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Pediatric Cor Triatriatum Workup

  • Author: M Silvana Horenstein, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Mar 05, 2014
 

Laboratory Studies

No specific laboratory studies are indicated in patients with suspected cor triatriatum.

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Electrocardiography

ECG findings are nonspecific and may range from normal in asymptomatic older patients to mimicking those findings of a patient with the clinical picture of pulmonary hypertension.

In some patients, ECG may reveal the following:

  • Atrial arrhythmias [8]
  • Right-axis deviation
  • Right atrial enlargement depicted by enlarged P waves
  • Right ventricular hypertrophy
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Chest Radiography

Chest radiographic findings are usually nonspecific but may include pulmonary congestion with diffuse haziness or Kerley B lines and the ground glass pattern of acute pulmonary edema in hilar areas.

Patients may have mild cardiac enlargement and prominence of the pulmonary arterial segment.

The dilated proximal chamber may produce the appearance of left atrial enlargement.

Presence of an atrial septal defect or of an associated partial anomalous pulmonary venous connection adds pulmonary overcirculation to the pulmonary venous obstruction. The radiograph may then reveal significant right ventricular enlargement.

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Echocardiography

Echocardiography is often sufficient for diagnosis and is the diagnostic modality of choice. See the image below.

Mean Doppler gradient of 7-8 mm Hg across left atr Mean Doppler gradient of 7-8 mm Hg across left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

The membrane dividing the left atrium can be visualized using 2-dimensional echocardiography, as can the presence of an associated atrial septal defect. The origin of each of the pulmonary veins should be identified to exclude the presence of anomalous pulmonary venous return.

The distinction between cor triatriatum and a supramitral ring should be made by the location of the left-atrial appendage. Differentiating between cor triatriatum and total anomalous pulmonary venous drainage to the coronary sinus may be difficult.

Common cardiac anomalies can also be demonstrated

Transesophageal echocardiography (TEE) and intracardiac echocardiography offer precise image definition and spatial relationship of the membrane.

TEE is very useful in larger and older patients in whom transthoracic images are suboptimal especially in visualizing the left atrium.[9]

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Angiography

This test is generally indicated to assess pulmonary venous return and pulmonary arterial pressures. Because approximately 10% of patients have partial anomalous venous return, angiography is helpful in defining the precise venous anatomy.

When performed, catheterization generally reveals pulmonary hypertension in a degree that varies directly with the severity of obstruction to pulmonary venous drainage.[8] Demonstration of a pressure gradient between the left atrium and capillary wedge pressure is classic.

The proximal chamber is visualized during the venous phase, and a delay then occurs before the true left atrium and left ventricle are visualized. The proximal chamber then remains opacified and does not contract with the distal chamber

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Cardiac CT Scanning and MRI

Both imaging modalities provide with very detailed anatomic images.[10, 11, 12] MRI has the advantage of not subjecting the patient to radiation.[13, 14]

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Procedures

In classic cor triatriatum, cardiac catheterization reveals pulmonary hypertension without a left-to-right shunt. Pulmonary wedge pressures are high and left atrial pressures are within normal limits. Angiography via pulmonary artery injection shows an opacified pulmonary venous chamber draining into the left atrium.

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Histologic Findings

Histology plays no part in the diagnosis; however, pulmonary hypertension results in well-defined structural changes.

There may be pulmonary edema and alveolar hemorrhage with lymphatic dilatation. Increased pulmonary arterial muscularity is present very early, with increased thickness of the arterial wall and extension of muscle into the arterioles. Necrotizing arteriolitis may be present.

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Contributor Information and Disclosures
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)

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.

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.

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 Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, 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.

References
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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.
Long-axis parasternal view depicting 2D image of cor triatriatum sinister membrane and color Doppler 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.
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.
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.
Apical 5-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.
Mean Doppler gradient of 7-8 mm Hg across 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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