An unusual case of two acute coronary syndrome episodes caused by allergic and non-allergic coronary artery dissection with potential coronary vasospasm association: a case report.

Background: Type I variant Kounis syndrome is characterized by coronary spasm following an allergic or anaphylactic reaction. Coronary spasm is also recognized as a contributing factor in spontaneous coronary artery dissection (SCAD). Case summary: A 46-year-old woman presented to the emergency room with a chief complaint of chest discomfort following the ingestion of a steamed bun. A marked decrease in systolic blood pressure and a prominent rash on her forearms and groin suggested anaphylactic shock. Upon stabilization of vital signs, acute coronary syndrome (ACS) was suspected based on electrocardiogram findings and symptoms, prompting an emergency coronary angiography (CAG). The CAG revealed severe stenosis with coronary artery dissection in the right coronary artery (RCA), and a stent implantation was performed. Given the suspicion of type I variant Kounis syndrome, a spasm provocation test was performed, yielding a positive result. Six years later, she experienced chest discomfort while sleeping and was admitted to our emergency department. An electrocardiogram showed ST-segment elevation in leads II, III, and aVF. An emergency CAG identified a severely stenotic lesion with coronary artery dissection in the RCA, leading to a diagnosis of SCAD. Direct stenting was performed at the stenotic site. The patient was discharged following intensification of medication. Discussion: This report describes a rare case of a middle-aged woman with two episodes of ACS caused by both allergic and non-allergic coronary artery dissection. These episodes suggest that a shared underlying coronary vasospasm in both conditions may be a common trigger for coronary artery dissection.

Multimodality Imaging of Fabry Disease Cardiomyopathy Complicated With Coronary Vasospasm.

A 41-year-old man with resting angina was diagnosed with a coronary vasospasm and subsequently with Fabry disease exhibiting low serum α-galactosidase A activity. High computed tomography (CT)-derived extracellular volume was detected in the apical inferior wall of the left ventricle suggesting myocardial fibrosis, potentially from vasospasm-related ischemia and/or microvascular dysfunction.

Severe ST-segment elevation and AV block during pulsed-field ablation due to vasospastic angina - a novel observation.

Catheter ablation of atrial fibrillation using non-thermal electroporation represents a promising ablation modality due to its believed superior safety profile. Still, if electroporation is delivered in proximity to a coronary artery, vasospasms can occur. We report the first case of severe right coronary artery vasospasm resulting in ST-segment elevation and AV block despite a remote distance from the ablation site to the right coronary artery, indicating a different mechanism. In this case, electroporation most likely triggered a previously unknown Prinzmetal vasospastic angina in the patient, resulting in the coronary vasospasm. Thus, meticulous monitoring of ST-segment changes following PFA delivery even from regions remote to coronary arteries is required.

Predicting the response to acetylcholine in ischemia or infarction with non-obstructive coronary arteries: The ABCD score.

BACKGROUND AND AIMS: Acetylcholine (ACh) provocation testing can detect vasomotor disorders in patients with ischemia and non-obstructed coronary arteries (INOCA) or myocardial infarction and non-obstructed coronary arteries (MINOCA). We aimed to derive and validate a simple risk score to predict a positive ACh test response. METHODS: We prospectively enrolled consecutive INOCA and MINOCA patients undergoing ACh provocation testing. Patients were split in two cohorts (derivation and validation) according to time of enrolment. The score was derived in 386 patients (derivation cohort) and then validated in 165 patients (validation cohort). RESULTS: 551 patients were enrolled, 371 (67.3%) INOCA and 180 (32.7%) MINOCA. ACh test was positive in 288 (52.3%) patients. MINOCA, myocardial bridge (MB), C-reactive protein (CRP) and dyslipidaemia were independent predictors of a positive ACh test in the derivation cohort. The ABCD (Acute presentation, Bridge, CRP, Dyslipidaemia) score was derived: 2 points were assigned to MINOCA, 3 to MB, 1 to elevated CRP and 1 to dyslipidaemia. The ABCD score accurately identified patients with a positive ACh test response with an AUC of 0.703 (CI 95% 0.652-0.754,p < 0.001) in the derivation cohort, and 0.705 (CI 95% 0.626-0.784, p < 0.001) in the validation cohort. In the whole population, an ABCD score ≥4 portended 94.3% risk of a positive ACh test and all patients with an ABCD score ≥6 presented a positive test. CONCLUSIONS: The ABCD score could avoid the need of ACh provocation testing in patients with a high score, reducing procedural risks, time, and costs, and allowing the implementation of a tailored treatment strategy. These results are hypothesis generating and further research involving larger cohorts and multicentre trials is needed to validate and refine the ABCD score.

