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

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.

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.

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).

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.