Cor triatriatum with supramitral ring: “cor tetratriatum”, associated with Raghib syndrome with Eisenmenger syndrome: multimodality imaging approach in this exceedingly rare case report

Abstract

Background Cor triatriatum and supramitral ring are congenital anomalies which result in formation of three chambers of atria. To the best of our knowledge, simultaneous presence of both entities in the same patient resulting in the formation of four chambers of atria has not been described in the literature. Here, we report a case of simultaneous presence of cor triatriatum and supramitral ring associated with Raghib syndrome and Eisenmenger syndrome. Case presentation We report the case of a middle-aged gentleman, who presented to us with features of atrial septal defect with Eisenmenger syndrome. Multimodality imaging confirmed the simultaneous presence of supramital ring and cor triatriatum resulting in “cor tetratriatum” along with Raghib syndrome. Presence of Eisenmenger syndrome compelled us to offer medical therapy for the patient. Conclusion This is the first case report describing the simultaneous presence of supramitral ring and cor triatriatum resulting in a new entity—“cor tetratriatum”.

Supramitral membrane with left atrial appendage thrombus: A rare entity

“…The membrane is characteristically present below the LAA and foramen ovale in SMM, while it is above the LAA in cor triatriatum. [ 4 ]…”

Raghib Syndrome and Pulmonary Arterial Hypertension in a Pediatric Patient: Case Report and Literature Review

“…After the case series published by Raghib et al, in which co-existing cardiac anomalies such as common atrioventricular canal and ventricular septal defect were reported, the associations between Raghib syndrome and other malformations have been subsequently described. Cor triatriatum has been by far the most frequent concurrent cardiac anomaly in patients with this malformation complex [ 13 , 14 , 15 ]. Nair et al described a unique association between Raghib syndrome and cor triatriatum in an 18-month-old child, who had developed tachypnea early, at the age of two months, and presented pre-operatory O2 saturations of 75% in ambient air.…”

“…In this case, the cor triatriatum was diagnosed intraoperatively [ 14 ]. Cor triatriatum and Raghib syndrome were also reported simultaneously with a supramitral ring (resulting in “cor tetratriatum”) and Eisenmenger syndrome in a middle-aged man [ 15 ]. Another association between Raghib syndrome and the double-orifice tricuspid valve has been reported as well, in a 42 year-old male with a history of Wolff–Parkinson–White syndrome, who was diagnosed after transthoracic and transesophageal cardiac ultrasounds [ 16 ].…”

“…After the case series published by Raghib et al, in which co-existing cardiac anomalies such as common atrioventricular canal and ventricular septal defect were reported, the associations between Raghib syndrome and other malformations have been subsequently described. Cor triatriatum has been by far the most frequent concurrent cardiac anomaly in patients with this malformation complex [13][14][15]. Nair et al described…”

Nonobstructive Vertical Folding Supramitral Membrane

“…[ 3 ] Cor triatriatum sinister and supramitral rings are rare congenital heart diseases dividing the LA into two chambers; however, the position of the membrane differs between these two anomalies. [ 4 ] In cor triatriatum sinister or cor triatriaum dexter, a sagittal membrane or a fibromuscular band divides the atrium into two chambers. [ 5 ] In cor triatriatum sinister one of them containing the MV and the LA appendage and the other one containing the pulmonary veins.…”