Anomalous Aortic Origin of the Right Coronary Artery: Invasive Haemodynamic Assessment in Adult Patients With High-Risk Anatomic Features.

Background: Anomalous aortic origin of a right coronary artery (AAORCA) with an interarterial course merits further evaluation; however, robust risk assessment strategies for myocardial ischemia and sudden cardiac death are currently lacking. The aim of this study is to explore the potential role of fractional flow reserve (FFR), instantaneous wave-free ratio (iFR), and intravascular ultrasound (IVUS) in patients with AAORCA. Methods: Consecutive adult patients with AAORCA with an interarterial course were included. Computed tomography angiography, noninvasive ischemia detection, and FFR, iFR, and IVUS were performed at baseline and during adrenaline-induced stress. External compression was evaluated with IVUS. Results: Eight patients (63% female, mean age: 53 ± 9.5 years) were included. Five patients (63%) were symptomatic, and computed tomography angiography revealed high-risk anatomy of the AAORCA in all patients. Only in 1 (12.5%) patient FFR and iFR were positive; however, this was attributed at large to concomitant diffuse atherosclerosis. In 2 of 8 (25%), IVUS revealed external compression; however, the ostial coronary surface area remained unchanged. In all patients, a conservative treatment strategy was pursued. During a mean follow-up of 29.3 months (standard deviation ±2.6 months), symptoms spontaneously disappeared in 4 of 5 (80%) and no adverse cardiac events occurred in any of the patients. Conclusions: Despite the presence of high-risk anatomy in all patients, none had proven ischemia prompting a conservative treatment strategy. No adverse cardiac events occurred during follow-up, and in the majority of patients, symptoms spontaneously disappeared. Therefore, FFR, iFR, and IVUS with pharmacologic stress merit further investigation and might contribute to ischemia-based risk stratification and management strategies in adult patients with AAORCA.

Contexte: L'anomalie de naissance de l'artère coronaire droite à partir de l'aorte (AAORCA, anomalous aortic origin of a right coronary artery) combinée à un trajet interartériel mérite un examen plus approfondi. Cependant, on observe à l'heure actuelle des lacunes en ce qui a trait à l'emploi de stratégies fiables d'évaluation du risque d'ischémie myocardique et de mort subite d'origine cardiaque. L'objectif de cette étude est d'examiner le rôle potentiel de la mesure de la réserve coronarienne (MRC), de l'évaluation du rapport instantané sans onde (iFR, instantaneous wave-free ratio) et de l'échographie intravasculaire chez des patients présentant une AAORCA. Méthodologie: Des cas de patients adultes consécutifs présentant une AAORCA combinée à un trajet interartériel ont été inclus à l'étude. Une angiographie par tomodensitométrie (TDM), une détection non invasive de la présence d'une ischémie, la MRC, l'évaluation de l'iFR et l'échographie intravasculaire ont été effectuées au début de l'étude ainsi que lors d'un stress induit par l'adrénaline. La compression externe a également été évaluée au moyen d'une échographie intravasculaire. Résultats: Huit patients (63 % de sexe féminin; âge moyen de 53 ans ± 9,5 ans) ont participé à l'étude. Cinq patients (63 %) présentaient des symptômes, et l'angiographie par TDM a révélé une AAORCA à risque élevé chez tous les patients. Les résultats de la MRC et de l'évaluation de l'iFR étaient positifs chez seulement un patient (12,5 %), ce qui est attribuable en majeure partie à une athérosclérose diffuse concomitante. Chez deux patients (25 %), l'échographie intravasculaire a montré une compression externe de l'artère coronaire droite même si l'aire de l'ostium de l'artère n'avait pas changé. Une stratégie thérapeutique prudente a été employée pour tous les patients. Pendant la période de suivi qui a duré en moyenne 29,3 mois (écart-type : ± 2,6 mois), les symptômes se sont résorbés de manière spontanée chez quatre des cinq patients (80 %), et aucun événement cardiaque indésirable n'est survenu. Conclusion: Malgré une anatomie à risque élevé chez tous les patients, aucun d'entre eux ne présentait une ischémie connue, ce qui justifiait une stratégie thérapeutique prudente. Aucun événement cardiaque indésirable n'est survenu durant la période de suivi, et les symptômes se sont résorbés de manière spontanée chez la majorité des patients. À la lumière de ces renseignements, la MRC, l'évaluation de l'iFR et l'échographie intravasculaire lors d'un stress pharmacologique devraient faire l'objet d'autres études et pourraient éventuellement être utiles dans la stratification du risque d'ischémie et dans le choix des stratégies de prise en charge des patients adultes présentant une AAORCA.

