Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0264733 (ventricular dilatation)
 2,163 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sodium nitroprusside (SNP) is rarely used in cardiology. It is reserved traditionally for severe episodes of arterial hypertension. Certain states of refractory heart failure represent new indications for use, which implies a double haemodynamic monitoring system: continuous control of systemic blood pressure by intra-arterial catheterization; control of pulmonary pressure and repeated measurements of cardiac output. Prolonged treatment requires continuous biological monitoring of toxicity and careful control of kidney function. As a moderator of blood pressure, SNP is remarkably effective. The hypotensive effect is immediate, readily reversible and generally tachyphylaxis is not observed. The effect of SNP on cardiac work is one of double load reduction: mainly a reduction in afterload or pressure and systemic resistance and a reduction in preload or pressure of ventricular filling. In this respect, SNP can be used effectively for severe cases of heart failure intractable to traditional cardio-stimulatory and diuretic treatments and stemming from diverse causes: acute stage of myocardial infarction, ventricular dilatation, mitral papillary syndrome, heart failure, either subacute or chronic, of various causes. As a rule, the immediate results are positive. Taking the patient off the drug can be difficult and may cause a return to the previous haemodynamic situation.
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PMID:[Use of sodium nitroprusside in cardiology]. 1 64

An 11-year-old girl with congenital myotonic dystrophy and infantile autism was reported. Her mother also suffered from typical myotonic dystrophy. Since her birth, the patient had been floppy, and showed bilateral talipes equinus at 1 year of age. Her subsequent psychomotor and speech development has been retarded. She showed autistic behavior and persistence to the sameness before 2 years old. She was admitted to Sawarabien at the age of 10 years. She could not talk anything but could understand simple, oral messages. Although she had severe degree of mental retardation, her ability for matching figures was relatively well reserved. Her autism was so manifest that it could not be explained by the degree of mental retardation. Neurological examinations revealed that she had facial diplegia, inverted V-shaped mouth, high-arched palate, talipes equinus, percussion myotonia of the tongue, generalized muscular atrophy and weakness, lordosis, areflexia, and congenital cataracta. The serum CPK was slightly elevated. EMG showed a myopathic pattern but did not show any myotonic discharge yet. The brachioradial muscle was biopsied and examined by light- and electron-microscopy. It mainly showed mild varieties of muscle fiber diameter and internal nuclei. Ultrastructurally, irregularly indented central nuclei and perinuclear degeneration of myofibrils associated with secondary lysosomes, lipid droplets and glycogen granules were revealed. Ventricular dilatation and some dysfunction of the brain were also revealed by CT scan and EEG respectively. The present case suggests that congenital myotonic dystrophy can be added into the disease group associated with infantile autism.
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PMID:[A case of congenital myotonic dystrophy with infantile autism]. 278 60

New indications have recently appeared for cardiac pacing with haemodynamic and antiarrhythmic objectives without any symptomatic bradycardia. The best documented indication, though relatively rare, is stimulation of obstructive hypertrophic cardiomyopathy; initially reserved for cases with favorable results of an acute haemodynamic test, it is now used in other cases without this criterion; hypertrophic cardiomyopathy without permanent obstruction, atrial fibrillation or left bundle branch block. The improvement observed during follow-up is always greater as a real remodeling of the myocardium seems to occur with ventricular dilatation and/or septal thinning. However, the position of the atrial, and above all, of the ventricular pacing catheters is critical as is regulation of the pacemaker which should allow complete ventricular capture with an AV delay allowing good filling. The follow-up of these patients must therefore be regular and the effects on longevity are unknown. DDD pacing has also been proposed in dilated cardiomyopathy. The results are contradictory and only very selected cases with left bundle branch block and long PR interval seem justified with, again, optimisation of the pacing sites with high septal or biventricular stimulation. Recurrent atrial tachycardia, special algorithms preventing extrasystoles have been tried with variable results. In cases with inter-atrial block, atrial resynchronisation by bi-atrial stimulation has been assessed with promising results but many technical problems remain unsolved.
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PMID:[New indications for cardiac pacing]. 872 1

