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Rare case of total anomalous pulmonary venous return into the right atrium in situs solitus

Published online by Cambridge University Press:  27 February 2024

Elio Caruso Open the ORCID record for Elio Caruso [Opens in a new window] ,
Silvia Farruggio Open the ORCID record for Silvia Farruggio [Opens in a new window]  and
Norman H. Silverman
Elio Caruso
Affiliation:
Mediterranean Pediatric Cardiology Center “Bambino Gesù”, San Vincenzo Hospital, Taormina, Italy
Silvia Farruggio*
Affiliation:
Mediterranean Pediatric Cardiology Center “Bambino Gesù”, San Vincenzo Hospital, Taormina, Italy
Norman H. Silverman
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA
*
Corresponding author: S. Farruggio; Email: silviafarruggio88@gmail.com
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Abstract

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein’s ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

Keywords

Total anomalous pulmonary venous returnCHDimagingechocardiographyCT scan
Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 34 , Issue 4 , April 2024 , pp. 919 - 921
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Copyright
© The Author(s), 2024. Published by Cambridge University Press

Case report

A full-term newborn was transferred to our hospital for the cardiological evaluation because of hypoxia resistant to ventilatory therapy with the addition of nitric oxide.

At birth, the body weight was 2.770 kg, the APGAR score was 9’–10’, and peripheral oxygen saturation was 85%. Electrocardiogram was normal. Chest X-ray showed bronchial situs solitus (Fig. 1). Echocardiogram revealed situs solitus, dilated right atrium and right atrial appendage, huge ostium secundum atrial septal defect of 8 mm and right-to-left atrial shunt, and three pulmonary veins draining into the right atrium near to the opening of the superior caval vein without obstruction. The superior caval vein was not dilated. (Figs. 2 and 3).

Figure 1. Posteroanterior chest radiography shows normal lungs, bronchial situs solitus, and normal heart size.

Figure 2. A. Subcostal view shows situs solitus, right-sided liver, left-sided stomach, left-sided aorta (red arrow), and right-sided inferior caval vein (white arrow). B. Four chamber view shows dilated right atrial appendage and right atrium, atrial septal defect (arrow), normal-size left atrium, mildly dilated and hypertrophied right ventricle, normal-size left ventricle, no shunts through the ventricular septum, normal left ventricle, normal left and right outflow tracts, and no pericardial effusion. No pulmonary veins are seen draining to either atrium. AO = aorta; ICV = inferior caval vein; LA = left atrium; LV = left ventricle; RA = right atrium; RAA = right atrial appendage; RV = right ventricle.

Figure 3. a . Subcostal view shows expanded right atrium and huge atrial septal defect (arrow) of 8 mm with right-to-left shunt; three pulmonary veins (the right superior and inferior veins, and the left superior one). b . Courtesy of Elio Caruso. Manual drawing of subcostal view of total anomalous pulmonary venous returns into the right superior caval vein’s ostium, huge atrial septal defect (arrow) with right-to-left shunt. CS = coronary sinus; ICV = inferior caval vein; LA = left atrium; LIPV = left inferior pulmonary vein; LSPV = left superior pulmonary vein; MV = mitral valve; RA = right atrium; RIPV = right inferior pulmonary vein; RSCV = right superior caval vein; RSPV = right superior pulmonary vein; SCV = superior caval vein; TV = tricuspid valve.

CT scan angiography (Fig. 4c) and maximum intensity projection (Fig. 4d) showed abdominal and bronchial situs solitus and confirmed the total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein ostium without vertical vein, better appreciable from the volume rendering by Horos® software (Fig. 4a,b). All four pulmonary veins appeared patent.

Figure 4. a , b . CT angiography, volume rendering by Horos® software, antero-posterior view (A) and posterior-anterior view (B), show four pulmonary veins draining at the base of the right superior caval vein’s ostium into the right atrium. c . Chest CT scan shows all four pulmonary veins draining C. CT angiography axial plane shows all four pulmonary veins draining into the right superior caval vein’s ostium in the right atrium. d . CT angiography multiplanar reconstruction with perfusion colour look up table by Horos® software, shows four pulmonary veins draining into the right atrium at the base of the right superior caval vein ostium. AO = aorta; DA = descending aorta; LA = left atrium; LIPV = left inferior pulmonary vein; LSPV = left superior pulmonary vein; LV = left ventricle; PT = pulmonary trunk; RA = right atrium; RAA = right atrial appendage; RIPV = right inferior pulmonary vein; RPA = right pulmonary artery; RIPV = right inferior pulmonary vein; RSCV= right superior caval vein; RSPV = right superior pulmonary vein; RV = right ventricle.