[Angina and no obstruction on coronary angiography : New approaches to the diagnosis and treatment of vasomotor disorders]. / Angina pectoris ohne Stenosen in der Koronarangiographie : Neue Wege zur Diagnose und Therapie von Vasomotionsstörungen.

BACKGROUND: Clinical management of patients with angina and no obstructive coronary artery disease (ANOCA) is still challenging. This scenario affects up to 50% of patients undergoing diagnostic coronary angiography due to suspected coronary artery disease. Many patients report a long and debilitating history before adequate diagnostics and management are initiated. OBJECTIVES: This article describes the current recommendations for diagnostic assessments and treatment in patients with ANOCA. Focus is placed on invasive diagnostics in the catheter laboratory, pharmacological/interventional treatment as well as the patient journey. RESULTS: In patients with ANOCA, the current European Society of Cardiology (ESC) guidelines suggest that invasive assessments using acetylcholine and adenosine for the diagnosis of an underlying coronary vasomotor disorder should be considered. Acetylcholine is used to diagnose coronary spasm, whereas adenosine is used in conjunction with a wire-based assessment for the measurement of coronary flow reserve and microvascular resistance. The invasive assessments allow the determination of what are referred to as endotypes (coronary spasm, impaired coronary flow reserve, enhanced microvascular resistance or a combination thereof). Establishing a diagnosis is helpful to: (a) initiate targeted treatment to improve quality of life, (b) reassure the patient that a cardiac cause is found and (c) to assess individual prognosis. CONCLUSIONS: Currently, patients with ANOCA are often not adequately managed. Referral to specialised centres is recommended to prevent long and debilitating patient histories until expertise in diagnosis and treatment becomes more widespread.

Capecitabine-induced-coronary-vasospasm leading to polymorphic ventricular tachycardia and cardiac arrest.

Capecitabine, a pro-drug of 5-fluorouracil, is commonly used in the treatment of breast and colorectal cancer. Its side effects, including nausea, vomiting, diarrhea, fatigue, loss of appetite, and bone marrow suppression, are well recognized. However, coronary vasospasm represents a less commonly recognized but significant complication of fluoropyrimidine-based therapies such as capecitabine. Proposed mechanisms for this adverse effect complication include direct endothelium-independent vasoconstriction, activation of protein kinase C, and activation of the cyclooxygenase pathway. In this report, we present a case of capecitabine-induced coronary vasospasm leading to progressive, focal ST-elevations, myocardial ischemia, and subsequently polymorphic ventricular tachycardia. These events were captured on telemetry, in a male in his early 40s, diagnosed with stage IIIB sigmoid colon cancer. Notably, the patient had no pre-existing coronary artery disease or other cardiovascular risk factors. Upon diagnosis, the patient was initiated on a calcium channel blocker, verapamil, to mitigate further coronary vasospasm events. After thorough discussions that prioritized the patient's input and values, an implantable cardioverter-defibrillator was placed subcutaneously. Following discharge, the patient restarted capecitabine therapy along with verapamil prophylaxis and did not experience any subsequent shocks from his ICD as assessed during his outpatient follow-up visits. This case emphasizes the need to involve patients in decision-making processes, especially when managing unexpected and serious complications, to ensure treatments align with their quality of life and personal preferences.

Focal Pulsed Field Ablation for Atrial Arrhythmias: Efficacy and Safety under Deep Sedation.

Focal pulsed field ablation (PFA) is a novel technique for treating cardiac arrhythmias. It has demonstrated positive results in initial studies and has a good safety profile. In recent studies, PFA was often utilized for first-time pulmonary vein isolation (PVI) and was performed under general anesthesia. In our study, we assessed the feasibility, safety, acute procedural efficacy, and efficiency of focal PFA under deep sedation in patients, 80% of whom had undergone at least one left atrial ablation previously. We treated 30 patients (71 ± 7, 46% male) using the CENTAURI system for various atrial arrhythmias, including atrial fibrillation, typical and atypical atrial flutter, and focal atrial tachycardia. The average procedure and fluoroscopy times were 122 ± 43 min and 9 ± 7 min, respectively. A total of 83.33% of patients received additional line ablations beyond PVI, specifically targeting the posterior box and anterior mitral line. All ablations were successfully performed in deep sedation with only one major and one minor complication observed. The major complication was a vasospasm of the right coronary artery during ablation of the cavotricuspid isthmus, which was treated successfully with intracoronary nitroglycerin. All patients could be discharged in sinus rhythm. Moreover, adenosine appears effective in identifying dormant conduction in some patients after focal PFA. In conclusion, focal PFA is an effective approach for complex left atrial ablations under deep sedation, offering both high efficacy and efficiency with a reliable safety profile. Studies on long-term outcomes are needed.

