Persistent 5th aortic arch — a great pretender: three new covert cases

Gerlis, Leon M.; Ho, Siew Yen; Anderson, Robert H.; Costa, Phillip Da

doi:10.1016/0167-5273(89)90253-2

Cited by 80 publications

(80 citation statements)

References 8 publications

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“…Gerlis and his colleagues 3) stated that the persistent fifth aortic arch is by no means rare but only underestimated and misdiagnosed, and we agree with their conclusion. Awareness of the protean manifestations of the anomaly and some understanding of the embryological principles involved may facilitate more frequent and accurate diagnosis in the future.…”

Section: -7)supporting

confidence: 69%

“…Although Gerlis and his colleagues reported an incidence of about 1 in 330 in autopsy studies, 3) still only rare cases have been presented since their publication.…”

Section: Discussionmentioning

confidence: 99%

“…The only possible structure that could communicate from the distal ascending aorta to the main pulmonary artery would be a persistent fifth aortic arch with systemic-to-pulmonary connection. 3) Although anatomically almost identical, the two conditions are embryologically quite distinct.…”

Section: -7)mentioning

confidence: 99%

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Persistent Fifth Aortic Arch. An Ignored and Underestimated Disease.

Chiu

Chen

et al. 2000

Jpn Heart J

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SUMMARYWe report two unique cases of persistent fifth aortic arch with a systemic-to-pulmonary connection. All previously reported cases with such a connection in the literature have either been cases of pulmonary atresia or an aortic arch anomaly, and the existence of a fifth aortic arch was a benefit to the underlying great vessel anomaly. However, our two cases did not have this associated great vessel anomaly, and the fifth arch resulted in a large left-to-right shunt with severe pulmonary hypertension and heart failure. The first case was misdiagnosed preoperatively; an accurate diagnosis was made after cardiac surgery. Because of its rerity and complexity, a persistent fifth aortic arch is often ignored and misdiagnosed. (Jpn Heart J 2000; 41: 665-671) Key words: Persistent fifth aortic arch, Systemic-to-pulmonary connection, Complex great vessel anomaly PERSISTENT fifth aortic arch is an extremely rare congenital cardiovascular malformation. It is an abnormal arterial branch originating from the distal ascending aorta proximal and opposite to the ostium of the innominate artery. 1)Most cases are associated with severe cardiac malformation and diagnosed fortuitously.2) By 1989, only 21 cases had been reported in the literature.3) Among them, 15 cases had a systemic-to-systemic connection, and six cases had a systemic-to-pulmonary connection. All six cases were associated with a variety of complex great vessel anomalies such as pulmonary atresia, aortic atresia or interruption of the aorta. The fifth arches in the reported cases were actually of some hemodynamic benefit to the patients. Recently, however, we encountered two unique cases of persistent fifth aortic arch with systemic-to-pulmonary connection without pulmonary atresia or an aortic arch anomaly. The fifth aortic arch caused a catastrophic event in both of these patients.

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Section: -7)supporting

confidence: 69%

“…Although Gerlis and his colleagues reported an incidence of about 1 in 330 in autopsy studies, 3) still only rare cases have been presented since their publication.…”

Section: Discussionmentioning

confidence: 99%

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Persistent Fifth Aortic Arch. An Ignored and Underestimated Disease.

Chiu

Chen

et al. 2000

Jpn Heart J

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“…By now it is accepted that a pair of 5th PAAs exists transitorily in human embryos [Gerlis et al, 1989;Hiruma and Hirakow, 1995;Hiruma et al, 2002]. Occasionally, remnants of one of the 5th arteries persist until adulthood and can contribute to a malformation named a double-lumen aortic arch [Van Praagh and Van Praagh, 1969;Geva et al, 1990;Yang et al, 2003;Kirsch and Julsrud, 2007;Kellenberger, 2010].…”

Section: Introductionmentioning

confidence: 99%

Some Mice Feature 5th Pharyngeal Arch Arteries and Double-Lumen Aortic Arch Malformations

Geyer

Weninger²

2012

Cells Tissues Organs

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A 5th pair of pharyngeal arch arteries (PAAs) has never been identified with certainty in mice. Murines in general are considered to not develop a 5th pair. If true, the significance of the mouse as a model for researching the genesis of malformations of the great intrathoracic arteries is limited. We aimed to investigate whether mouse embryos develop a 5th pair of PAAs and to identify malformations known to be caused by defective remodelling of the 5th PAAs. We employed the high-resolution episcopic microscopy method for creating digital volume data and three-dimensional (3D) computer models of the great intrathoracic arteries of 30 mouse embryos from days 12–12.5 post conception and 180 mouse fetuses from days 14.5 and 15.5 post conception. The 3D models of the fetuses were screened for the presence of a double-lumen aortic arch malformation. We identified such a malformation in 1 fetus. The 3D models of the embryos were analysed for the presence of 5th PAAs. Six of the 30 embryos (20%) showed a 5th PAA bilaterally, and an additional 9 (30%) showed a 5th PAA unilaterally. Our results prove that some mice do develop a 5th pair of PAAs. They also show that malformations which occur rarely in humans and result from defective remodelling of the left 5th PAA can be identified in mice as well. Thus, the mouse does represent an excellent model for researching the mechanisms driving PAA remodelling and the genesis of malformations of the great intrathoracic arteries.

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“…Indeed, persistent 5th aortic arch might be underdiagnosed because of diff iculties in echocardiographic evaluation, especially when both lumina are patent. 12,16 Regardless, echocardiography accurately reveals any associated defects. To confirm the diagnosis, conventional angiography 1 or magnetic resonance angiography 17 should also be performed.…”

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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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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Persistent 5th aortic arch — a great pretender: three new covert cases

Cited by 80 publications

References 8 publications

Persistent Fifth Aortic Arch. An Ignored and Underestimated Disease.

Persistent Fifth Aortic Arch. An Ignored and Underestimated Disease.

Some Mice Feature 5th Pharyngeal Arch Arteries and Double-Lumen Aortic Arch Malformations

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

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