Abstract Introduction Patients with cardiac amyloidosis (CA) frequently present with heart failure with various extra-cardiac red flags. However, some patients can present with typical or atypical chest pain or even mimicking an acute coronary syndrome. Significant epicardial coronary artery disease (CAD) and CA can coexist, but microvascular dysfunction might also play a role. The aims of this study were to systematically investigate clinical indications for invasive coronary angiography (ICA) in patients with CA, to verify the prevalence of significant epicardial CAD and its relation to the symptoms at presentation, and to start investigating the role cardiac troponin (cTn) in this setting of patients with multiple possible causes of myocardial injury. Methods Retrospective observational study of patients with CA, both transthyretin (ATTR-CA) and light chain (AL-CA) evaluated in our Cardiac Amyloidosis Outpatient Clinic and undergoing ICA for clinical indication. Significant epicardial CAD was defined as stenosis ≥ 50% of the left main coronary artery and/or ≥ 70% of the other epicardial vessels. Results Of the 161 patients initially evaluated, 67 (42%) underwent ICA; after exclusion of those with ICA older than 5 years than CA diagnosis, the final cohort was of 61 patients, 12 (20%) AL-CA and 49 (80%) ATTR-CA. Patients with significant epicardial CAD at the index ICA had ATTR-CA and were more frequently affected by systemic hypertension and dyslipidemia. In 42 (69%) patients ICA was performed during an urgent hospitalization and in 38 (62%) patients CA diagnosis was already established/suspected at the time of the index ICA. The most common indication for ICA was chest pain (n=24, 39%), followed by dyspnea (n=21, 34%); electrocardiographic alterations/arrhythmias were the main indication for 4 patients (6.6%) while imaging for 6 (9.8%). In the remaining 6 patients (9.8%), there were other indications. Prevalence of significant epicardial CAD at the index ICA was 28% (n=17), up to 36% (n=22) if patients with previous coronary revascularization but without actual critical stenoses at index ICA were included. Among those presenting with chest pain (n=24), 9 (38%) had significant epicardial CAD at the index ICA whereas 15 (62%) did not. Considering those presenting with an acute event, the most frequent indication for ICA was chest pain (n=20, 48%); among these patients with chest pain, 55% did not have evidence of significant epicardial CAD whilst 45% did. Regarding cardiac troponin (cTn) values, concentrations at all timepoints were similar between patients with and without significant epicardial CAD at index ICA, also considering those presenting acutely and for chest pain. Conclusion In our cohort of CA patients undergoing ICA, the main clinical indication was chest pain; however, the majority of those presenting with chest pain did not have evidence of significant epicardial CAD. Significant epicardial CAD was present in around one third of patient, which is slightly less than what reported in non-amyloidotic HFpEF patients. The role of cTn in this setting is controversial, due to the possible concomitant causes of chronic and acute myocardial injury in these patients.
During the coronavirus disease-19 (COVID-19) outbreak in spring 2020, people may have been reluctant to seek medical care fearing infection. We aimed to assess the number, characteristics and in-hospital course of patients admitted for acute cardiovascular diseases during the COVID-19 outbreak.We enrolled all consecutive patients admitted urgently for acute myocardial infarction, heart failure or arrhythmias from 1 March to 31 May 2020 (outbreak period) and 2019 (control period). We evaluated the time from symptoms onset to presentation, clinical conditions at admission, length of hospitalization, in-hospital medical procedures and outcome. The combined primary end point included in-hospital death for cardiovascular causes, urgent heart transplant or discharge with a ventricular assist device.A similar number of admissions were observed in 2020 (N = 210) compared with 2019 (N = 207). Baseline characteristics of patients were also similar. In 2020, a significantly higher number of patients presented more than 6 h after symptoms onset (57 versus 38%, P < 0.001) and with signs of heart failure (33 versus 20%, P = 0.018), required urgent surgery (13 versus 5%, P = 0.004) and ventilatory support (26 versus 13%, P < 0.001). Hospitalization duration was longer in 2020 (median 10 versus 8 days, P = 0.03). The primary end point was met by 19 (9.0%) patients in 2020 versus 10 (4.8%) in 2019 (P = 0.09).Despite the similar number and types of unplanned admissions for acute cardiac conditions during the 2020 COVID-19 outbreak compared with the same period in 2019, we observed a higher number of patients presenting late after symptoms onset as well as longer and more complicated clinical courses.