Successful Pregnancy After Cardiac Arrest in a Woman With Severe Coronary Vasospasm.

A 37-year-old gravida 5, para 3 woman presented with an unplanned pregnancy 6 weeks after experiencing a cardiac arrest caused by ventricular fibrillation from coronary vasospasm. She opted to continue the pregnancy with medical management despite ongoing chest pain and delivered a healthy female infant via vaginal delivery at 37 weeks.

Pathophysiology and Treatment of Intraseptal-Course Left Coronary Anomaly: Surgery for All?

Intraseptal-course, ectopic coronary anomalies are not well characterized as to anatomy, function, prognosis, and treatment. Recently, a revolutionary but unsupported new theory is claiming that most patients with a Left Anomalous Coronary Artery originating from the Opposite Sinus with anomalous Intra-Septal course (L-ACAOS-IS)-even small children-have significant stenoses and require open-heart surgery to prevent acute myocardial infarction and death. This surprising view has spurred ongoing discussions among adult and pediatric cardiologists and cardiac surgeons, compelling us (the conservative party in the discussion) to offer an in-depth and comprehensive review of this anomaly, based on objective but opposite data. We and other adult cardiologists have followed numerous L-ACAOS-IS patients for many years and have observed none of the claimed catastrophes. Rather, we have consistently found that L-ACAOS-IS generally has a benign clinical prognosis. We present the general principle of coronary artery dysfunction in anatomical congenital anomalies (that only significant luminal coronary stenosis can have clinical repercussions). We then review anatomical and functional details of L-ACAOS-IS related to prognosis and treatment indications, which could explain many of the clinical presentations recently mentioned. Finally, we encourage our more liberal colleagues to recognize that, compared with normal coronary arteries, those with anomalies of origin and course are associated with frequent coronary spasm. In particular, we underscore that some of the ischemic manifestations and other results might actually be caused by pressure wire-induced artifacts (rigid wires tend to cause coronary spasm when advanced into tortuous coronary arteries).

A case report of cardiac allograft vasculopathy complicated with coronary vasospasm: insights from near-infrared spectroscopy, intravascular ultrasound, and optical coherence tomography.

Background: Cardiac allograft vasculopathy (CAV) remains a major complication after heart transplantation. Although coronary vasospasm after heart transplantation has occasionally been reported, the association between CAV and coronary vasospasm remains unclear. Case summary: A 68-year-old male with a history of heart transplantation 21 years ago presented with atypical angina. Coronary angiography demonstrated intermediate stenoses in the proximal and mid left anterior descending artery (LAD) and right posterolateral artery. Intracoronary acetylcholine provocation testing resulted in subtotal occlusion of the coronary arteries bilaterally, which was resolved by nitroglycerine administration, but the intermediate stenoses remained. The stenosis in the proximal LAD was physiologically significant based on fractional flow reserve. The patient was diagnosed with a developed CAV and concomitant coronary vasospasm and treated with percutaneous coronary intervention and nifedipine. Near-infrared spectroscopy and intravascular ultrasound showed a large isoechoic plaque with a low lipidic burden, suggesting a non-atherosclerotic plaque. Optical coherence tomography revealed a layered homogenous plaque, an intravascular imaging finding common in both CAV and coronary vasospasm. A drug-eluting stent dilated the stenosis, and follow-up angiography at 4 months showed no CAV progression. Discussion: This case highlights the potential association between CAV and coronary vasospasm. Intravascular imaging detected similarities in plaque morphology between CAV and coronary vasospasm, suggesting that coronary vasospasm might contribute to the development of CAV. Although coronary vasospasm may be underdiagnosed in heart transplant recipients due to the lack of symptoms involving the denervated heart, this case showed that appropriate provocation testing may be beneficial for evaluating the cause of CAV.

Coronary Artery Vasospasm in Patients With Eosinophilia.

Coronary vasospasm is a relatively well-documented cause for ischemia and myocardial infarction in patients with nonobstructive coronary artery disease. Patients with coexisting eosinophilia present with severe manifestations and are often refractory to traditional therapies. There are few reported cases in the literature. We describe 3 cases occurring within 10 months. (Level of Difficulty: Intermediate.).

Coronary vasospasm during infusion of CD-19 directed chimeric antigen receptor T-cell therapy: a case report.

