5 Stroke Cases Not to Miss

Editor's note: In the following slideshow, Dr. Helmi Lutsep presents her perspective on 5 brief stroke cases representing actual patient encounters. Neuroimaging findings are presented along with recommended management approaches based on up-to-date evidence and clinical trial findings.

Patient 1

This patient is a woman in her early 50s who presented with recurrent episodes of right arm weakness. Cerebral angiogram revealed a high-grade calcified stenosis of the proximal right internal carotid artery.

Patient 1 (cont)

Cerebral angiogram shows a widely patent right internal carotid artery after stenting. Because the patient had symptomatic high-grade stenosis, she was enrolled in the CREST trial and was randomly assigned to the stenting arm. She has had no recurrent symptoms over the 6 years since the stent was placed. Although CREST did not show an interaction with the primary endpoint (including periprocedural events plus ipsilateral strokes up to 4 years) for sex, a preplanned analysis of the periprocedural endpoint alone showed more outcome events for stenting than for carotid endarterectomy (CEA) in women but not in men. The sex of the patient is one potential risk factor that must be weighed when selecting the most appropriate treatment for a patient.

Patient 2

This computed tomography angiogram (CTA) shows a high-grade stenosis of the proximal right middle cerebral artery. The patient is a woman in her 70s, who presented with left face and arm weakness.

Patient 2 (cont)

Cerebral angiography confirms the high-grade right M1 stenosis. The patient was enrolled in the SAMMPRIS trial and was randomly assigned to intensive medical therapy alone. She is receiving aggressive treatment to keep her blood pressure less than 140/90 mm Hg and she is being treated with rosuvastatin to lower her low-density lipoprotein level to less than 70 mg/dL. She has had no recurrent events over the 2 years since she was enrolled. The SAMMPRIS trial revealed that aggressive medical management was superior to percutaneous transluminal angioplasty and stenting with the use of the Wingspan stent system. This patient's management course would not have changed outside of the trial. 

Patient 3

Slurred speech, poor balance, and difficulty picking up his right leg developed suddenly in a 62-year-old man. He had right hand and foot sensory symptoms 7 years before that, as well as slowing of his thought processes over that time. He only had rare headaches. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) was confirmed by genetic testing. This diffusion-weighted MRI reveals an area of diffusion restriction within the left corona radiate consistent with a new stroke.

Patient 3 (cont)

Fluid-attenuated inversion recovery (FLAIR) MRI shows hyperintensity that involves the external capsule. The patient also had some anterior temporal pole involvement that is often seen in patients with CADASIL.

Patient 3 (cont)

This MRI shows diffuse periventricular hyperintensities typically found in patients with CADASIL. Although it has not been assessed in randomized clinical trials, the use of an antiplatelet agent and risk factor modification are generally recommended for stroke prevention in patients with CADASIL. Counseling and therapies for migraine prophylaxis and mood disorders may also be helpful.

Patient 4

Left face and arm numbness and tingling and incoordination of the left arm developed in a 57-year-old woman. This recurred 3 times over a period of 12 hours and finally persisted. The diffusion-weighted image revealed a right thalamic infarct. She received risk factor management and aspirin. Symptoms that stutter are a common feature of lacunar strokes. For ongoing stroke prevention, such patients should receive a single antiplatelet agent. The data safety monitoring board for the SPS3 trial in patients with lacunar strokes terminated the antiplatelet combination therapy portion of the trial because of futility and the occurrence of more bleeding events in patients treated with clopidogrel plus aspirin than in those on aspirin alone.

Patient 5

The patient is a 40-year-old woman with previously normal development in whom holocephalic headache, behavioral changes, and paraphasias developed. MRI obtained 4 days after symptom onset demonstrated an abnormality in the right temporal lobe seen best on T2-weighted imaging (shown). There was no diffusion restriction or enhancement with contrast.

Patient 5 (cont)

Three weeks later, the patient began experiencing auditory hallucinations of washing machine noises and seizures with left hand shaking. The T2-weighted MRI revealed that the lesion was now less apparent in the anterior right temporal lobe but had progressed to other brain areas. Although she improved initially, 6 months later headaches, aphasia, and disinhibition developed. The patient was diagnosed with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) after genetic testing. L-arginine may decrease the frequency and severity of the stroke-like episodes; however, other treatments remain unproven and the course of MELAS is progressive.

