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AUTHOR AND EDITOR INFORMATION
Section 1 of 12  
Author: Brian D Soriano, MD, Assistant Professor of Pediatrics, University of Washington School of Medicine; Consulting Staff, Pediatric Cardiology and Cardiac Imaging, Seattle Children's Hospital
Brian D Soriano is a member of the following medical societies: American College of Cardiology, American Heart Association, American Medical Association, and American Society of Echocardiography
Coauthor(s):
Charles Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston;
Renee E Margossian, MD, Instructor, Department of Cardiology, Children's Hospital, Harvard University; Consulting Staff, Department of Cardiology, Boston Medical Center and Brigham and Women's Hospital;
Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Editors: Christopher Johnsrude, MD, Associate Professor of Pediatrics, Director of Electrophysiology, University of Louisville School of Medicine; Consulting Staff, Pediatric Cardiology Associates, PSC; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Hugh D Allen, MD, Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine; Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Author and Editor Disclosure
Synonyms and related keywords:
constrictive pericarditis, pericardial constriction, chronic constrictive pericarditis, pericardial constraint, constrictive epicarditis, cardiac inflammation, pericardial inflammation, pericardium, pericardial disease, friction rub, congestive heart failure, CHF, pericardial resection, pericardial stripping, pericardiectomy, radical pericardiectomy, infectious constrictive pericarditis, postpericardiotomy syndrome, right-sided heart failure, tuberculous pericarditis, metabolic acidemia, hepatomegaly, ascites, pulsus paradoxus, tuberculosis, histoplasmosis, postpericardiotomy syndrome, uremia, sarcoidosis
Background
Although relatively more prevalent in adults, constrictive pericarditis in pediatric patients is an extremely rare condition in which the easily distensible, thin, parietal and visceral pericardial linings become inflamed, thickened, and fused. Because of these changes, the potential space between the linings is obliterated. Venous return to the heart becomes limited and ventricular filling is reduced. Symptoms consistent with congestive heart failure (CHF), especially right-sided heart failure, develop as a result of the inability of the heart to increase stroke volume.1 Cardiac output gradually becomes inadequate, at first with exercise and then at rest. Systolic function is rarely affected until late in the course of the disease, presumably secondary to infiltrative processes that affect the myocardium, atrophy, or scarring/fibrosis of the myocardium from the overlying adjacent pericardial disease.
Pathophysiology
Several hallmarks of constrictive pericarditis include the lack of ventricular distensibility, secondary to the thickened and inelastic pericardium. A subsequent inability to maintain adequate preload is noted. Filling pressures of the heart tend to become equal in both the ventricles and the atria. Myocardial function in early diastole is preserved, which aids in distinguishing this disease from restrictive cardiomyopathy. The following was determined through experimental models:
- A change in volume-elasticity curves was the fundamental pathophysiologic change associated with the disease.
- During development of the constriction, right and left ventricular diastolic pressure increased and stroke volume decreased.
- A small increase in volume resulted in a considerable increase in end-diastolic pressure.
Frequency
United States
Data are lacking for epidemiologic analysis. Although unusual in adults, the disease is even more rare in the pediatric population. In all age groups, prevalence is increased among patients who are hospitalized and among patients who have undergone cardiac surgery.
International
Tuberculous pericarditis is the most frequent known cause of chronic constrictive pericarditis.
Mortality/Morbidity
Failure of conventional medical therapy for CHF often follows an extensive diagnostic workup, leading to the final diagnosis of constrictive pericarditis. Decline in function is a result of decreased cardiac output with symptoms of CHF, along with morbidity stemming from chronic systemic venous congestion. Multisystemic failure can develop into the end-stage of illness when global tissue hypoxia leads to worsening metabolic acidemia. Life expectancy is reduced in untreated children and in patients with relatively acute onset of symptoms.
Race
No statistical evidence indicates a racial predilection.
Sex
No statistical evidence indicates a sex predilection.
Age
The incidence and prevalence rates reveal that the condition is rare in adults and even more rare in children.
History
Symptoms are usually similar to those associated with right-sided congestive heart failure.
- Precordial pain (frequent in acute pericarditis but unusual in chronic constrictive pericarditis)
- Dyspnea
- Easy fatigability
- Fever
- Tachycardia
- Abdominal fullness
- Palpitations
- Orthopnea
- Paroxysmal nocturnal dyspnea
- Diaphoresis
Physical
Unlike other forms of pericardial disease, such as acute pericarditis, a friction rub is usually not found. A protodiastolic knock, usually heard along the left sternal border, corresponds to the abrupt cessation of ventricular filling during diastole. Systemic venous pressures become elevated, and the following features are consistent with right-sided heart failure: - Neck vein distention with Kussmaul sign (absence of a drop in jugular venous pulsations during inspiration)
- Hepatojugular reflux
- Hepatomegaly
- Ascites
- Peripheral (dependent) edema
- Pulsus paradoxus (rare)
- A prominent third heart sound
- Diminished pulse volume
- Hepatic dysfunction
Causes
Chronic constrictive pericarditis is a disease with multiple etiologies that is associated with variable clinical findings, depending on the acuity of development.
