Cardiac Catheterization Hemodynamics Constrictive Pericarditis and Restrictive Cardiomyopathy Bassam Mushannen, MD King Fahad Specialist Hospital-Dammam KFSH-D

Anatomy Lt. Atrium is not completely intrapericardial All other cardiac chambers are intrapericardial Pulmonary Veins are completely intrathoracic

The pericardium Two-layered sac: A thin film of fluid (~ 50 ml). Inner serosal layer (visceral pericardium) adhering to outer wall of heart Reflected at the level of the great vessels Joins the tough fibrous outer layer (parietal pericardium ). A thin film of fluid (~ 50 ml). Pulmonary Trunk PL VL

Function of pericardium Fixes heart within mediastinum and limits its motion; Prevents extreme dilatation of heart during sudden rises of intracardiac volume A barrier to limit spread of infection from the adjacent lungs. But patients with complete absence of the pericardium (congenitally or surgically) generally do fine without it ? actual physiologic importance. *********************

Pathology of Constrictive Pericarditis Fibrotic, thickened, and adherent pericardium  restriction of diastolic filling of the heart. An initial episode of acute pericarditis (may be subclinical): Organisation & resorption of effusion Fibrous scarring and thickening of pericardium Obliteration of the pericardial space Uniform restriction of filling of all heart chambers. Calcium deposition  stiffening of pericardium.

Pathology of Constrictive Pericarditis Scarring and loss of normal elasticity of pericardial sac  § restriction of ventricular filling in mid and late diastole  majority of ventricular filling occurs rapidly in early diastole and ventricular volume does not increase after the end of early filling period. Restrictive cardiomyopathy: Non-dilated rigid ventricle  Severe diastolic dysfunction and restrictive filling  Hemodynamics similar to CP.

CP and Restrictive Cardiomyopathy Both Share: diastolic dysfunction Elevated diastolic pressures abnormal ventricular filling Decreased end diastolic volume (EDV). Differentiation important  Different management. Diagnosis: Mostly: Clinical and conventional tests. Others: May need Biopsy Surgical exploration

Pathophysiologic Similarity of: Restriction and Constriction Abnormal increase in ventricular pressure  impeding filling of RV & LV To NL EDV Constriction Restriction Myocardial Disorder Pericardial Disorder

Differences Constriction Restriction Myocardial compliance is NL Ab-Nl Myocardial compliance No impedance to Diastolic EARLY FILLING Impedance to filling increases throughout the diastole Total cardiac volume is fixed by Non-compliant pericardium Pericardium is compliant Septum is non-compliant Atria are able to empty into the ventricles, though at higher Press. proportional LV filling with atrial contraction  Atrial enlargement Marked Respiratory effect of RV on LV Minimal Respiratory effect of RV on LV ***********

Etiology of Constrictive Pericarditis Idiopathic: ~ 50% Others: Tuberculous: ~ 15% Post-viral pericarditis Post-surgical; Trauma Mediastinal Irradiation ESRD treated with HD Neoplastic pericardial infiltration Fungal and Parasitic P. Incomplete treatment of purulent pericarditis Post MI pericarditis (Dressler syndrome) Epicardial ICD patches implantation Pulmonary Asbestosis Methysergide Sarcoidosis Changing Etiology

Etiology of Restrictive CMP

TB Chronic Constrictive Pericarditis

CT volume rendered image : dense egg-shell calcification of the pericardial mimicking "snow fall on mountain"

Importance of CP Although uncommon Potential surgical cure. Last 15 years, declining incidence of TB pericarditis increase in therapeutic mediastinal radiation and cardiac surgery. ********************

Diagnosis

History Prior history of pericarditis, trauma, cardiac surgery, or a systemic disease (TB, connective tissue disease, malignancy)  CP A history of infiltrative disease that may involve the heart muscle (e.g., amyloidosis, sarcoidosis) RCMP Prior thoracic radiation treatment can result in either constrictive pericarditis, restrictive cardiomyopathy, or Combination of both constrictive pericarditis and restrictive cardiomyopathy.

