Introduction
Eisenmenger Syndrome (ES) is a heart defect that was first giving the name in 1897 (Fukushima, 2015). This syndrome happens when the birth defect is not treated before the lungs’ arteries become damaged. Eisenmenger Syndrome is named after Victor Eisenmenger a man who had a patient who showed symptoms such as, breathing complications and skin that was turning a bluish color. The autopsy of this patient lead him to discover a ventricular septal defect [VSD] (El-Chami, 2014), that causes a hole in the wall on the right and left ventricular. This is the defect that begins when signaling for pulmonary artery hypertension, which progresses into more advanced stages of ES. This birth defect eventually causes patients to have various
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This syndrome increase blood flow causes the heart to pump blood to the lungs at an increasing rate and destroys the blood vessels in the lungs. Several Heart defects that causes disorder is ventricular septal defect (VSD), atrial septal defect (ASD), Patent ductus arteriosus (PDS), and Atrioventricular canal defect (ACD) (Mayo Clinic,2016) This hole usually causes symptoms that include blue or gray skin pigments, shortness of breath, extreme fatigue, chest pains, racing or skipped heart beats, and dizziness. Other symptoms include coughing up blood, swelling in the abdominal region, and numb and/or enlarged fingers and toes. Some of the way ER syndrome can be diagnosed are Chest X-ray are used for heart and pulmonary artery enlargement. Electrocardiogram (ECG) electrical activity of the heart that help test for heart defect that are caused by ES, Echocardiogram is normally used for listing to sound of the heart during, but during ER testing it helps to see if the patient have a heart defect, Magnetic resonance imaging (MRI) is used to take images of blood vessels and lungs and blood test is use to check blood count, which ES would make it …show more content…
For example, heart and lung transplants. This is a solution if a patient’s hole in the heart cannot be healed or helped with another form treatment. Several different targeted therapies have been done to improve health of ES patients. Disease-targeting therapies have proven to be successful in Idiopathic Pulmonary Arterial Hypertension (IPAH) and have been analyzed to in their effectiveness against ES. Prostanoid therapy in patients with ES has also shown the possibility that therapy may improve oxygen saturation, exercise capacity, and even shows a decrease Pulmonary Vascular Resistance (PVR). This is backed by a study with eight patients that have ES, these results were after 3 months of therapy. Another study is being done in a method called Vasodilator therapy. In these case studies, a drug name prostacyclin improved hemodynamics sufficiently enough to ensure ES patients for surgery to repair the cardiac lesion. Other studies have shown that Phosphodiesterase Type-5 Inhibitors, were used in patients with ES. In these studies, observation over a six-month period allowed the researchers to see improvements in oxygen saturation and cardiopulmonary hemodynamics (Beghetti and Galiè, 2009). Research from these studies is also being combined with different medical treatment to target therapies and is it possible to reverse pulmonary vascular
The risk factors that Jessica presented with are a history that is positive for smoking, bronchitis and living in a large urban area with decreased air quality. The symptoms that suggest a pulmonary disorder include a productive cough with discolored sputum, elevated respiratory rate, use of the accessory respiratory muscles during quite breathing, exertional dyspnea, tachycardia and pedal edema. The discolored sputum is indicative of a respiratory infection. The changes in respiratory rate, use of respiratory muscles and exertional dyspnea indicate a pulmonary disorder since there is an increased amount of work required for normal breathing. Tachycardia may arise due to the lack of oxygenated blood available to the tissue stimulating an increase in heart rate. The pedal edema most probably results from decreased systemic blood flow.
Type I and II classic EDS are identifiable by the smooth hyperextensible skin, anomalous wound healing, and joint hypermobility (Malfait F, Wenstrup R, De Paepe A, 2007) (see figure 1). Type III hypermobile EDS is the least drastic type of EDS, musculoskeletal complications may occur. The skin is smooth and slightly hyperextensible, bruising is also common. The hypermobile EDS patient suffers from chronic pain associated with dislocation from a slight amount of trauma (Levy, 2004). Type IV vascular EDS is recognizable by the translucent thin skin, easy bruising, facial manifestation (only present for some EDS patients), and finally by the fragility of the arterial, intestinal and (in some cases) the uterine (Pepin and Byers, 1999). Type VI kyphoscoloitic EDS can be identified at birth from severe muscular hypotonia. The skin is hyperextensible, thin scarring, bruising from minimal trauma, and joint laxity (Yeowell and Steinmann, 2000). Type VII A and B arthrochalasia can be identified by joint hypermobility, as well as fragile skin and tissue deformities. The hypermobile joints lead to severe dislocations and paralyzation...