- Chronic constrictive pericarditis: This condition develops insidiously; an etiology is often never determined.
- Acute pericarditis: In some (approximately 10%) patients, an antecedent acute pericarditis is present. Other cases of constriction are postulated to have been preceded by a subclinical, or occult, form of pericarditis.
- Tuberculosis: Tuberculosis is the most frequently known infectious cause of chronic constrictive pericarditis. Other causal organisms include (among others) staphylococci, streptococci, and fungi such as histoplasmosis.
- Rheumatoid disease
- Sarcoidosis
- Mediastinal radiation
- Trauma (hemopericardium)
- Status post–cardiac surgery (postpericardiotomy syndrome)
- Uremia
- Neoplastic disease
- Postinfectious (bacterial) pericarditis
- Various metabolic and genetic disorders
Ascites
Budd-Chiari Syndrome
Cardiac Tumors
Cardiomyopathy, Dilated
Cardiomyopathy, Restrictive
Cor Triatriatum
Ebstein Anomaly
Endocardial Fibroelastosis
Endocarditis, Bacterial
Endocarditis, Fungal
Heart Failure, Congestive
Mitral Stenosis, Supravalvular Ring
Myocarditis, Nonviral
Myocarditis, Viral
Nephrotic Syndrome
Partial Anomalous Pulmonary Venous Connection
Pericardial Effusion, Malignant
Pericarditis, Bacterial
Pericarditis, Viral
Postpericardiotomy Syndrome
Pulmonary Hypertension, Eisenmenger Syndrome
Pulmonary Hypertension, High Altitude
Rheumatic Heart Disease
Superior Vena Cava Syndrome
Thoracic Trauma
Thromboembolism
Total Anomalous Pulmonary Venous Connection
Tricuspid Regurgitation
Tricuspid Stenosis
Veno-occlusive Hepatic Disease
Other Problems to be Considered
Cardiac tamponade
Scimitar syndrome
Effusive constrictive pericarditis
Lab Studies
- CBC count
- A CBC count may reveal evidence of dilutional anemia when CHF is also present.
- Leukocytosis may be evident if an infectious, bacteriologic, or rheumatologic source is the etiology or if patients are receiving treatment with steroid therapy.
- Leukopenia may be present in patients in whom chemotherapeutic agents are administered for malignancy.
- Electrolyte, BUN, and creatinine levels
- Dilution secondary to CHF may demonstrate hyponatremia or pseudohyponatremia.
- Contraction alkalosis (ie, hypochloremia with hypercarbia) may occur when diuretics are aggressively used.
- With renal insufficiency, short-term elevation of the BUN levels and long-term elevation of creatinine levels are observed.
- ABG: Metabolic acidosis (ie, low pH and low bicarbonate) with or without compensatory respiratory alkalosis (ie, decreased partial pressure of carbon dioxide) is frequently observed with right-sided CHF.
- Liver function profile
- Passive hepatic congestion from cor pulmonale may cause elevated transaminase levels.
- Hypoalbuminemia is the hallmark of a protein-losing enteropathy (PLE) that results from increased central venous pressure in the portal system of patients with hepatomegaly and ascites. In patients in whom PLE is suspected, stool a1-antitrypsinase should be measured.
- Acute phase reactants: Erythrocyte sedimentation rate and C-reactive protein level may be elevated in postpericardiotomy syndrome.
- Brain natriuretic peptide (BNP):2 Although elevated levels greater than 600 pg/mL can help differentiate constrictive pericarditis from restrictive cardiomyopathy in adults, no data are available in children.
Imaging Studies
- Two-dimensional echocardiography
- A thickened pericardium may be observed.
- Systemic veins may be dilated.
- The echocardiogram may reveal diminished intraventricular volumes.
- Pericardial effusions are easily depicted with this modality.
- Interventricular septal motion may be paradoxic or flat as a sign of ventricular interdependence.
- The inspiratory increase in chamber size is larger in patients with constrictive pericarditis than in those with restrictive cardiomyopathy.
- Pulsed-wave Doppler echocardiography
- Transmitral and transtricuspid early diastolic filling (E wave) is rapid with a shortened deceleration time; no significant change occurs in the atrial-filling phase (A wave).
- As opposed to restrictive cardiomyopathy, respiratory variation in the filling phases is more pronounced.
- Transmitral peak E velocity has a more pronounced decrease during inspiration in patients with constrictive pericarditis than in healthy persons and patients with restrictive cardiomyopathy.
- Peak E and peak A tricuspid velocities are significantly increased during inspiration.
