Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair

Cuttone, Fabio; Hadeed, Khaled; Lacour-Gayet, François; Lucron, Hugues; Hascoët, Sébastien; Acar, Philippe; Léobon, Bertrand; Praagh, Richard Van

doi:10.1093/icvts/ivx006

Cited by 6 publications

(12 citation statements)

References 14 publications

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“…This process is finalised by the drainage of the pulmonary veins into the anatomic right atrium despite their normal connection to the posterior wall of the left atrium. [4][5][6][7][8][9][10][11] Recently, new hypotheses have been proposed on the development of atrial septum based on the experimental data. 12,13 Goddeeris et al 12 used a genetic marker and novel magnetic resonance microscopy technique to demonstrate the origins of the dorsal mesenchymal protrusion in the dorsal mesocardium, and its valuable contribution to atrioventricular septation.…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, the atrial septal displacement technique without the substitution by a pericardial patch has been proposed as beneficial because it avoids patch retraction and allows natural septal growth. 6,8,16 In our study, the atrial septal displacement and pericardial patch techniques were both performed for surgical correction.…”

Section: Discussionmentioning

confidence: 99%

“…5 Although this anomaly usually occurs in conjunction with the polysplenia subset of heterotaxy syndrome or hypoplastic left heart syndrome, isolated cases have been reported. 4,6 Diagnoses can be established by transthoracic echocardiography. The deviation of the septum primum is best demonstrated in the subxiphoid coronal, apical four-chamber, and parasternal long-axis views.…”

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confidence: 99%

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A rare cause of abnormal pulmonary venous drainage: septum primum malposition

et al. 2020

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Objectives: This study aimed to evaluate the clinical features of patients with septum primum malposition, imaging tools used for diagnosis, and their effects on the surgical approach. Materials and methods: Patients diagnosed with septum primum malposition in our paediatric cardiac centre between 1 January, 2015 and 1 January, 2019 were included in the study. In all patients, the age, reason for admission, transthoracic echocardiography, cardiac multidetector CT angiography findings, and subsequent surgical data were evaluated. Results: Fifteen patients were diagnosed with septum primum malposition during the study period. The median age was 12 months (2 months–10 years). Six patients were left isomeric, and the rest were situs solitus; 80% of the patients (n = 12) had additional secundum atrial septal defect. There was cardiac pathology in 46% of the patients (n = 7) in addition to the abnormal pulmonary venous drainage, ventricular septal defect (n = 3), left ventricularhypoplasia (n = 2), cortriatriatum sinister (n = 2), double outlet right ventricle (n = 1), and atrioventricular septal defect (n = 1). There was bilateral superior caval vein in three patients, right-sided superior caval vein in 11 patients, and left-sided superior caval vein in one patient. All three patients with total abnormal pulmonary venous drainage were left atrial isomeric. There were differences between the results of transthoracic echocardiography and CT angiographies in two patients. The surgical strategy was changed in three patients after the preoperative diagnosis of septum primum malposition. Conclusion: Septum primum malposition should be kept in mind during the imaging of complex CHDs specifically during the segmental analysis of the pathologies with heterotaxy syndromes; it should be differentiated from other aetiologies of abnormal pulmonary venous drainage as accurate diagnosis would facilitate the ideal surgery in these complex pathologies requiring a detailed preoperative preparation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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A rare cause of abnormal pulmonary venous drainage: septum primum malposition

et al. 2020

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“…4,5 Isolated severe displacement of the septum primum in patients with situs solitus has been described in recent literature. 6 We present a previously undescribed case of septum primum malposition leading to anomalous pulmonary venous drainage and interatrial communication in a child with dextrocardia and situs inversus totalis.…”

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Rare case of septum primum malposition defect in dextrocardia and situs inversus totalis without heterotaxy syndrome

2018

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Septum primum malposition defect is an extremely rare CHD and is often found in patients with visceral heterotaxy, particularly of the polysplenia type. We describe a unique patient with dextrocardia, situs inversus totalis, and bilateral superior vena caval veins without heterotaxy syndrome who was diagnosed with an interatrial defect and partial anomalous pulmonary venous drainage due to malposition of the septum primum.

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“…Since we do not know for certain the nature of the shelf, is there any justification for continuing to presume that the lesion was initially the flap valve of the oval foramen, as is frequently the case? [5][6][7][8] Is it not better to opt for certainty, and to describe the lesion on the basis of the feature on which all agree? Thus, irrespective of the origin of the partition, it no longer subserves a septal function but instead divides the morphologically left atrium.…”

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Divided left atrium with totally anomalous drainage of normally connected pulmonary veins

2022

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In the December 2021 issue of Cardiology in the Young , Hubrechts and colleagues, from Brussels and Leuven in Belgium, describe their experience in which the pulmonary veins were normally connected to the morphologically left atrium. By virtue of the presence of a shelf dividing the morphologically left atrium, however, the venous return was to the morphologically right atrium, with no evidence of formation of the superior interatrial fold, meaning that there was no obstruction of flow into the systemic venous circulation. The question posed by the Belgian authors is whether the shelf dividing the morphologically left atrium is a deviated primary atrial septum, as the arrangement has previously been interpreted. As they discuss, it is currently impossible to arbitrate this conundrum. In our commentary, we discuss the background to the dilemma. We point out that, as yet, it is not possible to code accurately this congenital cardiac malformation within The International Paediatric and Congenital Cardiac Code (IPCCC), nor within the newly produced 11th Revision of the International Classification of Diseases (ICD-11).

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Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair

Abstract: This article reports the severe leftward displacement of the septum primum presented as an isolated cardiac malformation; 3D transthoracic echocardiography allowed an accurate diagnosis of this malformation and helped in choosing the best surgical strategy.

Cited by 6 publications

References 14 publications

A rare cause of abnormal pulmonary venous drainage: septum primum malposition

A rare cause of abnormal pulmonary venous drainage: septum primum malposition

Rare case of septum primum malposition defect in dextrocardia and situs inversus totalis without heterotaxy syndrome

Divided left atrium with totally anomalous drainage of normally connected pulmonary veins

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