Abstract Clinical Case A 80–year–old man underwent coronary angiography in 2006 because of stable angina. There was chronic total occlusion of middle left anterior descending artery requiring percutaneous intervention. In 2009 he underwent coronary angiography for recurrent angina: a severe stent restenosis was treated with stenting in stent (DES in BMS). The patient was subsequently asymptomatic till 2018 when he complained of exertional dyspnea and angina. Stent patency and severe aortic valve stenosis (AVA 0.85 cm2) were documented. The patient underwent successful transcatheter aortic valve implantation and discharged with dual antiplatelet therapy (DAPT) (Asa 100 mg and Clopidogrel 75 mg). Clinical and echocardiographic follow–up was unremarkable (mean prosthetic gradient 17 mmHg, DVI=0.45). In July 2021 during a cardiological check the patient complained of bruising: ASA was discontinued. In February 2022 the patient suffered from an exertional angina. A dipyridamole stress–echocardiography failed to demonstrate inducible ischemia, although the patient experienced angina at the peak load step. Transthoracic echocardiogram (TTE) revealed high mean trans–prosthetic aortic gradient (52 mmHg, DVI=0.18). A trans–esophageal echocardiogram (TEE) showed reduced mobility of the thickened left coronary and non–coronary aortic prosthetic leaflets. In the hypothesis of prosthetic valve thrombosis, warfarin (INR range 2–3) was started after stopping clopidogrel. After three months a TTE showed normalized mean trans–prosthetic gradients (17 mmHg, DVI=0.5). When a 6 month–period of anticoagulant therapy was accomplished, warfarin was stopped and DAPT administered again. In September 2022 the patient complained of worsening dyspnea and chest pain on exertion. At TTE mean trans–prosthetic gradients (46 mmHg) was increased. A new TEE showed thickening of non–coronary and right coronary leaflets, suggestive of recurrent valve thrombosis. After stopping DAPT, warfarin was started again with good result (mean prosthetic gradient 12 mmHg, DVI=0.5). Conclusion Thrombosis of aortic valve prothesis is an infrequent event usually occurring in early post–implantation period. In our case it occurred four years after implantation following DAPT interruption. The likely initial prosthetic valve degeneration together with a simplification of DAPT may have had a causative role. Since the proved efficacy of warfarin and the limited experience of DOAC in this field, the patient was given warfarin sine die.
Arrhythmogenic cardiomyopathy (ACM) is a genetic disorder characterized by fibrofatty replacement of myocardial tissue, predominantly affecting the right ventricle (RV), but often involving the left ventricle (LV) as well. The early detection of fibrosis, crucial for risk stratification, has been enhanced by advanced imaging techniques. Global longitudinal strain (GLS) has shown promise as a surrogate marker for late enhancement (LE) in identifying myocardial fibrosis, yet precise cut-off values for strain are lacking. The aim of the study is to evaluate LV strain as a predictor of LE in ACM and to define strain cut-offs for early fibrosis detection, enhancing non-invasive diagnostic accuracy.
Abstract Case n. 1 A 77 year–old woman with obstructive hypertrophic cardiomyopathy who had undergone alcohol septal ablation with a reduction in dynamic left ventricular outflow tract (LVOT) gradient from 95 to 65 mmHg was hospitalized for pulmonary oedema. Echocardiography and cardiac catheterization confirmed the presence of obstructive hypertrophic cardiomyopathy with a dynamic gradient of 65 mmHg, and identified a severe calcific mitral stenosis (3D planimetric area= 0,9 cm2). There was severe postcapillary pulmonary hypertension (pulmonary artery mean pressure of 60 mmHg and pulmonary capillary wedge pressure of 26 mmHg). The patient underwent a combined surgical intervention of mitral valve replacement (Epic 27 mm bioprosthesis) and surgical septal myectomy via a mini–thoracotomic approach. After the intervention there were dehiscence and infection of the thoracotomy and right inguinal surgical wounds, septic shock, hyporexia (with marked cachexia), emotional instability and delirium. After 21 days of hospitalization in the ICU, the patient was transferred to a medical department and died seven days later. Case n. 2 A 82 year–old woman with severe mitral stenosis associated with marked left ventricular hypertrophy and LVOT obstruction was admitted for heart failure and worsening renal function. Her comorbidities included diabetic neuropathy and stage 4 chronic kidney disease. Cardiac catheterization excluded significant coronary artery disease; mitral valve area was 1 cm2, and LVOT gradient was 75 mmHg. Pulmonary artery mean pressure was 32 mmHg, pulmonary capillary wedge pressure was 25 mmHg, and right atrial mean pressure was 8 mmHg. She was deemed a high–risk surgical candidate. The patient therefore was treated initially with peritoneal dialysis. However she demonstrated persisting pulmonary congestion. An atrial septostomy inserting a 8–mm Atrial Flow Regulator was subsequently performed, with an immediate reduction of capillary wedge pressure to 18 mmHg. In the following 18 months the patient had no more hospitalizations for heart failure. Conclusion The association between mitral stenosis and LVOT obstruction is associated with elevated capillary wedge pressure and frequent episodes of heart failure. Surgical intervention remains the first therapeutical option. Nevertheless, in older patients, the association between atrial septostomy and peritoneal dialysis may represent a valuable solution, with a lower operative risk.
