP not visualizedHis weight is 109 kgNEJM 2004, Vol 351,
5Case Presentation - Initial Tests HJSLabs were unremarkable including CBC, BMP, CPK, Troponin, LFTsph 7.47, pCO2 34, pO2 64CXR: Cardiomegaly and mildly increased vasculatureEKG: showed diffuse T-wave inversion, low voltage and sinustachycardiaEcho: nl LV size and function, RV nl. size but thickened, no valvular diseaseDobutamin-stress: no evidence for ischemiaNEJM 2004, Vol 351,
6Case Presentation - Initial Tests HJSSpiral-CT: no evidence for PE, right sided pleural effusion, no infiltratePFTs: FVC 2.5l (59%), FEV1 1.9l (65%), ratio 76%, TL 5.4l (85%).Sleep-Study: 21 apneic, 12 hypopneic episodes per hour, desaturation to 83%.Started on nocturnal CPAP and diureticsWorsening of symptomsNEJM 2004, Vol 351,
7Case Presentation - Final Tests HJSNo pulmonary disease but thickened pericardiumMild cardiomegalyincreased interstitial markingsNEJM 2004, Vol 351,
8Case Presentation - Heart Catheter HJSHemodynamic measurements were consistent with the diagnosis of constrictive pericarditisElevated and equal enddiastolic pressuresDiscordant peak sytolic pressuresThe patient underwent pericardectomy showing fibrosed pericardium and did well.NEJM 2004, Vol 351,
9Pericardium - AnatomyHJSForms a sac enclosing the origin of the aorta, pulmonary artery, Pulmonary veins, venae cavaeligamentous attachments to sternum, vertebral column, and diaphragmligaments help to fix the heart anatomically and prevent excessive movementsOtto, Textbook of clinical Echocardiography, 3rd ed.
10Pericardium - Anatomy Outer fibrous layer HJSOuter fibrous layerInner parietal layer forming a serous membrane composed of a single layer of mesothelial cellsVisceral layer is firmly attached to the surface of the heart
11Pericardium - AnatomyHJSMarked increase in surface area of the visceral pericardium by microvili and cilia.Microvilli and cilia permit movement and fluid transportPericardial fluid is an ultrafiltrate of plasma (nl 50ml)contains phospholipids that serve as a lubricant.
13Constrictive Pericarditis - Etiology HJSIdiopathic 42% (earlier inapparent viral pericarditis)Cardiac surgery 29%Radiation therapy to the mediastinumRenal failureConnective tissue diseaseTB (still highest in developing countries)less common in children (suspect TB)Braunwald, Heart Disease 4th ed., 1992
14Constrictive Pericarditis - Pathophysiology HJSFibrosed or calcified pericardium restricts diastolic filling of all 4 chambersconstriction leads to elevated and equilibrium of the diastolic pressuresIn early diastole filling is unimpaired => abnormally rapid fillingfilling is abruptly halted when cardiac volume meets the limits determined by the stiff pericardiumVirtually all filling occurs during early diastoleBraunwald, Heart Disease 4th ed., 1992
16Constrictive Pericarditis - Exam HJSKussmaul’s sign (increase of RA pressure during inspiration).described 1873 in combination with pulsus paradoxus in a patient with constrictive pericarditis.In Mayo clinic series found in 21% of patients referred for pericardectomy.Pulsus paradoxus (decrease in systolic pressure > 10 mmHg) infrequently found in constrictive pericarditisLancet 2002; 359,
17Constrictive Pericarditis - Exam HJSKussmaul’s sign (increase of RA pressure during inspiration).described 1873 in combination with pulsus paradoxus in a patient with constrictive pericarditis.In Mayo clinic series found in 21% of patients referred for pericardectomy.Pulsus paradoxus (decrease in systolic pressure > 10 mmHg with inspiration) found in 20% in constrictive pericarditisLancet 2002; 359,
18Constrictive Pericarditis - Exam HJSPericardial knock heard over the left sternal border.Corresponds with the sudden cessation of ventricular filling.Earlier than S3 and higher frequencymay be confused with opening sound of mitral stenosis.Braunwald, Heart Disease 4th ed., 1992
20Constrictive Pericarditis - CT/MRI HJSMay show thickened pericardiumMay exclude other abnormalities.Normal pericardium however does not exclude restrictive pericarditis.Nishimura, Heart 2001, 86,
21Constrictive Pericarditis - Echocardiography HJSUseful in the differential diagnosis of constrictive pericarditisExclusion of other causes of right sided heart failure (valve disease, left sided heart failure, pulmonary hypertension).Thickened ventricular walls with unusual texture found in restrictive and infiltrative CM are usually not found in restrictive pericarditisNishimura R., Contrictive pericarditis in the modern era: a diagnostic dilemma, heart 2001;86:619-23
22Constrictive Pericarditis - 2D Echo HJSPericardial thickening.abrupt posterior motion of the ventr. septum in early diastoleabrupt anterior motion following atrial contractioninspiratory septal shiftdilated inf. vena cavaOtto, Textbook of clinical Echocardiography, 3rd ed.
