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Severe coronary artery ectesia in a paediatric patient with Noonan syndrome presenting for transcatheter pulmonary valve placement

Published online by Cambridge University Press:  13 January 2025

Christopher Herron Open the ORCID record for Christopher Herron [Opens in a new window] ,
Karen Iacono  and
David Nykanen
Christopher Herron*
Affiliation:
Department of Pediatric Cardiology, Arnold Palmer Hospital for Children, Orlando, FL, USA
Karen Iacono
Affiliation:
Department of Pediatric Cardiology, Arnold Palmer Hospital for Children, Orlando, FL, USA
David Nykanen
Affiliation:
Department of Pediatric Cardiology, Arnold Palmer Hospital for Children, Orlando, FL, USA
*
Corresponding author: Christopher Herron; Email: christopher.herron@orlandohealth.com
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Abstract

Coronary ectasia is a very rare phenomenon seen in Noonan syndrome with only a few documented case reports. We describe a 14-year-old with Noonan syndrome and tetralogy of Fallot with described coronary artery ectasia since infancy who presented for possible transcatheter pulmonary valve placement and was found to have severe ectasia of bilateral coronary arteries.

Keywords

Noonan syndromecoronary dilationpulmonary stenosis
Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 35 , Issue 1 , January 2025 , pp. 192 - 194
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Coronary ectasia is a rare finding in Noonan syndrome with only a few documented case reports. Reference Aniol, Prokop and Rajasekaran1–Reference Loukas, Dabrowski, Kantoch, Ruzyłło, Waltenberger and Giannikopoulos5 A 14-year-old male with Noonan syndrome presented to our office for continued follow up of his CHD consisting of pulmonary valve stenosis that was surgically augmented at 2 months of age using a transannular patch in a foreign country, hypertrophic cardiomyopathy, and severely dilated bilateral coronary arteries. His surgical report from infancy noted prominent bilateral coronaries at the time of surgery. He had significant right ventricular dilation due to his free pulmonary insufficiency and, based on CT scan, was a possible self-expanding transcatheter pulmonary valve candidate, so he presented for cardiac catheterisation (Fig 1). Selective coronary angiography depicted a severely dilated left main coronary artery with ectasia of the left anterior descending and left circumflex coronary arteries (Fig 2). The right coronary artery was ectatic throughout its entire length (Fig 3). No discrete aneurysms were seen. The right and left pulmonary arteries were found to be severely dilated along with the main pulmonary artery (Fig 4). A 40 mm sizing balloon was placed across the right ventricular outflow tract with continued flow around the balloon so the patient was deemed too high risk for possible transcatheter pulmonary valve implantation with currently available devices. The patient was taken to surgery for a pulmonary valve replacement.

Figure 1. (a) Axial plane of CT angiogram depicting a severely dilated left main coronary artery (*), left anterior descending artery (^), and right coronary artery (<). (b) Axial plane of CT angiogram depicting a severely dilated main pulmonary artery as well as a right and left pulmonary artery.

Figure 2. (a) Right anterior oblique (RAO)/Cranial angulation and (b) lateral angulation with selective left main coronary angiography. There is a severely dilated left main coronary artery (measuring 7 mm at the ostium and 20 mm just prior to the bifurcation)(*) with significant ectasia of the left anterior descending artery (^)(measuring 9.9 mm) and left circumflex artery (<)(measuring 7 mm).

Figure 3. (a) Anterior posterior angulation and (b) lateral angulation with selective right coronary angiography. There is a severely dilated right coronary artery (*)(6.4 mm proximal) with significant ectasia of a conal branch (^).

Figure 4. (a) RAO/cranial angulation and (b) lateral angulation with angiography in the main and branch pulmonary arteries. There is severe dilation of the right, left, and main pulmonary arteries.

Financial support

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

Competing interests

None.

Compliance with ethical standard

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in this case report.

References

Aniol, CV, Prokop, JW, Rajasekaran, S et al. Dilated coronary arteries in a 2-month-old with RIT1-associated noonan syndrome: a case report. BMC Pediatr 2023; 23: 1.CrossRefGoogle Scholar
Gulati, GS, Gupta, A, Juneja, R, Saxena, A. Ectatic coronary arteries in Noonan syndrome. Tex Heart Inst J 2011; 38: 318–319.Google ScholarPubMed
Uçar, T, Atalay, S, Tekin, M, Tutar, E. Bilateral coronary artery dilatation and supravalvular pulmonary stenosis in a child with noonan syndrome. Pediatr Cardiol 2005; 26: 848–850.CrossRefGoogle Scholar
Calcagni, G, Baban, A, De Luca, E, Leonardi, B, Pongiglione, G, Digilio, MC. Coronary artery ectasia in noonan syndrome: report of an individual with SOS1 mutation and literature review. Am J Med Genet A 2016; 170: 665–669.CrossRefGoogle ScholarPubMed
Loukas, M, Dabrowski, M, Kantoch, M, Ruzyłło, W, Waltenberger, J, Giannikopoulos, P. A case report of noonan’s syndrome with pulmonary valvar stenosis and coronary aneurysms. Med Sci Monit 2004; 10: CS80–CS83.Google ScholarPubMed
Figure 0

Figure 1. (a) Axial plane of CT angiogram depicting a severely dilated left main coronary artery (*), left anterior descending artery (^), and right coronary artery (<). (b) Axial plane of CT angiogram depicting a severely dilated main pulmonary artery as well as a right and left pulmonary artery.

Figure 1

Figure 2. (a) Right anterior oblique (RAO)/Cranial angulation and (b) lateral angulation with selective left main coronary angiography. There is a severely dilated left main coronary artery (measuring 7 mm at the ostium and 20 mm just prior to the bifurcation)(*) with significant ectasia of the left anterior descending artery (^)(measuring 9.9 mm) and left circumflex artery (<)(measuring 7 mm).

Figure 2

Figure 3. (a) Anterior posterior angulation and (b) lateral angulation with selective right coronary angiography. There is a severely dilated right coronary artery (*)(6.4 mm proximal) with significant ectasia of a conal branch (^).

Figure 3

Figure 4. (a) RAO/cranial angulation and (b) lateral angulation with angiography in the main and branch pulmonary arteries. There is severe dilation of the right, left, and main pulmonary arteries.