A Cyanotic Newborn with a Pink Right Upper Extremity
Aberrant origin of the subclavian artery (SCA) is a well-known vascular anomaly as part of congenital heart diseases with the left subclavian artery (LSCA) being more frequently affected than the right subclavian artery (RSCA). Complete isolation of the SCA is an even more infrequent aortic arch ano...
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| Format: | Article |
| Language: | English |
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Wiley
2020-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2020/8873156 |
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| author | Matthias Beichl Andreas Hanslik Daniel Zimpfer Judith Rittenschober-Boehm Katrin Nagl Ina Michel-Behnke |
| author_facet | Matthias Beichl Andreas Hanslik Daniel Zimpfer Judith Rittenschober-Boehm Katrin Nagl Ina Michel-Behnke |
| author_sort | Matthias Beichl |
| collection | DOAJ |
| description | Aberrant origin of the subclavian artery (SCA) is a well-known vascular anomaly as part of congenital heart diseases with the left subclavian artery (LSCA) being more frequently affected than the right subclavian artery (RSCA). Complete isolation of the SCA is an even more infrequent aortic arch anomaly, occurring in less than 1% for the LSCA and even less for the RSCA. Isolation of the RSCA in patients with d-transposition of the great arteries (D-TGA) is even scanter with only a hand full of cases being reported in the literature. However, isolation of the RSCA has important implications on hemodynamics and surgical strategies. In this case report, we present a newborn patient with D-TGA which presented with distinct differential cyanosis. While the right upper extremity appeared pink with an oxygen saturation of 100%, the rest of the body was cyanotic. At first, this appearance was interpreted as the Harlequin phenomenon during primary care. However, detailed echocardiography revealed an aberrant origin of the RSCA from the right pulmonary artery, which led to the differential cyanosis. The patient underwent arterial switch operation on day of life two including dissection and reimplantation of the RSCA. The special hemodynamic situation of this is discussed in terms of pathophysiology and as well as its impact on perioperative and surgical management. |
| format | Article |
| id | doaj-art-e0a39faece5a49a3a37e0cffccacc440 |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2020-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-e0a39faece5a49a3a37e0cffccacc4402025-02-03T01:01:27ZengWileyCase Reports in Pediatrics2090-68032090-68112020-01-01202010.1155/2020/88731568873156A Cyanotic Newborn with a Pink Right Upper ExtremityMatthias Beichl0Andreas Hanslik1Daniel Zimpfer2Judith Rittenschober-Boehm3Katrin Nagl4Ina Michel-Behnke5Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, AustriaDivision of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, AustriaDivision of Cardiac Surgery, Department of Surgery, Medical University of Vienna, Vienna, AustriaDivision of Neonatology, Pediatric Intensive Care and Neuropediatrics, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, AustriaDivision of Pediatric Pulmonology, Allergology and Endocrinology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, AustriaDivision of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, AustriaAberrant origin of the subclavian artery (SCA) is a well-known vascular anomaly as part of congenital heart diseases with the left subclavian artery (LSCA) being more frequently affected than the right subclavian artery (RSCA). Complete isolation of the SCA is an even more infrequent aortic arch anomaly, occurring in less than 1% for the LSCA and even less for the RSCA. Isolation of the RSCA in patients with d-transposition of the great arteries (D-TGA) is even scanter with only a hand full of cases being reported in the literature. However, isolation of the RSCA has important implications on hemodynamics and surgical strategies. In this case report, we present a newborn patient with D-TGA which presented with distinct differential cyanosis. While the right upper extremity appeared pink with an oxygen saturation of 100%, the rest of the body was cyanotic. At first, this appearance was interpreted as the Harlequin phenomenon during primary care. However, detailed echocardiography revealed an aberrant origin of the RSCA from the right pulmonary artery, which led to the differential cyanosis. The patient underwent arterial switch operation on day of life two including dissection and reimplantation of the RSCA. The special hemodynamic situation of this is discussed in terms of pathophysiology and as well as its impact on perioperative and surgical management.http://dx.doi.org/10.1155/2020/8873156 |
| spellingShingle | Matthias Beichl Andreas Hanslik Daniel Zimpfer Judith Rittenschober-Boehm Katrin Nagl Ina Michel-Behnke A Cyanotic Newborn with a Pink Right Upper Extremity Case Reports in Pediatrics |
| title | A Cyanotic Newborn with a Pink Right Upper Extremity |
| title_full | A Cyanotic Newborn with a Pink Right Upper Extremity |
| title_fullStr | A Cyanotic Newborn with a Pink Right Upper Extremity |
| title_full_unstemmed | A Cyanotic Newborn with a Pink Right Upper Extremity |
| title_short | A Cyanotic Newborn with a Pink Right Upper Extremity |
| title_sort | cyanotic newborn with a pink right upper extremity |
| url | http://dx.doi.org/10.1155/2020/8873156 |
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