Prinzmetal angina in a child with actin gene ACTC1 mutation.

Prinzmetal angina is a rare cause of intermittent chest pain in paediatrics. Here, we report the case of a 2-year-old female who presented with episodic chest pain, malaise, diaphoresis, fatigue, and poor perfusion on exam. During her hospitalisation, these episodes were associated with significant low cardiac output as evidenced by lactic acidosis and low mixed venous oxygen saturations. Her workup revealed an actin alpha cardiac muscle 1 (ACTC1) gene mutation and associated left ventricular non-compaction with decreased systolic function. She was started on oral heart failure medications as well as a calcium channel blocker but continued to have episodes which were found to promptly resolve with nitroglycerine. She was ultimately listed for cardiac transplant given her perceived risk of sudden death.

ST-segment elevation and the Tpeak-Tend/QT ratio predict the occurrence of malignant arrhythmia events in patients with vasospastic angina.

BACKGROUND: ST-segment elevation (STE) and an increased Tpeak-Tend interval (Tp-e) have prognostic value for malignant arrhythmia events (MAEs) in patients with ST-segment elevation myocardial infarction (STEMI) and Brugada syndrome. Whether STE could predict MAEs and has an electrophysiological relationship with Tp-e in electrocardiogram (ECG) of vasospastic angina (VA) patients needs to be elucidated. METHODS: Sixty-five patients with VA and 23 patients with VA complicated by MAEs were enrolled. The relationship of ECG parameters and MAEs (defined as ventricular tachycardia/ventricular fibrillation (VT/VF), syncope, and aborted sudden death) was analyzed by t-test, regression and receiver operating characteristic (ROC) curve analyses. RESULTS: Patients with MAEs showed greater STE (P<0.001) and corrected QT dispersion (cQTd) (P=0.021), a longer corrected Tp-e interval (cTp-e) (P<0.001), and a larger Tp-e/QT ratio (P<0.001) than those in non-MAE groups. Univariate analysis revealed that cQTd (odds ratio (OR)=1.065; P=0.020), cTp-e (OR=1.159; P=0.001), Tp-e/QT (OR=1.344, P=0.002), and STE (OR=5.655, P<0.001) were significantly associated with MAEs. In the multivariate analysis, Tp-e/QT and STE remained predictors of MAEs. ROC curve analysis showed that the areas under curve (AUCs) for Tp-e/QT (AUC=0.944) and STE (AUC=0.974) were not significantly different (P>0.05), but both were significantly different than AUCs for cQTd (AUC=0.724) and cTp-e (AUC=0.841) (all P<0.05). STE was well fitted with the Tp-e/QT ratio in a multivariable linear regression model. CONCLUSIONS: STE and increased Tp-e/QT ratio had related electrophysiological properties and were independent prognostic indicators of MAEs in patients with VA.

Early repolarization is involved in ventricular fibrillation in patients with variant angina.

BACKGROUND: Variant angina (VA) is caused by reversible coronary artery spasm, which is characterized by chest pain with ST segment elevations on a standard 12-lead electrocardiogram (ECG) at rest. VA attack often causes lethal ventricular arrhythmia. The early repolarization (ER) pattern is associated with ventricular fibrillation (VF). However, whether the ER pattern is involved in VF in patients with VA is not known. We investigated the association between the ER pattern and VF in patients with VA. METHODS: Fifty patients underwent induction of ST elevation on 12-lead ECGs with total or nearly total occlusion by provocation test (VA patients). Twelve of these patients underwent induction of VF or had documented VF before hospital admission (VF occurrence group). The J-wave morphology was characterized as exhibiting notching or slurring. The amplitude of each J wave was measured manually with amplified waveforms. RESULTS: ER patterns were observed significantly more often in the VF occurrence group than in the non-VF occurrence group (P = 0.007). The J-wave amplitude was significantly higher in the VF occurrence group compared with the non-VF occurrence group (P = 0.02). Univariate analyses suggested that age, smoking, and ER patterns were associated with VF. Upon multivariate analyses, age (odds ratio [OR] = 0.880; 95% confidence interval [CI]: 0.794-0.975; P = 0.014) and ER patterns (OR = 8.937; 95% CI:1.661-48.06; P = 0.011) predicted VF independently. CONCLUSIONS: These data suggest that an ER pattern in VA patients is a risk factor for VF. The ER pattern may be one of the useful factors for adaptation of implantation of implantable cardioverter-defibrillator in patients with coronary spasm-induced VF.

