Coronary ectasia in different scenarios, primarily in myocardial infarction with nonobstructive coronary artery disease.

AIMS: Several causes have been reported for coronary artery ectasia (CAE), mostly atherosclerosis and tunica media abnormalities. The main aim of the present study was to investigate if CAE extension differs in distinct clinical settings. METHODS: Three hundred and forty-one patients with diagnosis of CAE were identified among 9659 coronary angiographies and divided into four groups according to the patient's admission diagnosis: stable or unstable angina (S-UA), myocardial infarction (MI), aortic disease, aortic valvular disease (AVD). S-UA and MI were subgrouped according to the presence of obstructive coronary artery disease (OCAD). Multivariable logistic regression was used to investigate the relationship between clinical diagnosis and CAE extension as expressed by Markis classification and number of coronary vessels affected by CAE. RESULTS: No significant differences in CAE extension were found among the four groups, in terms of vessels affected by CAE (P = 0.37) or Markis class (P = 0.33). CAE was not related to the extension of OCAD as assessed by the Gensini score, which was higher in MI and S-UA groups (P < 0.01). However, when ischemic patients were sub-divided on the basis of the presence of OCAD, MI without obstructive coronary artery disease (MINOCA) was associated with a higher extension of CAE in terms of Markis class 1 (OR 5.08, 95% CI 1.61-16.04; P < 0.01). CONCLUSION: The extension of CAE is comparable in patients referred to coronary angiography for different clinical scenarios, including S-UA, MI, aortic disease, and AVD; however, patients with MINOCA were associated with a higher extension of CAE.Graphical abstract: Difference in coronary artery ectasia extension in terms of Markis class severity, respectively, stratified by clinical presentation and obstructive coronary artery disease presence, http://links.lww.com/JCM/A519.

[Mitral valve prolapse and mitral annulus disjunction: be aware of a potential arrhythmogenic substrate]. / Prolasso valvolare mitralico e disgiunzione mitro-anulare. La consapevolezza di un possibile substrato aritmico.

Mitral valve prolapse is a relatively common disease with a good overall prognosis. However, in specific clinical and instrumental contexts, patients at high risk of ventricular arrhythmias and sudden cardiac death can be identified. Female sex, history of palpitations or syncope, bi-leaflet myxomatous valve, ECG repolarization abnormalities in the inferior leads, complex ventricular arrhythmias, left ventricular fibrosis detected by cardiac magnetic resonance correlate with a higher risk clinical profile. Additionally, morpho-functional abnormalities of the mitral valve annulus, particularly mitral annulus disjunction, may cause a mechanical stretch at the inferior basal ventricular wall and posterior papillary muscles, predisposing to myocardial fibrosis and arrhythmias. A risk stratification strategy is needed to identify patients with mitral valve prolapse and/or mitral annulus disjunction at high risk of arrhythmias; however, few data are available. Further prospective multicenter studies are warranted, focusing on medical therapy, the role of implantable cardioverter-defibrillators for primary prevention, efficacy of targeted catheter ablation or mitral valve surgery.

Clinical presentations leading to arrhythmogenic left ventricular cardiomyopathy.

OBJECTIVES: To describe a cohort of patients with arrhythmogenic left ventricular cardiomyopathy (ALVC), focusing on the spectrum of the clinical presentations. METHODS: Patients were retrospectively evaluated between January 2012 and June 2020. Diagnosis was based on (1) ≥3 contiguous segments with subepicardial/midwall late gadolinium enhancement in the left ventricle (LV) at cardiac magnetic resonance plus a likely pathogenic/pathogenic arrhythmogenic cardiomyopathy (AC) associated genetic mutation and/or familial history of AC and/or red flags for ALVC (ie, negative T waves in V4-6/aVL, low voltages in limb leads, right bundle branch block like ventricular tachycardia) or (2) pathology examination of explanted hearts or autoptic cases suffering sudden cardiac death (SCD). Significant right ventricular involvement was an exclusion criterion. RESULTS: Fifty-two patients (63% males, age 45 years (31-53)) composed the study cohort. Twenty-one (41%) had normal echocardiogram, 13 (25%) a hypokinetic non-dilated cardiomyopathy (HNDC) and 17 (33%) a dilated cardiomyopathy (DCM). Of 47 tested patients, 29 (62%) were carriers of a pathogenic/likely pathogenic DNA variant. Clinical contexts leading to diagnosis were SCD in 3 (6%), ventricular arrhythmias in 15 (29%), chest pain in 8 (15%), heart failure in 6 (12%) and familial screening in 20 (38%). Thirty patients (57%) had previously received a diagnosis other than ALVC with a diagnostic delay of 6 years (IQR 1-7). CONCLUSIONS: ALVC is hidden in different clinical scenarios with a phenotypic spectrum ranging from normal LV to HNDC and DCM. Ventricular arrhythmias, chest pain, heart failure and SCD are the main clinical presentations, being familial screening essential for the affected relatives' identification.

Myocardial infarction with non-obstructive coronary arteries in hypertrophic cardiomyopathy vs Fabry disease.

