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Indomethacin induced and prostaglandin relieved coarctation of the aorta in right aortic arch with left arterial duct: a case report

Published online by Cambridge University Press:  06 March 2017

Stevi Golden-Plotnik,
Herschel C. Rosenberg and
Luis A. Altamirano-Diaz
Stevi Golden-Plotnik
Affiliation:
Department of Paediatrics, Western University, London, Ontario, Canada
Herschel C. Rosenberg
Affiliation:
Department of Paediatrics, Western University, London, Ontario, Canada
Luis A. Altamirano-Diaz*
Affiliation:
Department of Paediatrics, Western University, London, Ontario, Canada
*
Correspondence to: Dr L. A. Altamirano-Diaz, Department of Paediatrics, Western University, 800 Commissioner’s Road East Room B5-122D London, Ontario, Canada, N6A 5W9. Tel: +519 685 8500, Ext 56061; Fax: +519 685 8334; E-mail: laltamir@uwo.ca
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Abstract

We describe the case of an infant with DiGeorge syndrome born with a right aortic arch and left arterial duct. Despite the remote location of the right aortic arch from the left arterial duct, he developed coarctation of the aorta during treatment with indomethacin. This was relieved by prostaglandin treatment. This case highlights the fact that, even in the absence of an arterial duct, ductal tissue can still be present in the aorta, and cause coarctation when exposed to indomethacin. We also demonstrate the utility of prostaglandin for relief of this type of obstruction.

Keywords

Right aortic archleft arterial ductcoarctation of the aorta

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 27 , Issue 5 , July 2017, pp. 1026 - 1029
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Copyright
© Cambridge University Press 2017 

We describe an interesting case that provides clinical evidence for the presence of ductal tissue in the aortic arch, distant from the arterial duct. This was identified in an infant with DiGeorge syndrome, who was born with a right aortic arch and left arterial duct. He was treated with indomethacin, and subsequently developed a coarctation, evident both clinically and on echocardiography. The obstruction was relieved by prostaglandin.

Edwards’ hypothetical modelReference Edwards 1 describes embryological arch development. Initially, there are two aortic arches and two ductus arteriosi (Fig 1a). Typically, the right arch and the right arterial duct involute and regress (Fig 1b). In a right aortic arch, the left arch involutes (Fig 1c). A right aortic arch with aberrant left subclavian artery forms when the left arch involutes at the region between the left arterial duct and the left carotid artery. The left subclavian artery and the left arterial duct then arise from the diverticulum of Kommerell (Fig 1d).

Figure 1

(a) Edward’s hypothetical model, AAo: ascending aorta, RCA:right carotid artery, RAD: right arterial duct, RPA: right pulmonary artery, RSA: right subclavian artery, RAA: right aortic arch, DAo: descending aorta, LAA: left aortic arch, LSA: left subclavian artery, LAD: left arterial duct, LCA: left carotid artery, LPA: left pulmonary artery. (b) Left aortic arch, BCT: brachiocephalic trunk, (c) Right aortic arch, (d) Right aortic arch with aberrant left subclavian artery and left arterial duct, ALSA: aberrant left subclavian artery. Cartoons by Stevi Golden-Potnik.

A right aortic arch is rarely seen in the general population (0.1%);Reference McElhinney, Tworetzky, Hanley and Rudolph 2 , Reference Ismat, Weinberg, Rychik, Karl and Fogel 3 however, it is common in patients with DiGeorge syndrome (30%) and is frequently associated with arch abnormalities.Reference McElhinney, Clark and Weinberg 4

Coarctation of the aorta has been shown to be caused by the presence of ductal tissue within the aorta.Reference Kim, Kim and Kim 5 After birth, and onset of ductal closure, this tissue constricts, causing aortic obstruction. In this setting, prostaglandins are used to re-open the arterial duct and the involved aorta. In addition, prostaglandins can relieve coarctation without opening the arterial duct.Reference Liberman, Gersony and Flynn 6

Case report

The patient was born at 34 weeks and 3 days of gestation in a community hospital following preterm premature rupture of membranes. The pregnancy was otherwise uncomplicated with no abnormalities on prenatal ultrasound. At 10 minutes of life, the patient developed respiratory distress and hypotension, refractory to fluid boluses and vasopressors. A left-sided obstructive lesion was suspected, and intravenous prostaglandin was commenced. He was transferred to our centre by 24 hours of age.

An echocardiogram while on prostaglandin showed mild left ventricular systolic dysfunction, a right aortic arch with aberrant left subclavian artery, and a large left-sided arterial duct. The aortic valve was normal, and there was no coarctation of the aorta or right arterial duct. Therefore, prostaglandin infusion was stopped.

The large left arterial duct remained open, with significant left-to-right shunt. At 7 days of life, a 3-day course of indomethacin was started to close the arterial duct. Following the second dose of indomethacin, the patient developed weak femoral pulses. Blood pressure in the left arm and left leg were 73/56 and 60/43 mmHg, respectively. After the third dose, he developed respiratory distress, absent femoral pulses, and un-recordable lower-limb blood pressure. A stat echocardiogram revealed a closing left arterial duct and a coarctation of the right aortic arch (Fig 2c and d). There was also reduced left ventricular function. Prostaglandins were re-started. His respiratory distress improved, and there was improvement in his blood pressures: 59/45 mmHg in the left arm and 62/44 mmHg in the left leg. At this time, an echocardiogram showed relief of the aortic obstruction and mild improvement in ventricular function.

