Cardiac/Vascular Nurse Exam Secrets Study Guide (8 page)

In infants, toddler and adolescents, renal vein thrombosis can occur due to severe dehydration, which is considered to be much more serious condition than when it occurs in adults. Other possible causes of renal vein thrombosis include blood clotting disorders such as hypercoagulability disorder, protein C or S deficiency, antiphospholipid antibody syndrome, pregnancy, post-renal transplant, Behçet syndrome, extrinsic compression such as lymph nodes, tumor, retroperitoneal fibrosis, or aortic aneurysm, sickle cell anemia, diabetes that affects the kidney, oral contraceptive use, illicit drug abuse, steroid use or thrombophlebitis migrans.

Symptoms

The symptoms of renal vein thrombosis are minimal, unless the vein becomes occluded suddenly. In patients who present with symptoms of renal vein thrombosis typically present with blood in urine or decreased urine volume upon excretion. Other signs of renal vein thrombosis include protein and/or red blood cells upon urinalysis.

Pulmonary embolism (from embolization of the thrombus) may be a sign that a patient has renal vein thrombosis. With pulmonary embolism, patients present with shortness of breath and chest pain made worse by breathing. Infants, toddlers, and adolescents typically present with lower back/abdominal pain, decreased urine excretion, fever, and blood in the urine.

Diagnosis and screening

Diagnosis of renal vein thrombosis is made by physical examination as well as the use of diagnostic imagining tools. Abdominal CT angiography scan is typically used to confirm the diagnosis of renal vein thrombosis, as physical examination does not always reveal renal vein thrombosis. Other diagnostic tools used for screening of renal vein thrombosis include abdominal MRI scan, X-ray, Doppler ultrasound, or abdominal ultrasound, which may reveal the occluded vein. Urinalysis may also be performed to detect increased protein levels and/or red blood cells. Blood tests may also indicate evidence of renal failure. Blood tests may involve lipid level testing, albumin levels and serum complement levels.

Renal biopsy may also be performed in patients who present with nephrotic syndrome as well.

Atheroembolic renal disease

Atheroembolic renal disease is a condition that involves lipid buildup and clot formation within the small arteries that serve the capillaries of the kidneys. The condition is also known as renal atheroembolism, cholesterol embolization syndrome or atherosclerotic disease. It is a condition that slowly progresses over time and can lead to severe complications such as heart attack, stroke, and brain damage. It is most commonly associated with atherosclerotic disease.

Approximately 25% of patients with renal failure due to atheroembolic renal disease recover renal function. The mortality rate among these patients is approximately 25% to 70%.

Causes and risk factors

Atheroembolic renal disease is strongly associated with atherosclerotic disease, which is very common disorder among patients with cardiovascular disease. The risk factors for atheroembolic renal disease are the same as for atherosclerotic disease.

Factors that can increase an individual’s risk for atherosclerosis include high low-density lipoprotein cholesterol, low high-density lipoprotein, evaluated triglyceride levels, menopause, lack of physical activity, obesity, infection of the vascular smooth muscle cells, high blood pressure, history of smoking and diabetes.

Smoking not only predisposes individuals for atherosclerosis but it increases the progression of the disease. The progression of atherosclerosis itself also further increases the extent and degree of the condition, as further accumulation of lipids, cholesterol, and other substances stimulates the endothelium to produce substances that cause increased plaque buildup.

Symptoms

The symptoms of atheroembolic renal disease include foot pain and/or ulcers, discoloration of feet such as blue or purple feet or toes, claudication, pain in the abdomen, nausea, vomiting, pancreatitis, hepatitis, strokes, blindness, flank pain, blood in urine, decrease or no urine excretion and uncontrolled high blood pressure.

In some patients, renal failure may also occur, which would present with nausea, vomiting, bad taste in mouth, chest pain, high blood pressure, high cholesterol, weight loss, decreased or no urine excretion, swelling, decreased sensation, skin pigmentation changes, dry itchy skin, drowsiness, fatigue, confusion and/or lethargy.