Tweet

Important Notice

Please, everybody try to comment here. Also, let us know, if you know any special things or share your views with us. Do you get any information of scholarships or admission news let us know at dr.rahulmallick@gmail.com  / rm0001de@yahoo.com , please. I think, everybody will try your best.


Tweet

Nasal polyps

Nasal polyps — Comprehensive overview covers symptoms, causes, treatment of these noncancerous nasal growths.

Definition

Nasal polyps are soft, painless, noncancerous growths on the lining of your nasal passages or sinuses. They hang down like teardrops or grapes. They result from chronic inflammation due to asthma, recurring infection, allergies, drug sensitivity or certain immune disorders.
Small nasal polyps may not cause symptoms. Larger growths or groups of nasal polyps can block your nasal passages or lead to breathing problems, a lost sense of smell, and frequent infections.
Nasal polyps can affect anyone, but they're more common in adults. Medications can often shrink or eliminate nasal polyps, but surgery is sometimes needed to remove them. Even after successful treatment, nasal polyps often return.

Symptoms

Nasal polyps are associated with inflammation of the lining of your nasal passages and sinuses that lasts more than 12 weeks (chronic rhinosinusitis, also known as chronic sinusitis). However, it's possible — and even somewhat more likely — to have chronic sinusitis without nasal polyps.

Nasal polyps themselves are soft and lack sensation, so if they're small you may not be aware you have them. Multiple growths or a large polyp may block your nasal passages and sinuses.

Common signs and symptoms of chronic sinusitis with nasal polyps include:

• A runny nose
• Persistent stuffiness
• Postnasal drip
• Decreased or absent sense of smell
• Loss of sense of taste
• Facial pain or headache
• Pain in your upper teeth
• A sense of pressure over your forehead and face
• Snoring
• Itching around your eyes

Causes

Scientists don't yet fully understand what causes nasal polyps. It's not clear why some people develop chronic inflammation or why ongoing inflammation triggers polyp formation in some people and not in others. The inflammation occurs in the fluid-producing lining (mucous membrane) of your nose and sinuses. There's some evidence that people who develop polyps have a different immune system response and different chemical markers in their mucous membranes than do those who don't develop polyps.
Nasal polyps can form at any age, but they're most common in young and middle-aged adults. Nasal polyps may form anywhere in your sinuses or nasal passages, but they appear most often in an area where sinuses near your eyes, nose and cheekbones all drain through winding passages into your nose (ostiomeatal complex).

Risk factors

Any condition that triggers chronic inflammation in your nasal passages or sinuses, such as infections or allergies, may increase your risk of developing nasal polyps. Conditions often associated with nasal polyps include:

• Asthma, a disease that causes overall airway inflammation and constriction
• An allergy-like response to aspirin or to pain relievers such as ibuprofen (Advil, Motrin, others) and naproxen (Aleve)
• Allergic fungal sinusitis, an allergy to airborne fungi
• Cystic fibrosis, a genetic disorder that results in the production and secretion of abnormally thick, sticky fluids, including thick mucus from nasal and sinus membranes
• Churg-Strauss syndrome, a rare disease that causes the inflammation of blood vessels

Your family history also may play a role. There's some evidence that certain genetic variations associated with immune system function make you more likely to develop nasal polyps.

Complications

Nasal polyps can cause complications because they block normal airflow and fluid drainage, and also because of the chronic inflammation underlying their development. Potential complications include:

• Obstructive sleep apnea. In this potentially serious condition, you stop and start breathing frequently during sleep.
• Asthma flare-ups. Chronic rhinosinusitis can trigger asthma attacks.
• Sinus infections. Nasal polyps can make you more susceptible to sinus infections that recur often or become chronic.
• Spread of infection to your eye socket. If an infection spreads to your eye socket, it can cause swelling or bulging of your eye, inability to move your eye, reduced vision or even blindness that can become permanent.
• Meningitis. Infection can also spread to the membranes and fluid surrounding your brain and spinal cord.
• Aneurysms or blood clots. Infection can cause problems in the veins surrounding the sinuses, interfering with the blood supply to parts of your brain and putting you at risk of a stroke.

