Subclavian and pulmonary artery steal phenomenon in a patient with isolated left subclavian artery and right aortic arch

Hayabuchi, Yasunobu; Inoue, Miki; Sakata, Miho; Ohnishi, Tatsuya; Kubo, Shoji

doi:10.1002/jcu.21874

Cited by 7 publications

(9 citation statements)

References 8 publications

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“…The surgical decision to reconnect the RSCA to the aortic arch, as done in our case, may be preventive for subclavian steal syndrome on the long run, as described in one case with D-TGA and isolated RSCA [ 2 ] or more frequently in other CHD with isolated LSCA [ 6 ]. Equally leaving the RSCA would account for the pulmonary steal phenomenon as soon as the pulmonary pressure and resistance normalize after arterial switch operation.…”

Section: Discussionmentioning

confidence: 99%

A Cyanotic Newborn with a Pink Right Upper Extremity

Beichl

Zimpfer

Rittenschober-Böhm

et al. 2020

Case Reports in Pediatrics

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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.

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Section: Discussionmentioning

confidence: 99%

A Cyanotic Newborn with a Pink Right Upper Extremity

Beichl

Zimpfer

Rittenschober-Böhm

et al. 2020

Case Reports in Pediatrics

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“…The presence of tetralogy of Fallot with a right-sided aorta should prompt the diagnostician to analyze the direction and the nature of flow in the left subclavian artery. Medical history and clinical examination indicative of reduced pulse in the left radial artery, headache, and impaired vision (due to the steal syndrome) help make a correct diagnosis (10,11) . It should be emphasized that the topographic proximity between LCCA and LSA (Fig.…”

Section: Discussionmentioning

confidence: 99%

Isolation of the left subclavian artery in an infant with tetralogy of Fallot, right aortic arch and DiGeorge syndrome. Echocardiographic diagnostic case study

2019

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We present a case of a 6-month-old infant with an isolated left subclavian artery coexistent with right-sided aortic arch, tetralogy of Fallot and DiGeorge syndrome, with an emphasis on echocardiographic detection of this extremely rare anomaly. Specific difficulties related to echocardiographic visualization of abnormally coursing artery were a result of significantly limited ultrasonographic access due to the absence of thymus and a very close proximity of the left subclavian artery and left common carotid artery, mimicking a normal brachiocephalic trunk, which is usually present in patients with right-sided aortic arch. Precise analysis of the course of carotid and vertebral arteries as well as the nature and direction of flow in these vessels (particularly in the left vertebral and subclavian artery) suggested ductal rather than aortic origin of the left subclavian artery. Precise delineation of anatomical relationships between major arteries prior to surgical closure of the arterial duct was necessary to prevent potential postoperative ischemia of the left upper extremity; therefore the diagnosis was completed with CT angiography.

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“…Isolation of the left subclavian artery usually presents with no apparent symptoms in neonates, but it may present with congenital pulmonary steal syndrome, subclavian steal syndrome, or may even present in adults with late symptoms due to sporadic progression. Hayabuchi et al [ 6 ] reported the case of a three-month-old girl with cerebral atrophy and an underdeveloped left arm. Jesudian et al [ 7 ] reported the case of a 15-year-old boy with an underdeveloped left arm.…”

Section: Discussionmentioning

confidence: 99%

“…Some authors have suggested that adequate collateral circulation must be ensured, meaning that reconstruction of the isolated subclavian artery is optional, regardless of the symptoms and signs. Successful results have been reported after ligation or device closure of the PDA and ligation of the left subclavian artery [ 1 , 6 ]. However, reconstruction of the left subclavian artery due to pulmonary steal syndrome after right PDA closure in bilateral PDA has been reported.…”

Section: Discussionmentioning

confidence: 99%

Isolation of the Left Subclavian Artery with Right Aortic Arch in Association with Bilateral Ductus Arteriosus and Ventricular Septal Defect

Lee

Park

et al. 2015

Korean J Thorac Cardiovasc Surg

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Right aortic arch with isolation of the left subclavian artery is a rare anomaly. The incidence of bilateral ductus arteriosus is sporadic, and a right aortic arch with isolation of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. Since the symptoms and signs of isolation of the left subclavian artery can include the absence or underdevelopment of the left arm, subclavian steal syndrome, or pulmonary artery steal syndrome, the proper therapeutic approach is controversial. We report a case in which surgical reconstruction was used to treat isolation of the left subclavian artery with right aortic arch in association with bilateral ductus arteriosus and a ventricular septal defect.

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Subclavian and pulmonary artery steal phenomenon in a patient with isolated left subclavian artery and right aortic arch

Cited by 7 publications

References 8 publications

A Cyanotic Newborn with a Pink Right Upper Extremity

A Cyanotic Newborn with a Pink Right Upper Extremity

Isolation of the left subclavian artery in an infant with tetralogy of Fallot, right aortic arch and DiGeorge syndrome. Echocardiographic diagnostic case study

Isolation of the Left Subclavian Artery with Right Aortic Arch in Association with Bilateral Ductus Arteriosus and Ventricular Septal Defect

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