Pediatric Patent Foramen Ovale Atrial Septal Defects Workup

  • Author: Barry A Love, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Nov 2, 2010
 

Laboratory Studies

  • No laboratory tests are specific for patent foramen ovale (PFO).
  • In patients with a cryptogenic stroke and a patent foramen ovale, a complete hypercoagulable workup is indicated to rule out a hypercoagulable state. If a hypercoagulable condition is found, specific treatment of this condition with antiplatelet therapy or anticoagulation may be indicated. Consultation with a hematologist should be considered for patients in whom a hypercoagulable state is suspected. Closure of the patent foramen ovale may be indicated in patients with a hypercoagulable state, although one needs to be aware that surgical closure should be considered because the risk of thrombosis on transcatheter occlusion devices may be increased in the presence of a hypercoagulable state.
  • A hypercoagulable workup typically consists of the following tests:
    • CBC count (for platelet count)
    • Prothrombin time (PT), activated partial thromboplastin time (aPTT), international normalized ratio (INR)
    • Factor V Leiden assessment
    • Prothrombin gene mutation
    • Protein C and protein S assessment (Note that proteins C and S are vitamin K–dependent factors and should not be measured while the patient is taking warfarin [Coumadin]).
    • Antithrombin III assessment
    • Homocysteine assessment
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Imaging Studies

  • Transthoracic 2-dimensional echocardiography can generally resolve the atrial septum and show the flap of the foramen ovale in infants and small children. Color Doppler flow across the atrial septum proves the presence of the foramen ovale. A foramen ovale may be differentiated from an atrial septal defect by the overlap of septal tissue. In older children and adults, transthoracic echocardiography does not visualize the atrial septum as well.
  • Transesophageal echocardiography (TEE) is preferred in patients where the atrial septum is inadequately visualized by transthoracic echocardiography. Older children and adults fall into this category. In addition to the patent foramen ovale, redundancy of septum primum can also be seen. When the redundancy of the septum moves more than 1 cm, it is called an atrial septal aneurysm. In the presence of a patent foramen ovale in patients who have had a prior stroke, atrial septal aneurysm confers an increased risk for a subsequent neurologic event.[14]
  • Bubble-contrast echocardiography is mandatory to diagnose a patent foramen ovale and to demonstrate a potential right-to-left shunt.
    • A bubble-contrast study is performed by inserting a peripheral intravenous line and agitating 8 mL of saline with 1 mL of the patient's blood and 1 mL of air. The air is agitated into the solution and the bubble contrast is injected. The atrial septum and left atrium are visualized during the injections. In the presence of a patent foramen ovale, bubbles can be seen crossing the atrial septum and entering the left atrium. Transesophageal echocardiogram showing the atrial Transesophageal echocardiogram showing the atrial septum. The "flap" of the septum primum is seen. The diagnosis of patent foramen ovale (PFO) cannot be made until right-to-left bubble contrast is demonstrated. LA = Left atrium; RA = Right atrium.
    • The bubble-contrast injection should be performed at rest and with a Valsalva maneuver. The Valsalva maneuver transiently increases right atrial pressure above left atrial pressure, encouraging potential right-to-left shunting. Bubble-contrast injection during Valsalva maneuverBubble-contrast injection during Valsalva maneuver. The "flap" of the foramen ovale is opened and bubbles are seen crossing from the right atrium to the left atrium (arrow).
    • TEE is usually the best echocardiographic imaging tool to use with a bubble-contrast injection, although it can be performed using transthoracic echocardiography. The sensitivity of transthoracic echocardiography with bubble-contrast injection is poor compared with transesophageal imaging.
  • Transcranial Doppler imaging with bubble-contrast injection is a useful screening tool for right-to-left shunting via a patent foramen ovale (see image and video below). An ultrasound probe is placed on the head, and the ultrasound beam is used to sample the middle cerebral arteries. A bubble-contrast injection is performed at rest and with the Valsalva maneuver. Any right-to-left shunt is revealed by the appearance of bubble artifacts on the transcranial Doppler signal. The number of bubble counts correlates with the potential for right-to-left shunting. Modern transcranial Doppler with bubble-contrast injection is as sensitive as TEE with bubble-contrast injection in identifying a patent foramen ovale.[15] Transcranial Doppler (TCD) study with bubble-contrTranscranial Doppler (TCD) study with bubble-contrast study. A Doppler probe is used to interrogate the right middle cerebral artery. Frame 1 shows normal findings. Note the absence of bubble artifact of Doppler signal in the middle cerebral artery. Frame 2 shows strongly positive (5/5) bubble transit seen in a patient with a patent foramen ovale (PFO) during Valsalva maneuver. TCD is a useful screening tool for PFO because of its ease-of-use and ability to easily quantify the amount of potential right-to-left shunt. One of the pitfalls is the inability to differentiate between other sources of right-to-left shunt, such as pulmonary arteriovenous malformation and a PFO.
    Transesophageal echocardiogram of bubble contrast study showing right-to-left passage of bubble-contrast with a Valsalva maneuver. RA = Right atrium. LA = Left atrium. Arrow shows bubble passage to LA.
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Contributor Information and Disclosures
Author