Diagnosis and screening

Physical examination and diagnostic testing are used to diagnose atheroembolic renal disease. Practicing clinicians will look for signs of swelling of the ankles, wrists and/or parts of the body (signs of kidney failure), particles in blood vessels of the retina, abnormal blood flow from the heart, increased blood pressure, as well as skin ulcers on lower feet.

Diagnostic imaging tests used include abdominal CT scan, abdominal MRI, abdominal x-ray and kidney or abdominal ultrasound. Blood work performed may include chem.-7, chem.-20, complete blood count, serum complement levels, and serum lipid levels. More invasive diagnostic tools include kidney biopsy and renal arteriography. Urinalysis may also be performed to look for increases in protein excretion and/or red blood cells.

Marfan syndrome

Marfan syndrome is a connective tissue disorder, affecting the skeleton, lungs, eyes, heart, and major blood vessels. It is a genetic disorder that causes a reduction in fibrillin, leading to decrease in the elasticity and weakening of connective tissue.

Marfan syndrome affects men and women equally as well as individuals of all races and ethnic groups. In the United States, approximately 60,000 individuals have been diagnosed with Marfan syndrome. Most individuals with Marfan syndrome have a family history of disease. However, approximately 15% to 30% of individuals diagnosed with the disease have new genetic mutations.

Causes and risk factors

Marfan syndrome is a genetic disorder that results from a defect in the gene that enables the production of fibrillin. In this syndrome, the defect leads to lower production of fibrillin, which leads to weakened connective tissue in the skeletal system, aorta, and ligaments. The greatest risk factor for the condition is a parent with the disease.

Marfan syndrome is an autosomal dominant condition, which means that only one parent needs to have the gene for the condition to pass it on to off spring. Offspring have a 50% chance of inheriting the disease from one parent diagnosed with the condition.

Symptoms

Some patients with Marfan syndrome present with few or no symptoms. Also, patients typically present with varying symptoms dependent on the organ system affected. Symptoms can vary from very mild in some patients to severe in others. The degree and extent of symptoms also varies from patient to patient, which makes diagnosis difficult. However, most patients have common traits and characteristics such as being tall, thin with tapering fingers, chest wall abnormalities, high arched palate, long arms and legs, narrow face, loose joints, curvature of the spine, crowded teeth, flat feet, weakened part of aorta, heart murmur, nearsightedness, glaucoma, cataracts, detached retina, stretch marks, hernia, painful abdomen, weakened legs, collapsed lung, stiff air sacs, and snoring.

Diagnosis and screening

Physical examination and medical history are key in diagnosing Marfan syndrome. There are no key diagnostic tools solely used to confirm Marfan syndrome. However, patients with Marfan syndrome are typically tall, thin with tapering fingers, narrow face, loose joints, long arms and legs, curvature of the spine, chest wall abnormalities, and eye problems.

Therefore, diagnosis is made by a combination of clinical practitioners including cardiologists, ophthalmologist, orthopedic surgeon, and/or medical geneticist. Diagnostic tools used to diagnose Marfan syndrome include echocardiogram, chest X-ray, MRI and genetic testing. The Ghent criterion is typically used to make a diagnosis of Marfan syndrome.

Ghent criteria

Practicing clinicians use the Ghent criteria to diagnose patients with Marfan syndrome correctly, since the symptoms of the condition resemble those of other connective tissue disorders. The criteria are divided into 2 categories, major and minor. In patients with a family history of Marfan syndrome, they must have 1 major criterion in 1 organ system and involvement of another organ system to be diagnosed with the condition. If patients do not have a family history of the condition, then these individuals need to have 2 major criteria affecting different organs and the involvement of another organ system.

The major criteria for Marfan syndrome include an enlarged aorta, with or without aortic dissection; aortic dissection affecting ascending aorta; dislocation of the lens of an eye; dural ectasia; at least 4 skeletal problems such as chest deformities, long thin arms and legs, flat footedness and scoliosis; family history; and/or having an abnormal gene for Marfan syndrome. Minor criteria occur are symptoms and signs that occur in both patients with the disease as well as in the general population.