Background: Cardiovascular events have been reported to occur in one in five patients receiving chimeric antigen receptor T-cell (CAR-T) therapy. Commonly reported effects including cardiomyopathy, heart failure, myocardial infarction (MI), and arrhythmia. Here, we present a novel case of a patient who developed acute ST segment elevations during CAR-T cell infusion. Case summary: A 76-year-old man with diffuse large B cell lymphoma was admitted for an investigational CD-19 directed, autologous CAR-T cell therapy. Less than 5 min into the CAR-T cell infusion, he developed severe chest pain, dyspnea, flushing, hypotension, and tachycardia. Electrocardiogram (EKG) showed inferior ST elevations and reciprocal lateral ST depressions. Emergent coronary angiography revealed mild non-obstructive coronary disease. ST segment changes and patient symptoms resolved after catheterization. Discussion: Given the complete resolution of symptoms and EKG abnormalities in the context of non-obstructive coronary artery disease, this clinical presentation was thought to be most consistent with ST elevation MI due to coronary vasospasm. The mechanism of this vasospasm is as yet not understood and may be related to an anaphylactic reaction or a cardiotoxicity related to the cell therapy agent. As the use of CAR-T therapy continues to expand, there is a need to further characterize the full spectrum of its cardiotoxic effects.

Worsening of Angina Following Nitroglycerin Administration: A Case Report of the Interplay With Undiagnosed Myocardial Bridge.

Myocardial bridge (MB) is a congenital abnormality where part of a coronary epicardial artery runs under the myocardium fibers and is compressed in systole; this becomes more pronounced when nitroglycerin (NTG) is administered. In this report, we describe the case of a 40-year-old African American man who presented with chest pain that did not respond to NTG or isosorbide mononitrate and was only partially relieved by narcotics. His past medical history was significant for coronary artery disease (CAD) with a stent into the left anterior descending artery (LAD) several months prior, hypertension, hyperlipidemia, paroxysmal atrial fibrillation, sick sinus syndrome, permanent pacemaker, pulmonary embolism, and cerebral vascular accident. No explanation for his angina was found either in the previous outpatient left heart catheterization (LHC) procedures demonstrating LAD stent patency or initial chest pain workup upon admission. Functional LHC procedure with adenosine infusion and acetylcholine provocation demonstrated endothelial dysfunction with notable epicardial spasm and MB of the LAD that worsened with NTG. Cardiology advised dual antiplatelet therapy and a statin as part of treatment for CAD and a calcium channel blocker with a bradycardic effect (e.g., diltiazem, verapamil) for the MB and coronary vasospasm, and avoidance of NTG and long-acting nitrates (e.g., isosorbide mononitrate), which can cause reflex tachycardia and worsen angina from MB. A selective serotonin reuptake inhibitor was added for increased cardiac nociception. The patient's pain resolved, and he was discharged. MB is an important alternate etiology to consider when chest pain does not respond to NTG administration for adjustment of treatment modalities. The initial treatment for this patient's pain with NTG likely exacerbated symptoms by reducing intrinsic coronary wall tension and subsequently increasing reflex sympathetic stimulation of contractility of the left ventricular myocardium, which can, in turn, increase anginal symptoms and ischemia.

Coronary Microvascular Spasm: Clinical Presentation and Diagnosis.

Professor Maseri pioneered the research and treatment of coronary vasomotion abnormalities represented by coronary vasospasm and coronary microvascular dysfunction (CMD). These mechanisms can cause myocardial ischaemia even in the absence of obstructive coronary artery disease, and have been appreciated as an important aetiology and therapeutic target with major clinical implications in patients with ischaemia with non-obstructive coronary artery disease (INOCA). Coronary microvascular spasm is one of the key mechanisms responsible for myocardial ischaemia in patients with INOCA. Comprehensive assessment of coronary vasomotor reactivity by invasive functional coronary angiography or interventional diagnostic procedure is recommended to identify the underlying mechanisms of myocardial ischaemia and to tailor the best treatment and management based on the endotype of INOCA. This review highlights the pioneering works of Professor Maseri and contemporary research on coronary vasospasm and CMD with reference to endothelial dysfunction, Rho-kinase activation and inflammation.

Vasospasm in the First Septal Perforator Branch and Late High-Grade Atrioventricular Block Following Successful Primary Percutaneous Coronary Intervention for the Proximal Left Anterior Descending Coronary Artery: A Case Report.

We present a case of a high-degree advanced atrioventricular block (AVB), which occurred 24 hours after successful primary percutaneous coronary intervention (PCI) in the proximal left anterior descending coronary artery (LAD), the culprit of ST-segment elevation myocardial infarction (STEMI). The methylergometrine provocation test for coronary vasospasms, which was performed on the eighth hospital day, revealed transient total occlusion of the first septal perforator branch. After prescribing a calcium channel blocker to the patient, AVB did not recur for three years, as confirmed using an implantable loop recorder (ILR). In this patient, delayed high-grade AVB following primary PCI in the proximal LAD might be caused by the spasm of the first septal perforator branch. Documented cases of spasms in this branch are rare.