Original Article

The Presence of Patent Foramen Ovale in the Superior Type of Sinus Venosus Atrial Septal Defect


Background: The superior type of sinus venosus atrial septal defect (SVASD) is a rare form of the atrial septal defect (ASD) in which the upper part of the atrial septum does not exist. The presence of other cardiac anomalies such as anomalous pulmonary venous connections has been reported in this type of congenital heart disease. This study aimed to assess the presence of the patent foramen ovale (PFO) in patients with the superior type of SVASD.
Methods: This retrospective case-control study on 387 patients, consisting of 187 patients with a definite SVASD and 200 patients with problems other than the ASD, was conducted in Rajaie Cardiovascular Medical and Research Center between February 2005 and July 2014. Seven patients with inadequate data were excluded from the analysis. The presence/absence of the PFO was also evaluated in the case and control groups.
Results: The analyses were performed on 182 male and 198 female patients at a mean age of 39.07±14.41 and 51.01±15.80 years in the case and control groups, respectively. The PFO was significantly more frequent in the patients with the superior type of SVASD than in those without the condition (P<0.001). The persistence of the left superior vena cava was seen in 34 out of 180 patients with SVASD and in 1 out of 200 patients without the condition (18.9% vs 0.5%; P<0.001).
Conclusion: This study was the first to highlight the coexistence of the PFO and the superior type of SVASD. Physiological, genetic, or fetal factors may play an important role in the association between the PFO and the SVASD.