Variant Angina and Aborted Sudden Cardiac Death.

PURPOSE OF REVIEW: Variant angina, which is characterized by recurrent chest pain and transient ECG changes along with angiographic evidence of coronary artery spasm, generally has a favorable prognosis. However, episodes of ischemia caused by vasospasm may lead to potentially life-threatening ventricular arrhythmias and cardiac arrest, even in patients with no history of prior cardiac disease. This review describes the epidemiology, pathogenesis, clinical spectrum, and management of variant angina, as well as outcomes in patients who present with aborted sudden cardiac death (ASCD). RECENT FINDINGS: Contrary to prior opinions, evidence from recent observational studies indicate that patients with variant angina presenting with ASCD face a worse prognosis than those without this type of presentation. Predictors of ASCD include age, hypertension, hyperlipidemia, family history of sudden cardiac death, multi-vessel spasm, and left anterior descending artery spasm. Medical therapy alone with calcium channel blockers and nitrates may not be sufficiently protective in these patients and there is lack of concrete data on the optimal management strategy. Current guidelines recommend implantable cardiac defibrillator (ICD) therapy in patients who are survivors of cardiac arrest caused by ventricular fibrillation or unstable ventricular tachycardia after reversible causes are excluded, and should strongly be considered in these patients. Although medical therapy is absolutely imperative for patients with variant angina and a history of ASCD, ICD therapy in these patients is justified. Further large-scale studies are required to determine whether ICD therapy can improve survival in this high-risk group of patients.

Alternative causes of myocardial ischemia in women: An update on spontaneous coronary artery dissection, vasospastic angina and coronary microvascular dysfunction.

Although coronary obstruction due to atherosclerosis is the most common cause of myocardial ischemia, a significant proportion of patients have myocardial ischemia in the absence of obstructive epicardial coronary artery disease (CAD). This finding is more common among women and alternative causes can mediate myocardial ischemia. Abnormalities in vascular structure, alterations in coronary vasomotion and dysfunction of the coronary microcirculation can all cause ischemia in the absence of obstructive CAD due to atherosclerosis. In this review, we provide an update on three alternative causes of myocardial ischemia: spontaneous coronary artery dissection (SCAD), vasospastic angina (VSA) and coronary microvascular dysfunction (CMVD). We review pathophysiology, clinical presentation, diagnosis, treatment and outcomes related to these important clinical entities. There is increasing interest in better defining this patient population with use of advanced imaging and testing tools. Despite the increased associated risk with future cardiac events, evidence-based treatments for these diagnoses remain under-studied and poorly defined. These alternative diagnoses should be kept in mind when evaluating women with myocardial ischemia without obstructive CAD due to atherosclerosis.

Vasospastic Angina Presenting With Syncope and Chest Pain: A Case Report and Brief Literature Review.

A 65-year-old male presented to the hospital with chest pain associated with recurrent syncope. He had a history of coronary artery disease and a long-standing history of smoking. While he was hospitalized, he had an episode of chest pain during which he was found to have transient ST segment elevation in the inferior leads. He was also noted to have a brief cardiac tachyarrhythmia. Coronary arteriography revealed vasospasm of the left anterior descending artery and right coronary artery, which were relieved to a significant extent after administration of intracoronary nitroglycerin. Subsequent angiograms and fractional flow reserve studies, demonstrated underlying non-obstructive coronary artery disease at the sites of spasm. No percutaneous coronary intervention was pursued. The patient was started on a calcium channel blocker on dismissal from the hospital. Upon follow up several months later, he remained free of symptoms that brought him to the hospital.

