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Total Anomalous Pulmonary Venous Connection Workup

  • Author: Allen D Wilson, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: May 02, 2015
 

Laboratory Studies

Assess and improve (as possible) the oxygenation, acid-base status, and hemogram status in newborns or young infants with total anomalous pulmonary venous connection (TAPVC) in preparation for surgery.

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Electrocardiography

ECG reveals significant right ventricular hypertrophy in most of these patients, usually with a qR pattern in the right chest leads by age 5-7 days. Right atrial enlargement rarely occurs in these younger patients.

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Chest Radiography

In patients with total anomalous pulmonary venous connection with pulmonary venous obstruction, chest radiographs reveal a normal heart size with a diffuse reticular pattern fanning out from the hilum.

When the pulmonary veins are unobstructed, the heart is enlarged (right atrial and right ventricular enlargement), and pulmonary markings reveal active increase in size of the pulmonary hilar and midzone vessels.

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Echocardiography

Echocardiographic findings, which are usually definitive, have been vital in pinpointing the exact cardiac defect. Hyaline membrane disease may demonstrate similar findings initially. In this setting, ECG helps identify right ventricular hypertrophy in patients with total anomalous pulmonary venous connection, especially in premature babies, particularly because premature babies usually have a greater level of left ventricular force.

Echocardiography of the precordium in patients with total anomalous pulmonary venous connection reveals right ventricular and pulmonary artery volume loading with flattened or paradoxic septal motion on M-mode imaging. Apical and subcostal 4-chamber views usually best identify individual pulmonary veins and their confluence in patients with total anomalous pulmonary venous connection. Then, using multiple views, the common pulmonary vein can usually be tracked to its point of entry to the systemic venous system or to the coronary sinus.

Subcostal long- and short-axis views can also help evaluate size and flow patterns across the foramen ovale.

Total anomalous pulmonary venous connection may be difficult to diagnose, especially in an ill newborn on a ventilator, if views of the atrial septum are difficult to obtain or if the common pulmonary vein is small or at an obtuse angle to the left atrial back wall. The addition of color Doppler ultrasonography greatly aids in the diagnosis of individual pulmonary veins and in analysis of the abnormal flow pattern across the atrial septal defect

Color-flow mapping may be helpful in finding individual pulmonary veins and confirming whether they enter the left atrium. Color-flow ultrasonography may also be used to assess directional flow at the foramen ovale. In patients with total anomalous pulmonary venous connection, flow across the atrial septum predominantly occurs from the right to left.

Altogether, echocardiography with additional color Doppler can help make the diagnosis in the vast majority of patients with total anomalous pulmonary venous connection. In patients with pulmonary inflow obstruction, further diagnostic studies may be needed.

With fetal echocardiography, an attempt should be made to see the individual pulmonary veins, but most consistent diagnostic findings in total anomalous pulmonary venous connection have involved a confluence (chamber) behind the left atrium or a vertical vein.

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Magnetic Resonance Imaging

MRI serves to confirm the diagnosis in patients with total anomalous pulmonary venous connection (especially in those with associated lung disease).

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Selective Pulmonary Vein or Pulmonary Artery Angiography

This study may precisely reveal a vessel's anatomy.

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Procedures

In some patients with multiple sites of pulmonary venous connection, cardiac catheterization serves to better define sites of pulmonary venous obstruction, when other associated cardiac defects are present (ie, pulmonary atresia), and to directly measure foramen ovale size when surgery is delayed.

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Contributor Information and Disclosures
Author

Allen D Wilson, MD Professor, Department of Pediatrics, University of Wisconsin School of Medicine and Public Health; Department of Pediatrics, University of Wisconsin Hospital and Clinics

Allen D Wilson, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, 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.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

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

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.

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Types of total anomalous pulmonary venous connection.
 
 
 
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