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.

Background Current guidelines on coronary anomalies are primarily based on expert consensus and a limited number of trials. A gold standard for diagnosis and a consensus on the treatment strategy in this patient group are lacking, especially for patients with an anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) with an interarterial course. Aim To provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries. Methods A clinical care pathway for patients with ACAOS was established by six Dutch centres. Prospectively included patients undergo work-up according to protocol using computed tomography (CT) angiography, ischaemia detection, echocardiography and coronary angiography with intracoronary measurements to assess anatomical and physiological characteristics of the ACAOS. Surgical and functional follow-up results are evaluated by CT angiography, ischaemia detection and a quality-of-life questionnaire. Patient inclusion for the first multicentre study on coronary anomalies in the Netherlands started in 2020 and will continue for at least 3 years with a minimum of 2 years of follow-up. For patients with a right or left coronary artery originating from the pulmonary artery and coronary arteriovenous fistulas a registry is maintained. Results Primary outcomes are: (cardiac) death, myocardial ischaemia attributable to the ACAOS, re-intervention after surgery and intervention after initially conservative treatment. The influence of work-up examinations on treatment choice is also evaluated. Conclusions Structural evidence for the appropriate management of patients with coronary anomalies, especially (interarterial) ACAOS, is lacking. By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group.

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Coarctation of the aorta (CoA) is a congenital heart defect that is associated with a bicuspid aortic valve (BAV), ascending aorta dilatation, intracerebral aneurysms, and premature atherosclerotic disease. The first presentation during late adulthood is rare and is frequently driven by late sequelae. Hypertrophic collateral arteries can develop aneurysms which are at risk for spontaneous rupture, however, treatment recommendations for these aneurysms are scarce. Here, we describe the clinical course and percutaneous treatment strategy of a patient with a late diagnosis of a pin-point CoA, a BAV with moderate regurgitation, and an exceptionally large aneurysm of a collateral artery. A 59-year-old woman was diagnosed with Streptococcus bovis endocarditis of a BAV with moderate aortic valve regurgitation and small vegetation (<5 mm) on the non-coronary cusp. Work-up revealed hypertension and adenocarcinoma in situ of the ascending colon, considered the bacteremia porte d’entrée, for which a curative hemicolectomy was performed. Echocardiography showed a narrowing of the aorta distal from the origin of the left subclavian artery with the antegrade diastolic flow with a pathognomonic “sawtooth” pattern and an estimated pressure gradient of >70 mmHg. Computed tomography angiography (CTA) showed a network of well-developed collateral arteries and a levoatriocardinal vein. One of the collateral arteries arising from the left subclavian artery revealed an exceptionally large aneurysmatic dilation (29 × 24 × 24 mm). The invasive assessment confirmed a hemodynamically significant CoA. Treatment involved balloon dilatation and placement of a covered stent at the site of the pin-point CoA and a percutaneous coronary intervention (PCI) of the stenosis in the left anterior descending artery. No residual gradient over the CoA was observed. Antihypertensive drugs could be discontinued, and CTA performed 4 months later showed regression and thrombosis of the numerous collaterals and, importantly, thrombosis of the large aneurysm. This case illustrates the late diagnosis of CoA with associated congenital heart defects and late sequelae including hypertension, BAV endocarditis, coronary artery disease, and aneurysm formation of the extensive collateral network. The patient underwent pharmacological and percutaneous treatment, ultimately resulting in the alleviation of the CoA, normalization of the blood pressure, reduction of collateral flow, and thrombosis of the large aneurysm of the collateral artery.

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