ORIGINAL ARTICLE Benign Outcome of Pulmonary Hypertension in Neonates With a Restrictive Patent Foramen Ovale Versus Result for Neonates With an Unrestrictive Patent Foramen Ovale Umang Gupta • Ra-id Abdulla • John Bokowski Received: 27 April 2011 / Accepted: 9 June 2011 / Published online: 28 June 2011 Ó Springer Science+Business Media, LLC 2011 Abstract Premature closure or restriction of foramen ovale (FO) is a rare but known entity. FO diameter \ 2 mm and Doppler velocity [ 120 cm/s, diameter \ 3 mm with Doppler velocity measured gradient [ 5 mmHg have all being used by various authors to describe this entity. Some neonates with restrictive FO have been noted to have severe pulmonary hypertension with no clinical signs or symptoms and with spontaneous resolution without any intervention. Seven consecutive neonates were indentified in the database between 01/01/2003 and 06/30/2010 with diagnosis of restrictive PFO (diameter \ 2 mm) with structurally normal heart and their initial and follow-up echocardiogram as well as hospital medical records were reviewed. As a control, seven neonates with diagnosis of pulmonary hypertension and respiratory distress syndrome or meconium aspiration syndrome were randomly selected. Eighty-six percent of the patients in the control group were symptomatic and required treatment as compared to 14% in the restrictive FO group (p = 0.03). Further, the fall in the peak instantaneous pulmonary artery pressure on follow-up echocardiogram was greater in the restrictive FO group compared with the non-restrictive group (p = 0.03). Patients with pulmonary hypertension and a restrictive FO with no other associated congenital heart disease and/or lung pathology behave differently when compared to neonates with non-restrictive FO and pulmonary hyper- tension with associated lung disease. They seldom manifest symptoms requiring intervention and tend to show a faster drop in their pulmonary artery pressure toward the normal. Keywords Neonates Á Pulmonary hypertension Á Restrictive foramen ovale Premature closure or restriction of the foramen ovale (FO) is a rare but known entity. One of the earliest case reports describing this condition was published in 1939 [1], and the first prenatal diagnosis of this disorder was published in 1982 by Hansmann and Redel [6]. Different authors have used various pathologic/echocardiogram-based criteria for diagnosis including a diameter less than 2 mm with a Doppler velocity exceeding 120 cm/s and an FO smaller than 3 mm with a Doppler velocity measured gradient exceeding 5 mmHg [2, 3, 5, 7, 8, 10]. In the earlier decades, restrictive FO was thought to be one of the pathologies responsible for hypoplastic left heart syndrome (HLHS), but that notion currently has been questioned, and this condition is considered to be an effect rather than the cause for HLHS. Nevertheless, a restrictive foramen ovale has been indicated as a factor for poor outcome among patients with congenital heart disease (CHD). In one review of 370 fetal echocardiograms by Donofrio et al. [4], FO restriction was present in six fetu- ses, all of whom had CHD, and associated with a dismal outcome. Some authors have suggested performing serial ultra- sound assessment for at-risk fetuses with CHD to ensure fetal well-being, plan the timing and mode of delivery, and develop management protocols for in utero and postnatal care. Some authors even suggest stenting of the restrictive septum [9]. Others have noted a relationship between restrictive patent FO (PFO) and poor outcome for patients with dextro-transposition of the great arteries (D-TGA) [10]. U. Gupta (&) Á R.-i. Abdulla Á J. Bokowski Department of Pediatric Cardiology, Rush University Medical Center, Chicago, IL, USA e-mail: umanggupta@yahoo.com 123 Pediatr Cardiol (2011) 32:972–976 DOI 10.1007/s00246-011-0032-7