Pediatric Patent Foramen Ovale Atrial Septal Defects Clinical Presentation
- Author: Barry A Love, MD; Chief Editor: Stuart Berger, MD more...
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.
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].
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].
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].
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].
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].
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].
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].
Post MC, Budts W. The relationship between migraine and right-to-left shunt: Fact or fiction?. Chest. Sep 2006;130(3):896-901. [Medline].
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].
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].
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].
Schwedt TJ, Demaerschalk BM, Dodick DW. Patent foramen ovale and migraine: a quantitative systematic review. Cephalalgia. May 2008;28(5):531-40. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Hanzel GS. Complications of patent foramen ovale and atrial septal defect closure devices. J Interv Cardiol. Apr 2006;19(2):160-2. [Medline].
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].
Maisel WH, Laskey WK. Patent foramen ovale closure devices: moving beyond equipoise. JAMA. Jul 20 2005;294(3):366-9. [Medline].
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].
Rao PS. Transcatheter management of platypnea-orthodeoxia syndrome. J Invasive Cardiol. Oct 2004;16(10):583-4. [Medline].
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].
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].
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].