Barry A Love, MD  Assistant Professor, Department of Medicine, Division of Cardiology, Assistant Professor, Division Pediatric Cardiology, Pediatrics and Medicine, Division of Pediatric Cardiology, Mount Sinai School of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Michael A Portman, MD  Research Director, Department of Pediatrics, Division of Cardiology, Associate Professor, Childrens' Hospital

Michael A Portman, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Physiological Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul M Seib, MD  Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Alvin J Chin, MD  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

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.

References

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This 2-dimensional echocardiogram in an infant (subcostal long-axis view) shows a patent foramen ovale. Right atrium (RA) and left atrium (LA).
Color Doppler of the patent foramen ovale (PFO) seen in the previous image. A small amount of left-to-right flow is present. This left-to-right flow pattern is typical for PFO seen in newborn infants.
Transesophageal echocardiogram showing the atrial septum. The "flap" of the septum primum is seen. The diagnosis of patent foramen ovale (PFO) cannot be made until right-to-left bubble contrast is demonstrated. LA = Left atrium; RA = Right atrium.
Bubble-contrast injection during Valsalva maneuver. The "flap" of the foramen ovale is opened and bubbles are seen crossing from the right atrium to the left atrium (arrow).
Transesophageal echocardiogram revealing a 25-mm Amplatzer patent foramen ovale (PFO) occluder in place across the PFO shown in the previous 2 images.
Transesophageal echocardiogram of a patent foramen ovale (PFO) closed with 25-mm Amplatzer PFO occluder. Bubble-contrast study with Valsalva post-device placement shows no residual right-to-left bubble passage.
Transcranial Doppler (TCD) study with bubble-contrast study. A Doppler probe is used to interrogate the right middle cerebral artery. Frame 1 shows normal findings. Note the absence of bubble artifact of Doppler signal in the middle cerebral artery. Frame 2 shows strongly positive (5/5) bubble transit seen in a patient with a patent foramen ovale (PFO) during Valsalva maneuver. TCD is a useful screening tool for PFO because of its ease-of-use and ability to easily quantify the amount of potential right-to-left shunt. One of the pitfalls is the inability to differentiate between other sources of right-to-left shunt, such as pulmonary arteriovenous malformation and a PFO.
Transesophageal echocardiogram of bubble contrast study showing right-to-left passage of bubble-contrast with a Valsalva maneuver. RA = Right atrium. LA = Left atrium. Arrow shows bubble passage to LA.
Transesophageal echocardiogram showing a 10-mm Amplatzer Septal Occluder in place across a patent foramen ovale.
 
 
 
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