Prof. R.V. Kumar & Dr.Sai Surbhai Discussant - Prof.Nitin Kabra

From WINCARS Association

46 yr old lady presented in Dec 2015 with complaints of acute onset of breathlessness.

2d echo done then revealed pericardial effusion. Drained under radiological guidance. Fluid analysis-inconclusive. ATT x 9 months.

Sept 2016-DOE and cough x 1 month.

HR-102/min BP-110/70mm Hg CVP-elevated RR-24/min SpO2-97% room air CVS-S1, S2 heard. RS-AEBE

Hb-10.8gm% TLC-9200 LFT-WNL S.Creat-0.78mg% S. Electrolytes-Normal

Increased cardio thoracic ratio.

Left cardio phrenic angle obscured.

Ill defined heart borders

Moderate pericardial effusion Significant Respiratory variation in Tricuspid

Inflow velocities. Mitral annular velocities show annulus

reversus. Mild PAH-RVSP=40mm Hg IVC-dilated Diastolic flow reversal in hepatic vein Large homogenous mass bt LA & aorta and

extending anteriorly surrounding aorta & PA

Cardiomegaly

Moderate pericardial effusion

Thickened pericardium

Thickened pericardium measuring

25mm-aorta 35mm-PA 30mm-RA 20mm-LV 18mm bt aorta and LA

Coronary angiogram

normal study

Cardiac catheterization

Diastolic equalization of pressures.

Constrictive Pericarditis with no PAH with normal Epicardial coronaries with Good LV function

Metabolically active soft tissue thickening of the pericardium

Involved the pericardial sinuses, encasing the aortic arch and pulmonary vessels.

Maximum thickness 52mm.

BY

WINCARS Association

Dr.Nitin K.Kabra MD,DM,FACC.

Prof &HOD Department of cardiology

Gandhi Medical College Chief Cardiologist Gandhi Hospital

Secunderabad, TS.

46y non hypertensive , non diabetic hospitalised female.

Presenting Complaints: Dyspnea on exertion : 1 month.

H/o present Illness: Progressive exertional dyspnea Leg edema Generalised weakness. Fatigue Palpitations. No H/o Orthopnea , fever , cough , weight loss.

Anaemia with hypoproteinemia - ? Nutritional.

CHF

Biventricular

Forward & backward

Low Output state

Underlying Heart Disease Precipitating Factors

Mitral Valve Disease Arrhythmias/esp. Atrial Fibrillation

Dilated Cardiomyopathy RTI

Restrictive Cardiomyopathy Anemia

Cor-Pulmonale Myocardial Ischemia

Coronary Artery Disease Recurrent Rheumatic Fever

Pericardial disease Infective Endocarditis

Connective Tissue Disorders Non compliance with prescribed drugs

Dietary Indiscretion.

Drugs : NSAIDS, Steroids etc.

Pulmonary Embolism

Thyrotoxicosis

Excessive Physical exertion

Past History:

Cardiac Tamponade : drained 1 year back.

ATT for 9 months.

Pericardial Fluid Analysis : Not available.

No H/o :

▪ HIV

▪ Radiation

▪ Prior cardiac surgery.

Underlying Heart Disease Precipitating Factors

Mitral Valve Disease Arrhythmias/esp. Atrial Fibrillation

Dilated Cardiomyopathy RTI

Restrictive Cardiomyopathy Anemia

Cor-Pulmonale Myocardial Ischemia

Coronary Artery Disease Recurrent Rheumatic Fever

Pericardial disease Infective Endocarditis

Connective Tissue Disorders Non compliance with prescribed drugs

Dietary Indiscretion.

Drugs : NSAIDS, Steroids etc.

Pulmonary Embolism

Thyrotoxicosis

Excessive Physical exertion

Pericardial Disease

500 consecutive patients -1st episode of acute pericarditis.