In the last few years, a phenotypic variant of arrhythmogenic cardiomyopathy (ACM) labeled arrhythmogenic left ventricular cardiomyopathy (ALVC) has been defined and researched. This type of cardiomyopathy is characterized by a predominant left ventricular (LV) involvement with no or minor right ventricular (RV) abnormalities. Data on the specific risk and management of pregnancy in women affected by ALVC are, thus far, not available. We have sought to characterize pregnancy course and outcomes in women affected by ALVC through the evaluation of a series of childbearing patients.A series of consecutive female ALVC patients were analyzed in a cross-sectional, retrospective study. Study protocol included 12-lead ECG assessments, 24-h Holter ECG evaluations, 2D-echocardiogram tests, cardiac magnetic resonance assessments, and genetic analysis. Furthermore, the long-term disease course of childbearing patients was compared with a group of nulliparous ALVC women.A total of 35 patients (mean age 45 ± 9 years, 51% probands) were analyzed. Sixteen women (46%) reported a pregnancy, for a total of 27 singleton viable pregnancies (mean age at first childbirth 30 ± 9 years). Before pregnancy, all patients were in the NYHA class I and none of the patients reported a previous heart failure (HF) episode. No significant differences were found between childbearing and nulliparous women regarding ECG features, LV dimensions, function, and extent of late enhancement. Overall, 7 patients (20%, 4 belonging to the childbearing group) experienced a sustained ventricular tachycardia and 2 (6%)-one for each group-showed heart failure (HF) episodes. The analysis of arrhythmia-free survival patients did not show significant differences between childbearing and nulliparous women.In a cohort of ALVC patients without previous episodes of HF, pregnancy was well tolerated, with no significant influence on disease progression and degree of electrical instability. Further studies on a larger cohort of women with different degrees of disease extent and genetic background are needed in order to achieve a more comprehensive knowledge regarding the outcome of pregnancy in ALVC patients.
Abstract Background Lamin A (LMNA) protein is one of the main constituents of the nuclear lamina. Pathogenic variants of LMNA gene have been demonstrated to be linked to a wide spectrum of disorders, including cardiac diseases. The first reported heart disease linked to LMNA was dilated cardiomyopathy (DCM), in a form typically characterized by the presence of conduction disorders and high incidence of sudden cardiac death (SCD) disrespectfully of left ventricular systolic function. Genetic variants of the same gene have been associated with Arrhythmogenic Cardiomyopathy (ACM), but its putative role as disease causing gene is still debated. Case summary a man with positive familial history for DCM presented to the Emergency Department because of palpitations. The electrocardiogram showed first degree AV-block, complete right bundle branch block and low QRS voltages on limb leads. Cardiac magnetic resonance revealed severe dilatation of the right ventricle with wall motion abnormalities in keeping with ACM, associated with mild dilatation and moderate dysfunction of left ventricle. The genetic test identified a pathogenic variant in LMNA. Moreover, non-sustained ventricular tachycardias were present on the ECG Holter. According to international guidelines on SCD prevention, the patient underwent ICD implantation, with one appropriate ICD discharge on a sustained ventricular tachycardia episode after few months. Discussion LMNA pathogenic variants can lead to a broad spectrum of cardiac manifestations, including Arrhythmogenic Cardiomyopathy. A genetic test should be considered if there is a familial cardiomyopathy associated with conduction disorders, in order to achieve a proper risk stratification for sudden death.
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