24Constrictive Pericarditis - Doppler HJSDoppler echocardiography provides useful information in patients with constrictive pericarditis.The pathophysiologic features of constrictive pericarditis (diastolic filling) are assessed by the analysis ofthe mitral inflowhepatic vein flowpulmonary vein flowSimilar flow pattern can be found in restrictive cardiomyopathy
25Constrictive Pericarditis - Doppler HJSavxyCorresponds with right atrial fillingProminent a-wavedeep y-descentHigh initial E velocityshort deceleration timereduced velocity at atrial contractionDecrease in E velocity during inspirationOtto, Textbook of clinical Echocardiography, 3rd ed.
26Constrictive Pericarditis - Echocardiography HJSA comprehensive echocardiogram may be considered diagnostic in a subset of patients with classical findingsseptal bouncerespiratory septal shifttypical doppler findings with respiratory variationpericardial thickeningHowever in up to 1/3 of the patients the echocardiographic findings are equivocalcombination of pericardial and myocardial diseaseCOPDAFIBNishimura R., Contrictive pericarditis in the modern era: a diagnostic dilemma, heart 2001;86:619-23
27Constrictive Pericarditis - Catheterization HJSConfirm presence of restrictive physiology and assess severitydifferentiating constrictive pericarditis from restrictive cardiomyopathyexclude major coexisting caused such as severe pulmonary hypertensionexclude rare causes of valvular constriction or pinching of coronary arteries.Grossman Cardiac catheterization, Angiography, and Intervention, th edition
28Constrictive Pericarditis - Catheterization HJSElevated RA pressurevery prominent Y decent indicating rapid RA emtyingNadir of Y descent corresponds to the abrupt cessation of early diastolic ventricular fillingCharacteristic W or M formavGrossman Cardiac catheterization, Angiography, and Intervention, th edition
29Constrictive Pericarditis - Catheterization HJSLeft and right ventricular pressures should be recorded simultaneously at the same scaleRV and LV diastolic pressures are elevated and equal within 5 mm or lessdip and plateau configuration of RV and LV wave formsall filling occurs during early diastoletachycardia may obscure some of the findingsBraunwald, Heart Disease 4th ed., 1992
30Constrictive Pericarditis - Catheterization HJSIncrease of RA pressure during inspirationKussmaul’s signGrossman Cardiac catheterization, Angiography, and Intervention, th edition
31Constrictive Pericarditis - Restrictive CM HJSOtto, Textbook of clinical Echocardiography, 3rd ed.
32Constrictive Pericarditis - Restrictive CM HJSVentricular interdependence not seen in restrictive cardiomyopathyDiscordant change in left and right peak systolic pressure with repiratory changes.Grossman Cardiac catheterization, Angiography, and Intervention, th edition
34Constrictive pericarditis Cause-specific survival after pericardectomy HJSPericardectomy at the Cleveland clinic foundation January1977-December 2000, 163 patientsIdiopathic 75 (46%)Postsurgical 60 (37%)Irradiation 15 (9%)Miscellaneous13 (8%)Perioperative MortalityLong term SurvivalJ Am Coll Cardiol 2004;43:
35Constrictive pericarditis Cause-specific survival after pericardectomy HJSOverall perioperative mortality 6.1%Idiopathic 2.7%Postsurgical 8.3%Irradiation 21.4%Miscellaneous0%J Am Coll Cardiol 2004;43:
36Constrictive pericarditis Cause-specific survival after pericardectomy HJSIdiopathic 88% 7-year survivalpostsurgical 66% 7-year survivalirradiation 27% 7-year survivalJ Am Coll Cardiol 2004;43:
37Constrictive Pericarditis - Summary HJSContrictive Pericarditis is a rare disease often posing a diagnostic challenge.Echocardiography is an essential part in the diagnostic process and the diagnosis can be made if the classical fechocardiographic features are present.Outcome after pericardectomy is excellent except in patients with irradiation as cause.