A comprehensive analysis of the intramural segment in interarterial anomalous coronary arteries using computed tomography angiography.

Aims: An anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using computed tomography angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, proximal luminal narrowing, and an intramural segment. To date, no robust CTA criteria exist to determine the presence of an intramural segment. We aimed to deduct new CTA parameters to distinguish an intramural course of interarterial ACAOS. Methods and results: Twenty-five patients with an interarterial ACAOS (64% female, mean age 46 years, 88% right ACAOS) from two academic hospitals were evaluated. Inclusion criteria were the availability of a preoperative CTA scan (0.51 mm slice thickness) and peroperative confirmation of the intramural segment. Using multiplanar reconstruction of the CTA, the distance between the lumen of the aorta and the lumen of the ACAOS [defined as 'interluminal space' (ILS)] was assessed at 2 mm intervals along the intramural segment. Analysis showed a mean ILS of 0.69 ± 0.15 mm at 2 mm from the ostium. At the end of the intramural segment where the ACAOS becomes non-intramural, the mean ILS was significantly larger (1.27 ± 0.29 mm, P < 0.001). Interobserver agreement evaluation showed good reproducibility (intraclass correlation coefficient 0.77, P < 0.001). Receiver operator characteristic analysis demonstrated that at a cut-off ILS of <0.95 mm, an intramural segment can be diagnosed with 100% sensitivity and 84% specificity. Conclusion: The ILS is introduced as a novel and robust CTA parameter to identify an intramural course of interarterial ACAOS. An ILS of <0.95 mm is indicative of an intramural segment.

Computed Tomography Derived Coronary Triangulated Orifice Area-Deduction of a New Parameter for Follow-up After Surgical Correction of Anomalous Aortic Origin of Coronary Arteries and Call for Validation.

Introduction: Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus of Valsalva is a rare congenital abnormality. Computed tomography angiography (CTA) is primarily used as a diagnostic tool to evaluate the anatomy and identify potentially malignant AAOCA variants. Limited data is available on the role of CTA during postoperative follow-up. We aimed to develop an objective CTA derived parameter for diagnostic evaluation and follow-up after surgical correction of AAOCA and correlate the anatomical features to the postoperative outcome. Methods: All consecutive patients who underwent surgical repair of AAOCA from 2001 to 2018 and had pre and postoperative CTA imaging available were included. A retrospective analysis of the pre- and postoperative CTA and the outcomes was performed. The origin and course of the anomalous coronary artery and the ostial dimensions were evaluated and correlated with restenosis of operated coronary artery. To allow an accurate evaluation of the effective orifice area at diagnosis and after surgical repair we deduce and propose a new parameter-the coronary triangulated orifice area (CTOA). Results: Out of the 54 patients who underwent surgical treatment for AAOCA, 11 fulfilled the inclusion criteria. The median follow-up was 19 months [IQR 3;42]. The mean age at surgery was 41 ± 16 years, with six patients (55%) being male. Postoperatively, the angle between the proximal coronary artery and the aortic wall increased from 20 ± 5° to 28 ± 9° (p < 0.01) and ostial diameter in the transversal plane increased from 4.1 ± 2.5 mm to 6.2 ± 2.7 mm (p < 0.01). The median CTOA increased significantly from 1.6 mm2 [IQR 0.9;4.9] to 5.5 mm2 [IQR 3;11.8] (p < 0.005). During follow-up, in three patients a restenosis of the operated coronary artery was suspected. In these patients, the CTOA only showed a limited postoperative increase of ≤ 1.4 mm2. Conclusions: CTA can play an important role in the evaluation of the pre- and postoperative anatomy in AAOCA patients. CTOA may be of use in conjunction with the acute angle take-off and ostial diameter order to comprehensively evaluate the operated ostium after unroofing or patch angioplasty.