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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

Maksym Delikatnyi
Maksym Delikatnyi
, 
Raad Tammo
Raad Tammo
, 
Hanna Morkovkina
Hanna Morkovkina
, 
Oleksandr Teterin
Oleksandr Teterin
, 
Tetyana Yalynska
Tetyana Yalynska
 und 
Roman Sekelyk
Roman Sekelyk
   | 21. Sept. 2023
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Article Category: Case Presentation
Online veröffentlicht: 21. Sept. 2023
Seitenbereich: 107 - 111
DOI: https://doi.org/10.2478/rjc-2023-0016
Schlüsselwörter
© 2023 Maksym Delikatnyi et al., published by Sciendo
This work is licensed under the Creative Commons Attribution 4.0 International License.

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.
eISSN:
2734-6382
Sprache:
Englisch
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Fachgebiete der Zeitschrift:
Medizin, Klinische Medizin, Allgemeinmedizin, Innere Medizin, Kardiologie, Chirurgie, Herzchirurgie, Kinder- und Jugendmedizin, Kinderkardiologie
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