[A CASE OF ASPIRIN-EXACERBATED RESPIRATORY DISEASE COMPLICATED BY REFRACTORY VARIANT ANGINA, WHICH SHOWED EXCELLENT RESPONSE TO STEROID TREATMENT].

A 47-year-old woman with aspirin-exacerbated respiratory disease visited our hospital complaining of persistent chest pain that manifested in the evenings and early mornings. Holter monitoring revealed ST elevation during chest pain and coronary angiography showed coronary vasospasm, which led to the diagnosis of variant angina. Chest pain persisted despite administration of a coronary vasodilator. The patient experienced an increase in peripheral blood eosinophils during the clinical course and received prednisolone for the same, which resulted in the resolution of her chest pain. Prednisolone was therefore seen to be effective for treating variant angina that manifested as a non-respiratory tract symptom of aspirin-exacerbated respiratory disease.

Structural and Functional Coronary Artery Abnormalities in Patients With Vasospastic Angina Pectoris.

Coronary spasm is involved in many clinical scenarios, such as stable angina, acute coronary syndrome, sudden cardiac death, non-ischemic cardiomyopathy, arrhythmia and syncope. In recent years, imaging tools such as computerized tomographic angiography, intravascular ultrasound or optical coherence tomography have been applied to study the coronary pathology in patients with vasospastic angina. Patients with vasospastic angina represent a heterogeneous cohort of patients with regard to the extent of concomitant coronary atherosclerosis. They share the common pathophysiological phenomenon of vascular smooth muscle hyperreactivity leading to spasm caused by various factors that may also overlap. Focal coronary spasm is related to epicardial atherosclerosis and in the presence of obstructive coronary artery disease it may be useful to treat the lesion to prevent further spasm. The aim of this article is to review structural and functional coronary artery abnormalities in patients with vasospastic angina.

Double hazards of ischemia and reperfusion arrhythmias in a patient with variant angina pectoris.

Variant angina pectoris, also called Prinzmetal's angina, is a syndrome caused by vasospasms of the coronary arteries. It can lead to myocardial infarction, ventricular arrhythmias, atrioventricular block and even sudden cardiac death. We report the case of a 53 year-old male patient with recurrent episodes of chest pain and arrhythmias in the course of related variant angina pectoris. It is likely that the reperfusion following myocardial ischemia was responsible for the ventricular fibrillation while the ST-segment returned to the baseline. This case showed that potential lethal arrhythmias could arise due to variant angina pectoris. It also indicated that ventricular fibrillation could be self-terminated.

[Ventriculer fibrillation due to Prinzmetal angina in a pregnant patient]. / Hamile bir hastada ventrikül fibrilasyonuna neden olan Prinzmetal anjina.

Variant angina, which is also referred to as prinzmetal or coronary vasospastic angina, is a clinical entity characterized by episodes of angina pectoris, usually at rest and often between midnight and early morning, in association with ST-segment elevation on the electrocardiogram. Angina is usually caused by focal spasm of a major coronary artery resulting in a high-grade obstruction, and myocardial infarction may develop in some cases. We report a prinzmetal angina which caused ventricular fibrillation and cardiac arrest in an 18-week pregnant woman.

Vasospastic angina: a review on diagnostic approach and management.

Vasospastic angina (VSA) refers to chest pain experienced as a consequence of myocardial ischaemia caused by epicardial coronary spasm, a sudden narrowing of the vessels responsible for an inadequate supply of blood and oxygen. Coronary artery spasm is a heterogeneous phenomenon that can occur in patients with non-obstructive coronary arteries and obstructive coronary artery disease, with transient spasm causing chest pain and persistent spasm potentially leading to acute myocardial infarction (MI). VSA was originally described as Prinzmetal angina or variant angina, classically presenting at rest, unlike most cases of angina (though in some patients, vasospasm may be triggered by exertion, emotional, mental or physical stress), and associated with transient electrocardiographic changes (transient ST-segment elevation, depression and/or T-wave changes). Ischaemia with non-obstructive coronary arteries (INOCA) is not a benign condition, as patients are at elevated risk of cardiovascular events including acute coronary syndrome, hospitalization due to heart failure, stroke and repeat cardiovascular procedures. INOCA patients also experience impaired quality of life and associated increased healthcare costs. VSA, an endotype of INOCA, is associated with major adverse events, including sudden cardiac death, acute MI and syncope, necessitating the study of the most effective treatment options currently available. The present literature review aims to summarize current data relating to the diagnosis and management of VSA and provide details on the sequence that treatment should follow.