- Isovolumetric relaxation time (IVRT) is also affected by respiratory variation in constrictive pericarditis, with an increase of more than 25% during inspiration.
- During constriction, the pulmonary venous flow pattern demonstrates systolic and diastolic forward flow, with a marked decrease in diastolic flow on inspiration and an increase on expiration. This measurement may help determine if a pseudonormalized diastolic pattern is present on the mitral inflow tracing.
- In the pediatric population, no single Doppler measurement can fully characterize left ventricular diastolic function, and no single measurement is free of complicating factors. Most of the parameters are dependent on load, heart rate, and age. In addition, indices of constriction in adults, such as hepatic vein flow reversal and alterations in pulmonary venous Doppler patterns, are confounded by the fact that such patterns are detected in healthy children.
- Tissue Doppler echocardiography (TDE): TDE is useful in distinguishing constrictive versus restrictive physiology. Early mitral annular velocity is usually reduced in restriction, whereas it is normal in constrictive pericarditis.
- Chest radiography
- Radiographic findings are usually unremarkable.
- Pericardial calcifications are present in 40-50% of patients.
- MRI: MRI can reveal pericardial thickening, right atrial dilation, and a characteristic intraventricular septal "bounce" in early diastole.
- Cardiac CT: Both CT and MRI can detect a thickened pericardium (>4 mm), but this is an insensitive finding. The absence of pericardial thickening does not rule out hemodynamically significant restrictive pericarditis.
Other Tests
- Electrocardiography
- ECG usually reveals nonspecific ST-T wave changes.
- Atrial dysrhythmias are common.
- QRS complexes may demonstrate low voltage.
Procedures
- Diagnostic cardiac catheterization
- Cardiac catheterization can be performed to measure intracardiac pressures. The hallmark finding in patients with chronic constrictive pericarditis is elevation of end-diastolic pressures, which are at equal levels in the right atrium, right ventricle, pulmonary artery, left atrium, and left ventricle.
- Loss or reversal of respiratory variation of right atrial pressure is noted. When hemodynamic studies are equivocal, response to bolus fluid administration is recorded.
- The intraventricular pressure pulse contour characteristically reveals an early rapid fall in diastolic pressure in the right ventricle, followed by a rapid rise to an elevated diastolic plateau (square root sign). The left ventricular pressure pulse tracing is usually similar.
- Pulmonary artery systolic pressure should be less than 50 mm Hg. Higher pressures may suggest other diseases, such as restrictive cardiomyopathy and pulmonary arteriolar hypertension.
Histologic Findings
- Myocardial histologic findings include fibrotic thickening, chronic inflammation, granulomas, and calcification.
Medical Care
Care is primarily surgical. Medical management, such as careful observation or symptomatic treatment, has been suggested in less severe cases. However, this option is controversial.
Surgical Care
Pericardiectomy is the predominant treatment. Hemodynamic and symptomatic improvements are rapid.
No medications are required when the diagnosis is definitive because the patient is usually referred for surgery. To help maintain a euvolemic state, diuretics and afterload reducing medications should be used cautiously; decreasing preload or afterload can cause greater compression of the heart and sudden cardiac decompensation. This is especially so when general anesthetic agents are administered just before the pericardiectomy procedure is performed.
Further Inpatient Care
- Low cardiac output may occur postoperatively in patients who are debilitated and who have ascites or other findings of fluid retention.
- Patients with low cardiac output may require maintenance of high left atrial pressure, sympathomimetic infusions, or both.
- Mechanical support of the circulation, such as extracorporeal membrane oxygenation (ECMO) or intra-aortic balloon counterpulsation, can be used in patients who are critically ill.
Transfer
- Transfer to a tertiary care center with cardiothoracic surgeons and critical care services for pediatric patients is the only referral needed.
Prognosis
- The prognosis after pericardial resection/stripping is excellent as long as the underlying myocardium is unaffected.
- Occasionally, the etiology of the constriction may cause coincident myocardial dysfunction.
Patient Education
- Pericardial constriction does not recur in the absence of an intact pericardium.
Medical/Legal Pitfalls
- Failure to diagnose a surgically correctable disease
- Failure to recognize and exclude a restrictive cardiomyopathy
- Failure to appropriately evaluate and treat the etiology of the constriction (if one is determined)
- Excessive use of diuretics or afterload-reducing agents in an already compromised patient
The authors would like to thank Kurt Pflieger, MD, FAAP for his significant contributions and original work for this article.
| Media file 1:
MRI image of constrictive pericarditis in a 13-year-old and an otherwise structurally normal heart. Infectious workup was negative. (Image courtesy of Tal Geva, M.D.) |
 |  View Full Size Image | |
Media type: MRI
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| Media file 2:
Left ventricular volume curve in constrictive pericarditis. |
 | View Full Size Image | |
Media type: Graph
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Pericarditis, Constrictive excerpt Article Last Updated: Aug 1, 2008
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