BACKGROUND: Little is known about prevalence and predictors of myocardial infarction with non-obstructive coronary arteries (MINOCA) in Fabry disease (FD) and hypertrophic cardiomyopathy (HCM). We assessed and compared the prevalence and predictors of MINOCA in a large cohort of HCM and FD patients. METHODS: In this multicenter, retrospective study we enrolled 2870 adult patients with HCM and 267 with FD. The only exclusion criterion was documented obstructive coronary artery disease. MINOCA was defined according to guidelines. For each patient we collected clinical, ECG and echocardiographic data recorded at initial evaluation. RESULTS: Overall, 36 patients had MINOCA during a follow-up period of 4.5 ± 11.2 years. MINOCA occurred in 16 patients with HCM (0.5%) and 20 patients with FD (7.5%; p < 0.001). The difference between the 2 groups was highly significant, also after adjustment for the main clinical, ECG and echocardiographic variables (OR 6.12; 95%CI 2.80-13.3; p < 0.001). In the FD population MINOCA occurred in 17 out of 96 patients with left ventricle hypertrophy (LVH, 17.7%) and in 3 out of 171 patients without LVH (1.7%; OR 12.0; 95%CI 3.43-42.3; p < 0.001). At multivariable analysis, voltage criteria for LVH at ECG (OR 7.3; 95%CI 1.93-27.7; p = 0.003) and maximal LV wall thickness at echocardiography (OR 1.15; 95%CI 1.05-1.27; p = 0.002) maintained an independent association with MINOCA. No major significant differences were found in clinical, ECG and echocardiographic findings between HCM patients with or without MINOCA. CONCLUSIONS: MINOCA was rare in HCM patients, and 6-fold more frequent in FD patients. MINOCA may be considered a red flag for FD and aid in the differential diagnosis from HCM.

Correlation between aortic root dimension and coronary ectasia.

BACKGROUND: Aortic aneurysms are associated with coronary artery ectasia (CAE). However, the relation between the extent of CAE and the severity of aortic dilatation is not understood. This study was undertaken to investigate the relationship between angiographic extension of CAE and aortic dimension. PATIENTS AND METHODS: We retrospectively include 135 patients with angiographic diagnosis of CAE defined as dilatation of coronary segment more than 1.5 times than an adjacent healthy one. Study population was divided in four groups according to the maximum diameter of ascending aorta beyond sinus of Valsalva obtained in the parasternal long-axis view (group 1: <40 mm; group 2: 40-45 mm; group 3: 45-55 mm; group 4: >55 mm or previous surgery because of aortic aneurysm/dissection). The relationship between aortic dimension and the extension of CAE was investigated by means of multivariable linear regression, including variables selected at univariable analysis (P < 0.1). The total estimated ectatic area (EEA total) was used as dependent variable. RESULTS: Baseline characteristics of study groups were well balanced. Patients in group 4 were more likely to have both higher neutrophil count and neutrophil to lymphocyte ratio. On univariable analysis ascending aorta diameter [Coef. = 0.075; 95% confidence interval (CI) 0.052-0.103, P < 0.01] and c-reactive protein (CRP) values [Coef. = 0.033, 95% CI 0.003-0.174, P = 0.04] showed a linear association with total EEA. After adjustment for CRP values only the ascending aorta diameter was still associated with the extent of CAE (95% CI 0.025-0.063, P < 0.01). CONCLUSION: In patients with diagnosis of CAE, a strong linear association between aortic dimension and coronary ectasia extent exists.

Sino-tubular junction to sinuses of Valsalva ratio: An echocardiographic parameter to predict coronary artery ectasia in patients with aortic enlargement.

BACKGROUND: Coronary artery ectasia (CAE) is associated with ascending aortic (AA) ectasia. The purpose of this study is to evaluate the diagnostic performance of different echocardiographic parameters (EP) in predicting the presence of CAE. METHODS: Four hundred-eighteen patients with AA ectasia candidate to coronary angiography were identified and divided in two groups in respect of the presence of CAE. Receiver-operating characteristic curves areas (AUC) were used to assess the discrimination power of the following EP: aortic annulus diameter, sinuses of Valsalva (SV) diameter, sino-tubular junction (STJ) diameter, AA diameter, STJ to SV ratio (STJ-to-SV) and STJ to AA ratio (STJ-to-AA). All these parameters were indexed by body surface area. The relationship between the best EP and the presence of CAE was investigated by means of multivariable logistic regression. RESULTS: The rate of CAE in the study population was 32%. On univariable logistic regression, aortic annulus, STJ, STJ-to-SV and STJ-to-AA were associated with the presence of CAE after Bonferroni correction. STJ-to-SV emerged as the parameter with the best discrimination power (AUC = 0.81) compared to STJ (AUC = 0.69), STJ-to-AA (AUC = 0.68), aortic annulus (AUC = 0.59), AA (AUC = 0.56) and SV (AUC = 0.55); (p for comparison <0.01). An 89.6% value for STJ-to-SV ratio emerged as the best cut-off to diagnose CAE with a sensitivity = 75%, specificity = 82%, positive predictive value = 66% and negative predictive value = 88%. On multivariable analysis, STJ-to-SV was still associated with the presence of CAE (OR = 1.15;95%CI:1.11-1.19;p < 0.01). CONCLUSION: In patients with dilated aorta, STJ-to-SV sampled by transthoracic echocardiography shows a good diagnostic performance in predicting the presence of CAE.

Pediatric Restrictive Cardiomyopathies.

Restrictive cardiomyopathy (RCM) is the least frequent phenotype among pediatric heart muscle diseases, representing only 2.5-3% of all cardiomyopathies diagnosed during childhood. Pediatric RCM has a poor prognosis, high incidence of pulmonary hypertension (PH), thromboembolic events, and sudden death, is less amenable to medical or surgical treatment with high mortality rates. In this scenario, heart transplantation remains the only successful therapeutic option. Despite a shared hemodynamic profile, characterized by severe diastolic dysfunction and restrictive ventricular filling, with normal ventricle ejection fraction and wall thickness, RCM recognizes a broad etiological spectrum, consisting of genetic/familial and acquired causes, each of which has a distinct pathophysiology and natural course. Hence, the aim of this review is to cover the causes, clinical presentation, diagnostic evaluation, treatment, and prognosis of pediatric RCM.