Constrictive Pericarditis

Nisha I. Parikh, MD MPH

July 21st 2009

Echo Conference

Summary of Talk

Background Clinical features Echocardiographic diagnosis

M-modeDoppler

Constriction versus restriction Treatment and prognosis

Historical Perspective

The history of constrictive pericarditis is replete with famous names in medicine

Richard Lower described a patient with dyspnea and an intermittent pulse in 1669

Lancisi first reported on the constrictive syndrome in 1828

Corrigan described the pericardial knock in 1842

Kussmaul described his sign and the associated paradoxical pulse in 1873.

Pericardium

Usually 5-10 mL fluid

Parietal and visceral layers

Pericardium

When larger amounts of fluid accumulate (pericardial effusion) or when the pericardium becomes scarred and inelastic, one of three pericardial compressive syndromes may occur

1. Cardiac tamponade — characterized by the accumulation of pericardial fluid under pressure.

2. Constrictive pericarditis —result of scarring and consequent loss of elasticity of the pericardial sac. Typically chronic. The pathological changes are inflammation, sometimes calcification. Grossly, pericardium thicker than normal -80% of time.

3. Effusive-constrictive pericarditis —characterized by constrictive physiology with a coexisting pericardial effusion, usually with tamponade.

Epidemiology

9% of patients with acute pericarditis for any reason go on to develop constrictive physiology.

Acute pericarditis is only clinically diagnosed in 1 in 1,000 hospital admissions

Frequency of a diagnosis of constrictive pericarditis is less than 1 in 10,000 hospital admissions.

Constrictive Pericarditis - HPI

67 % presented with symptoms of heart failure (HF)

8 % with chest pain 6 % with abdominal symptoms 4 % with atrial arrhythmia 5 % with symptoms of cardiac

tamponade

Constrictive Pericarditis - Etiology

Idiopathic or viral — 42 to 49 % Post cardiac surgery — 11 to 37 % Post radiation therapy — 9 to 31 % Connective tissue disorder — 3 to 7 % Postinfectious (tuberculous or purulent

pericarditis) — 3 to 6 % Miscellaneous causes (malignancy, trauma,

drug-induced, asbestosis, sarcoidosis, uremic pericarditis) — 1 to 10 %

Constricitve Pericarditis - PE

Elevated JVP Peripheral edema Ascites Hepatomegaly Pleural effusion S3 Pulsus paradoxus Kussmaul’s sign Cachexia- late stages

The observation of a jugular venous pressure (JVP) that rises with inspiration.

Respiratory variation in intrathoracic pressure with inspiration is not transmitted to the heart chambers.

Kussmaul’s sign

Physiology of constriction

In the pericardial compressive syndromes, the pericardium is inelastic and total cardiac volume cannot change

The result is enhanced ventricular interaction or…ventricular interdependence

Physiology of constriction

Pericardial constriction leads to impairment of ventricular filling, usually affecting all four cardiac chambers, preventing ventricular filling in mid and late diastole.

As a result, the majority of ventricular filling occurs rapidly in early diastole and the ventricular volume does not increase after the end of the early filling period.

Pericardial Effusion

M-Mode

Pericardial effusion

M-mode Cannot determine volume of accumulated fluid accurately

Pericardial thickening

This can be visualized by transesophageal echo (often requiring multiple views), however, this is best seen using other imaging modalities such as CT or MRI.

Calcified Pericardium

Pericardial calcifications CT

Pericardial calcification on echo

Normal pericardium is highly reflective

Bright pericardial echo cannot alone diagnose constrictive pericarditis

Specific echo exam for constriction

Neither sensitive nor specific Must diagnose via a combination of

physical exam/ history findings and echo findings

M-mode findings in constriction Abrupt relaxation of the posterior wall

with flattening of endocardial motion during diastole

Abnormal septal motion:Mimics conduction disturbancesMimics RV p/v overloadEarly diastolic notching followed by

paradoxical and then normal motion of the ventricular septum

diastolic septal bounce:

Thought to be due to the rapid filling during early diastole leading to asymmetrical filling of the right and left ventricals which creates a fluctuating pressure gradient that manifests as an abrupt shift of the septum.

? Subtle septal bounce

“Bouncy Septum”

Dilation and lack of respiratory variation in IVC

Doppler echo findings in constriction Mitral inflow

Exaggerated E/A ratio Short deceleration time Exaggerated respiratory variation in E-wave

velocity >25% Seen more reliably when patients are well

hydrated Can also be seen in pulmonary disease

Hepatic Veins Expiratory increase in diastolic flow reversal

Hepatic flow reversal

Secondary to elevated right atrial pressures. Hepatic vein doppler reveals pressure tracings significant for a prominant "a" wave and prominent "y" descent.

Atrial dilation

Mild Secondary to elevated atrial

pressures More severe atrial dilatation seen in

restrictive cardiomyopathy.