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetically determined disease that progresses continuously from conception and throughout life. ARVC/D manifests predominantly in young adulthood. Early identification of the concealed cases in childhood is of utmost importance for the prevention of sudden cardiac death later in life. Magnetic resonance imaging (MRI) is routinely requested in patients with a confirmed or suspected diagnosis of ARVC/D and in family members of the patients with ARVC/D. Although the utility of MRI in the assessment of ARVC/D is well recognized in adults, MRI is a low-yield test in children as the anatomical, histological, and functional changes are frequently subtle or not present in the early phase of the disease. MRI findings of ARVC/D include morphologic changes such as right ventricular dilatation, wall thinning, and aneurismal outpouchings, as well as abnormal tissue characteristics such as myocardial fibrosis and fatty infiltration, and functional abnormalities such as global ventricular dysfunction and regional wall motion abnormalities. Among these findings, regional wall motion abnormalities are the most reliable MRI findings both in children and adults, while myocardial fibrosis and fat infiltration are rarely seen in children. Therefore, an MRI protocol should be tailored according to the patient's age and compliance, as well as the presence of other findings, instead of using the protocol that is used for adults. We propose that MRI in children with ARVC/D should focus on the detection of regional wall motion abnormalities and global ventricular function by using a cine imaging sequence and that the sequences for myocardial fat and late gadolinium enhancement of the myocardium are reserved for those who show abnormal findings at cine imaging. Importantly, MRI should be performed and interpreted by experienced examiners to reduce the number of false positive and false negative readings.
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PMID:Magnetic resonance imaging assessment of arrhythmogenic right ventricular cardiomyopathy/dysplasia in children. 2083 Feb 48

The tricuspid valve has historically received less attention than any other cardiac valve with regard to pathophysiology and surgical management. The tricuspid valve is part of a complex functional apparatus that includes the right atrium, right ventricle, and pulmonary circulation. Functional tricuspid regurgitation is the most common tricuspid valve disease and it occurs secondary to dilatation of tricuspid annulus and/or tethering of valve leaflets from right ventricular dilatation and dysfunction. However, recent expansion in the use of right-sided transvenous devices such as pacemakers and implantable defibrillators has been met with a parallel increase in the risk of organic tricuspid disease. Recent data suggest that tricuspid regurgitation is not benign, and many patients will benefit from intervention at the time of left-sided valve surgery, or early in isolated tricuspid valve disease. This review describes the various surgical options and strategies in the treatment of common tricuspid valve diseases. Current American and European guidelines advocate for a more proactive surgical approach in the treatment of tricuspid regurgitation and/or annular dilatation at the time of left-sided valve surgery. Tricuspid annuloplasty is the preferred technique given its superior long-term outcomes. This renewed interest in surgical repair has been ushered by the development of a newer generation of tricuspid annuloplasty rings, as well as technological strides in transcatheter therapies which now extend to include tricuspid pathologies in otherwise inoperable patients with advanced tricuspid disease and cardiomyopathy. Tricuspid disease is not a benign entity. An aggressive approach to surgical management as well as prophylactic interventions are becoming more widely adopted. Aggressive tricuspid surgery however, remains an area of controversy in cardiac surgery, although surgical repair remains the gold standard for functional tricuspid regurgitation with replacement reserved for advanced or organic tricuspid disease. Transcatheter therapy is primarily directed toward non-surgical patients and is not included in this review.
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PMID:Tricuspid valve surgery: repair and replacement. 2964 93

The decision to administer thrombolysis in submassive pulmonary embolism is undertaken based on risk stratification to prevent further cardiorespiratory deterioration. Although right ventricular dysfunction has been used to risk stratify haemodynamically stable patients with acute pulmonary embolism, there is still much controversy in the use of thrombolysis for its treatment. The European Society of Cardiology guidelines suggest thrombolysis should be reserved for rescue reperfusion. However, we present a unique case of submassive pulmonary embolism in which transthoracic echocardiography visualised dynamic left ventricular outflow tract obstruction secondary to right ventricular dilatation, which led to the decision to instigate thrombolysis therapy. A 68-year-old man presented with submassive pulmonary embolism with evidence of right ventricular dysfunction but was haemodynamically stable. He was initially commenced on anticoagulation but echocardiography revealed significant right ventricular dilatation and left ventricular outflow tract obstruction, signifying a high risk of impending cardiac arrest. After deliberation, full-dose thrombolysis was administered. Subsequently the patient's symptoms and haemodynamics improved significantly and repeat echocardiography demonstrated that the right ventricular and left ventricular size and function had returned to normal. We suggest echocardiography is used to assess right heart, left heart and outflow dynamics to individualise thrombolysis therapy in patients with submassive pulmonary embolism.
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PMID:Thrombolysis for submassive pulmonary embolism with left ventricular outflow tract obstruction. 3205 59