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

Published online by Cambridge University Press:  29 April 2022

Robert H. Anderson Open the ORCID record for Robert H. Anderson [Opens in a new window] ,
Jeffrey P. Jacobs Open the ORCID record for Jeffrey P. Jacobs [Opens in a new window]  and
Rodney C.G. Franklin
Robert H. Anderson
Affiliation:
Cardiovascular Research Centre, Biosciences Institute, Newcastle University, Newcastle upon Tyne, UK
Jeffrey P. Jacobs*
Affiliation:
Congenital Heart Center, Division of Cardiovascular Surgery, Departments of Surgery and Pediatrics, University of Florida, Gainesville, FL, USA
Rodney C.G. Franklin
Affiliation:
Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK
*
Author for correspondence: J. P. Jacobs, MD, Congenital Heart Center, UF Health Shands Hospital, Division of Cardiovascular Surgery, Departments of Surgery and Pediatrics, University of Florida, 1600 SW Archer Road, Gainesville, FL, 32608, USA. Tel: 352-273-7770; Fax: 352-392-0547. E-mail: [email protected]
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Abstract

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).

Keywords

Pulmonary venous drainagecor triatriatum sinisternomenclaturecardiac development
Type
Commentaries
Information
Cardiology in the Young , Volume 32 , Issue 4 , April 2022 , pp. 641 - 643
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Copyright
© The Author(s), 2022. Published by Cambridge University Press

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References

Hubrechts, J, Pollenus, J, Gewillig, M. Leftward deviation of the primary septum or dividing left atrial shelf. 2021; 31: 1893–1900.Google ScholarPubMed
Franklin, RCG, Beland, M, Colan, S, et al. Nomenclature for congenital and paediatric cardiac disease. The International Paediatric and Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11). Cardiol Young 2017; 27: 1872–1938.CrossRefGoogle Scholar
Jacobs, JP, Franklin, RCG, Béland, MJ, et al. Nomenclature for pediatric and congenital cardiac care: unification of clinical and administrative nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). World J Pediatr Congenit Heart Surg 2021; 12: E1–E18. DOI 10.1177/21501351211032919.CrossRefGoogle Scholar
Jacobs, JP, Franklin, RCG, Béland, MJ, et al. Nomenclature for pediatric and congenital cardiac care: unification of clinical and administrative nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). Cardiol Young 2021; 31: 1057–1188. DOI 10.1017/S104795112100281X.10.1017/S104795112100281XCrossRefGoogle Scholar
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 primary septum: anatomic and echocardiographic findings and surgical treatment: a study based on 36 cases. Chest 1995; 107: 1488–1498.CrossRefGoogle ScholarPubMed
Cohen, MS, Weinberg, P, Coon, PD, Gaynor, JW, Rychik, J. Deviation of atrial primary septum in association with normal left atrioventricular valve size. J Am Soc Echocardiogr 2001; 14: 732–737.10.1067/mje.2001.111875CrossRefGoogle Scholar
Cuttone, F, Hadeed, K, Lacour-Gayet, F, et al. Isolated severe leftward displacement of the primary septum: anatomic and 3D echocardiographic findings and surgical repair. Interact Cardiovasc Thorac Surg 2017; 24: 772–777.10.1093/icvts/ivx006CrossRefGoogle Scholar
Ayylldlz, P, Aztürk, E, Kasar, T, Türkvatan, A, Onan, IS, Güzeltaş, A. A rare cause of abnormal pulmonary venous drainage: primary septum malposition. Cardiol Young 2020; 30: 1716–1721.Google Scholar
Walsh, MJ, Ungerleider, RM, Aiello, VD, Spicer, D, Giroud, JM. Anomalous pulmonary venous connections and related anomalies: nomenclature, embryology, anatomy, and morphology. World J Pediatr Congenit Heart Surg 2013; 4: 30–43.CrossRefGoogle ScholarPubMed
Bharucha, T, Spicer, DE, Mohun, TJ, Black, D, Henry, GW, Anderson, RH. Cor triatriatum or divided atriums: which approach provides the better understanding? Cardiol Young 2015; 25: 193–207.10.1017/S1047951114000572CrossRefGoogle ScholarPubMed
Brown, DW, Geva, T. Totally anomalous pulmonary venous connection. In: Lang, F (eds). Encyclopedia of Molecular Mechanisms of Disease. Springer, Berlin/Heidelberg, 2009.Google Scholar