eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Atrial Septal Defect, Patent Foramen Ovale

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
Coauthor(s): Michael A Portman, MD, Research Director, Department of Pediatrics, Division of Cardiology, Associate Professor, Childrens' Hospital
Contributor Information and Disclosures

Updated: Mar 18, 2009

Introduction

Background

The foramen ovale is a normal cardiac structure found in all newborns and can be best described as a "door" between the right and left atria.

The foramen ovale is essential for proper fetal circulation, directing oxygenated, nutrient-rich blood from the placenta, preferentially to the developing fetal brain. During fetal life, the "door" is open, and blood passes from the right to left atrium. However, with separation from the placenta and with the first few breaths, the left atrium fills with blood returning from the lungs and closes the "door."

During the first years of life, the foramen ovale seals shut and becomes a true wall that separates the right and left atria. However, in a significant proportion of people, the foramen ovale does not seal shut and remains a potential trapdoor between the 2 atria. A patent foramen ovale (PFO) is defined as a foramen ovale that does not seal.

This 2-dimensional echocardiogram in an infant (s...

This 2-dimensional echocardiogram in an infant (subcostal long-axis view) shows a patent foramen ovale. Right atrium (RA) and left atrium (LA).

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This 2-dimensional echocardiogram in an infant (s...

This 2-dimensional echocardiogram in an infant (subcostal long-axis view) shows a patent foramen ovale. Right atrium (RA) and left atrium (LA).


All newborns are expected to have a patent foramen ovale. The time frame over which most seal shut varies. However, adult autopsy studies have shown that 20-34% of adults from the third to ninth decades of life have at least a small patent foramen ovale.1

Although it is a normal structure, a foramen ovale has several special circumstances under which it may be implicated in disease.

Pathophysiology

The foramen ovale is an interatrial communication that permits blood from the inferior vena cava to freely enter the left atrium in utero. Anatomically, a thick muscular ridge, the limbus of the fossa ovalis, borders the foramen ovale. A thin tissue flap on the left atrial side of the septum, which represents an embryological remnant of the septum primum, forms the valve of the fossa ovalis. At birth, the left atrial pressure exceeds the right atrial pressure and forces the valve against the limbus, thus achieving physiological closure. During the first weeks of life, Doppler echocardiographic studies in healthy newborns can often demonstrate incompetence of the valve that allows some degree of left-to-right shunting. Shunting generally resolves by age 1 year as the foramen ovale seals shut.

Persistent left atrial enlargement associated with specific cardiac lesions, such as mitral valve stenosis, mitral valve regurgitation, patent ductus arteriosus, or ventricular septal defect, can render the foramen ovale "incompetent." Atrial left-to-right shunting can continue as a result.

Right-to-left shunting can occur through a patent foramen ovale, especially in conditions associated with elevated right atrial pressure such as tricuspid valve stenosis or right ventricular hypoplasia with decreased right ventricle compliance. Patients with persistent or transient elevation of right atrial pressure can experience a paradoxical embolus through a patent foramen ovale. Some congenital heart lesions depend on the foramen ovale for obligatory left-to-right (mitral atresia) or right-to-left (tricuspid atresia, total anomalous pulmonary venous return) shunting to maintain adequate cardiac output.

Frequency

International

Several echocardiography and postmortem studies indicate that the foramen remains competent in 30% of patients with otherwise normal cardiac anatomy.

Mortality/Morbidity

The vast majority of patients with a patent foramen ovale experience no symptoms throughout life.

Morbidity, although rare, is predominantly due to paradoxical embolism. Cerebrovascular ischemic events can be attributed to paradoxical embolism through a patent foramen ovale.2,3,4,5 This usually occurs in patients without other risk factors, although deep venous thrombosis and hypercoagulable states may significantly increase this risk.