1. Naqvi N, McCarthy KP, Ho SY. Anatomy of the atrial septum and interatrial communications. J Thorac Dis 2018;10(Suppl 24):S2837-S2847.
2. Windecker S, Wahl A, Nedeltchev K, Arnold M, Schwerzmann M, Seiler C, Mattle HP, Meier B. Comparison of medical treatment with percutaneous closure of patent foramen ovale in patients with cryptogenic stroke. J Am Coll Cardiol 2004;44:750-758.
3. Turc G, Calvet D, Guérin P, Sroussi M, Chatellier G, Mas JL; CLOSE Investigators. Closure, anticoagulation, or antiplatelet therapy for cryptogenic stroke with patent foramen ovale: systematic review of randomized trials, sequential meta-analysis, and new insights from the CLOSE study. J Am Heart Assoc 2018;7:e008356.
4. Kutty S, Sengupta PP, Khandheria BK. Patent foramen ovale: the known and the to be known. J Am Coll Cardiol 2012;59:1665-1671.
5. Morton SU, Brodsky D. Fetal Physiology and the Transition to Extrauterine Life. Clin Perinatol 2016;43:395-407.
6. Chubb H, Whitaker J, Williams SE, Head CE, Chung NA, Wright MJ, O'Neill M. Pathophysiology and management of arrhythmias associated with atrial septal defect and patent foramen ovale. Arrhythm Electrophysiol Rev 2014;3:168-172.
7. Falanga G, Carerj S, Oreto G, Khandheria BK, Zito C. How to understand patent foramen ovale clinical significance: Part I. J Cardiovasc Echogr 2014;24:114-121.
8. Lynch JJ, Schuchard GH, Gross CM, Wann LS. Prevalence of right-to-left atrial shunting in a healthy population: detection by Valsalva maneuver contrast echocardiography. Am J Cardiol 1984;53:1478-1480.
9. Webb G, Gatzoulis MA. Atrial septal defects in the adult: recent progress and overview. Circulation 2006;114:1645-1653.
10. Attenhofer Jost CH, Connolly HM, Danielson GK, Bailey KR, Schaff HV, Shen WK, Warnes CA, Seward JB, Puga FJ, Tajik AJ. Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients. Circulation 2005;112:1953-1958.
11. Pascoe RD, Oh JK, Warnes CA, Danielson GK, Tajik AJ, Seward JB. Diagnosis of sinus venosus atrial septal defect with transesophageal echocardiography. Circulation 1996;94:1049-1055.
12. Donovan MS, Kassop D, Liotta RA, Hulten EA. Sinus venosus atrial septal defect as a cause of palpitations and dyspnea in an adult: a diagnostic imaging challenge. Case Rep Med 2015;2015:128462.
13. Kafka H, Mohiaddin RH. Cardiac MRI and pulmonary MR angiography of sinus venosus defect and partial anomalous pulmonary venous connection in cause of right undiagnosed ventricular enlargement. AJR Am J Roentgenol 2009;192:259-266.
14. Kivistö S, Hänninen H, Holmström M. Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography. J Cardiothorac Surg 2011;6:126.
15. Hoey ET, Lewis G, Yusuf S. Multidetector CT assessment of partial anomalous pulmonary venous return in association with sinus venosus type atrial septal defect. Quant Imaging Med Surg 2014;4:433-434.
16. Kessel-Schaefer A, Linka A, Pretre R, Buser P. Inferior sinus venosus defect associated with incomplete cor triatriatum dexter and patent foramen ovale. Eur J Echocardiogr 2006;7:239-242.
17. Sojak V, Sagat M, Balazova E, Siman J. Outcomes after surgical repair of sinus venosus atrial septal defect in children. Bratisl Lek Listy 2008;109:215-219.
18. al Zaghal AM, Li J, Anderson RH, Lincoln C, Shore D, Rigby ML. Anatomical criteria for the diagnosis of sinus venosus defects. Heart 1997;78:298-304.
19. Silvestry FE, Cohen MS, Armsby LB, Burkule NJ, Fleishman CE, Hijazi ZM, Lang RM, Rome JJ, Wang Y; American Society of Echocardiography; Society for Cardiac Angiography and Interventions. Guidelines for the echocardiographic assessment of atrial septal defect and patent foramen ovale: from the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. J Am Soc Echocardiogr 2015;28:910-958.
20. Hubail Z, Lemler M, Ramaciotti C, Moore J, Ikemba C. Diagnosing a patent foramen ovale in children: is transesophageal echocardiography necessary? Stroke 2011;42:98-101.
21. González-Alujas T, Evangelista A, Santamarina E, Rubiera M, Gómez-Bosch Z, Rodríguez-Palomares JF, Avegliano G, Molina C, Alvarez-Sabín J, García-Dorado D. Diagnosis and quantification of patent foramen ovale. Which is the reference technique? Simultaneous study with transcranial Doppler, transthoracic and transesophageal echocardiography. Rev Esp Cardiol 2011;64:133-139.
22. Yamashita E, Murata T, Goto E, Fujiwara T, Sasaki T, Minami K, Nakamura K, Kumagai K, Naito S, Kario K, Oshima S. Inferior vena cava compression as a novel maneuver to detect patent foramen ovale: a transesophageal echocardiographic study. J Am Soc Echocardiogr 2017;30:292-299.
23. Homma S, Sacco RL. Patent foramen ovale and stroke. Circulation 2005;112:1063-1072.
24. Sharma RK, Houston BA, Lima JA, Cameron DE, Tedford RJ. Never too old for congenital heart disease: sinus venosus atrial septal defect with anomalous pulmonary venous return in an octogenarian. Pulm Circ 2015;5:587-589.
25. Anuwatworn A, Gedela M, Bendaly E, Prescott-Focht JA, Yee J, Clark R, Jonsson O. Sinus venosus atrial septal defect complicated by Eisenmenger syndrome and the role of vasodilator therapy. Case Rep Cardiol 2016:8164923.
26. Ghosh S, Ghosh AK, Ghosh SK. Patent foramen ovale and atrial septal aneurysm in cryptogenic stroke. Postgrad Med J 2007;83:173-177.
27. Mügge A, Daniel WG, Angermann C, Spes C, Khandheria BK, Kronzon I, Freedberg RS, Keren A, Denning K, Engberding R. Atrial septal aneurysm in adult patients. A multicenter study using transthoracic and transesophageal echocardiography. Circulation 1995;91:2785-2792.
28. Razaq M, Parihar RK, Saini G. Atrial septal aneurysm and stroke. Ann Pediatr Cardiol 2012;5:98-99.
29. Disha B, Prakashini K, Shetty RK. Persistent left superior vena cava in association with sinus venosus defect type of atrial septal defect and partial pulmonary venous return on 64-MDCT. BMJ Case Rep 2014:bcr2013202999.
30. Sahay S, Krasuski RA, Tonelli AR. Partial anomalous pulmonary venous connection and pulmonary arterial hypertension. Respirology 2012;17:957-963.
31. Sahin T, Kilic T, Celikyurt U, Bildirici U, Ural D. Persistent left superior vena cava and partial anomalous pulmonary venous return in an old asymptomatic female patient. Cardiol Res Pract 2009:152164.
32. Kurtoglu E, Cakin O, Akcay S, Akturk E, Korkmaz H. Persistent left superior vena cava draining into the coronary sinus: a case report. Cardiol Res 2011;2:249-252.
IssueVol 15 No 3 (2020): J Teh Univ Heart Ctr QRcode
SectionOriginal Article(s)
DOI https://doi.org/10.18502/jthc.v15i3.4218
Foramen ovale; patent Atrial septal defect Sinus venosus Echocardiography; transesophageal

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
Samiei N, Akiash N, Djafari Naeini S, Nikpajouh A, Pazoki M. The Presence of Patent Foramen Ovale in the Superior Type of Sinus Venosus Atrial Septal Defect. J Tehran Heart Cent. 15(3):98-104.