"You have a big heart." These five words have almost as many implications. Being "big hearted" can describe a generous and giving nature. In a different context, Secretariat's heart was unusually large because he carried an X-linked gene(1) conferring on him rare physiological attributes that in 1973 propelled him to ...

Under normal baseline conditions the unique anatomy, myocardial ultrastructure, and coronary physiology of the right ventricle (RV) reflect a high-volume low-pressure pump. Early work described the RV as a passive conduit with minimal pumping capability. It is now appreciated that through a mechanism of ventricular interdependence, RV systolic function and ...

Ventricular tachycardia as a late complication of blunt chest trauma has never been reported. We present a case of combined endocardial and epicardial delineation of the right ventricular arrhythmogenic substrate, where other causes of cardiomyopathy were excluded. The epicardial scar was more extensive than endocardial scar, and the central isthmus ...

Objective: There is a reciprocal association between major depressive disorder (MDD) and coronary heart disease (CHD). These conditions are linked by a causal network of mechanisms. This causal network should be quantitatively studied and it is hypothesised that the investigation of vagal function represents a promising starting point. Heart rate ...

Cardiovascular diseases are the prime cause of death in the world. The kallikrein-kinin system has been implicated in the pathophysiology of the vascular smooth muscle and cardiac dysfunctions. In recent years, numerous observations obtained from clinical and experimental models of diabetes, hypertension, cardiac failure, ischemia, myocardial infarction and left ventricular ...

Hereditary transthyretin-related amyloidosis remains a widely underdiagnosed condition, owing to its extreme phenotypic variability: the clinical spectrum of the disease ranges from an almost exclusive neurologic involvement to strictly cardiac manifestations. This heterogeneity is linked to several factors including specific transthyretin mutations, geographic distribution and endemic vs. non-endemic aggregation type. ...

Cardiac sarcoid is an infiltrative, granulomatous disease of the myocardium. It is more prevalent entity than once believed, especially subclinical disease. It affects heart mechanics causing ventricular failure, and disrupts the cardiac electrical system leading to third degree heart block, malignant ventricular arrhythmias, and sudden cardiac death. This makes early ...

Mutations involving cardiac ion channels result in abnormal action potential formation or propagation, leading to cardiac arrhythmias. Despite the large impact on society of sudden cardiac death resulting from such arrhythmias, understanding of the underlying cellular mechanism is poor and clinical risk stratification and treatment consequently limited. Basic research using ...

Takayasu's arteritis is an inflammatory vasculitis that primarily affects the aorta and its major branches. Involvement of the thoracic and abdominal aortas, although rare, causes marked hypertension and may lead to severe heart failure. We report the improvement of cardiac function after axillofemoral bypass grafting in a 59-year-old woman who ...

Active contiguous abnormalities can frequently involve the pericardium. Prominent among these are cardiac conditions which encroach on the pericardium, particularly transmural myocardial infarction (newly always with Q-waves). Complications of infarctions, notably myocardial pseudoaneurysm, have one wall which is pericardium. Furthermore, dissecting aneurysm of the aorta and the intramural aortic hemorrhage ...

BACKGROUND: In patients presenting with an acute coronary syndrome, left over right coronary dominance appears to be independently associated with increased long-term mortality. This could lead to decreasing numbers of patients with a left dominant coronary artery system in older age groups. METHODS: We assessed the type of coronary dominance ...

The myocardial extracellular matrix (ECM), an interwoven meshwork of proteins, glycoproteins, proteoglycans, and glycosaminoglycans that is dominated by polymeric fibrils of type I collagen, serves as the mechanical scaffold on which myocytes are arrayed for coordinated and synergistic force transduction. Following ischemic injury, cardiac ECM remodeling is initiated via localized ...

Pediatric sudden cardiac arrest (SCA), which can cause sudden cardiac death if not treated within minutes, has a profound effect on everyone: children, parents, family members, communities, and health care providers. Preventing the tragedy of pediatric SCA, defined as the abrupt and unexpected loss of heart function, remains a concern ...

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is heritable cardiomyopathy that may result in arrhythmia, heart failure, and sudden cardiac death (SCD). Approximately 50-60% of ARVD/C patient will have an identifiable pathogenic mutation in one of seven genes associated with the cardiac desmosome and other cardiac pathways. Genetic counseling remains complicated, however, ...

AimsLimited information is available regarding the relationship between coronary vessel dominance and prognosis. Therefore, the purpose of this study was to determine the prognostic value of coronary vessel dominance in relation to significant coronary artery disease (CAD) in patients referred for computed tomography coronary angiography (CTA).Methods and resultsThe study population ...

Mitral valve competence requires complex interplay between structures that comprise the mitral apparatus - the mitral annulus, mitral valve leaflets, chordae tendineae, papillary muscles, and left atrial and left ventricular myocardium. Myxomatous mitral valve degeneration is prevalent in the canine, and most adult dogs develop some degree of mitral valve ...

Degenerative mitral valve disease (MVD), the most common acquired heart disease in small-sized dogs, is characterized by valvular degeneration resulting in systolic mitral valve regurgitation (MR). Worsening of MR leads to several combined complications including cardiac remodeling, increased left ventricular filling pressure, pulmonary arterial hypertension, and myocardial dysfunction. Conventional two-dimensional, ...

