Cor triatriatum with Raghib complex in partial atrioventricular septal defect and common atrium: a rare combination

Baek, Wan Ki; Kim, Young Sam; Yoon, Yong Han; Kim, Joung Taek

doi:10.1007/s11748-019-01159-7

Cited by 2 publications

(4 citation statements)

References 8 publications

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“…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.…”

Section: Literature Reviewmentioning

confidence: 99%

“…The same coexisting anomalies (cor triatriatum and Raghib syndrome) were reported in a 42-year-old female who also presented with an atrioventricular septal defect. 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 ].…”

Section: Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

“…An 18-month-old male patient diagnosed with Raghib syndrome in whom extracardiac correction was performed, which implied anastomosis of the LSVC to the right atrial appendage 8 Baek et al [14].…”

Section: Literature Reviewmentioning

confidence: 99%

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

Gozar,

Săsăran,

Cosma

et al. 2024

JCM

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Background: Raghib syndrome is a rare malformation complex consisting of the drainage of the left superior vena cava (LSVC) into the left atrium, ostial atresia of the coronary sinus and an atrial septal defect (ASD). Case Report: This report aims to present the case of a child newly diagnosed with Raghib syndrome, complicated by pulmonary arterial hypertension, and to review previously published cases with the same diagnosis. A six-year-old female patient presented with signs and symptoms of heart failure (Ross III), reduced exercise tolerance and severe delay in stature and ponderal development. The imagistic work-up included echocardiography, followed by computer tomography (CT) and magnetic resonance imaging (MRI), through which a diagnosis of Raghib syndrome was established, complicated by pulmonary hypertension. As in other cases presented in the literature, MRI allowed for an accurate diagnosis, detecting the absent coronary sinus. The decision regarding the surgical closure of the ASD was made, with the patient having a favorable clinical evolution but with the persistence of elevated pulmonary artery pressure, for which Sildenafil therapy was instituted. Conclusions: The malformation complex consisting of an atrial septal defect, ostium atresia of the coronary sinus, uncovered coronary sinus, and persistent left superior vena cava, as identified through multiple imagistic investigations, was suggestive of the rare diagnosis of Raghib syndrome in this case. Among the limited number of cases of Raghib syndrome available in the literature, the present case is distinguished by the severity of the pulmonary artery hypertension at a very young age and in the absence of other concurrent cardiac malformations.

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Section: Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

Section: Literature Reviewmentioning

confidence: 99%

See 2 more Smart Citations

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

Gozar,

Săsăran,

Cosma

et al. 2024

JCM

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A 4-month-old male with Raghib syndrome: a rare case report from Syria

Sleiay,

Batrash

et al. 2024

Annals of Medicine &Amp; Surgery

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Introduction and Importance: Raghib syndrome is a rare congenital complication consisting of the termination of the left superior vena cava (LSVC) in the left atrium, an unroofed coronary sinus, and an atrial septal defect most often found in the posterior-inferior angle of the atrial septum. Both a right-to-left and a left-to-right intracardiac shunt exist. In most circumstances, they do not show any symptoms. Presentation of Case: Our patient presented with a persistent left superior vena cava draining into the left atrium, an unroofed coronary sinus, and a secondary atrial septal defect (ASD). Transthoracic echocardiography was used to diagnose the condition, and surgery was applied as the primary treatment. Clinical Discussion: It was formerly believed that this complex was exclusive to Raghib Syndrome; however, cases have been found in which the interatrial connection is the aperture of the unroofed coronary sinus in patients with a normal atrial septum. Conclusion: Extracardiac treatment for this illness reduces the load on the left atrial suture and may stop further arrhythmias. There is no possibility of pulmonary vein flow restriction when there are no atrial tunnels.

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Cor triatriatum with Raghib complex in partial atrioventricular septal defect and common atrium: a rare combination

Cited by 2 publications

References 8 publications

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

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

A 4-month-old male with Raghib syndrome: a rare case report from Syria

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