Jan 2000-dec.2008 Aetiologies:

Viral : 83.2%

CTD : 7.2%

Neoplastic : 5%

T.B : 4%

Purulent : 0.3% Median F/u: 72 months

(24-120 months)

Recurrent pericarditis: 11%-32%

5 year survival : 45-50% (~ 60 % malignant).

Constrictive Pericarditis : 17-40% of tuberculous pericardial effusions.

Circulation. 2011 Sep 13;124(11):1270-5. Imazio M1, Brucato A, Maestroni S, Cumetti D et.al

Constrictive Pericarditis : 1.8%(9 out of 500 patients).

Incidence rate of CP:

Aetiology Incidence Rate per 1000 person years

Idiopathic/Viral 0.76

Connective tissue diseases 4.4

Neoplastic pericarditis 6.33

Tuberculous pericarditis 31.65

Purulent pericarditis 52.74

Circulation. 2011 Sep 13;124(11):1270-5. doi: 10.1161/CIRCULATIONAHA.111.018580. Epub 2011 Aug 15. Risk of constrictive pericarditis after acute pericarditis. Imazio M1, Brucato A, Maestroni S, Cumetti D, Belli R, Trinchero R, Adler Y.

Constriction generally develops within 6 months of presentation with effusive pericarditis.

Rifampicin based ATT has reduced the incidence of constictive pericarditis in T.B. pericarditis from 50 % to 17 – 40%.

Two interventions may reduce the incidence of constriction: intrapericardial urokinase

high-dose adjunctive prednisolone(reduces

the incidence of constrictive pericarditis by 46% )

The initiating event results in a chronic inflammatory pericardial process, resulting in fibrinous scarring and occasionally calcification of the pericardium.

Visceral and parietal pericardium are fibrosed and fused together.

Impaired ventricular filling in diastole- rigid pericardium restricts inflow into the ventricles after an initial expansion.

During early to mid diastole, ventricular filling abruptly ceases when intra cardiac volume reaches the limit set by the stiff pericardium.

This results in elevation and equilibration of filling pressures in all chambers and the systemic and pulmonary veins

Abnormally rapid ventricular filling in early distole because of elevated atrial pressures and accentuated early diastolic ventricular suction..

Signs and symptoms of right-sided heart failure (due to Systemic venous congestion): lower extremity edema, vague abdominal complaints, and passive hepatic congestion. ascites, anasarca, and Jaundice due to cardiac cirrhosis

Signs and symptoms ascribable to elevated pulmonary venous pressures: exertional dyspnea, cough, and orthopnea.

End-stage constrictive pericarditis: chronically low cardiac output as a result of impaired ventricular filling: severe fatigue, muscle wasting, and cachexia.

No cyanosis , pallor or icterus. BMI: 20.2 kg/m²

BP: 97/45 mmHg.

HR : 92 bpm.

SPo2: 98% (RA).

JVD +

Bipedal Edema + : Pitting Type.

Mild Ascites & Hepatomegaly +

Heart& Breath sounds : Unremarkable.

Signs of Right heart failure

Markedly elevated JVP Rapid Y descent ( M shaped JVP contour) Kussmaul Sign( Inspiratory increase in venous pressure) :

reflects loss of the normal increase in right-sided heart venous return on inspiration.

Pulsus paradoxus : in 1/3rd of Patients. Pericardial Knock:

high-pitched early diastolic sound, corresponds in timing to the abrupt cessation of ventricular

expansion after atrioventricular valve opening and with the prominent y descent seen in the jugular venous

waveform. Lower extremity edema is the rule.

Hb.: 14g/dl. WBC Count: 17000 /mm³.

N-60%,L-37%,E-2%, M-1%.

ESR:45mm.

TFT/LFT/RFT: Normal.

Tuberculin skin test : Negative.

HIV: Negative.

BNP: 322.8 pg/mL.

Brain Natriuretic Peptide Levels in Constrictive Pericarditis and Restrictive Cardiomyopathy

Luciano Babuin et al. JACC 2006;47:1489-1491

American College of Cardiology Foundation

ECG:

Sinus Rhythm.