Clinical Features - Symptoms and signs Reduced cardiac output: fatigue, hypotension, reflex tachycardia Elevated systemic venous pressure (Rt. Heart Failure) JVP distension, hepatomegaly with marked ascites and peripheral edema. Pulmonary venous congestion exertional dyspnea, cough and orthopnea Chest pain typical of angina Under-perfusion of the coronary arteries or compression of an epicardial coronary artery by the thickened pericardium.

Physical examination JVP: deep, steep Y descent. Elevated jugular venous pressure (JVP). ◊ JVP: deep, steep Y descent. constrictive pericarditis, restrictive cardiomyopathy, TR with enlarged compliant RA, or Rt. heart failure (e.g., RV infarction or PH). Kussmaul's sign (lack of inspiratory decline in JVP) Pulsus paradoxus (rare in classic CP) Peripheral edema Ascites and hepatomegaly Pleural effusions. Pericardial knock (50%) in CP. Not in restrictive cardiomyopathy. Audible S3: in RCMP; abrupt cessation of rapid ventricular filling. Not usually present in CP.

Diagnostic Testing 2-D and Doppler Echo: rule out other causes of right heart failure differentiation between CP and RMD may be difficult. CT and MRI can help in detecting an abnormal pericardium, provide anatomical information But not pathophysiological abnormality. Patients with surgically proven CP may have a normal-appearing pericardium on imaging studies (Talreja  D.R., Edwards  W.D., Danielson  G.K.;  et al. Circulation 108 2003 1852-1857). Or Pericardial thickness without constriction: after radiation therapy or prior cardiac operation. ********************

Cardiac Cath. Hemodynamics

Cardiac Catheterization Not necessary for patients with typical CP:  Classic clinical presentation Typical features on noninvasive testing: restrictive mitral inflow velocity, typical respiratory changes in transmitral and hepatic vein Doppler velocities normal to increased (e’) mitral annular tissue velocity. Indicated: If still a question of diagnosis after a comprehensive Clinical Noninvasive evaluation.

Cardiac Cath. Hemodynamics High-Fidelity Micromanometers: § Useful for studies of Cardiac Mechanics Combined with quantitative volume measurements to examine chamber function. Volumetric data acquired by: ventricular angiography, simultaneous echocardiography. Standard fluid-filled catheter systems: Pressure data is sufficient for most clinical hemodynamic studies Many artifacts and suboptimal frequency-response to accurately assess ventricular properties in research studies.

Measurements Obtain Rt. and Lt. cardiac pressure waveforms. These measurments are only Possible using High-fidelity Micromanometer systems . Measurements Obtain Rt. and Lt. cardiac pressure waveforms. All right-sided pressure recorded simultaneously with LV pressure. Pressures recorded during: normal quiet respiration (for measurement of end-expiratory pressures) and exaggerated respiration Volume loading of 1 liter NS if on diuretics and RAP (<15 mm Hg). Overdrive pacing if in A. Fib RAP, PASP, RVEDP, PCWP, LVEDP, & height of rapid filling wave (RFW). ********************

Traditional Criteria for CP Diastolic equalization of Pressures: LVED – RVEDP ≤ 5 mm Hg Narrow RV Pulse Pressure: RVEDP/RVSP > 1/3 (33%) Lack of significant pulmonary HTN: SPAP < 55 mm Hg Dip and Plateau Pressure (Square Root Sign): Height of LV rapid filling wave (RFW) > 7 mm Hg Kussmaul’s Sign: Inspiratory fall in mean RA pressure < 3 mm Sometime elevation of mean RA pressure with inspiration

1- RV and LV Tracings In Systole: 1) RV & LV diastolic pressures In diastole: 1) RV & LV diastolic pressures - Elevated - Equalized 4) Square root sign Rapid filling wave (RFW) due to tithering effect of pericardium, pulling the ventricular muscle back to its diastolic configuration Muscle is compliant  steep drop in early diastole Because of restraint  RFW, then plateau 2) Pulse Pressure of RV > 1/3 « In Systole: 3) SRVp < 55 mmHg RV SP=60 RV DP=30 RVDP/RVSP=30/60=1/2 (50%) (>1/3; >33%)

RV, LV pressure tracings - rapid pressure deceleration (dip) § rapid filling wave (RFW) pressure equalization of RV and LV (plateau) Square Root Sign Stawowy P et al. Circ Cardiovasc Imaging 2008;1:173-174

What Does it say?

