Diagnostic Considerations

Important considerations

Do not fail to diagnose cor triatriatum and other structural abnormalities in children with pulmonary hypertensive disease.

Special concerns

Patients should have an experienced congenital cardiac surgeon to treat this rare lesion. One of the few late complications is recurrence secondary to incomplete resection of the intra-atrial diaphragm.

Differential Diagnoses

Mitral Stenosis, Valvar
Partial Anomalous Pulmonary Venous Connection
Pediatric Idiopathic Pulmonary Artery Hypertension
Persistent Pulmonary Hypertension of the Newborn (PPHN)
Pulmonary Hypoplasia Imaging
Pulmonary Vein Stenosis
Supravalvular Ring Mitral Stenosis
Total Anomalous Pulmonary Venous Connection

Workup

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Media Gallery

Pediatric Cor Triatriatum. Long-axis parasternal view demonstrating a left atrial membrane separating pulmonary vein inflow from left ventricular (mitral valve) inflow. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. Long-axis parasternal view depicting a two-dimensional image of cor triatriatum sinister membrane and a color Doppler image of pulmonary venous flow through the orifice of the membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. Short-axis parasternal view depicting right and left pulmonary vein flow proximal to the cor triatriatum left atrial membrane and left atrial appendage orifice distal to the cor triatriatum left atrial membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. Subxiphoid coronal image of the posterior left atrial chamber that receives pulmonary venous flow separated from the rest of the left atrium by the cor triatriatum membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. Apical five-chamber view demonstrating a 4-5 mm left atrial membrane orifice with mild pulmonary venous inflow restriction. Note the presence of an associated perimembranous ventricular septal defect (VSD) with tricuspid septal aneurysmal tissue. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Pediatric Cor Triatriatum. This sonogram shows a mean Doppler gradient of 7-8 mmHg across the left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

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Author

M Silvana Horenstein, MD Assistant Professor, Department of Pediatrics, University of Texas Medical School at Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc

M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Maria Victoria T Tantengco, MD Associate Professor of Pediatrics, Division of Cardiology, Department of Pediatrics, University of Massachusetts Medical School; Medical Director, Echocardiography Laboratory, Child Heart Associates, LLC

Maria Victoria T Tantengco, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, Massachusetts Medical Society, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

Specialty Editor Board

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.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous coauthors Manuel Caceres, MD; James Jaggers, MD; and Jeff L Myers, MD, PhD, to the writing and development of this article.