Low QRS amplitude.

Atrial fibrillation : 22%

Low voltage QRS complexes : 27%

CT ratio : Increased obscured left cardio-

phrenic angle ill defined cardiac

borders.

in a retrospective review of 135 patients with constrictive pericarditis, confirmed surgically or at autopsy; 27 percent had pericardial calcification.

Isolation of the cardiac chambers from intra thoracic respiratory pressure changes and a fixed end-diastolic ventricular volume.

These pressure changes continue to be transmitted to the pulmonary circulation.

Inspiration causes a decrease in intrathoracic pressure - lower driving force from the lungs into the left side of the heart and the left ventricle becomes underfilled.

Because of ventricular interaction, the right heart volume expands via shift of the interventricular septum.

The rigid, noncompliant fibrous pericardial sac couples both ventricles.

Consequently, an increase in filling on one side of the heart impedes contralateral filling through the motion of the interventricular septum (IVS), thereby making both ventricles markedly interdependent.

These physiologic abnormalities that occur as a result of the ventricular interdependence and respiratory influenced changes are the fundamental processes that account for the clinical, hemodynamic, and echocardiographic findings of CP.

Moderate pericardial effusion Significant Respiratory variation in Tricuspid

Inflow velocities. Mitral annular velocities show annulus reversus. Mild PAH-RVSP=40mm Hg. IVC-dilated Diastolic flow reversal in hepatic vein Large homogenous mass between LA & aorta

and extending anteriorly surrounding aorta & PA

Septal shudder and bounce: Abrupt posterior motion of the ventricular septum in early diastole with inspiration.

Rapid posterior motion of the left ventricular posterior wall in early diastole followed by a flattening .

Inferior vena cava and hepatic veins (plethora).

Increased pericardial thickness (TEE better). Premature opening of the pulmonic valve. Moderate biatrial enlargement.

Increased E velocity / EDT <160ms. The reduction in left heart filling during inspiration causes a

reduction in mitral inflow velocity ~ 25% and a shift of the interventricular septum toward the left ventricle.

• Tricuspid velocity greatly increases ~ 40% in the first beat after inspiration.

With expiration, left heart filling increases which shifts the interventricular septum back toward the right ventricle, leading to reduced filling to right side of the heart and a late-diastolic reversal of flow in the hepatic veins.

Normal or increased mitral annular velocity (>7 cm/sec).

Annulus paradoxus: Transmitral flow velocity to mitral annular velocity ratio (E/e’) is inversely proportional to pulmonary capillary wedge pressure in patients with constrictive pericarditis.

In normal subjects, mitral lateral e’ velocity is higher than the medial e’velocity.

In patients with constrictive pericarditis the averaged lateral e’ velocity is lower than the medial e’velocity.

The presence of ventricular septal shift in combination with

either medial e'≥9 cm/s or hepatic vein expiratory diastolic reversal ratio

≥0.79 corresponded to a desirable combination of

sensitivity (87%) and specificity (91%) in diagnosing CP.

Equivocal echocardiographic findings may be present in up to one third of patients.

Computed tomography is the

imaging modality of choice to evaluate the thickness of the pericardium(upper limit of normal being 2 mm.) and for pericardial calcification.

Thickened pericardium by CT can be found in a multitude of situations, including the early post-operative period, uraemia, rheumatic heart disease, sarcoidosis, or as a consequence of radiation therapy . Increased thickness of the pericardium per se does not constitute proof of constriction

Up to 18% of patients with constriction confirmed by other modalities do not have pericardial thickening

Density of pericardial effusions can be determined, with simple effusions having uniform low attenuation (with a Hounsfield unit of 0), whereas complex effusions, e.g. those resulting from blood or infection, are of increased density.

Computed tomography can also assist surgical planning.