Tests and diagnosis

Your doctor can usually make a diagnosis based on your answers to questions about your symptoms, a general physical exam and an examination of your nose. Polyps may be visible with the aid of a simple lighted instrument.
Other diagnostic tests include:

• Nasal endoscopy. A nasal endoscope, a narrow, flexible tube with a lighted magnifying lens or tiny camera, enables your doctor to perform a detailed examination inside your nose and sinuses. He or she inserts the endoscope into a nostril and guides it into your nasal cavity.
• Imaging studies. Images obtained with computerized tomography (CT) or magnetic resonance imaging (MRI) can help your doctor pinpoint the size and location of polyps in deeper areas of your sinuses and evaluate the extent of inflammation. These studies may also help your doctor rule out the presence of other possible obstructions in your nasal cavity, such as structural abnormalities or another type of cancerous or noncancerous growth.
• Allergy tests. Your doctor may suggest skin tests to determine if allergies are contributing to chronic inflammation. With a skin prick test, tiny drops of allergy-causing agents (allergens) are pricked into the skin of your forearm or upper back. The drops are left on your skin for 15 minutes before your doctor or nurse observes your skin for signs of allergic reactions. If a skin test can't be performed, your doctor may order a blood test that screens for specific antibodies to various allergens.
• Test for cystic fibrosis. If you have a young child diagnosed with nasal polyps, your doctor may suggest testing for cystic fibrosis, an inherited condition affecting the glands that produce mucus, tears, sweat, saliva and digestive juices. The standard diagnostic test for cystic fibrosis is a noninvasive sweat test, which determines whether your child's perspiration is saltier than most people's sweat.

Treatments and drugs

Chronic sinusitis, with or without polyps, is a challenging condition to clear up completely. You'll work with your health care team to develop the best long-term treatment plan to manage your symptoms and to treat factors, such as allergies, that may contribute to chronic inflammation.
The treatment goal for nasal polyps is to reduce their size or eliminate them. Medications are usually the first approach. Surgery may sometimes be needed, but it may not provide a permanent solution because polyps tend to recur.
Medications
Nasal polyp treatment usually starts with drugs, which can make even large polyps shrink or disappear. Drug treatments may include:

• Nasal corticosteroids. Your doctor is likely to prescribe a corticosteroid nasal spray to reduce inflammation. This treatment may shrink the polyps or eliminate them completely. Nasal corticosteroids include fluticasone (Flonase, Veramyst), budesonide (Rhinocort), flunisolide, mometasone (Nasonex), triamcinolone (Nasacort) and beclomethasone (Beconase AQ).
• Oral and injectable corticosteroids. If a nasal corticosteroid isn't effective, your doctor may prescribe an oral corticosteroid, such as prednisone, either alone or in combination with a nasal spray. Because oral corticosteroids can cause serious side effects, you usually take them only for a brief period. If your polyps don't respond to nasal and oral corticosteroids, your doctor may recommend injecting a corticosteroid directly into your polyps.
• Other medications. Your doctor may prescribe drugs to treat conditions that contribute to chronic inflammation in your sinuses or nasal passages. These may include antihistamines to treat allergies and antibiotics to treat a chronic or recurring infection.

Surgery
If drug treatment doesn't shrink or eliminate nasal polyps, your doctor may recommend surgery. The type of surgery depends on the size, number and location of your polyps and the extent of inflammation. Surgery options for nasal polyps include:

• Polypectomy. Small or isolated polyps can often be completely removed using a small mechanical suction device or a microdebrider — an instrument that cuts and extracts soft tissue. This procedure, called a polypectomy, is performed on an outpatient basis.
• Endoscopic sinus surgery. You may need surgery to remove polyps and to correct problems with your sinuses that make them prone to inflammation and polyp development. The surgeon inserts an endoscope, a small tube with a magnifying lens or tiny camera, into your nostrils and guides it into your sinus cavities. He or she uses tiny instruments to remove polyps and other obstructions that block the flow of fluids from your sinuses. Your surgeon may also enlarge the openings leading from your sinuses to your nasal passages. Endoscopic surgery is usually performed as an outpatient procedure.

After surgery, you'll likely use a corticosteroid nasal spray to help prevent the recurrence of nasal polyps. Your doctor may also recommend the use of a saltwater (saline) rinse to promote healing after surgery.

