Constrictive Pericarditis Nisha I. Parikh, MD MPH July 21st 2009 Echo Conference

Summary of Talk Background Clinical features Echocardiographic diagnosis M-mode Doppler 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 Parietal and visceral layers Usually 5-10 mL fluid

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

Kussmaul’s sign 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.

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 disturbances Mimics RV p/v overload Early 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 Cardiomyopathy Pure diastolic dysfunction Systolic function preserved Usually due to infiltrative process Several echo signs overlap with constrictive pericarditis

Restrictive versus Constrictive Restrictive Cardiomyopathy Constrictive Pericarditis History Infiltrative disease Pericarditis, trauma, 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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