Isolated noncompaction of the left ventricle (LV) is a rare disorder, classified as a primary genetic cardiomyopathy by the American Heart Association. The European Society of Cardiology Working Group on Myocardial and Pericardial Diseases classified LV noncompaction as an unclassified cardiomyopathy. LV noncompaction cardiomyopathy characterized by the following features: 1) ...

Mitral valve prolapse is defined as abnormal bulging of the mitral valve leaflets into the left atrium during ventricular systole. Mitral valve prolapse is a common condition that is a risk factor for mitral regurgitation, congestive heart failure, arrhythmia, and endocarditis. Myxomatous degeneration is the most common cause of mitral ...

PURPOSE OF REVIEW: The presence of tricuspid regurgitation in the setting of right ventricular dysfunction is associated with poor prognosis. The purpose of this review is to describe the pathophysiology of functional tricuspid regurgitation, summarize the current reports favoring a more aggressive approach toward tricuspid valve surgery, and discuss the ...

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by deep intertrabecular recesses communicating with the main ventricular chamber. Cardiac magnetic resonance offers high spatial resolution, and thereby substantial aptitude for the diagnosis of LVNC. Additional clinically relevant information, including thrombus and myocardial fibrosis evaluation, can be readily acquired. These ...

Left ventricular noncompaction (LVNC) is a cardiomyopathy associated with sporadic or familial disease, the latter having an autosomal dominant mode of transmission. The clinical features associated with LVNC vary from asymptomatic to symptomatic patients, with the potential for heart failure, supraventricular and ventricular arrhythmias, thromboembolic events, and sudden cardiac death. ...

Cardiomyopathies are categorized as extrinsic, being caused by external factors, such as hypertension, ischemia, inflammation, valvular dysfunction, or as intrinsic, which correspond to myocardial diseases without identifiable external causes. These so called primary cardiomyopathies can be categorized in four main forms: hypertrophic, dilated, restrictive, and arrhythmogenic right ventricular cardiomyopathy. Cardiomyopathies ...

Heart failure is a clinical syndrome that results when the heart is unable to provide sufficient blood flow to meet metabolic requirements or accommodate systemic venous return. This common condition affects over 5 million people in the United States at a cost of $10-38 billion per year. Heart failure results ...

Russell's viper (Daboia russelli) bites are well known to cause bleeding complications. However, thrombotic complications are rare. We present the case details of a female who was bitten by a Russell's viper (Daboia russelli) in her village. She then developed features of envenomation in the form of hemorrhagic episodes. She ...

Pontine infarcts are often part of a large ischemia involving the brainstem, although infarcts may be restricted to the pons. In both cases, infarcts in the pons are characterized by interesting clinical patterns resulting from a variety of cranial nerve dysfunctions, eye movement disorders and motor, sensory and cerebellar manifestations, ...

Cerebellar infarction accounts for about 3.4% of all ischemic strokes. Cerebellar syndromes are principally characterized by cerebellar symptoms and signs which depend on the involved vascular territories. In the Perugia Stroke Registry, infarct areas have included the superior cerebellar artery region in 36% of patients, the anterior inferior cerebellar artery ...

Border-zone (BZ) and watershed infarcts occur at the junction of two artery territories and are precipitated by a hemodynamic impairment although they cannot be excluded from microembolic etiology. These strokes may often be preceded by specifically precipitating circumstances that induce hypotension and/or hypovolemia (rising from a supine position, exercise, Valsalva's ...

Spinal cord infarctions are rare, especially at the cervical level and in the posterior spinal artery territory. Moreover, this diagnosis is difficult to establish as the clinical picture varies. Even if sensory patterns appear as cardinal signs, their distribution can be very variable. We report a rare case of posterior ...

Ischemia-reperfusion injury is a composite of damage accumulated during reduced perfusion of an organ or tissue and the additional insult sustained during reperfusion. Such injury occurs in a wide variety of clinically important syndromes, such as ischemic heart disease and stroke, which are responsible for a high degree of morbidity ...

Positional vertigo and nystagmus without associated neurological symptoms and signs are characteristic features of benign paroxysmal positional vertigo (BPPV). Although positional nystagmus may occur with caudal cerebellar infarction including the nodulus, positional nystagmus is usually associated with other neurological signs such as spontaneous or gaze-evoked nystagmus, perverted head-shaking nystagmus, cerebellar ...

The aim of this article was to provide a comprehensive review of current knowledge regarding ocular hemodynamic alterations affecting the retinal neuroglial cells and optic nerve head (ONH) function during cardiac surgery. Literature indicates that visual loss after heart surgery is a rare but devastating complication provoked by two main ...

INTRODUCTION: Although hypoplastic left heart syndrome (HLHS) be extensively studied, this disease still has a high mortality rate compared to other diseases treated as univentricular physiology. In this way, morphological differences between phenotypes within the spectrum of HLHS may be risk factors and their identification can assist in choosing treatment ...