Diagnosis and treatment of epicardial coronary artery spasmVasospastic angina (VSA) refers to chest pain experienced as a consequence of a sudden narrowing of the epicardial coronary arteries. VSA can occur in patients with non-obstructive coronary arteries and obstructive coronary artery disease, with transient spasm causing chest pain and persistent spasm potentially leading to acute myocardial infarction. Reduced blood and oxygen supply in patients with non-obstructive coronary arteries is not a benign condition, as patients are at elevated risk of adverse cardiovascular events. These patients also experience impaired quality of life and associated increased healthcare costs. This review aims to summarise current data relating to the diagnosis of VSA and provides details on treatment strategies.

Morphological findings in typical variant angina presenting as acute coronary syndrome using optical coherence tomography.

BACKGROUND: Coronary vasospasm causes variant angina, as well as acute myocardial infarction, ventricular tachycardia, and sudden cardiac death. We evaluated morphological changes due to vasospastic lesions, which may cause acute coronary syndrome (ACS), using a novel technique called optical coherence tomography (OCT). METHODS: Twenty patients (40-83 years old, 19 males) with vasospasm-induced ACS who visited the emergency room because of continuous chest pain and displayed transient ST segment elevation in their electrocardiogram were enrolled in the study. None of these patients had significant coronary artery disease and all had positive results in the provocation test. OCT examinations were performed for evaluation of vasospastic lesions. RESULTS: Intraluminal thrombi and intimal erosion were found in 6 (33.3%) and 2 patients (10%), respectively. High-sensitivity C-reactive protein levels were significantly higher in patients with microthrombi (2.66 ± 3.33 mg/L) compared with those in patients without microthrombi (0.49 ± 0.30 mg/L; P = 0.022). Serum cardiac troponin-I levels were not significantly different between patients with or without microthrombi (2.37 ± 5.31 ng/mL vs. 1.45 ± 4.68 ng/mL; P = 0.704). Other parameters, including creatinine kinase-myocardial band isoenzyme, total cholesterol, pain duration, residual stenosis, lesion length, and coronary risk factors, were not significantly different between the 2 groups. CONCLUSION: In patients with vasospasm-induced ACS, microthrombi with or without intimal erosion are major abnormal morphologic findings of OCT examinations. However, further large-scale studies are required for validation.

Vasospastic Angina With ST-Segment Elevation Seen During Mobile Cardiac Outpatient Telemetry (MCOT) Monitoring.

A 54-year-old man presented with significant ST-segment elevations noted on both channels displayed on the mobile cardiac outpatient telemetry (MCOT). Pertinent cardiac history was remarkable for syncope and episodes of atypical chest pain. The latter were described as infrequent and not associated with exercise intolerance. His syncopal episodes were described as occurring mostly in the mornings after the use of the restroom. Episodes happen 1 or 2 times a year since 2015. Patient had undergone thorough investigation with no significant findings. An MCOT was prescribed since frequency of symptoms has recently increased. Significant ST-segment elevations were noted. The patient described atypical chest pain and a sensation of presyncope during these recordings. He was urgently admitted, and a coronary angiogram revealed no epicardial luminal stenosis. However, the presence of sluggish coronary flow was suggestive of possible vasospastic angina. No ST-segment changes were noted during his coronary angiogram. The remarkable element portrayed by this case hinges in showing the unique utility of MCOT, as the most uncharacteristic diagnostic tool, in identifying transient ST-segment elevations that finally led to the diagnosis.