2- RA pressure tracings Elevated RA pressure Sharp, deep Y descent a v Stawowy P et al. Circ Cardiovasc Imaging 2008;1:173-174 a v c Elevated RA pressure Sharp, deep Y descent

RA and LV pressures RA and LV represent inflow and outflow of heart In CP: < 5 mm Hg difference in diastole Sometimes: Steep X and Y descents  “W” shape of RA tracing a v a x y

Kussmaul Sign ? (5) Deep inspiration: - ve pressure in intrathoracic IVC + ve pressure in intraabdominal IVC  PG in IVC  pulling blood to chest. Normal pericardium allows transmission of this PG to cardiac chambers  flow to RA. In CP: less transmission of – ve pressure  less drop in RA pressure in inspiration < 3 mmHg (5)

Hepato-jugular Reflux

Accuracy ********************

Assessment of Respiratory Variation

Dissociation of Intrathoracic and Intracardiac Pressures 3- LV and PCWP Tracing Dissociation of Intrathoracic and Intracardiac Pressures Hatle LK, et. al. Circ. 1989;79357-370 v v v v

3- LV and PCWP Tracing Catheter in PA: Outside heart Pressure changes with respiratory variation preserved (PA & PCWP). Intracardiac (LV) p less changes  Drop in PCWP-LV diastolic PG during inspiration  flow into LV. Insp. Exp.

Dissociation of Intrathoracic and Intracardiac Pressures

Dynamic, Respiratory Variations in CP In Inspiration: dissociation of intrathoracic and intracardiac pressures  increase in ventricular interaction  Increasing RV filling Decreasing LV filling. Alternative hypothesis for discordance: increase in inspiratory flow to RV  decreased transseptal gradient  decrease in early diastolic suction of LV. Doppler Echo: transmitral and hepatic vein flows. §

PCWP and LV- Restriction ? PCWP and LV- Restriction Simultaneous drop in LVDP and PCWP with respiration Negative intrathoracic pressure is transmitted to Intracardiac chambers

4- RV and LV pressure Tracing Exp. De-Coupling Insp. Coupling Insp. Exp. Systolic LV & RV pressure Respiratory Discordance

Discordance

Concordance

Constrictive Pericarditis Restrictive Cardiomyopathy Ventricular Interdependence During Respirations Constrictive Pericarditis vs. Restrictive Cardiomyopathy Constrictive Pericarditis (LV and RV discordant) Restrictive Cardiomyopathy (LV and RV concordant) Hurrell et al, Circulation 1996; 93:2007

Simultaneous Echo and Cath Data Effect of Inspiration: Constriction Simultaneous Echo and Cath Data Insp. Expir. Inspir. Expir. Inspir. Expir. PCWP No proportionate decrease in LV diastolic pressure Decreased transmitral gradient  Transmitral flow RV SV LV SV

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Constrictive Pericarditis in the Modern Era: Novel Criteria for Diagnosis in the Cardiac Catheterization Laboratory J Am Coll Cardiol. 2008;51(3). Deepak R. Talreja,; Rick A. Nishimura,; Jae K. Oh,; David R. Holmes Criterion Sensitivity (%) Specificity (%) Positive Predictive Accuracy (%) Negative Predictive Accuracy (%) LVEDP − RVEDP ≤5 mm Hg 46 54 58 40 PASP <55 mm Hg 90 29 73 66 RVEDP/RVSP >1/3 93 71 79 LV RFW >7 mm Hg 45 44 62 42 Inspiratory decrease in RAP <5 mm Hg 37 39 Systolic area index >1.1 97 100 95

“Classical” Criteria of CP vs. RCMP J Am Coll Cardiol. 2008;51(3):315-319. doi:10.1016/j.jacc.2007.09.039 ≤ 5 ≤ 55 > 33% > 7

Pressure Waveforms During Exaggerated Respiration The area under ventricular pressure curve was used to determine change in relative volumes of LV and RV a better determinant of beat-to-beat stroke volume than the peak pressure alone The systolic area index: RV area (mm Hg × s) to LV area (mm Hg × s) in inspiration versus expiration.

