Type III persistent fifth aortic arch in association with transposition of great arteries and pulmonary atresia—An unreported case

Lee, Meng-Hsun; Chiu, Chow-Chi; Dai, Zen‐Kong; Lin, Wei-Che; Wu, Ding-Kwo; Wu, Jiunn‐Ren

doi:10.1016/j.ijcard.2006.07.067

Cited by 9 publications

(9 citation statements)

References 6 publications

(6 reference statements)

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“…Persistent 5th aortic arch has been classified into 3 variants: 1) double-lumen aortic arch with both lumina patent, 2) interruption of the superior arch with persistence of the 5th arch (inferior part), and 3) systemic-to-pulmonary arterial connection. 6 The first formation appears to be the most common. The clinical presentation is usually silent in patients with the first variant; however, patients with the 2nd variant frequently have coarctation of the distal end of the 5th embryonic arch.…”

Section: Discussionmentioning

confidence: 99%

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Coarctation of Persistent 5th Aortic Arch: First Report of Catheter-Based Intervention

Uysal¹,

Bostan²,

Çil³

2014

Texas Heart Institute Journal

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P ersistent 5th aortic arch, an apparently rare congenital anomaly, is defined as a single aortic arch with separate superior and inferior parts.1 It was initially called a double-lumen aortic arch.2 The 5th aortic arch forms between the true aortic arch (a derivative of the 4th aortic arch) and the pulmonary artery (a derivative of the 6th aortic arch).3 Without additional coarctation of the existing aorta, this anomaly alone has no physiologic significance and does not necessitate surgical intervention.We report our catheter-based treatment of an infant who had aortic coarctation of a persistent 5th aortic arch with no associated cardiac defects. Case ReportIn July 2012, a 7-month-old infant girl was referred to our department for evaluation of a heart murmur. The healthy-looking patient had a normal prenatal and postnatal history. Her blood pressure was normal, and her systemic oxygen saturation was 98% on room air. Auscultation revealed normal heart sounds and a grade 2/6 midsystolic murmur that was audible at the 3rd left sternal border and at the back. The patient's diminished femoral pulses raised the suspicion of aortic coarctation.Electrocardiographic results were normal. Echocardiograms showed left ventricular hypertrophy and an unusual double-lumen aortic arch. There was coarctation of the inferior part of the arch (Fig. 1), and mild obstruction was evident upon color-flow Doppler analysis. Continuous-wave Doppler images revealed a 50-mmHg peak instantaneous gradient at the coarctation site, with typical diastolic runoff. Conventional angiograms showed that the common origin of all brachiocephalic vessels from the ascending aorta ended after the left subclavian artery. A distinct vessel in the inferior part of the interrupted arch originated from the ascending aorta and connected with the descending aorta (Fig. 2). We concluded that this lower arch was a persistent 5th aortic arch. The definitive diagnosis was interrupted 4th arch with persistent 5th arch. The coarctation was at the distal end of the 5th arch.During catheterization, after consulting a cardiac surgeon, we performed balloon angioplasty to correct the coarctation. Afterwards, the peak-to-peak gradient decreased from 50 to 20 mmHg (Fig. 3). At age 1 year, the patient was normotensive. Echocardiograms revealed mild repeat coarctation at the distal end of the persistent 5th arch and a 30-mmHg peak instantaneous gradient across the coarctation site. As of June 2014, we continue to monitor the patient for hypertension and any increase in the gradient. Surgical intervention might be necessary in the future.

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Section: Discussionmentioning

confidence: 99%

“…7 The presentation of the persistent 5th arch depends on the anatomic connections and the associated cardiac defects. 8 The persistent arch is frequently associated with tetralogy of Fallot, 9 complete D-transposition of the great arteries, 6 truncus arteriosus, 10 and ventricular septal defect.…”

Section: Discussionmentioning

confidence: 99%

Coarctation of Persistent 5th Aortic Arch: First Report of Catheter-Based Intervention

Uysal¹,

Bostan²,

Çil³

2014

Texas Heart Institute Journal

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“…The persistent fifth aortic arch (PFAA) was first described in 1969 2 . It has since been classified into three groups: (I) double‐lumen aortic arch with both lumens patent; (II) atresia/interruption of the superior arch with persistence of the inferior (fifth) arch; and (III) systemic‐to‐pulmonary arterial connection 3 . This anomaly has been previously diagnosed using angiography, MRI and CT. Silverman et al suggested that this diagnosis can accurately be made by echocardiography 1 .…”

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confidence: 99%

“…2 It has since been classified into three groups: (I) double-lumen aortic arch with both lumens patent; (II) atresia/interruption of the superior arch with persistence of the inferior (fifth) arch; and (III) systemic-to-pulmonary arterial connection. 3 This anomaly has been previously diagnosed using angiography, MRI and CT. Silverman et al suggested that this diagnosis can accurately be made by echocardiography. 1 We used echocardiography to diagnose PFAA in a neonate born at 36 6/7 weeks gestational age with a diagnosis of plate-like pulmonary atresia with intact ventricular septum.…”

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confidence: 99%

Persistent Fifth Aortic Arch: Echocardiographic Diagnosis of a Persistent Fifth Aortic Arch

2010

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“…This malformation, per se, is usually not hemodynamically significant, thereby its real incidence might be underestimated. However, PFAA is frequently associated with other anomalies, such as tetralogy of Fallot, transposition of the great arteries, truncus arteriosus, aortic arch coarctation or interruption [2][3][4][5]. Ductal-dependent tissue present at the coarctation site plays an important role in the development of a discrete coarctation and prostaglandin infusion could be effective in relieving…”

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confidence: 99%

Aortic coarctation with persistent fifth left aortic arch

Santoro¹,

Caianiello

Palladino³

et al. 2009

International Journal of Cardiology

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Type III persistent fifth aortic arch in association with transposition of great arteries and pulmonary atresia—An unreported case

Cited by 9 publications

References 6 publications

Coarctation of Persistent 5th Aortic Arch: First Report of Catheter-Based Intervention

Coarctation of Persistent 5th Aortic Arch: First Report of Catheter-Based Intervention

Persistent Fifth Aortic Arch: Echocardiographic Diagnosis of a Persistent Fifth Aortic Arch

Aortic coarctation with persistent fifth left aortic arch

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