Migraine headaches, especially with aura, have been found to be associated with the presence of a patent foramen ovale. As many as 50% of patients with migraine headaches can be found to have a patent foramen ovale, compared with a 15-30% prevalence in the normal population.6 The reason for this correlation is not established. Paradoxical embolization of small clots is a possible etiology. Eight percent of patients with migraine headaches have been shown to have evidence of asymptomatic strokes on brain MRI.7 Another possible etiology is the transit of vasoactive substances from the venous circulation to the arterial circulation without being modified by the lungs, which then causes cerebral hyperreactivity.8

Race

Based on transesophageal echocardiography (TEE) findings in the setting of cryptogenic stroke in adults, some studies have shown black patients to be about half as likely to have patent foramen ovale as white patients;9 however, a more recent study showed no racial difference.10

Sex

The prevalence of patent foramen ovale appears to be similar in men and women.10

Age

The foramen ovale is a potential opening in virtually all newborns. The foramen ovale seals shut over the first months of life in most infants. Studies of adults by contrast TEE shows a patent foramen ovale incidence rate of approximately 13% in adults in the fourth decade of life that decreases to approximately 6% by the eighth decade of life.11  Autopsy series reveal a somewhat higher prevalence, with an incidence rate of approximately 30% in the third decade of life, decreasing to 20% by the ninth decade of life.1

Clinical

History

  • The trivial amount of left-to-right shunting through a patent foramen ovale (PFO) generally produces no symptoms.
  • Patients with right-to-left shunting can experience transient or persistent periods of cyanosis. This can be exacerbated by acute increases in pulmonary vascular resistance, such as those that occur during breath holding, crying, or the Valsalva maneuver. Persistent cyanosis due to right-to-left shunting may also occur during the neonatal period until pulmonary vascular resistance falls.
  • Premature closure of the foramen ovale in-utero may lead to underdevelopment of the left-sided structure of the heart and hypoplastic left-sided heart syndrome. About 10% of patients with hypoplastic left-sided heart syndrome have an intact or nearly intact atrial septum in-utero.
  • Paradoxical emboli through a patent foramen ovale can cause a constellation of neurologic symptoms, such as stroke or transient ischemic attacks. Paradoxical embolization more often produces symptoms when the embolization occurs in the posterior cerebral circulation.
  • Migraine headaches are associated with a patent foramen ovale.12 The exact mechanism is not yet clear.
  • Rarely, the clinical constellation of orthodeoxia-platypnea may be seen as a result of a patent foramen ovale.13 Orthodeoxia is desaturation with upright posture, whereas platypnea is dyspnea with upright posture. This occurs in the absence of pulmonary hypertension and with relatively low or normal right and left atrial pressures and is sometimes seen following pneumonectomy. Transcatheter patent foramen ovale closure eliminates the right-to-left shunt and restores normal arterial oxygen saturation.

Physical

  • No physical findings clearly indicate a patent foramen ovale without an associated congenital heart defect; however, the presence of a patent foramen ovale with right-to-left shunting should be considered in an infant with generalized cyanosis.
  • Right-to-left atrial level shunting results in symmetric central cyanosis rather than differential cyanosis.

More on Atrial Septal Defect, Patent Foramen Ovale

Overview: Atrial Septal Defect, Patent Foramen Ovale
Differential Diagnoses & Workup: Atrial Septal Defect, Patent Foramen Ovale
Treatment & Medication: Atrial Septal Defect, Patent Foramen Ovale
Follow-up: Atrial Septal Defect, Patent Foramen Ovale
Multimedia: Atrial Septal Defect, Patent Foramen Ovale
References
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References

  1. Hagen PT, Scholz DG, Edwards WD. Incidence and size of patent foramen ovale during the first 10 decades of life: an autopsy study of 965 normal hearts. Mayo Clin Proc. Jan 1984;59(1):17-20. [Medline].

  2. Agnetti A, Carano N, Sani E, et al. Cryptogenic stroke in children: possible role of patent foramen ovale. Neuropediatrics. Feb 2006;37(1):53-6. [Medline].

  3. Bartz PJ, Cetta F, Cabalka AK, et al. Paradoxical emboli in children and young adults: role of atrial septal defect and patent foramen ovale device closure. Mayo Clin Proc. May 2006;81(5):615-8. [Medline].

  4. Filippi L, Palermo L, Pezzati M, et al. Paradoxical embolism in a preterm infant. Dev Med Child Neurol. Oct 2004;46(10):713-6. [Medline].

  5. Petty GW, Khandheria BK, Meissner I, et al. Population-based study of the relationship between patent foramen ovale and cerebrovascular ischemic events. Mayo Clin Proc. May 2006;81(5):602-8. [Medline].

  6. Reisman M, Christofferson RD, Jesurum J, et al. Migraine headache relief after transcatheter closure of patent foramen ovale. J Am Coll Cardiol. Feb 15 2005;45(4):493-5. [Medline].