(A) constrictive pericarditis. LV and RV Pressure From 2 Patients During Expiration and Inspiration (A) constrictive pericarditis. During inspiration: increase in area of RV pressure curve (orange) compared with expiration. Enhancement of LV-RV coupling. area of LV pressure curve (yellow) decreases in height and width as compared with expiration. B) restrictive myocardial disease decrease in area of RV pressure curve (orange) as compared with expiration. area of the LV pressure curve (yellow) is unchanged as compared with expiration. CP 3 1 LV-RV coupling 2 RP 2 2 1

97 100 95 1.1 Systolic area index >1.1 J Am Coll Cardiol. 2008;51(3):315-319. doi:10.1016/j.jacc.2007.09.039 1.1 This ratio is a measurement of the degree of ventricular interaction.

Another example

Other Cath. And Coronary Angiogram Features

Pericardial Calcification

RA Angiogram Abnormal contour of RA Thickening of heart border Attenuated convex edge Thickening of heart border

Coronary Angiogram

LV Angiogram Needle broke off during IV injection, embolized to right heart  cardiac perforation entering pericardium  chronic constrictive pericarditis, presented as congestive heart failure. Pericardectomy and removal of the foreign body was successful. ********************

Management CCP is a progressive irreversible disease Minority survive for years with modest elevated JVP and peripheral edema controlled by diet and diuretics. Drugs that slow HR, eg beta blockers and Ca2+ channel blockers should be avoided as mild sinus tachycardia is a compensatory mechanism. The majority of patients become progressively more disabled and subsequently suffer the complications of severe cardiac cachexia.

Surgical & Pathology Findings in CP obliteration of pericardial space bulging of heart upon incision of pericardium, abnormal pericardial thickening and/or calcification post-operatively - decrease in RA - increase in cardiac index.

EFFUSIVE CONSTRICTIVE PERICARDITIS (ECP) Rare clinical syndrome Pericardial effusion and pericardial constriction, Constrictive hemodynamics persistent after p. effusion is removed. Mechanism: visceral pericardial constriction  Visceral pericardiectomy Pericardial effusions vary in size and age. Type: Transudative, exudative, sanguineous, or chylous. An effusion for months to years may evolve into ECP Any point of time; from occurrence of P. Effusion to development of constriction. Symptoms due to limitation of end-diastolic volume. pericardial effusion/ tampnade, And - pericardial constriction.

EFFUSIVE CONSTRICTIVE PERICARDITIS Etiology Idiopathic factors Irradiation Cardiac surgery Neoplasm - Most commonly lung, breast, or hematologic Infectious disease - Particularly in immunocompromised states most commonly tuberculosis and fungal disease, Streptococcus species reported Myocardial infiltration Connective tissue disease Uremia

EFFUSIVE CONSTRICTIVE PERICARDITIS (ECP) Hancock in 1960: helped in current understanding of ECP. Hancock, EW. Subacute effusive-constrictive pericarditis. Circulation 1971. Hancock, EW. On the elastic and rigid forms of constrictive pericarditis. Am Heart J 1980. 24 patients undergoing pericardiectomy for CP, 9 had concurrent effusion. 6 of 9 had hemodynamic studies before surgery. Sagrista-Sauldea et al in 2004; prospective study of 1184 patients with pericarditis, 6.9% of 218 patients with tamponade had confirmed EFP. Sagrista-Sauleda J, Angel J, Sanchez A, et al. Effusive-constrictive pericarditis. N Engl J Med. 2004

Clinical clues to ECP: Pulsus paradoxus rare in classical CP (absence of transmission of the inspiratory decline in pressure to right heart chambers)    Absence of pericardial knock (effusion) The Y descent less dominant than expected Kussmaul's sign frequently absent

PA and Ao. Tracing ? SPAP> 55 mm Hg Pulsus Paradoxus (Ao.) Insp. Exp. Pulsus Paradoxus

EFFUSIVE CONSTRICTIVE PERICARDITIS (ECP) Diagnosis: during pericardiocentesis in patients considered to have uncomplicated cardiac tamponade. Despite lowering pericardial pressure to normal, persistence of elevated RA pressure development of y decent dominance impaired respiratory variation. § because of visceral constrictive component of the syndrome  persistent elevation and equalization of intracardiac diastolic pressures.

