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

Part of: Cardiac Morphology

Published online by Cambridge University Press:  26 December 2018

Simone Jhaveri ,
Francine Erenberg  and
Malek Yaman
Simone Jhaveri*
Affiliation:
Department of Pediatric Cardiology, Cleveland Clinic Children’s, Cleveland, OH, USA
Francine Erenberg
Affiliation:
Department of Pediatric Cardiology, Cleveland Clinic Children’s, Cleveland, OH, USA
Malek Yaman
Affiliation:
Department of Pediatric Cardiology, Cleveland Clinic Children’s, Cleveland, OH, USA
*
Author for correspondence: Simone Jhaveri, 9500 Euclid Avenue, Cleveland, OH 44195, USA. Tel: +1 216-337-4433; Fax: +1 216-445-5679; E-mail: [email protected]
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Abstract

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.

Keywords

Septum primum malpositionpartial anomalous pulmonary venous drainageheterotaxysitus inversus totalisdextrocardia
Type
Brief Report
Information
Cardiology in the Young , Volume 29 , Issue 3 , March 2019 , pp. 419 - 421
Copyright
© Cambridge University Press 2018 

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Footnotes

Cite this article: Jhaveri S, Erenberg F, Yaman M. (2019) Rare case of septum primum malposition defect in dextrocardia and situs inversus totalis without heterotaxy syndrome. Cardiology in the Young29: 419–421. doi: 10.1017/S1047951118002263

References

1. Neill, CA. Development of the pulmonary veins with reference to embryology of the anomalies of pulmonary venous return. Pediatrics 1956; 18: 880–887.Google Scholar
2. Van Praagh, S, Carrera, ME, Sanders, S, Mayer, JE, Van Praagh, R. Partial or total direct pulmonary venous drainage to right atrium due to malposition of septum primum: anatomic and echocardiographic findings and surgical treatment: a study based on 36 cases. Chest 1995; 107: 1488–1498.Google Scholar
3. Chin, AJ, Weinberg, PM, Barber, G. Subcostal two-dimensional echocardiographic identification of anomalous attachment of septum primum in patients with left atrioventricular valve underdevelopment. J Am Coll Cardiol 1990; 15: 678–681.Google Scholar
4. Hiramatsu, MD T, Takanashi, MD Y, Imai, MD Y, et al. Atrial septal displacement for repair of anomalous pulmonary venous return into the right atrium. Ann Thorac Surg 1998; 65: 1110–1114.Google Scholar
5. Kouchoukos, NT, Blackstone, EH, Hanley, FL, Kirklin, JK. 30 Atrial septal defect and partial anomalous pulmonary venous connection. Kirklin/Barratt-Boyes Card Surg Fourth Ed 2013: 1149–1181.Google Scholar
6. Cuttone, F, Hadeed, K, Lacour-Gayet, F, et al. Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair. Interact Cardiovasc Thorac Surg 2017; 24: 772–777.Google Scholar
7. Moller, JH, Nakib, A, Anderson, RAYC, Edwards, E. Congenital cardiac disease associated with polysplenia. Circulation 1967; 36: 789–799.Google Scholar
8. Silvestri, LM, Scarabotti, A, Marino, B. Leftward displacement of septum primum in children with congenital heart disease. Pediatr Cardiol 2014; 35: 185.Google Scholar
9. Park, M V, Fedderly, RT, Frommelt, PC, et al. Leftward displacement of septum primum in hypoplastic left heart syndrome. Pediatr Cardiol 2013; 34: 942–947.Google Scholar
10. Tomar, M, Radhakrishnan, S, Shrivastava, S. Partial or total anomalous pulmonary venous drainage caused by malposition of septum primum: echocardiographic description of a rare variant of anomalous pulmonary venous drainage. J Am Soc Echocardiogr 2005; 18: 15–18.Google Scholar
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