Moderate pericardial effusion

ADVANTAGES

Short acquisition time of a few seconds, compared with up to 1 h for a CMR examination.

Eliable assessment of pericardial calcification, whereas the appearance of calcification on CMR is variable and unreliable.

Secondary functional information, such as enlargement of the atria and venae cavae in cases of pericardial constriction, can be obtained by CT

DISADVANTAGES

The principal disadvantage of CT is the lack of information on ventricular function, which is routinely provided by CMR

Ventricular functional parameters can be acquired only with retrospective ECG-gated acquisitions, which are associated with an increased radiation dose.

Spin-echo black-blood imaging. Steady-state free precession gradient echo

cine sequences. Spatial modulation of magnetisation cine

images. Real-time cine imaging during dynamic

respiration. Phase-contrast flow imaging

MR imaging is often helpful in these cases, offering superior soft tissue contrast and the ability to image the entire pericardium and its relationship to cardiac structure and function

Showed thickened pericardium measuring 25mm-aorta 35mm-PA 30mm-RA 20mm-LV 18mm between aorta and LA

On CMR, normal pericardium measures 1.2 mm in diastole to 1.7 mm in systole. larger than those found in anatomical studies of the heart, ie, 0.4–1 mm. Pericardial thickening on MRI of greater than 4 mm differentiates CP from

restrictive cardiomyopathy with a sensitivity and specificity of 88% and 100%, respectively (Masui T, Finck S, Higgins CBRadiology. 1992 Feb; 182(2):369-73.)

increased pericardial thickness does not necessarily imply pericardial constriction, and vice versa.

Up to 18% of patients with histologically proven constrictive pericarditis have a normal or near normal pericardial thickness (i.e. < 2 mm) (non or minimally thickened constrictive pericarditis)

.

Dilatation of the superior and inferior vena cavae, hepatic veins, coronary sinus and atria,

Ascites and pleural effusion Distortion of the ventricular chambers such that they have a

tubular morphology, particularly around the AV groove. Flattening of the RV free wall is also typical.

Real-time cine MRI sequence made during dynamic respiration, which highlights ventricular septal inversion during early inspiration. This finding differentiates CP from restrictive cardiomyopathy with a sensitivity of 81%, specificity of 100% and a positive predictive value of 100%

Real-time cine images of a patient with constrictive pericarditis. (a) Four-chamber and (b) short-axis views taken in the diastolic phase during (1) expiration and (2) early inspiration. The interventricular septum is normally positioned during expiration (arrows) but inverts during inspiration (arrowheads).

Showed metabolically active soft tissue thickening of the pericardium involving the pericardial sinuses, encasing the aortic arch and pulmonary vessels with maximum thickness of 52mm.

In selected cases, positron emission tomography (PET) alone, or preferably in combination with CT (PET/CT), can be indicated to depict the metabolic activity of pericardial disease.

Of value in identifying the nature of inflammatory pericarditis.

In particular, tuberculous pericarditis yields higher FDG uptakes than idiopathic forms.

[18F] Fluorodeoxyglucose PET/CT Predicts Response to Steroid Therapy in Constrictive Pericarditis

Chamber Pressures Interpretation

Right Atrium a = 20/ v = 19 mean = 15 mmHg.

Elevated RA pressure Equal a and v waves

Right Ventricle 53/20 mmHg. Elevated RVEDP > 1/3 of RVSP

Pulmonary Artery 51/20 mmHg. Mildly elevated PASP

PCWP 18 mmHg. Elevation & Equalization of Diastolic Pressures. Left Ventricle 94/19 mmHg.

Aorta 100/64 mmHg

Cardiac index 3.4 l/min/m2 Systematic vascular resistance (dyne・sec・cm-5) 400 Pulmonary vascular resistance (dyne・sec・cm-5) 128 Hemodynamic evaluation or RV using a Swan-Ganz catheter revealed exhibited a dip and plateau pattern .

Cardiac catheterization is not routinely used for the diagnosis of pericardial disease.