ECP post pericardiocentesis: Pericardial pressure normalized Y ECP post pericardiocentesis: Pericardial pressure normalized RA pressure remains elevated with features of CP (deep Y descent).

Persistently elevated RA pressure after pericardiocentesis Cardiac tamponade complicating right heart failure or tricuspid regurgitation.

Conclusion CP still challenging diagnosis for clinicians, especially when both myocardial and pericardial disease present. Although noninvasive testing help in the diagnosis of CP, some cases remain unclear. Dynamic respiratory changes reflecting the enhancement of ventricular interaction at cardiac catheterization is most useful. The ratio of RV to LV systolic pressure X area during inspiration vs. expiration (systolic area index) is a novel measurement of enhanced ventricular interaction.

End

Cardiac Catheterization Typical hemodynamic response in CP: early rapid filling and equalization of end-diastolic pressures in all 4 cardiac chambers but also in RMD. More severe pulmonary hypertension greater difference between LVEDP and RVEDP in patients with RMD. Though statistically significant differences in overall values of these criteria, but the – ve and + ve predictive value were of limited.

RA and RV Tracing

Pressure Waveforms During Exaggerated Respiration Peak inspiratory beat was selected as the systolic impulse preceded by the lowest early diastolic nadir of the LV pressures. Selection of the peak inspiratory beat required that the early diastolic nadir was at a minimum for the diastolic filling period before and after the systolic pressure contours. Peak expiratory beat was selected as the systolic impulse that was preceded by the highest early diastolic nadir of the LV pressure. In CP: inspiratory decrease in the LV volume and enhancement of ventricular coupling (obligatory increase in RV volume) The LV pressure curves become smaller in terms of both the height and width of curve, The RV pressure curve becomes larger during peak inspiration. Previously, RV index based on RV peak systolic pressure variation between inspiration and expiration was used as a measure of ventricular coupling. We subsequently found that changes in the peak pressure alone were not sensitive enough to detect all patients with CP. Therefore, the area under the ventricular pressure curve was used to determine the change in the relative volumes of the LV and RV a better determinant of beat-to-beat stroke volume than the peak pressure alone The systolic area index: the ratio of RV area (mm Hg × s) to LV area (mm Hg × s) in inspiration versus expiration.

Constrictive Pericarditis in the Modern EraNovel Criteria for Diagnosis in the Cardiac Catheterization Laboratory Deepak R. Talreja, MD, FACC; Rick A. Nishimura, MD, FACC; Jae K. Oh, MD, FACC; David R. Holmes, MD, FACC J Am Coll Cardiol. 2008;51(3):315-319. doi:10.1016/j.jacc.2007.09.039 Abstract Objectives  This study sought to determine the clinical utility of a new catheterization criterion for the diagnosis of constrictive pericarditis (CP). Background  The finding of early rapid filling and equalization of end-diastolic pressures obtained by cardiac catheterization are necessary for the diagnosis of CP, but these findings are also present in patients with restrictive myocardial disease (RMD). Enhanced ventricular interaction is unique to CP. Methods  High-fidelity intracardiac pressure waveforms from 100 consecutive patients undergoing hemodynamic catheterization for diagnosis of CP versus RMD were examined. Fifty-nine patients had surgically documented CP and comprised group 1; the remaining 41 patients with RMD comprised group 2. The ratio of the right ventricular to left ventricular systolic pressure-time area during inspiration versus expiration (systolic area index) was used as a measurement of enhanced ventricular interaction. Results  There were statistically significant differences in the conventional catheterization criteria between CP and RMD, but the predictive accuracy of any of the criteria was <75%. The systolic area index had a sensitivity of 97% and a predictive accuracy of 100% for the identification of patients with surgically proven CP. Conclusions  The ratio of right ventricular to left ventricular systolic area during inspiration and expiration is a reliable catheterization criterion for differentiating CP from RMD, which incorporates the concept of enhanced ventricular interdependence.