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

Published online by Cambridge University Press:  28 August 2020

Pelin Ayyıldız ,
Erkut Öztürk Open the ORCID record for Erkut Öztürk [Opens in a new window] ,
Taner Kasar ,
Aysel Türkvatan ,
İsmihan Selen Onan  and
Alper Güzeltaş
Pelin Ayyıldız*
Affiliation:
Department of Pediatric Cardiology, University of Health Sciences, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center, İstanbul, Turkey
Erkut Öztürk
Affiliation:
Department of Pediatric Cardiology, University of Health Sciences, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center, İstanbul, Turkey
Taner Kasar
Affiliation:
Department of Pediatric Cardiology, University of Health Sciences, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center, İstanbul, Turkey
Aysel Türkvatan
Affiliation:
Department of Radiology, University of Health Sciences, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center, İstanbul, Turkey
İsmihan Selen Onan
Affiliation:
Department of Pediatric Cardiovascular Surgery, University of Health Sciences, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center, İstanbul, Turkey
Alper Güzeltaş
Affiliation:
Department of Pediatric Cardiology, University of Health Sciences, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center, İstanbul, Turkey
*
Author for correspondence: Pelin Ayyıldız, MD, Department of Pediatric Cardiology, University of Health Sciences, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Center, Istasyon Mah. Turgut Ozal Bulvarı No: 11 Kucukcekmece, İstanbul34303, Turkey. Tel: +90 212 692 20 00 – 4020; Fax: +90 212 471 94 94. E-mail: [email protected]
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Abstract

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.

Keywords

Abnormal pulmonary venous drainageseptum primum malpositionCHDchildren
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 11 , November 2020 , pp. 1716 - 1721
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

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