Prevention

You may help reduce your chances of developing nasal polyps or having nasal polyps recur after treatment with the following strategies:

• Manage allergies and asthma. Follow your doctor's treatment recommendations for managing asthma and allergies. If your symptoms aren't well controlled, talk to your doctor about changing your treatment plan.
• Avoid nasal irritants. As much as possible, avoid breathing airborne substances that are likely to contribute to inflammation or irritation of your nose and sinuses, such as allergens, tobacco smoke, chemical fumes, and dust and fine debris.
• Practice good hygiene. Wash your hands regularly and thoroughly. This is one of the best ways to protect against bacterial and viral infections that can cause inflammation of the nasal passages and sinuses.
• Humidify your home. Use a humidifier if the air in your home tends to be dry. This may help moisten your breathing passages, improve the flow of mucus from your sinuses, and help prevent blockage and inflammation.
• Use a nasal rinse or nasal lavage. Use a saltwater (saline) spray or nasal lavage to rinse your nasal passages. This may improve mucus flow and remove allergens and other irritants. You can purchase over-the-counter saline sprays or nasal lavage kits with devices, such as a neti pot, to administer a rinse. You can make your own saltwater solution by mixing 1/8 teaspoon (0.6 milliliters) of table salt in 8 ounces (about 237 milliliters) of distilled or purified warm water.

Tweet

Bone Density Scan

What is a Bone Density Scan (DXA)?

Bone density scanning, also called dual-energy x-ray absorptiometry (DXA) or bone densitometry, is an enhanced form of x-raytechnology that is used to measure bone loss. DXA is today's established standard for measuring bone mineral density (BMD).

An x-ray (radiograph) is a noninvasive medical test that helps physicians diagnose and treat medical conditions. Imaging with x-rays involves exposing a part of the body to a small dose of ionizing radiation to produce pictures of the inside of the body. X-rays are the oldest and most frequently used form of medical imaging.

DXA is most often performed on the lower spine and hips. In children and some adults, the whole body is sometimes scanned. Peripheral devices that use x-ray or ultrasound are sometimes used to screen for low bone mass. In some communities, a CT scan with special software can also be used to diagnose or monitor low bone mass (QCT). This is accurate but less commonly used than DXA scanning.

What are some common uses of the procedure?

DXA is most often used to diagnose osteoporosis, a condition that often affects women after menopause but may also be found in men. Osteoporosis involves a gradual loss of calcium, as well as structural changes, causing the bones to become thinner, more fragile and more likely to break.

DXA is also effective in tracking the effects of treatment for osteoporosis and other conditions that cause bone loss.

The DXA test can also assess an individual's risk for developing fractures. The risk of fracture is affected by age, body weight, history of prior fracture, family history of osteoporotic fractures and life style issues such as cigarette smoking and excessive alcohol consumption. These factors are taken into consideration when deciding if a patient needs therapy.

Bone density testing is strongly recommended if you:

·         are a post-menopausal woman and not taking estrogen.

·         have a personal or maternal history of hip fracture or smoking.

·         are a post-menopausal woman who is tall (over 5 feet 7 inches) or thin (less than 125 pounds).

·         are a man with clinical conditions associated with bone loss.

·         use medications that are known to cause bone loss, including corticosteroids such as Prednisone, various anti-seizure medications such as Dilantin and certain barbiturates, or high-dose thyroid replacement drugs.

·         have type 1 (formerly called juvenile or insulin-dependent) diabetes, liver disease, kidney disease or a family history of osteoporosis.

·         have high bone turnover, which shows up in the form of excessive collagen in urine samples.

·         have a thyroid condition, such as hyperthyroidism.

·         have a parathyroid condition, such as hyperparathyroidism.

·         have experienced a fracture after only mild trauma.

·         have had x-ray evidence of vertebral fracture or other signs of osteoporosis.

The Lateral Vertebral Assessment (LVA), a low-dose x-ray examination of the spine to screen for vertebral fractures that is performed on the DXA machine, may be recommended for older patients, especially if:

·         they have lost more than an inch of height.

·         have unexplained back pain.

·         if a DXA scan gives borderline readings.

How should I prepare?

On the day of the exam you may eat normally. You should not take calcium supplements for at least 24 hours before your exam.

You should wear loose, comfortable clothing, avoiding garments that have zippers, belts or buttons made of metal. Objects such as keys or wallets that would be in the area being scanned should be removed.

You may be asked to remove some or all of your clothes and to wear a gown during the exam. You may also be asked to remove jewelry, removable dental appliances, eye glasses and any metal objects or clothing that might interfere with the x-ray images.

Inform your physician if you recently had a barium examination or have been injected with a contrast material for a computed tomography (CT) scan or radioisotope scan. You may have to wait 10 to 14 days before undergoing a DXA test.