Constrictive Pericarditis – other tests?

CT – not very sens/spec Cardiac MRI – growing in favor BNP – usually only a mild elevation

due to limited wall stretch Cath – GOLD STANDARD

Effusive constrictive pericarditis

Combination of tamponade and constriction

Common etiologies: malignancy and radiation therapy

Pericardial thickening may prevent RA collapse

Hemodynamic compromise and JVD persist even after tap

Effusive Constrictive Pericarditis- Prospective Study Methods From 1986 through 2001, all patients with effusive–constrictive pericarditis were prospectively

evaluated. Combined pericardiocentesis and cardiac catheterization were performed in all patients, and pericardiectomy was performed in those with persistent constriction. Follow-up ranged from 1 month to 15 years (median, 7 years).

Results 1184 patients with pericarditis were evaluated, 218 with tamponade. 190 underwent combined pericardiocentesis and catheterization. Fifteen of these patients had effusive–constrictive pericarditis and were included in the study. All patients

presented with clinical tamponade; however, concomitant constriction was recognized in only seven patients. At catheterization, all patients had elevated intrapericardial pressure (median, 12 mm Hg; interquartile range,

7 to 18) and elevated right atrial and end-diastolic right and left ventricular pressures. After pericardiocentesis, the intrapericardial pressure decreased (median value, –5 mm Hg; interquartile range, –5 to 0), whereas right atrial and end-diastolic right and left ventricular pressures, although slightly reduced, remained elevated, with a dip–plateau morphology. The causes were diverse, and death was mainly related to the underlying disease.

Pericardiectomy was required in seven patients, all of whom had involvement of the visceral pericardium. Three patients had spontaneous resolution.

Conclusions Effusive–constrictive pericarditis is an uncommon pericardial syndrome that may be missed in some patients who present with tamponade. Although evolution to persistent constriction is frequent, idiopathic cases may resolve spontaneously. In our opinion, extensive epicardiectomy is the procedure of choice in patients requiring surgery.

Constriction versus Restriction

Restrictive CardiomyopathyPure diastolic dysfunctionSystolic function preservedUsually due to infiltrative processSeveral echo signs overlap with

constrictive pericarditis

Restrictive versus Constrictive

Restrictive Cardiomyopathy Constrictive Pericarditis

History Infiltrative disease Pericarditis, trauma, surgery

Mantle radiation, cardiac surgery

Mantle radiation, cardiac surgery

Respiratory effects

No bulging Increased ventricular interaction- bulging of the septum towards LV

CMR C/w infiltrative disease Increased pericardial thickness (> 5 mm

Comparison of Pericardial Constriction and Restrictive Cardiomyopathy

Constrictive Pericarditis Restrictive Cardiomyopathy

Right Atrial Pressure

RV/LV filling pressures RV=LV LV > RV

PASP Mild elevation 35-40 mmHg

Moderate-to-severe (≥ 60 mmHg)

2D Echo Pericardial thickening, no effusion

LVH, normal systolic function

Doppler Echo * E > a on LV inflow

* Prominent y descent in hepatic vein

* Pulm venous flow = prominent a wave, reduced systolic phase

* Resp variation in IVRT and E velocity

* Atria: mildly enlarged

* Early in disease E < a

* Late in disease E > a

* Constant IVRT

* Absence of significant respiratory variation

* Marked enlarged atria

Tissue Doppler to distinguish entities

Dimunitive E’<8 cm/s

E’ similar to E>12cm/s

Treatment

Definitive treatment is surgical Earlier the better Extensive decortication favored, especially at the diaphragmatic-

ventricular contact regions.  Complications

excessive bleeding atrial and ventricular arrhythmias ventricular wall ruptures.

Published surgical mortality 5-15%. Perioperative mortality rate (within 30 days) was found to be

6.1%.  progressive heart failure Sepsis renal failure respiratory failure arrhythmia

Post-op course

80-90% achieve NYHA class I or II postoperatively.

Abnormal diastolic filling (which can be correlated with clinical status) often remains

Only 60% of patients have complete normalization of cardiac hemodynamics.

In 58 patients who underwent total pericardectomy for constriction, 30% still had some significant symptoms after 4 years.

These patients were more likely to have a persistent restrictive or constrictive pattern to their transmitral and transtricuspid Doppler signals as determined by respiratory recording.

Survival post pericardiectomy Long-term survival after pericardiectomy depends on

the underlying cause. Idiopathic with best prognosis (88% survival at 7 yrs), Constriction due to cardiac surgery (66% at 7 years). Worst prognosis occurs in postradiation constrictive

pericarditis (27% survival at 7 years). (likely represents confounding comorbidities). 

Predictors of poor outcomes in patients who undergo pericardiectomy history of prior radiation worsening renal function pulmonary hypertension systolic heart failure Hyponatremia advanced age.

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Listening!!