BACKGROUND: Bilateral sensory permanent hearing loss (PHL) has been reported after neonatal respiratory failure but has rarely been noted in survivors after cardiac operations. We report the prevalence and severity of PHL after Norwood right ventricular-pulmonary artery shunt for hypoplastic left heart syndrome (HLHS), document progressive loss, and explore markers ...

Background and objectivesData collected by the US Renal Data System (USRDS) identify sudden cardiac death (SCD) as the leading cause of death among hemodialysis patients. However, evidence suggests that clinical events captured on the USRDS death notification form may be inaccurate. A new method for classifying SCD was recently developed ...

Pure motor monoparesis (PMM), an isolated motor deficit that occurs after ischemic stroke (IS) in a single arm or leg without accompanying cranial or sensory dysfunction, is rare and easily misdiagnosed as other causes of weakness. Cortical infarctions of the precentral knob and the anterior cerebral artery territory (for upper ...

Monomorphic and polymorphic Purkinje-related ventricular tachycardias (VTs) may occur in patients with and without underlying structural heart disease. Monomorphic Purkinje-related VTs can be divided into different entities: verapamil-sensitive left fascicular VTs; bundle branch reentry tachycardias (BBRT); interfascicular VTs and focal Purkinje VTs. The most frequent fascicular VT is left posterior ...

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare adrenergically mediated arrhythmogenic disorder classically induced by exercise or emotional stress and found in structurally normal hearts. It is an important cause of cardiac syncope and sudden death in childhood. Catecholaminergic polymorphic ventricular tachycardia is a genetic cardiac channelopathy with known mutations ...

Barth syndrome (OMIM #302060) (BTHS) is an X-linked disorder of lipid metabolism characterized by skeletal myopathy, neutropenia, growth delay, and cardiomyopathy. It is caused by mutations in the tafazzin gene (TAZ), which lead to decreased production of an enzyme required to produce cardiolipin, a component of the inner mitochondrial membrane ...

A 52-year-old woman presented with left ventricular (LV) dysfunction and incessant narrow complex tachycardia. Electrocardiogram and clinical picture were suggestive of a permanent form of junctional reciprocating tachycardia, but electrophysiology study showed the tachycardia to be a fast-slow form of atrioventricular nodal reentrant tachycardia (AVNRT). Slow pathway ablation terminated the ...

One of the unmet clinical needs in heart surgery is the prevention of myocardial stunning and necrosis that occurs as a result of ischemia-reperfusion. Myocardial stunning, a frequent consequence after heart surgery, is characterized by a requirement for postoperative inotropic support despite a technically satisfactory heart operation. In high-risk patients ...

Familial hypertrophic cardiomyopathy (FHC) is an inherited disorder affecting roughly one in 500 people. Its hallmark is abnormal thickening of the ventricular wall, leading to serious complications that include heart failure and sudden cardiac death. Treatment is complicated by variation in the severity, symptoms and risks for sudden death within ...

There are many disorders that may involve the left ventricular (LV) apex; however, they are sometimes difficult to differentiate. In this setting cardiac imaging methods can provide the clue to obtaining the diagnosis. The purpose of this review is to illustrate the spectrum of diseases that most frequently affect the ...

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder. This autosomal dominant condition is defined by left ventricular hypertrophy and associated with functional limitation and premature death. In fact, many individuals are asymptomatic and the annual mortality in most modern series is 1% or less. However, severe symptoms may ...

The paramedian artery arises from P1 segment of posterior cerebral artery and supplies a variable extent of thalamus but usually the dorsomedian, median, internal medullary lamina and the intralaminar nuclei. The typical clinical picture of unilateral paramedian thalamic infarctions consist of arousal and memory disorders, language or visuospatial disorders depending ...

We describe a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM) who later developed cardiac arrhythmias, and briefly discuss the diagnostic modalities, differential diagnosis and treatment option for this condition. AHCM is a rare form of hypertrophic cardiomyopathy which classically involves the apex of the left ventricle. AHCM can be an ...

PURPOSE: To quantify periods of low motion and cross-sectional area changes of the coronary veins during the cardiac cycle for planning magnetic resonance coronary venograms (MRCV). MATERIALS AND METHODS: Images were acquired from 19 patients with coronary artery disease (CAD) and 13 patients scheduled for cardiac resynchronization therapy (CRT). The ...

A general review was made of studies involving: (1) The experimental evidence of remote ischemic preconditioning (RIPC) and relative clinical studies, (2) The experimental and clinical evidences of remote ischemic postconditioning (RIPOC), (3) The potential mechanistic pathways underlying their protective effects. The data used in this review were mainly from ...

TRPV1 (transient receptor potential cation channel, subfamily V, member 1) is best studied in peripheral sensory neurons as a pain receptor; however TRPV1 is expressed in numerous tissues and cell types including those of the cardiovascular system. TRPV1 expression is upregulated in the hypertrophic heart, and the channel is positioned ...

Paediatric cardiomyopathy and heart failure are distinct but frequently associated conditions, which have a high mortality. Traditional medical therapy has evolved to incorporate newer classes of heart failure drugs, although the evidence to support efficacy in children is limited. This perspective article discusses the rationale, benefits and limitations of the ...