Women should always inform their physician and x-ray technologist if there is any possibility that they are pregnant. Many imaging tests are not performed during pregnancy so as not to expose the fetus to radiation. If an x-ray is necessary, precautions will be taken to minimize radiation exposure to the baby. 

What does the equipment look like?

There are two types of DXA equipment: a central device and a peripheral device.

Central DXA devices measure bone density in the hip and spine and are usually located in hospitals and medical offices. Central devices have a large, flat table and an "arm" suspended overhead.

Peripheral devices measure bone density in the wrist, heel or finger and are often available in drugstores and on mobile health vans in the community. The pDXA device is much smaller than the Central DXA device, weighing only about 60 pounds. It is a portable box-like structure with a space for the foot or forearm to be placed for imaging. Other portable technologies such as specially designed ultrasound machines, are also sometimes used for screening.

How does the procedure work?

The DXA machine sends a thin, invisible beam of low-dose x-rays with two distinct energy peaks through the bones being examined. One peak is absorbed mainly by soft tissue and the other by bone. The soft tissue amount can be subtracted from the total and what remains is a patient's bone mineral density.

DXA machines feature special software that compute and display the bone density measurements on a computer monitor.

How is the procedure performed?

This examination is usually done on an outpatient basis.

In the Central DXA examination, which measures bone density in the hip and spine, the patient lies on a padded table. An x-ray generator is located below the patient and an imaging device, or detector, is positioned above.

To assess the spine, the patient's legs are supported on a padded box to flatten the pelvis and lower (lumbar) spine. To assess the hip, the patient's foot is placed in a brace that rotates the hip inward. In both cases, the detector is slowly passed over the area, generating images on a computer monitor.

You must hold very still and may be asked to keep from breathing for a few seconds while the x-ray picture is taken to reduce the possibility of a blurred image. The technologist will walk behind a wall or into the next room to activate the x-ray machine.

The peripheral tests are simpler. The finger, hand, forearm or foot is placed in a small device that obtains a bone density reading within a few minutes.

An additional procedure called Lateral Vertebral Assessment (LVA) is now being done at many centers. LVA is a low-dose x-ray examination of the spine to screen for vertebral fractures that is performed on the DXA machine.

The LVA test adds only a few minutes to the DXA procedure.

The DXA bone density test is usually completed within 10 to 30 minutes, depending on the equipment used and the parts of the body being examined.

You will probably be asked to fill out a questionnaire that will help the doctor determine if you have medical conditions or take certain medications that either increase or decrease your risk of a fracture. The World Health Organization has recently released an online survey that combines the DXA results and a few basic questions and can be used to predict 10-year hip fracture risk for post-menopausal women. This will be coming into more use in the next few years.

What will I experience during and after the procedure?

Bone density tests are a quick and painless procedure.

Routine evaluations every two years may be needed to see a significant change in bone mineral density, decrease or increase. Few patients, such as patients on high dose steroid medication, may need follow-up at six months.

Who interprets the results and how will I get them?

A radiologist, a physician specifically trained to supervise and interpret radiology examinations, will analyze the images and send a signed report to your primary care or referring physician, who will discuss the results with you.

DXA scans are also interpreted by other physicians such as rheumatologists and endocrinologists.

Your test results will be in the form of two scores:

T score — This number shows the amount of bone you have compared with a young adult of the same gender with peak bone mass. A score above -1 is considered normal. A score between -1 and -2.5 is classified as osteopenia (low bone mass). A score below -2.5 is defined as osteoporosis. The T score is used to estimate your risk of developing a fracture.

Z score — This number reflects the amount of bone you have compared with other people in your age group and of the same size and gender. If this score is unusually high or low, it may indicate a need for further medical tests.

Small changes may normally be observed between scans due to differences in positioning and usually are not significant.

What are the benefits vs. risks?

Benefits

·         DXA bone densitometry is a simple, quick and noninvasive procedure.

·         No anesthesia is required.

·         The amount of radiation used is extremely small—less than one-tenth the dose of a standard chest x-ray, and less than a day's exposure to natural radiation.

·         DXA bone density testing is the most accurate method available for the diagnosis of osteoporosis and is also considered an accurate estimator of fracture risk.

·         DXA equipment is widely available making DXA bone densitometry testing convenient for patients and physicians alike.

·         No radiation remains in a patient's body after an x-ray examination.

·         X-rays usually have no side effects in the diagnostic range.

Risks

·         There is always a slight chance of cancer from excessive exposure to radiation. However, the benefit of an accurate diagnosis far outweighs the risk.