Ehlers Danlos Syndrome Ehlers-Danlos syndrome (EDS) is a rare inherited group of connective tissue disorders characterized by defects of the major structural protein in the body (collagen). Collagen is a tough fibrous protein that plays an essential role in binding, holding together, strengthening, and providing elasticity to bodily cells and tissues. There are six major types of EDS that I will discuss, however I will only go into a detailed discussion on two of the six types of EDS. The two major types of EDS are Classical EDS and Hypermobile EDS. These two types make up 90% of all EDS cases.
It incorporates dietary advising, exercise training, and mental guiding. There are also numerous surgical treatment alternatives. For example, the removal of nasal polyps that block breathing, oxygen treatment to prevent pulmonary hypertension, endoscopy and lavage to suction mucus from airways, the surgical insertion of a feeding tube may be important to convey supplements while sleeping. In cases where life-threatening lung complexities arise, a lung transplant may be viewed as an option.
P3 – Describe the investigations that are carried out to enable the diagnosis of these physiological disorders
“Hypoplastic left heart syndrome accounts for 9% of all critically ill newborns with congenital cardiac disease, causing the largest number of cardiac deaths in the first year of life.(2) ” HLHS is a severe heart defect that is present at birth. HLHS combines different defects that result in an underdeveloped left side of the heart. This syndrome is one of the most challenging and difficult to manage of all of the congenital heart defects. Multiple portions on the left side of the heart are affected including the left ventricle, the mitral and aortic valve, and the ascending aorta. These structures are greatly reduced in size, or completely nonexistent causing the functionality of the left heart to be reduced, or non-functional all together.
There are four different categories of treatment: lifestyle changes, surgical procedures, non-surgical procedures, and medications. Lifestyle changes include having a healthy diet; increasing physical activity; eliminating cigarettes, alcoholic beverages, and illicit drugs; and getting enough rest and sleep; losing excess weight. These lifestyle changes are to lower the patient’s blood pressure, cholesterol, and reducing any other future medical conditions. There are also surgical options to help cure, prevent, or control cardiomyopathy. Surgical method include a septal myectomy, surgically implanted devices, and a heart transplant. A septal myectomy is used to specifically treat hypertrophy cardiomyopathy which is where the heart muscle cells enlarge and cause the walls of the ventricles to thicken. The thickening of the walls may not affect the size of the ventricles but instead may affect the blood flow out of the ventricle. Usually along with the ventricles swelling, the septum in between the ventricles can become enlarged and block the blood flow causing a heart attack. When medication is not working well to treat hypertrophic cardiomyopathy, a surgeon will open the chest cavity and remove part of the septum that is blocking blood flow. Surgically implanted devices include a pacemaker, a cardiac resynchronization therapy device, a left ventricular assist device, and an implantable
First and foremost, Eisenmenger syndrome was initially described in 1897 when German physician, Victor Eisenmenger, reported on a patient with symptoms of dyspnea and cyanosis from infancy that subsequently developed heart failure (Connolly, 2014). The postmortem description was revealed and a ventricular septal defect was discovered (El-Chami et al., 2014a). With that being said, this had been the first time that the link between a large congenital cardiac shunt defect and the development of pulmonary hypertension had ever been noted (El-Chami et al., 2014b). The normal heart has four chambers. The two upper chambers are separated from each other by the atrial septum (NORD, 2014a). The two lower chambers are known as ventricles and are separated from each other by the ventricular septum (NORD, 2014b).
Plenty of people love the fact that they can enjoy life normal because their brain functions accordingly. Well not every one has the gift of normality. In this harsh world people are dying of all types of diseases and things that are undetectable or even treatable due to the fact that they have unknown origins. Many of the world’s diseases such as HIV, AIDS, some cases of Multiple Sclerosis and Muscular Dystrophies. Among these fatal and life threatening ailments Creutzfeldt-Jakob disease, other wise known as (CJD), takes precedence.
