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A case report of prenatal diagnostics and surgical correction of pulmonary agenesis associated with total anomalous pulmonary venous connection and vascular ring Cover

A case report of prenatal diagnostics and surgical correction of pulmonary agenesis associated with total anomalous pulmonary venous connection and vascular ring

Romanian Journal of Cardiology
Volume 33 (2023): Issue 3 (September 2023)
By: Maksym Delikatnyi,  Raad Tammo,  Hanna Morkovkina,  Oleksandr Teterin,  Tetyana Yalynska and  Roman Sekelyk  
Open Access
|Sep 2023

Figures & Tables

Figure 1

Fetal echocardiography at 32 weeks gestation shows a complete vascular ring made by a right aortic arch, left ductus arteriosus with the aberrant left subclavian artery (A, B). Retroaortic, retroesophageal innominate vein drained into superior vena cava (C, D). Fetal MRI at 32 weeks gestation showed left pulmonary agenesis, the right pulmonary veins drained into the retroaortic, retroesophageal innominate vein (E, F), complete vascular ring made by a right aortic arch with retroesophagal left subclavian artery, which encircles the esophagus and trachea (G, H). LIV – left innominate vein; DAo – descending aorta; RAA – right aortic arch; ALSA – aberrant left subclavian artery; DA – ductus arteriosus; SVC – superior vena cava; RV – right ventricle; LV – left ventricle; RPV – right pulmonary veins; Tr – trachea; Es – esophagus; RAA – right aortic arch; Rbr – right bronchus.
Fetal echocardiography at 32 weeks gestation shows a complete vascular ring made by a right aortic arch, left ductus arteriosus with the aberrant left subclavian artery (A, B). Retroaortic, retroesophageal innominate vein drained into superior vena cava (C, D). Fetal MRI at 32 weeks gestation showed left pulmonary agenesis, the right pulmonary veins drained into the retroaortic, retroesophageal innominate vein (E, F), complete vascular ring made by a right aortic arch with retroesophagal left subclavian artery, which encircles the esophagus and trachea (G, H). LIV – left innominate vein; DAo – descending aorta; RAA – right aortic arch; ALSA – aberrant left subclavian artery; DA – ductus arteriosus; SVC – superior vena cava; RV – right ventricle; LV – left ventricle; RPV – right pulmonary veins; Tr – trachea; Es – esophagus; RAA – right aortic arch; Rbr – right bronchus.

Figure 2

Postnatal CT shows the left pulmonary agenesis with left rudimentary bronchus (A – minimum intensity projection (MinIP)), trachea shifted to the left side and compressed by the vascular ring, hypoplastic right pulmonary artery (B – maximum intensity projection (MIP)), diminutive right pulmonary veins drained into the retroaortic, retroesophageal innominate vein (C, D, F – 3D volume rendering technique (VRT) and E – MIP). Tr – trachea; Rbr – main right bronchus; rLbr – rudimentary left bronchus; PA – pulmonary artery; RAA – right aortic arch; DA – ductus arteriosus; ALSA – aberrant left subclavian artery; LIV – left innominate vein; SVC – superior vena cava; RPV – right pulmonary veins; DAo – descending aorta; Es – esophagus; RPA – right pulmonary artery; R – right; L – left; A – anterior; P – posterior.
Postnatal CT shows the left pulmonary agenesis with left rudimentary bronchus (A – minimum intensity projection (MinIP)), trachea shifted to the left side and compressed by the vascular ring, hypoplastic right pulmonary artery (B – maximum intensity projection (MIP)), diminutive right pulmonary veins drained into the retroaortic, retroesophageal innominate vein (C, D, F – 3D volume rendering technique (VRT) and E – MIP). Tr – trachea; Rbr – main right bronchus; rLbr – rudimentary left bronchus; PA – pulmonary artery; RAA – right aortic arch; DA – ductus arteriosus; ALSA – aberrant left subclavian artery; LIV – left innominate vein; SVC – superior vena cava; RPV – right pulmonary veins; DAo – descending aorta; Es – esophagus; RPA – right pulmonary artery; R – right; L – left; A – anterior; P – posterior.

Figure 3

The diagram shows the anatomy of the heart malformation confirmed intraoperatively. Tr – trachea; Es – esophagus; PA – pulmonary artery; DA – ductus arteriosus; ALSA – aberrant left subclavian artery; LIV – left innominate vein; SVC – superior vena cava; RPV – right pulmonary veins; RPA – right pulmonary artery; RA – right atrium; RV – right ventricle; LA – left atrium; LV – left ventricle; RSA – right subclavian artery; RCA – right common carotid artery; LCA – left common carotid artery; DAo – descending aorta; AAo – ascending aorta. The figure was drawn by B. Hulitskyi.
The diagram shows the anatomy of the heart malformation confirmed intraoperatively. Tr – trachea; Es – esophagus; PA – pulmonary artery; DA – ductus arteriosus; ALSA – aberrant left subclavian artery; LIV – left innominate vein; SVC – superior vena cava; RPV – right pulmonary veins; RPA – right pulmonary artery; RA – right atrium; RV – right ventricle; LA – left atrium; LV – left ventricle; RSA – right subclavian artery; RCA – right common carotid artery; LCA – left common carotid artery; DAo – descending aorta; AAo – ascending aorta. The figure was drawn by B. Hulitskyi.
DOI: https://doi.org/10.2478/rjc-2023-0016 | Journal eISSN: 2734-6382 | Journal ISSN: 1220-658X
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Language: English
Page range: 107 - 111
Published on: Sep 21, 2023
Published by: Romanian Society of Cardiology
In partnership with: Paradigm Publishing Services
Publication frequency: 4 issues per year
Keywords:
Pulmonary agenesis,
total anomalous pulmonary venous connection,
vascular ring,
congenital cardiac malformation
Related subjects:
Medicine,
Clinical medicine,
Internal medicine,
Cardiology,
Surgery,
Cardiac surgery,
Pediatrics and juvenile medicine,
Paediatric cardiology

© 2023 Maksym Delikatnyi, Raad Tammo, Hanna Morkovkina, Oleksandr Teterin, Tetyana Yalynska, Roman Sekelyk, published by Romanian Society of Cardiology
This work is licensed under the Creative Commons Attribution 4.0 License.

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