Figure 2

Figure 2: (a) Cartoon shows right aortic arch with aberrant left subclavian artery and left artery duct, AAo: ascending aorta, LCA: left carotid artery, RCA: right carotid artery, RSA: right subclavian artery, AA: aortic arch, DAo: descending aorta, ALSA: aberrant left subclavian artery, LDA: left arterial duct. (b) Echocardiogram: Colour compare showing right aortic arch and descending aorta with and without colour. (c) Cartoon shows coarctation in the right aortic arch and constriction of the left arterial duct, CoA: coarctation. (d) Echocardiogram colour compare showing the coarctation of the aorta on the right aortic arch. Cartoons by Stevi Golden-Potnik.

He was continued on prostaglandin infusion while being transferred to a tertiary paediatric cardiac centre for surgery, where he underwent patch augmentation of the aorta and ligation of the left arterial duct. His postoperative course was uneventful. A FISH assay showed 22q11 deletion, confirming the diagnosis of DiGeorge syndrome.

Follow-up assessment 2 months after the procedure showed no evidence of re-coarctation. His blood pressure was 94/42 mmHg in the right arm and 87/54 mmHg in the left leg. His echocardiogram showed that the aortic arch was wide open with a normal velocity in the descending aorta of 1.8 m/s.

Discussion

To our knowledge, this is the first reported case of a newborn with DiGeorge syndrome and a right aortic arch and left arterial duct, who developed a coarctation that was induced by indomethacin and relieved with prostaglandin. The clinical findings are consistent with coarctation secondary to constriction of ectopic ductal tissue present in the aorta. This constriction was induced by indomethacin. Prostaglandin was able to relieve the obstruction in the right aortic arch, irrespective of the opening effect in the left arterial duct.

Conclusions

This case demonstrates that the clinical presence of ductal tissue in a right aortic arch with no ipsilateral arterial duct can become constricted in response to indomethacin, causing coarctation. Even if an arterial duct is not visualised, prostaglandin can relieve aortic obstruction. In the acute setting, this allows for stabilisation before surgical correction or intervention is performed.

Acknowledgements

The authors would like to thank the Pediatric Cardiopulmonary laboratory, Children’s Hospital, London Health Science Center, London, Ontario, Canada for its support.

Financial Support

This research received no specific grant funding from any funding agency or from commercial or not-for-profit sectors.

Conflicts of Interest

None.

References

1. Edwards, JE. Anomalies of the derivatives of the aortic arch system. Med Clin North Am 1948; 32: 925–948.CrossRefGoogle ScholarPubMed
2. McElhinney, DB, Tworetzky, W, Hanley, FL, Rudolph, AM. Congenital obstructive lesions of the right aortic arch. Ann Thorac Surg 1999; 67: 1194–1202.CrossRefGoogle ScholarPubMed
3. Ismat, FA, Weinberg, PM, Rychik, J, Karl, TR, Fogel, MA. Right aortic arch and coarctation: a rare association. Congenit Heart Dis 2006; 1: 217–223.Google Scholar
4. McElhinney, DB, Clark, J, Weinberg, PM, et al. Association of 22q11 deletion syndrome with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol 2001; 37: 2114–2119.Google Scholar
5. Kim, JE, Kim, E-K, Kim, W-H, et al. Abnormally extended ductal tissue into the aorta is indicated by similar histopathology and shared apoptosis in patients with coarctation. Int J Cardiol 2010; 145: 177–182.Google Scholar
6. Liberman, L, Gersony, WM, Flynn, PA, et al. Effectiveness of prostaglandin E1 in relieving obstruction in coarctation of the aorta without opening the ductus arteriosus. Pediatr Cardiol 2004; 25: 49–52.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1(a) Edward’s hypothetical model, AAo: ascending aorta, RCA:right carotid artery, RAD: right arterial duct, RPA: right pulmonary artery, RSA: right subclavian artery, RAA: right aortic arch, DAo: descending aorta, LAA: left aortic arch, LSA: left subclavian artery, LAD: left arterial duct, LCA: left carotid artery, LPA: left pulmonary artery. (b) Left aortic arch, BCT: brachiocephalic trunk, (c) Right aortic arch, (d) Right aortic arch with aberrant left subclavian artery and left arterial duct, ALSA: aberrant left subclavian artery. Cartoons by Stevi Golden-Potnik.

Figure 1

Figure 2Figure 2: (a) Cartoon shows right aortic arch with aberrant left subclavian artery and left artery duct, AAo: ascending aorta, LCA: left carotid artery, RCA: right carotid artery, RSA: right subclavian artery, AA: aortic arch, DAo: descending aorta, ALSA: aberrant left subclavian artery, LDA: left arterial duct. (b) Echocardiogram: Colour compare showing right aortic arch and descending aorta with and without colour. (c) Cartoon shows coarctation in the right aortic arch and constriction of the left arterial duct, CoA: coarctation. (d) Echocardiogram colour compare showing the coarctation of the aorta on the right aortic arch. Cartoons by Stevi Golden-Potnik.