Current non-invasive techniques are usually able to solve the differential diagnosis of a patient with the suspicion of heart disease involving the pericardium.

Differentiation between constrictive pericarditis and restrictive cardiomyopathy is sometimes difficult and may require an invasive test.

Right atrial, right ventricular diastolic, pulmonary capillary wedge, and pre–a wave left ventricular diastolic pressures are elevated and equal, or nearly so, at around 20 mm Hg.

Differences of more than 3 to 5 mm Hg between left- and

right-sided heart filling pressures are rarely encountered. Square root“(Dip & Plateau) signs in the RV and LV diastolic

pressure tracings often with an absent a wave. Equalization of LV and RV diastolic plateau pressure tracing.

Increased RV end-diastolic pressure, usually to a level one-third or more of RV systolic pressure.

A greater inspiratory fall in pulmonary capillary wedge pressure compared to left ventricular diastolic pressure.

Pulmonary artery and right ventricular systolic pressures are often modestly elevated, in the range of 35 to 45 mm Hg.

Discordance between RV and peak LV systolic pressures during inspiration, another sign of increased ventricular interdependence. During peak inspiration, an increase in RV pressure occurs when LV pressure is lowest.

Figure 4. Suggested diagnostic approach for the evaluation of patients with

constrictive pericarditis.

David Verhaert et al. Circ Cardiovasc Imaging. 2010;3:333-

343

Copyright © American Heart Association, Inc. All rights reserved.

What could be the possible diagnosis? what is the approach of management?

Chronic Constrictive Pericarditis of Tuberculous Aetiology

Pericardiectomy: In experienced centres(Centres with special interest in pericardial disease) remove as much of the pericardium as possible with all constricting parietal

and epicardial layers, taking care of preserving the phrenic nerves bilaterally. necessary to liberate all of the right atrium, the superior vena cava and

especially the inferior vena cava and the inferior part of the right ventricle adjacent to the diaphragm as far as possible.

surgery should be considered cautiously in patients with either mild or very advanced disease and in those with radiation-induced constriction, myocardial dysfunction, or significant renal dysfunction.

Preoperative diuretics should be used sparingly with the goal of reducing, not eliminating elevated venous pressure, ascites, and edema.

significant operative mortality.: ▪ 1970-1985:12% ▪ 1977-2000: 6%

After pericardiectomy, 70% to 80% of patients remain free from adverse cardiovascular outcomes at 5 years and 40% to 50% at 10 years.

derive little or no benefit from pericardiectomy . the operative risk is inordinately high. Manifestations of end-stage disease include cachexia,

atrial fibrillation,

a low cardiac output (cardiac index !1.2 L/m2 per min) at rest, and

hypoalbuminemia due to protein losing enteropathy

and/or impaired hepatic function due to chronic congestion or cardiogenic cirrhosis.

Induction CVP-22mm Hg

Fibrotic pericardium of varying thickness and consistency

Encasing the heart and great vessels

Infiltrating into left ventricular myocardium.

Partial removal of the pericardium from the aorta, PA, RA and RV done.

Post Procedure CVP-10-12mm Hg. Post Op course- Symptomatic improvement

and uneventful. Follow Up- 2 months follow up.

Mesothelioma is a primary malignant tumour of the epithelial cells of the mesothelium.

Pleura, peritoneum, pericardium. Primary cardiac mesotheliomas-less than 3%

of cardiac tumours. Incidence-0.7%. More than 75% of diagnosis is post mortem

Diagnosis-locally advanced stage. CT/MRI Fluid analysis-negative. Tissue diagnosis Survival-10 months after diagnosis.

Surgery-

Tissue diagnosis

Debulking of tumour

Symptomatic relief

Chemotherapy

Adriamycin

Cisplatin

Primary pericardial mesothelioma

Presentation as primary tumour

Rare to diagnose ante mortem

Presentation as constrictive pericarditis

Surgery