Atrial septal defect

Atrial septal defect is a congenital birth defect that involves an abnormality of the atrial chambers of the heart, which causes them not to close appropriately. Smaller defects may close on their own during infancy, toddler years, or early childhood. However, larger defects may cause problems later in life damaging the heart, lung, or other organ systems.

The disease is relatively uncommon and typically difficult to diagnose due to lack of symptoms. The risk of congenital heart disease in the general population is less than 1%. In children of parents with congenital heart disease, the risk of atrial septal defects increases to between 2% and 20%. The condition occurs equally in men and women as well as in race or ethnic descent.

Causes and risk factors

Atrial septal defect is a genetic condition that is congenital. It occurs during fetal circulation, which leads to the formation of a shunt, either left to right shunt or right to left shunt, depending on the nature of the condition. It can occur in combination with other genetic conditions such as Down syndrome. It can occur due to genetic inheritance or mutations during pregnancy or birth.

The following conditions during pregnancy may increase the risk of an offspring having a congenital heart defect such as atrial septal defects including rubella infection, poorly controlled diabetes, and illicit drug and/or alcohol abuse.

Symptoms

Individuals may present with few or no symptoms, making diagnosis rather difficult. However, symptoms may begin to present in infancy through childhood. Yet, some individuals may not present with symptoms until later in life due to presence of other comorbid conditions or progression of the defect. Individuals with small or minor defects may present with no symptoms or present with symptoms later in life.

Symptoms associated with atrial septal defect include frequent respiratory infections in children, difficulty breathing, shortness of breath with activity, fatigue, swelling of legs, feet, or abdomen, and heart palpitations in adults.

Diagnosis and screening

Physical examination and diagnostic tools are used to diagnose atrial septal defect, but diagnosis is difficult in patients who present with few or no symptoms. Practicing clinicians typically use a stethoscope to determine the presence of abnormal heart sounds. Murmur is a quiet ejection murmur heard at the left upper sternal border often with a split second heart sound. Murmur is not usually heard until after 1 year of age. Other diagnostic tools used to determine atrial septal defect include chest x-ray, echocardiography, Doppler ultrasound, transesophageal echocardiography, magnetic resonance imaging, pulse oximetry, and electrocardiogram. A more invasive approach such as cardiac catheterization may also be used.

Patients experiencing poor appetite, failure to gain weight, bluish discoloration of skin, shortness of breath, easy tiring, swelling of legs, abdomen or abdomen, and heart palpitations should seek medical attention.

Types of thromboembolism

The types of thromboembolism include pulmonary embolism and deep vein thrombosis. Pulmonary embolism is a condition that involves the blockage of arteries supplying the lungs. Typically, pulmonary embolism occurs due to the traveling of a blood clot to the lungs from another organ system. Deep venous thrombosis is a condition that involves the formation of blood clots in the veins deep within the body (usually the legs), which can dislodge and embolize into other organ systems such as the lungs, heart and brain. Of note, the superficial femoral vein in the leg is considered a “deep” vein.

In the United States, pulmonary embolism and deep venous thrombosis occur in approximately 2.5% to 5% of adults.

Pulmonary embolism

Pulmonary embolism is a condition that involves blockage of arteries that supplies the lungs. Typically, pulmonary embolism occurs due to the traveling of a blood clot to the lungs from another organ system.

In the United States, the incidence of pulmonary embolism is 0.9 cases per 100,000 population per year. The mortality rate associated with untreated pulmonary embolism in the United States averages between 18% and 30%. The mortality rate upon early diagnosis is approximately 8%.

Pregnant women in their third trimester are at high risk of pulmonary embolism as well as women undergoing treatment with hormone replacement therapy or oral contraceptives. The prevalence of pulmonary embolism increases with age.