·         Women should always inform their physician or x-ray technologist if there is any possibility that they are pregnant.  The effective radiation dose for this procedure varies. No complications are expected with the DXA procedure.

A Word About Minimizing Radiation Exposure

Special care is taken during x-ray examinations to use the lowest radiation dose possible while producing the best images for evaluation. National and international radiology protection councils continually review and update the technique standards used by radiology professionals.

State-of-the-art x-ray systems have tightly controlled x-ray beams with significant filtration and dose control methods to minimize stray or scatter radiation. This ensures that those parts of a patient's body not being imaged receive minimal radiation exposure.

What are the limitations of DXA Bone Densitometry?

·         A DXA test cannot predict who will experience a fracture but can provide indications of relative risk.

·         Despite its effectiveness as a method of measuring bone density, DXA is of limited use in people with a spinal deformity or those who have had previous spinal surgery. The presence of vertebral compression fractures or osteoarthritis may interfere with the accuracy of the test; in such instances, CT scans may be more useful.

·         Central DXA devices are more sensitive than pDXA devices but they are also somewhat more expensive.

·         A test done on a peripheral location, such as the heel or wrist, may help predict the risk of fracture in the spine or hip. These tests are not helpful in following response to treatment, however, and if they indicate that drug therapy is needed, a baseline central DXA scan should be obtained.

Tweet

The uses of spinach

1-it is useful for pregnant woman for making R.B.C.because it has a lot of heme.
2- useful for weak people or those who are infected with tuberculosis, it is better to boil it with water and then drink the water after boiling.


Tweet

The uses of Grape

1-for curing anemia.
2-for pregnant woman.
3-making stomach, heart and intestine stronger.
4-for thirsty.
5-activating kidney.
6-for tiredness.
7-making the person more intelligence.

Congenital heart disease

Congenital heart disease refers to a structural or functional anomaly of the heart. Congenital heart disease is categorized into cyanotic and noncyanotic disease. Depending on the type of anomaly, patients with congenital heart disease may present at birth with frank cyanosis, heart failure, or extremis; however, many asymptomatic defects are found incidentally. Congenital heart disease occurs most commonly as an isolated defect, but it can also be a part of genetic syndromes such as Down, Turner, or Noonan. The red arrow highlights a congenital heart disease atrial septal defect (between the right and left atrium), seen on contrast-enhanced CT of the heart.



An infant is brought by his mother to the pediatric cardiologist's office for workup of transient cyanosis noted during periods of breath holding, crying, or the Valsalva maneuver. The child is otherwise healthy and growing normally. The cardiologist notes his examination to be within normal limits. He orders an echocardiogram (shown; LA, left aorta; RA, right aorta).
What is the most likely diagnosis?
A. Ventricular septal defect
B. Patent foramen ovale
C. Pulmonary hypertension
D. Tricuspid atresia



Answer: B. Patent foramen ovale
The foramen ovale is a normal fetal structure that allows oxygenated blood from the placenta to bypass the lungs by passing from the right to left atrium (arrow) and into the systemic circulation. At birth, the acute drop in pulmonary vascular resistance creates a pressure differential between the right and left sides of the heart. The increased pressure in the left heart should shut the thin remnant of the septum primum and prevent further right-to-left blood flow. Most children with patent foramen ovale are asymptomatic; however, cyanosis can occur with episodes of increased pulmonary resistance when blood is forced across the patent foramen ovale rather than into the lungs, as with breath holding, the Valsalva maneuver, and crying.



Diagnosis of patent foramen ovale is made via echocardiography and sometimes a "bubble study" (shown). A bubble study is performed by injecting an agitated mixture of saline and air into the peripheral circulation. In the presence of patent foramen ovale, bubbles can be seen crossing the septal defect (yellow arrow) entering the left atrium. In most instances, no therapy is required. Patients who have experienced paradoxical emboli (most notably a cerebral emboli) may require anticoagulation or closure of the patent foramen ovale; however, this is widely debated. Patients who participate in deep sea scuba diving should consider closure of an asymptomatic patent foramen ovale because they are at increased risk for decompression illness after dives.