  7. Kruit MC, van Buchem MA, Hofman PA, Bakkers JT, Terwindt GM, Ferrari MD. Migraine as a risk factor for subclinical brain lesions. JAMA. Jan 28 2004;291(4):427-34. [Medline].

  8. Post MC, Budts W. The relationship between migraine and right-to-left shunt: Fact or fiction?. Chest. Sep 2006;130(3):896-901. [Medline].

  9. Kizer JR, Silvestry FE, Kimmel SE, Kasner SE, Wiegers SE, Erwin MB. Racial differences in the prevalence of cardiac sources of embolism in subjects with unexplained stroke or transient ischemic attack evaluated by transesophageal echocardiography. Am J Cardiol. Aug 15 2002;90(4):395-400. [Medline].

  10. Gupta V, Yesilbursa D, Huang WY, Aggarwal K, Gupta V, Gomez C. Patent foramen ovale in a large population of ischemic stroke patients: diagnosis, age distribution, gender, and race. Echocardiography. Feb 2008;25(2):217-27. [Medline].

  11. Fisher DC, Fisher EA, Budd JH, et al. The incidence of patent foramen ovale in 1,000 consecutive patients. A contrast transesophageal echocardiography study. Chest. Jun 1995;107(6):1504-9. [Medline].

  12. Schwedt TJ, Demaerschalk BM, Dodick DW. Patent foramen ovale and migraine: a quantitative systematic review. Cephalalgia. May 2008;28(5):531-40. [Medline].

  13. Toffart AC, Bouvaist H, Feral V, Blin D, Pison C. Hypoxemia-orthodeoxia related to patent foramen ovale without pulmonary hypertension. Heart Lung. Sep-Oct 2008;37(5):385-9. [Medline].

  14. Mas JL, Arquizan C, Lamy C, Zuber M, Cabanes L, Derumeaux G. Recurrent cerebrovascular events associated with patent foramen ovale, atrial septal aneurysm, or both. N Engl J Med. Dec 13 2001;345(24):1740-6. [Medline].

  15. Telman G, Yalonetsky S, Kouperberg E, Sprecher E, Lorber A, Yarnitsky D. Size of PFO and amount of microembolic signals in patients with ischaemic stroke or TIA. Eur J Neurol. Sep 2008;15(9):969-72. [Medline].

  16. Homma S, Sacco RL, Di Tullio MR, Sciacca RR, Mohr JP. Effect of medical treatment in stroke patients with patent foramen ovale: patent foramen ovale in Cryptogenic Stroke Study. Circulation. Jun 4 2002;105(22):2625-31. [Medline].

  17. Mammoto T, Hayashi Y, Ohnishi Y, Kuro M. Incidence of venous and paradoxical air embolism in neurosurgical patients in the sitting position: detection by transesophageal echocardiography. Acta Anaesthesiol Scand. Jul 1998;42(6):643-7. [Medline].

  18. Torti SR, Billinger M, Schwerzmann M, et al. Risk of decompression illness among 230 divers in relation to the presence and size of patent foramen ovale. Eur Heart J. Jun 2004;25(12):1014-20. [Medline]. [Full Text].

  19. von Bardeleben RS, Richter C, Otto J, Himmrich L, et al. Long term follow up after percutaneous closure of PFO in 357 patients with paradoxical embolism: Difference in occlusion systems and influence of atrial septum aneurysm. Int J Cardiol. Aug 18 2008;[Medline].

  20. Majunke N, Baranowski A, Zimmermann W, et al. A suture not always the ideal solution: Problems encountered in developing a suture-based PFO closure technique. Catheter Cardiovasc Interv. Oct 27 2008;73(3):376-382. [Medline].

  21. Sievert H, Ruygrok P, Salkeld M, et al. Transcatheter closure of patent foramen ovale with radiofrequency: Acute and intermediate term results in 144 patients. Catheter Cardiovasc Interv. Oct 27 2008;73(3):368-373. [Medline].

  22. Messe SR, Silverman IE, Kizer JR, Homma S, Zahn C, Gronseth G. Practice parameter: recurrent stroke with patent foramen ovale and atrial septal aneurysm: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. Apr 13 2004;62(7):1042-50. [Medline].