Vasospastic angina with J-wave pattern and polymorphic ventricular tachycardia effectively treated with quinidine.

BACKGROUND: Myocardial ischemia during coronary spasm may generate malignant ventricular arrhythmias. The J-wave pattern was suggested to be a marker of a disorder associated with life-threatening arrhythmias. RESULTS: We report the case of a patient with vasospastic angina and J-wave pattern in inferior and lateral leads associated with polymorphic ventricular tachycardia which was effectively treated only with quinidine-vasodilating drugs were not able to prevent the arrhythmia although they were effective in preventing ischemic events. CONCLUSION: The J-wave pattern in inferolateral leads may be a sign of electrical vulnerability to lethal ventricular arrhythmia in patients suffering from vasospastic angina--quinidine can effectively prevent such arrhythmias in these patients.

[High-degree atrioventricular block induced by Prinzmetal angina]. / Bloqueio aurículo-ventricular de alto grau induzido por angina de Prinzmetal.

We present the case of a 46-year-old woman with episodes of chest pain with ST-T segment elevation and paroxysmal high-degree atrioventricular block and syncopal episodes. Coronary angiography revealed no significant atheromatous lesions. The patient was treated with nitrates and calcium channel blockers but syncopal episodes with atrioventricular block persisted. A DDD-R pacemaker was implanted and four-month follow-up was uneventful.

Spontaneous Multivessel Coronary Spasm During Diagnostic Coronary Angiography.

Acute vasospastic angina, formerly known as Prinzmetal angina, is characterized by transient electrocardiographic changes that are not related to exertion. Its atypical presentation makes it difficult to establish the diagnosis, so it is probably underrecognized and therefore mismanaged. We treated a 49-year-old woman who presented with a 2-day history of chest pain associated with palpitations. Abnormal radionuclide stress test results prompted diagnostic coronary angiography, during which the patient reported chest pain and became hemodynamically unstable. Active coronary vasospasm at multiple sites was treated with intracoronary nitroglycerin and nicardipine, leading to immediate recovery. Our case highlights the importance of accurate, timely diagnosis of vasospastic angina, and of early recognition and management of spontaneous coronary spasm during angiography.

Variant Angina with Spontaneously Documented Ischemia- and Tachycardia-induced "Lambda" Waves.

In a patient with variant angina of the proximal left anterior descending coronary artery, myocardial ischemia changed the QRS-ST-T configurations without J-waves into those resembling "lambda" waves at maximal ST-segment elevation, and couplets or triplets of supraventricular extrasystole (SVE) changed the ischemia-induced "lambda" waves into QRS-ST-T configurations resembling a "tombstone" morphology or "monophasic QRS-ST complex." At the resolution phase of coronary spasm, the QRS-ST-T configurations returned to those without J-waves and were changed by SVE into "lambda" waves. Interestingly, neither ischemia- nor SVE-induced "lambda" waves or SVE-induced "tombstone" morphology or "monophasic QRS-ST complex" were complicated by ventricular tachyarrhythmia.

Cardiac arrest induced by submucosal injection of epinephrine in a patient with variant angina.

This case report describes a 35-year old male who experienced ventricular tachycardia induced by intramucosal injection of epinephrine (1:100,000). Under general anaesthesia with desflurane inhalation, 1.5% lidocaine containing 1:100,000 epinephrine was injected into the nasal mucosa for septoplasty. ST segment elevation and QRS widening occurred after 10 minutes and progressed to pulseless ventricular tachycardia. A sinus rhythm was restored after cardiopulmonary resuscitation with electrical cardioversion. The cardiac enzymes were significantly elevated after the event. Exercise-stress testing and coronary angiography were normal. However, an injection of acetylcholine into the coronary artery provoked vasospasm in the left anterior descending and circumflex arteries. This case illustrates an unusual response to low dose epinephrine with cardiac arrest induced in a patient with undiagnosed variant angina.