Pediatric Constrictive Pericarditis 

  • Author: Brian D Soriano; Chief Editor: Stuart Berger, MD   more...
 
Updated: May 11, 2012
 

Background

Although relatively more prevalent in adults, constrictive pericarditis (see image below) in pediatric patients is an extremely rare condition in which the easily distensible, thin, parietal and visceral pericardial linings become inflamed, thickened, and fused.

MRI image of constrictive pericarditis in a 13-yeaMRI 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.)

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] Over time, 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.

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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 (see image below) was the fundamental pathophysiologic change associated with the disease.Left ventricular volume curve in constrictive periLeft ventricular volume curve in constrictive pericarditis.
  • 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.
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Epidemiology

Frequency

United States

Pediatric 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.

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Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Brian D Soriano  MD, Assistant Professor of Pediatrics, Cardiology Division, University of Washington School of Medicine; Attending Physician, Pediatric Cardiology and Cardiac Imaging, Seattle Children's Hospital

Brian D Soriano is a member of the following medical societies: American Heart Association, American Medical Association, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Coauthor(s)

Charles I Berul, MD  Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, and Society for Pediatric Research

Disclosure: Johnson & Johnson Consulting fee Consulting

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

Renee E Margossian, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, and Heart Failure Society of America

Disclosure: Nothing to disclose.

Kurt Pflieger, MD, FAAP  Active Staff, Department of Pediatrics, Lake Pointe Medical Center

Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Christopher Johnsrude, MD, MS  Chief, Division of Pediatric Cardiology, University of Louisville School of Medicine; Director, Congenital Heart Center, Kosair Children's Hospital

Christopher Johnsrude, MD, MS is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology

Disclosure: St Jude Medical Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Hugh D Allen, MD  Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

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

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

The authors would like to thank Kurt Pflieger, MD, FAAP for his significant contributions and original work for this article.

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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.)
Left ventricular volume curve in constrictive pericarditis.
 
 
 
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