Organic acid disorders and disorders of fatty acid oxidation
Steve Hannigan in Inherited Metabolic Diseases: A Guide to 100 Conditions, 2018
Symptoms of this condition may become apparent at any time from birth to 10 years of age, but usually appear during infancy or early childhood. The main symptoms include weakening of the heart muscle, which usually leads to enlargement of the lower chambers (dilated cardiomyopathy). There may also be thickening of the heart with overgrowth of fibrous and elastic tissues (endocardial fibroelastosis), or dilation of just part of the heart (left ventricular non-compaction). The dilation and thickening of the heart leads to a reduced ability to pump blood around the body (cardiac failure), and can cause breathlessness and fatigue on exertion, bluish discoloration of the skin (cyanosis) and irregular heartbeats (arrhythmias). Symptoms of heart failure are usually worst during infancy, but may improve and then deteriorate again during puberty. Arrhythmias are particularly common during the teenage years. There may be reduced levels of muscle tone (hypotonia) and muscle weakness, as well as a delay in physical development and growth, with ‘failure to thrive’ and short stature (although boys continue to grow late, and consequently often end up well grown). Some children may show falls in their blood sugar level (hypoglycaemia). Many children have food cravings, especially for savoury foods such as cheese, pickles and crisps. There are frequently low levels of the white blood cells known as neutrophils, which help to fight infections. This leads to a weakened immune system, and afected individuals become prone to infections and mouth ulcers.
Clinical studies on Shengmai San
Kam-Ming Ko in Shengmai San, 2002
Since there is no curative treatment for dilated cardiomyopathy, symptomatic treatment is mainly used at the present time. While the majority of cases of dilated cardiomyopathy were not diagnosed until the initial strike of heart failure, the treatment regimen is generally similar to that of treating heart failure, with the aims being to 1) relieve symptoms and thereby improve the quality of living; and 2) to protect the myocardium in order to prolong the survival time. For mild cases of heart failure, the treatment is mainly focused on protecting the myocardium, while the amelioration of symptoms and protection of the myocardium are more critically needed in severe cases of heart failure. If all these treatments remain ineffective, cardiac transplantation would be required.
Elements of Case Analysis
Julie Dickinson, Anne Meyer, Karen J. Huff, Deborah A. Wipf, Elizabeth K. Zorn, Kathy G. Ferrell, Lisa Mancuso, Marjorie Berg Pugatch, Joanne Walker, Karen Wilkinson in Legal Nurse Consulting Principles and Practices, 2019
Analysis: This case fact pattern describes a claim that would garner consideration for further investigation. Symptoms that do not respond as expected or worsen under treatment always require further evaluation. Unusual fatigue or new onset shortness of breath, particularly in women, should raise the suspicion of heart disease. Regarding causation, idiopathic dilated cardiomyopathy can be reversed or stabilized with proper treatment in the majority of cases. Disease progression will occur in a small minority of patients, whose hope for long-term survival rests with heart transplantation. The epidemiology of idiopathic dilated cardiomyopathy suggests that, with earlier diagnosis and treatment, this young woman more likely than not could have avoided transplantation and possibly reversed her disease. The delay in diagnosis left her with heart transplantation as her only viable treatment option.
CPR Induced Inappropriate Shocks from a Subcutaneous Implantable Cardioverter Defibrillator during Out-of-Hospital Cardiac Arrest
Published in Prehospital Emergency Care, 2020
Patrik Cmorej, Eva Smrzova, David Peran, Tana Bulikova
We present the case of a 30-year-old man who in 2006 underwent a heart transplant for terminal heart failure due to dilated cardiomyopathy. Twelve years after transplant the patient was placed on the waiting list for a second heart transplant. Given his high risk of sudden death, he underwent pre-transplant implantation of an S-ICD (EMBLEM MRI S-ICD, Boston Scientific) with a Conditional Shock Zone programed at 200 beats/min and a Shock Zone programed at 230 beats/min. The Shock Zone determines the heart rate threshold, which once reached triggers shock delivery based solely on the heart rate. In the Conditional Shock Zone, the device uses other elements to differentiate whether a shock is truly needed. Apart from the heart rate, these mainly involve the morphology of the QRS complexes (4).
Total artificial heart implantation as a bridge to transplantation: a viable model for the future?
Published in Expert Review of Medical Devices, 2018
Rachel A. Beaupré, Oscar Howard Frazier, Jeffrey A. Morgan
Indications for TAH implantation are numerous and varied with the most common being known or high projected risk of biventricular failure. The overwhelming majority of TAH patients present in cardiogenic shock, a physiology corresponding to a greater than two-fold risk for severe right ventricular failure. Over 80% of the adult TAH patient group present with high risk of right heart failure [3]. In patients under the age of 19, cardiomyopathy comprises 61% of the indications, with dilated cardiomyopathy being the most common [4]. These patients demonstrate much higher rates of multi-system organ failure, pre-operative need for dialysis, poor pre-operative hepatic and nutritional function, and higher rates of tricuspid regurgitation: patient characteristics all associated with right heart dysfunction after LVAD support. Therefore, this patient population is composed of poor candidates for single ventricle support due to their underlying physiology.
Analysis of the influence of modelling assumptions on the prediction of the elastic properties of cardiac fibres
Published in Computer Methods in Biomechanics and Biomedical Engineering, 2018
Jacobo Córdova Aquino, Hugo I. Medellín-Castillo
On the other hand, Taber et al. (1996) studied the mechanics of ventricular torsion considering a compressible cylinder model and an incompressible ellipsoid. Their results showed that the magnitude of the ventricular twist is sensitive to the ventricular geometry, the fiber architecture, and the compressibility and contractility of the myocardium. Azhari et al. (1999) used five analytically defined shapes (a cone, a sphere, a cylinder, a truncated ellipsoid, and the discrete cosine transform of the normal LV shape) to characterize the geometric properties of the LV shape of 10 healthy volunteers and nine pathological hearts. The results revealed that conicity is an important feature of the LV geometry, and it is the most prominent shape difference between normal and abnormal hearts. More recently, van Dalen et al. (2010) studied the influence of the cardiac shape on the left ventricular twist in the normal and dilated human heart. The study comprised 45 dilated cardiomyopathy patients and 60 healthy volunteers. They concluded that the LV apical rotation and twist are significantly influenced by the LV configuration and shape.
Related Knowledge Centers
- Arrhythmia
- Cardiomegaly
- Chest Pain
- Edema
- Shortness of Breath
- Syncope
- Blood
- Heart Failure
- Shortness of Breath
- Valvular Heart Disease
- Genetics