ABSTRACT
Lamin A/C gene mutations can be associated with cardiac diseases, usually referred to as 'cardiolaminopathies' characterized by arrhythmic disorders and/or left ventricular or biventricular dysfunction up to an overt picture of heart failure. The phenotypic cardiac manifestations of laminopathies are frequently mixed in complex clinical patterns and specifically may include bradyarrhythmias (sinus node disease or atrioventricular blocks), atrial arrhythmias (atrial fibrillation, atrial flutter, atrial standstill), ventricular tachyarrhythmias and heart failure of variable degrees of severity. Family history, physical examination, laboratory findings (specifically serum creatine phosphokinase values) and ECG findings are often important 'red flags' in diagnosing a 'cardiolaminopathy'. Sudden arrhythmic death, thromboembolic events or stroke and severe heart failure requiring heart transplantation are the most dramatic complications of the evolution of cardiolaminopathies and appropriate risk stratification is clinically needed combined with clinical follow-up. Treatment with cardiac electrical implantable devices is indicated in case of bradyarrhythmias (implant of a device with pacemaker functions), risk of life-threatening ventricular tachyarrhythmias (implant of an ICD) or in case of heart failure with wide QRS interval (implant of a device for cardiac resynchronization). New technologies introduced in the last 5 years can help physicians to reduce device-related complications, thanks to the extension of device longevity and availability of leadless pacemakers or defibrillators, to be implanted in appropriately selected patients. An improved knowledge of the complex pathophysiological pathways involved in cardiolaminopathies and in the determinants of their progression to more severe forms will help to improve clinical management and to better target pharmacological and non-pharmacological treatments.
Subject(s)
Arrhythmias, Cardiac/physiopathology , Arrhythmias, Cardiac/therapy , Clinical Decision-Making , Heart Diseases/physiopathology , Heart Diseases/therapy , Arrhythmias, Cardiac/genetics , Arrhythmias, Cardiac/pathology , Heart Diseases/genetics , Heart Diseases/pathology , Humans , Lamin Type A/genetics , Lamin Type A/metabolism , Musculoskeletal Diseases/genetics , Musculoskeletal Diseases/pathology , Musculoskeletal Diseases/physiopathology , Musculoskeletal Diseases/therapyABSTRACT
BACKGROUND: Chronic kidney disease (CKD) is associated with adverse outcomes in presence of atrial fibrillation (AF). However, the literature shows limited data on non-pharmacological management of AF in CKD patients. AIM: summarizing the available data on outcomes associated with electrical cardioversion (ECV) and AF catheter ablation (CA) in CKD patients. METHODS: We searched MEDLINE and the Cochrane Central Register of Controlled Trials and performed a metaanalysis. The primary outcome was recurrence of AF. The secondary outcomes were occurrence of thromboembolic events (TEs) and estimated glomerular filtration rate (eGFR) modification. RESULTS: Literature search yielded 26 eligible papers: 22 on CA and 4 concerning ECV. CKD patients presented more AF recurrences 30 days after ECV (OR 2.62, 95%CI 1.28-5.34; p <0.001). Patients with eGFR<60-68 ml/min and on dialysis presented a higher incidence of AF recurrences after CA, median follow up 26.0 and 29.9 months (HR 1.75, 95%CI 1.46-2.09, p <0.001; and HR 1.69, 95%CI 1.22-2.33, p <0.001; respectively). Periprocedural TEs were rare and not associated with CKD or dialysis. However, patients with CKD were at increased risk for delayed TEs after CA (HR 2.61, 95%CI 1.04-6.54; p <0.001). No significant modification of eGFR was associated with ECV or CA in the overall population. CONCLUSION: ECV and CA for sinus rhythm restoration/maintenance in AF patients, albeit theoretically promising, seem to be associated with lower efficacy at medium to long-term in patients with CKD. Further studies are needed to better define the role of ECV and CA in CKD.
Subject(s)
Atrial Fibrillation/complications , Catheter Ablation/adverse effects , Electric Countershock/adverse effects , Renal Insufficiency, Chronic/complications , HumansABSTRACT
The presence of clinical peripheral arterial disease (PAD) is associated with an increased risk for adverse cardiovascular outcomes in patients with coronary artery disease. However, there are few data regarding the impact of the presence and degree of the subclinical PAD on outcomes in patients with coronary artery disease. The aim of this study was to assess prospectively the grade of subclinical PAD in the setting of patients who underwent primary percutaneous coronary intervention for the prediction of intermediate- and long-term clinical outcomes. A total of 971 consecutive patients without histories of clinical PAD who under went primary percutaneous coronary intervention for ST-segment elevation myocardial infarction were included in a prospective follow-up. Subclinical PAD severity was blindly assessed on the basis of an ultrasound arterial morphologic classification defined with the assessment of wall carotid and femoral artery bifurcations. This classification included 4 increasing classes of subclinical carotid and femoral arterial wall lesions, and the total group was divided accordingly. Death and major cardiovascular and cerebrovascular events were evaluated. During a median follow-up period of 40 months, a total of 109 patients (11.2%) died, 9 (2.8%) in class I, 12 (3.1%) in class II, 37 (23.7%) in class III, and 51 (49.0%) in class IV (p <0.001). On multivariate analysis, mortality in class IV was sevenfold higher (hazard ratio [HR] 7.34, 95% confidence interval [CI] 3.3 to 16.33, p <0.001) compared to class I and was also increased in class III (HR 5.38, 95% CI 2.42 to 11.92, p <0.001). Similar results were obtained for major adverse cardiovascular and cerebrovascular events in class IV (HR 7.50, 95% confidence interval 5.36 to 10.50, p <0.0001), class III (HR 6.44, 95% CI 4.45 to 9.32, p <0.001), and class II (HR 1.73, 95% CI 1.23 to 2.43, p = 0.002). In conclusion, ultrasound arterial morphologic classification may be applied in patients with ST-segment elevation myocardial infarctions who undergo primary percutaneous coronary intervention and can stratify patients for poor clinical outcomes during long-term follow-up.