  23. Arquizan C, Coste J, Touboul PJ, Mas JL. Is patent foramen ovale a family trait? A transcranial Doppler sonographic study. Stroke. Jul 2001;32(7):1563-6. [Medline]. [Full Text].

  24. Berger F, Ewert P, Bjornstad PG, et al. Transcatheter closure as standard treatment for interatrial defects: experience in 200 patients treated with the Amplatzer Septal Occluder. Cardiol Young. 1999;9:468-473. [Medline].

  25. Chatterjee T, Petzsch M, Ince H, et al. Interventional closure with Amplatzer PFO occluder of patent foramen ovale in patients with paradoxical cerebral embolism. J Interv Cardiol. Jun 2005;18(3):173-9. [Medline].

  26. Dearani JA, Ugurlu BS, Danielson GK, et al. Surgical patent foramen ovale closure for prevention of paradoxical embolism-related cerebrovascular ischemic events. Circulation. Nov 9 1999;100(19 Suppl):II171-5. [Medline].

  27. Duke DA, Lynch JJ, Harner SG, et al. Venous air embolism in sitting and supine patients undergoing vestibular schwannoma resection. Neurosurgery. Jun 1998;42(6):1282-6; discussion 1286-7. [Medline].

  28. Fuchs G, Schwarz G, Stein J, et al. Doppler color-flow imaging: screening of a patent foramen ovale in children scheduled for neurosurgery in the sitting position. J Neurosurg Anesthesiol. Jan 1998;10(1):5-9. [Medline].

  29. Guntheroth WG, Schwaegler R, Trent E. Comparative roles of the atrial septal aneurysm versus patent foramen ovale in systemic embolization with inferences from neonatal studies. Am J Cardiol. Nov 15 2004;94(10):1341-3. [Medline].

  30. Hansen LK, Oxhoj H. High prevalence of interatrial communications during the first three months of life. Pediatr Cardiol. Mar-Apr 1997;18(2):83-5. [Medline].

  31. Hanzel GS. Complications of patent foramen ovale and atrial septal defect closure devices. J Interv Cardiol. Apr 2006;19(2):160-2. [Medline].

  32. Kizer JR, Silvestry FE, Kimmel SE, et al. Racial differences in the prevalence of cardiac sources of embolism in subjects with unexplained stroke or transient ischemic attack evaluated by transesophageal echocardiography. Am J Cardiol. Aug 15 2002;90(4):395-400. [Medline].

  33. Maisel WH, Laskey WK. Patent foramen ovale closure devices: moving beyond equipoise. JAMA. Jul 20 2005;294(3):366-9. [Medline].

  34. Meissner I, Khandheria BK, Heit JA, et al. Patent foramen ovale: innocent or guilty? Evidence from a prospective population-based study. J Am Coll Cardiol. Jan 17 2006;47(2):440-5. [Medline].

  35. Rao PS. Transcatheter management of platypnea-orthodeoxia syndrome. J Invasive Cardiol. Oct 2004;16(10):583-4. [Medline].

  36. Schuchlenz HW, Weihs W, Berghold A, et al. Secondary prevention after cryptogenic cerebrovascular events in patients with patent foramen ovale. Int J Cardiol. May 11 2005;101(1):77-82. [Medline].

  37. Wilmshurst PT, Pearson MJ, Nightingale S, et al. Inheritance of persistent foramen ovale and atrial septal defects and the relation to familial migraine with aura. Heart. Nov 2004;90(11):1315-20. [Medline]. [Full Text].

  38. Wyland J, Krulak D. U.S. navy diver/aviator/skydiver with AGE from a previously unknown PFO. Undersea Hyperb Med. Mar-Apr 2005;32(2):129-33. [Medline].

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Further Reading

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Keywords

atrial septal defect, patent foramen ovale, ASD PFO, interatrial communication, congenital heart defect, left-to-right shunting, right-to-left shunting, incompetent valve of the fossa ovalis, right atrial enlargement, left atrial enlargement, paradoxical embolism, mitral valve stenosis, mitral valve regurgitation, patent ductus arteriosus, ventricular septal defect, tricuspid valve stenosis, right ventricular hypoplasia, tricuspid atresia, total anomalous pulmonary venous return, migraine headaches, deep venous thrombosis, hypoplastic left-sided heart syndrome, stroke, transient ischemic attacks

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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.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

CME Editor

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.

 
 
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