We believe this is an uncommon case report of total anomalous pulmonary venous return to the right atrium at the base of the superior caval vein’s ostium without a sinus venosus defect, in situs solitus, and without vertical vein or a posterior pulmonary venous confluence. Reference Gaynor, Burch, Dollery, Sullivan, Deanfield and Elliott1

The two-dimensional echocardiography with colour Doppler allowed to accurately describe the anatomy of the pulmonary venous drainage; however, advanced imaging scan may be needful for defining the anatomy and confirming the diagnosis. Reference Rocamora Salort, Ruiz González, Cano Sánchez and Fernández Tudela2

Acknowledgements

None.

Author contribution

Elio Caruso wrote article and critical revision.

Silvia Farruggio wrote article and data interpretation.

Norman H Silverman developed concept of publication, critical revision, and approval of article.

Financial support

Additional supporting information may be found in the online version of the article at the publisher’s website.

Competing interests

All authors have read and approved submission of the manuscript and have no conflict of interest to disclose.

References

Gaynor, JW, Burch, M, Dollery, C, Sullivan, ID, Deanfield, JE, Elliott, MJ. Repair of anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995; 59: 1471–1475. DOI: 10.1016/0003-4975(95)00150-j.CrossRefGoogle Scholar
Rocamora Salort, S, Ruiz González, E, Cano Sánchez, A, Fernández Tudela, B. Total anomalous pulmonary venous return with mixed drainage and double connection: a rare case report not previously described. Eur Heart J Case Rep 2022; 6: ytac415. DOI: 10.1093/ehjcr/ytac415.CrossRefGoogle Scholar
Figure 0

Figure 1. Posteroanterior chest radiography shows normal lungs, bronchial situs solitus, and normal heart size.

Figure 1

Figure 2. A. Subcostal view shows situs solitus, right-sided liver, left-sided stomach, left-sided aorta (red arrow), and right-sided inferior caval vein (white arrow). B. Four chamber view shows dilated right atrial appendage and right atrium, atrial septal defect (arrow), normal-size left atrium, mildly dilated and hypertrophied right ventricle, normal-size left ventricle, no shunts through the ventricular septum, normal left ventricle, normal left and right outflow tracts, and no pericardial effusion. No pulmonary veins are seen draining to either atrium. AO = aorta; ICV = inferior caval vein; LA = left atrium; LV = left ventricle; RA = right atrium; RAA = right atrial appendage; RV = right ventricle.

Figure 2

Figure 3. a. Subcostal view shows expanded right atrium and huge atrial septal defect (arrow) of 8 mm with right-to-left shunt; three pulmonary veins (the right superior and inferior veins, and the left superior one). b. Courtesy of Elio Caruso. Manual drawing of subcostal view of total anomalous pulmonary venous returns into the right superior caval vein’s ostium, huge atrial septal defect (arrow) with right-to-left shunt. CS = coronary sinus; ICV = inferior caval vein; LA = left atrium; LIPV = left inferior pulmonary vein; LSPV = left superior pulmonary vein; MV = mitral valve; RA = right atrium; RIPV = right inferior pulmonary vein; RSCV = right superior caval vein; RSPV = right superior pulmonary vein; SCV = superior caval vein; TV = tricuspid valve.

Figure 3

Figure 4. a, b. CT angiography, volume rendering by Horos® software, antero-posterior view (A) and posterior-anterior view (B), show four pulmonary veins draining at the base of the right superior caval vein’s ostium into the right atrium. c. Chest CT scan shows all four pulmonary veins draining C. CT angiography axial plane shows all four pulmonary veins draining into the right superior caval vein’s ostium in the right atrium. d. CT angiography multiplanar reconstruction with perfusion colour look up table by Horos® software, shows four pulmonary veins draining into the right atrium at the base of the right superior caval vein ostium. AO = aorta; DA = descending aorta; LA = left atrium; LIPV = left inferior pulmonary vein; LSPV = left superior pulmonary vein; LV = left ventricle; PT = pulmonary trunk; RA = right atrium; RAA = right atrial appendage; RIPV = right inferior pulmonary vein; RPA = right pulmonary artery; RIPV = right inferior pulmonary vein; RSCV= right superior caval vein; RSPV = right superior pulmonary vein; RV = right ventricle.