Pediatric Patent Foramen Ovale Atrial Septal Defects Clinical Presentation
- Author: Barry A Love, MD; Chief Editor: P Syamasundar Rao, MD more...
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.[14, 15] 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. 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.
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. 1984 Jan. 59(1):17-20. [Medline].
Kutty S, Sengupta PP, Khandheria BK. Patent foramen ovale: the known and the to be known. J Am Coll Cardiol. 2012 May 8. 59(19):1665-71. [Medline].
Menon SC, Grove A, McFadden M, et al. Clinical practice, resource utilization, and outcomes of device closure of patent foramen ovale in pediatrics. Pediatr Neurol. 2014 Mar. 50(3):213-7. [Medline].
Agnetti A, Carano N, Sani E, et al. Cryptogenic stroke in children: possible role of patent foramen ovale. Neuropediatrics. 2006 Feb. 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. 2006 May. 81(5):615-8. [Medline].
Filippi L, Palermo L, Pezzati M, et al. Paradoxical embolism in a preterm infant. Dev Med Child Neurol. 2004 Oct. 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. 2006 May. 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. 2005 Feb 15. 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. 2004 Jan 28. 291(4):427-34. [Medline].
Post MC, Budts W. The relationship between migraine and right-to-left shunt: Fact or fiction?. Chest. 2006 Sep. 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. 2002 Aug 15. 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. 2008 Feb. 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. 1995 Jun. 107(6):1504-9. [Medline].
Schwedt TJ, Demaerschalk BM, Dodick DW. Patent foramen ovale and migraine: a quantitative systematic review. Cephalalgia. 2008 May. 28(5):531-40. [Medline].
McCandless RT, Arrington CB, Nielsen DC, Bale JF Jr, Minich LL. Patent foramen ovale in children with migraine headaches. J Pediatr. 2011 Aug. 159(2):243-7.e1. [Medline].
Toffart AC, Bouvaist H, Feral V, Blin D, Pison C. Hypoxemia-orthodeoxia related to patent foramen ovale without pulmonary hypertension. Heart Lung. 2008 Sep-Oct. 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. 2001 Dec 13. 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. 2008 Sep. 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. 2002 Jun 4. 105(22):2625-31. [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. 2004 Jun. 25(12):1014-20. [Medline]. [Full Text].
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. 1998 Jul. 42(6):643-7. [Medline].
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. 2008 Aug 18. [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. 2008 Oct 27. 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. 2008 Oct 27. 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. 2004 Apr 13. 62(7):1042-50. [Medline].
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. 2005 Jun. 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. 1999 Nov 9. 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. 1998 Jun. 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. 1998 Jan. 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. 2004 Nov 15. 94(10):1341-3. [Medline].
Hansen LK, Oxhoj H. High prevalence of interatrial communications during the first three months of life. Pediatr Cardiol. 1997 Mar-Apr. 18(2):83-5. [Medline].
Hanzel GS. Complications of patent foramen ovale and atrial septal defect closure devices. J Interv Cardiol. 2006 Apr. 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. 2002 Aug 15. 90(4):395-400. [Medline].
Maisel WH, Laskey WK. Patent foramen ovale closure devices: moving beyond equipoise. JAMA. 2005 Jul 20. 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. 2006 Jan 17. 47(2):440-5. [Medline].
Rao PS. Transcatheter management of platypnea-orthodeoxia syndrome. J Invasive Cardiol. 2004 Oct. 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. 2005 May 11. 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. 2004 Nov. 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. 2005 Mar-Apr. 32(2):129-33. [Medline].