A 38 year old well functioning Starr-Edwards valve prosthesis (original) (raw)
Related papers
A familial cluster of idiopathic dilatation of the right atrium
2010
We report two cases of idiopathic atrial dilatation in two adult siblings, a brother and a sister. The first patient was a 36-year-old man who was referred to our institution for evaluation of atrial fibrillation and syncopes. Transthoracic echocardiography revealed an enlarged right atrium accompanied by a severe tricuspid regurgitation associated with annular dilatation. The diagnosis of idiopathic atrial enlargement was made after all other lesions known to produce it have been excluded. The patient did not consent to the proposed cardiothoracic surgical treatment so he remained on conservative therapy. On electrocardiography, atrial standstill was noted, resulting in the implantation of a VVI cardiac pacemaker one year later. After an asymptomatic period, the patient suffered sudden death at the age of 40 years. His 45-year-old sister suffering from the same symptoms was also referred for examination, also to be diagnosed with idiopathic atrial dilatation and severe tricuspid regurgitation. Idiopathic dilatation of the right atrium, although a rare disorder, should not be forgotten as differential diagnosis for enlarged right atrium. Transthoracic echocardiography is the most commonly used technique and in our cases it was sufficient for establishing the diagnosis.
A familial cluster of idiopathic dilatation of the right atrium—A two-case report
Journal of Cardiology Cases, 2010
We report two cases of idiopathic atrial dilatation in two adult siblings, a brother and a sister. The first patient was a 36-year-old man who was referred to our institution for evaluation of atrial fibrillation and syncopes. Transthoracic echocardiography revealed an enlarged right atrium accompanied by a severe tricuspid regurgitation associated with annular dilatation. The diagnosis of idiopathic atrial enlargement was made after all other lesions known to produce it have been excluded. The patient did not consent to the proposed cardiothoracic surgical treatment so he remained on conservative therapy. On electrocardiography, atrial standstill was noted, resulting in the implantation of a VVI cardiac pacemaker one year later. After an asymptomatic period, the patient suffered sudden death at the age of 40 years. His 45-year-old sister suffering from the same symptoms was also referred for examination, also to be diagnosed with idiopathic atrial dilatation and severe tricuspid regurgitation. Idiopathic dilatation of the right atrium, although a rare disorder, should not be forgotten as differential diagnosis for enlarged right atrium. Transthoracic echocardiography is the most commonly used technique and in our cases it was sufficient for establishing the diagnosis.
Atrial fibrillation in patients with inherited cardiomyopathies
Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, 2018
Atrial fibrillation (AF) often complicates the course of inherited cardiomyopathies and, in some cases, may be the presenting feature. Each inherited cardiomyopathy has its own peculiar pathogenetic characteristics that can contribute to the development and maintenance of AF. Atrial fibrillation may occur as a consequence of disease-specific defects, non-specific cardiac chamber changes secondary to the primary illness, or a combination thereof. The presence of AF can denote a turning point in the progression of the disease, promoting clinical deterioration and increasing morbidity and mortality. Furthermore, the management of AF can be particularly challenging in patients with inherited cardiomyopathies. In this article, we review the current information on the prevalence, pathophysiology, risk factors, and treatment of AF in three different inherited cardiomyopathies: hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia/cardiomyopathy, familial dilated cardiomyo...
Heart Rhythm, 2013
exclusion of events that are likely due to vasovagal events such as those occurring during abrupt postural changes, exposure to heat and dehydration, emotional reactions to events such as blood drawing, etc. We refer to the guidelines of ESC and AHA/ACCF for the differential diagnoses of syncope [3, 4]. Symptomatic individuals: the term "symptomatic" refers to individuals who have experienced ventricular arrhythmias (usually ventricular tachycardia or resuscitated ventricular fibrillation), or syncopal episodes (see definition above). The presence of symptoms is, in some of the channelopathies, an independent predictor of cardiac arrest at follow up. Arrhythmic events: the term refers to the occurrence of symptomatic or asymptomatic sustained or nonsustained spontaneous ventricular tachycardia, or unexplained syncope/resuscitated cardiac arrest. Concealed mutation-positive patients: this term is used to refer to individuals without clinical symptoms or phenotype of a channelopathy who carry the genetic defect present in clinically affected members of the family. Methodological aspects and instructions for the consultation of the document When considering the guidance from this document, it is important to remember that there are no absolutes governing many clinical situations. The final judgment regarding care of a particular patient must be made by the health care provider and the patient in light of all relevant circumstances. Recommendations are based on consensus of the writing group following the Heart Rhythm Society's established consensus process. It is recognized that consensus does not mean unanimous agreement among all writing group members. We identified the aspects of patients' care for which a true consensus could be found. Surveys of the entire writing group were used. The authors received an agreement that was equal to or greater than 84% on all recommendations; most recommendations received agreement of 94% or higher. This statement is directed to all health care professionals who are involved in the management of 1) individuals who survived a cardiac arrest at a young age (usually defined as <40 years) in the absence of a clinical diagnosis of cardiac disease, despite extensive clinical assessment; 2) family members of individuals who died suddenly at young age with a negative autopsy; 3) in patients and family members in whom the diagnosis of a channelopathy is clinically possible, likely, or established, and 4) young patients with unexplained syncope.