Waardenburg Syndrome is a group of genetic conditions that can lead to hearing loss and changes in the color of hair, skin, and eyes (Genetics 2013). Cases of Waardenburg Syndrome are not very common. There are different types of symptoms of the syndrome. Waardenburg Syndrome can be inherited either on an autosomal dominant pattern or autosomal recessive pattern (Calendar 2013). The ways of diagnosing Waardenburg Syndrome include certain tests to detect the disorder. While Waardenburg Syndrome cannot be cured, treatments can be given to lessen the effects. Like other diseases, Waardenburg Syndrome has certain symptoms, inheritance patterns, diagnosis and treatments.
...a are bronchodilators like anticholinergic, beta agonists, theophylline and oxygen, which are for the advance cases of the disease. In addition, the best treatment for people whom have emphysema is for them to stop smoking.
Hypertrophic cardiomyopathy is an inherited disease that affects the cardiac muscle of the heart, causing the walls of the heart to thicken and become stiff. [1] On a cellular level, the sarcomere increase in size. As a result, the cardiac muscles become abnormally thick, making it difficult for the cells to contract and the heart to pump. A genetic mutation causes the myocytes to form chaotic intersecting bundles. A pathognomonic abnormality called myocardial fiber disarray. [2,12] How the hypertrophy is distributed throughout the heart is varied. Though, in most cases, the left ventricle is always affected. [3] The heart muscle can thicken in four different patterns. The most common being asymmetrical septal hypertrophy without obstruction. Here the intraventricular septum becomes thick, but the mitral valve is not affected. Asymmetrical septal hypertrophy with obstruction causes the mitral valve to touch the septal wall during contraction. (Left ventricle outflow tract obstruction.) The obstruction of the mitral valve allows for blood to slowly flow from the left ventricle back into the left atrium (Mitral regurgitation). Symmetrical hypertrophy is the thickening of the entire left ven...
Oxygen is vital to a beating, healthy heart because it is needed to perform cellular respiration in order to pump the blood. When there is no oxygen being transported to the heart, the heart cannot pump blood, and eventually after being inactive, the heart tissue dies . This results in a heart attack. Heart attacks can be diagnosed before it is too late. One method of diagnosis is through a blood test. Blood tests are capable of sorting through the material found in blood, and if heart cell contents are found in the specimen, a heart attack can be diagnosed. If the levels of the cell contents are higher, this signifies that the heart attack is more severe. Another method of diagnosis is an electrocardiogram (ECG). An ECG measures the rhythm of the heart in order to detect defects. There are many other strategies that are used by doctors in order to diagnose a heart attack, such as chest x-rays, stress tests, tilt table tests, and echocardiograms, to name a few. A myocardial biopsy involves looking at the heart cells, and is accomplished by collecting a sample of heart cells from the patient. If the results are positive, a heart attack is diagnosed. A heart MRI involves looking at images of the heart in order to detect heart failure. The doctor uses the images to look for disorders in the structure of the
Emphysema causes the patient to use more muscles to push the air out of the alveoli which causes barrel chest. Other symptoms that can occur includes shortness of breath and dyspnea that will increase. Circumoral cyanosis and symptoms of right ventricular heart failure can also occur as the disease progresses. Emphysema diagnosis is normally symptomatic, which helps in the proper diagnosis. Emphysema can be diagnosed by patient history and clinical exam, pulmonary function studies and radiologic chest studies also help in the diagnosis. Due to the fact that emphysema is not asymptomatic, the diagnosis is much easier when present in the medical office. Certain signs that doctors look for any breathing difficulties, hypertension, polycythemia, wheezing and barrel chest appearance. Other symptoms that can occur include hepatomegaly, edema, blue-ish discoloration around the mouth and clubbed fingers which encourages treatment options that need to be considered. Drug therapy treatment options for emphysema includes adrenergic sympathomimetic drug or can be used in combination with corticosteroid. Other treatment options include medications for GERD, oxygen therapy and in certain cases surgery could be considered. Treatment methods encourage the patient to avoid any pulmonary irritants, getting their flu shot annually, and pulmonary rehabilitation. Unfortunately, the prognosis for emphysema is very poor. This disease is actually one of the most common causes of death in the U.S.. Prevention of emphysema is basic education about health risk that can occur from long-term smoking. Also parents should make sure to prevent repeated respiratory infection from occurring to limit the
Creutzfeldt-Jakob Disease is an uncommon, deteriorating, consistently fatal brain disorder that is caused by prions. The symptoms of CJD are similar of Alzheimer’s but progress much faster. There are three variations of CJD, sporadic, familial, and acquired. All variations affect the brain the same way and have the same result of death. CJD is an untreatable and incurable disease.