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Intermittent obstruction of the left coronary artery ostia by a congenital supravalvular aortic membrane in an adult.

ABSTRACT: Left main coronary artery obstruction due to a congenital supra-aortic membrane is a rare anomaly, but it can have serious consequences, including significant myocardial ischemia that may result in impaired ventricular function or even sudden death. Therefore, early diagnosis and treatment of this condition are crucial for improving patient outcomes. We present, in this report, a patient with intermittent obstruction of the left coronary arteries caused by a congenital ostial membrane diagnosed in adulthood that resulted in myocardial ischemia.

SUBMITTER: El Ghali T

PROVIDER: S-EPMC10856603 | biostudies-literature | 2023 Jul-Aug

REPOSITORIES: biostudies-literature

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Intermittent obstruction of the left coronary artery ostia by a congenital supravalvular aortic membrane in an adult.

El Ghali Tanae T Fellat Nadia N Doghmi Nawal N Cherti Mohamed M

Annals of pediatric cardiology 20230701 4

Left main coronary artery obstruction due to a congenital supra-aortic membrane is a rare anomaly, but it can have serious consequences, including significant myocardial ischemia that may result in impaired ventricular function or even sudden death. Therefore, early diagnosis and treatment of this condition are crucial for improving patient outcomes. We present, in this report, a patient with intermittent obstruction of the left coronary arteries caused by a congenital ostial membrane diagnosed ...[more]

PMID: 38343497

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Project description:ObjectivesThe aim of this study was to identify the prevalence and anatomic characteristics of coronary artery lesions and their associated postoperative risk in patients undergoing supravalvular aortic stenosis repair.MethodsThe association between structural risk factors, postoperative ST-segment changes, and major adverse cardiac events was explored using logistic regression and the Fisher's exact test.ResultsIn 51 consecutive patients with supravalvular aortic stenosis treated between 2000 and 2017, a total of 48 coronary lesions were identified in 27 patients (53%). Prominent ostial ridge (type I) was the most common coronary lesion, followed by small ostium with (IIIb) or without (IIIa) diffuse long-segment coronary narrowing, and adhesion of the coronary cusp (type II). There were 54 concomitant coronary procedures, including 43 primary corrections and 11 revisions. Thirty-three patients underwent supravalvular aortic stenosis repair with a bifurcated patch, of which 13 (39.4%) had right coronary artery distortion/kinking requiring patch plication (n = 8) and reimplantation (n = 5). Postoperative major adverse cardiac events (MACE) occurred in 9 patients (17.6%), including 3 deaths, 4 needing mechanical circulatory support, and 6 experiencing ventricular arrhythmias. Twenty-two patients (43.1%) had postoperative ST-segment changes, including 13 early changes that resolved within 24 h and 9 persistent changes lasting >24 h. Patients with type III lesions were associated with postoperative persistent ST-segment change (P = 0.04) and these lesions independently predicted postoperative MACE (P = 0.02). Patients with pre-existing coronary lesions were at elevated risk of right coronary artery distortion/kinking (P = 0.045).ConclusionsThe prevalence of ST-segment changes and MACE is high in patients undergoing supravalvular aortic stenosis repair. The preoperative presence of complex coronary lesions is the most important predictor for postoperative major adverse cardiac events.

Project description:Obstructive and flow limiting coronary artery dissections can be a catastrophic clinical scenario, requiring urgent treatment and tailored approach for each case. A 55-year-old female patient, with a history of breast cancer, chest radiation treatments and hypertension presented with episodes of angina and significant area of reversible ischemia on single-photon emission computerized tomography (SPECT). Coronary angiogram revealed separate ostia of the left coronary arteries and three-vessel disease (SYNTAX (Synergy between percutaneous coronary intervention (PCI) with Taxus and Cardiac Surgery) = 15); subsequent full revascularization was achieved successfully with two drug-eluting stents (DES) (mid left anterior descending artery (LAD), left circumflex coronary artery (LCx)) and one drug-coated balloon (posterior descending artery (PDA)). However, after a few hours the patient underwent an urgent second angiography due to ongoing chest pain and electrocardiogram (ECG) changes. Proximal complete occlusion of the anomalous LAD was displayed and a long dissection attributable to an intimal tear following first stent implantation was recorded (well expanded and apposed stent (proximal stent edges were implanted in an unhealthy vessel area infiltrated with fibrotic and calcified plaque) not detectable by conventional angiography). A second 3.5 × 38 mm DES was implanted optimally in the proximal LAD segment and overlapped with the first one, with immediate restoration of the flow and relief of the patient's symptoms. The patient was discharged symptom free and with recommendation for optimal medical treatment for secondary coronary artery disease (CAD) prevention. Conventional coronary angiography in patients with history of chest radiation treatment might not detect accurately the extent and characteristics of the underlying CAD. Appropriate use of intravascular imaging in these cases secures a safe approach for ambiguous lesions and facilitates treatment of iatrogenic coronary dissections following PCI.

Dataset Information

Intermittent obstruction of the left coronary artery ostia by a congenital supravalvular aortic membrane in an adult.

Publications

Intermittent obstruction of the left coronary artery ostia by a congenital supravalvular aortic membrane in an adult.

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