Hypertension, 2014
S everal studies have shown that alterations in left ventricular (LV) structure and function, such as an increased LV mass (LVM), a reduction of systolic contractility, and an impairment of LV diastolic function, have an adverse prognostic value (ie, that they are associated with an increased risk of cardiovascular morbid and fatal events, independently on other cardiovascular risk factors). 1,2 More recently, evidence has been obtained that this is the case also for anatomic alterations of the left atrium (LA), the enlargement of which has been found to predict independently the development of heart failure, 3 and other cardiovascular events. 4,5 LA enlargement, however, frequently occurs with an increased LVM or a frank LV hypertrophy (LVH), 6,7 both changes being commonly generated by an increase in blood pressure (BP), 8 and limited information exists on whether LA enlargement increases cardiovascular risk also when associated with or superimposed on LV structural changes. This has been the aim of the present study, which has examined the long-term risk of cardiovascular morbid and fatal events (as well as of all-cause mortality) in a population in which LA enlargement and LV hypertrophy were present in isolation or combination, their combined absence representing the control group. A strength of the study is that BP was measured in and outside the physician's office, which allowed to correct the data for the effect of BP elevation on cardiovascular risk more accurately than by using office BP. 9 Methods Subjects and Measurements The details of the Pressioni Arteriose Monitorate E Loro Associazioni (PAMELA) study have been reported elsewhere. 10 Briefly, the study was performed in 1990 to 1991 on 3200 individuals aged between 25 and 74 years, who were selected to be representative of the Monza population, based on the criteria of the World Health Organization Monitoring Diseases (WHO-MONICA) project performed in the same geographic area. 11 The participation rate was 64%, and the demographic characteristics and medical history of nonparticipants were similar to those of participants. All subjects underwent a Abstract-We estimated the risk of cardiovascular events, cardiovascular mortality, and all-cause mortality associated with left atrium (LA) enlargement alone or combined with echocardiographic left ventricular hypertrophy (LVH) in 1785 representatives of the general population of Monza recruited for the Pressioni Arteriose Monitorate E Loro Associazioni (PAMELA) study. LA enlargement was assessed by measuring LA diameter via echocardiography. LA enlargement was defined as a LA diameter >2.3 cm/m 2 , whereas LVH was defined as a left ventricular mass index ≥114 g/m 2 and 99 g/m 2 in men and women, respectively. Death certificates and hospital diagnoses were collected over an average 148 months follow-up. During follow-up, there were 175 deaths (of which 59 for cardiovascular causes) and 139 cardiovascular fatal and nonfatal events. Compared with subjects with neither LA enlargement nor LVH, subjects with isolated LA enlargement exhibited a significant increase in the adjusted risk of combined fatal and nonfatal cardiovascular events (hazard ratio, 2.0; confidence interval, 1-4.1; P=0.04), although not of cardiovascular death or all-cause death. The adjusted (for baseline covariates, including ambulatory blood pressure) risk of fatal and nonfatal cardiovascular events, cardiovascular death, and all-cause death was significantly increased also in subjects with isolated LVH (hazard ratio, 2.2, 3.4, 2.1, respectively; P=0.001 for all), whereas no further increase was seen in subjects with both LA and left ventricular abnormalities. Thus, like LVH, LA enlargement is an independent long-term predictor of cardiovascular events. The cardiovascular risk, however, is not further increased when LA enlargement is superimposed on an increase