A 20-month-old child presents with blue fingers and toes over the past few weeks. He has always been sickly with difficulty feeding. However, since developing blue extremities, he breathes easier and feeds without difficulty. On exam, no respiratory distress is noted, but cyanosis is evident. A holosystolic murmur is heard at the cardiac apex. Bedside hematocrit shows the child to be polycythemic. Chest radiograph shows right ventricular hypertrophy and a dilated pulmonary artery. A transesophageal echocardiogram is obtained (shown; Asc Ao, ascending aorta; PA, pulmonary artery).
What is the most likely cause of this child's cyanosis?
A. Aortic stenosis
B. Pulmonary atresia
C. Eisenmenger syndrome
D. Transposition of the great arteries


Answer: C. Eisenmenger syndrome
Eisenmenger syndrome is a sequela of a chronic left-to-right shunt, most commonly a ventricular septal defect (VSD, arrow). Pulmonary overcirculation occurs from left-to-right shunting via the more efficient pumping of the left ventricle, manifested in an infant as failure to thrive, difficulty feeding, and tachypnea. Increased blood flow induces smooth muscle proliferation of pulmonary capillaries leading to pulmonary hypertension. This induces pulmonary vascular dilation (see previous slide), increased pulmonary resistance, and eventual reversal of the shunt yielding cyanosis. Treatment is difficult once the right-to-left shunt has developed because the pulmonary changes are usually irreversible. (LV, left ventricle; RV, right ventricle)



A 9-year-old girl reports that she cannot keep up during play and that her feet are "always freezing" compared with her hands. On exam, the physician notes a short stature, wide shoulders, and webbing of the neck. Her cardiac exam is without murmur, but the blood pressure in her right arm is 146/79 mm Hg and in the right thigh is 91/45 mm Hg. By palpation, her femoral pulse is delayed compared with her radial artery pulse. The physician orders numerous diagnostic tests including an aortic angiogram (shown).
What is the most likely diagnosis causing the patient's symptoms?
A. Coarctation of the aorta
B. Hypoplastic left heart syndrome
C. Aortic stenosis
D. Patent ductus arteriosus (PDA)



Answer: A. Coarctation of the aorta
Coarctation of the aorta is a constricted aortic segment (red arrows), often occurring just distal to the branch point of the left subclavian artery. Symptoms depend on the degree of impediment to flow and include left ventricular hypertrophy, upper extremity hypertension, acute congestive heart failure, and shock. In neonates with critical limits to blood flow through the aorta, closure of the ductus arteriosus at birth can cause abrupt onset of heart failure and shock. Because aortic outflow is acutely diminished, left heart pressure rises secondary to the elevated afterload and blood begins to engorge the left atrium. Elevated left heart pressures can cause new left-to-right shunting through the foramen ovale



Children with coarctation are often asymptomatic, and diagnosis is commonly incidental after noting differences in blood pressure or pulse between the upper and lower extremities. Children may present with leg pain or weakness with exertion. Chest radiograph may show "rib notching" (arrows) as engorged intercostal collateral arteries notch out portions of the inferior border of the ribs. Coarctation is a common manifestation in Turner syndrome (XO karyotype in girls) as in the previous case. Treatment is surgical; however, medical stabilization and presurgical optimization with prostaglandin E1 to either prevent closure or reopen the ductus arteriosus to improve postcoarctation blood flow has become the mainstay of treatment. Image courtesy of Radiopaedia.



A 32-week gestation premature infant is examined in the nursery and found to have a persistent "machinelike" holosystolic murmur heard best over the left sternal border. She has persistent tachycardia and is developing signs of heart failure. An aortogram of her heart is obtained (shown) with dye injected into the descending aorta (PA, pulmonary artery; DAo, descending aorta).
What anomaly is highlighted by the white arrow and is most likely the cause of her symptoms?
A. Aortic stenosis
B. Pulmonary atresia
C. Coarctation of the aorta
D. PDA



Answer: D. PDA
PDA is a persistent fetal connection between the pulmonary artery and the descending aorta. In fetal life, blood is diverted from the pulmonary (high-resistance) to the systemic (low-resistance) system via the ductus. With expansion of the lungs at birth, resistance in the pulmonic system falls below the systemic system and flow passively increases to the lungs. Risk factors for pathologic PDA include prematurity, maternal rubella infection, or exposure to prostaglandins. Symptoms of PDA are based on the degree of blood flow, most commonly a machinelike murmur, but also heart failure due to volume overload. Once recognized, treatment is surgical with ligation of the PDA. Image courtesy of Wikimedia Commons.



Hypoplastic left heart syndrome is a rare congenital defect caused when the left side of the heart underdevelops, resulting in thickened and fibrous left ventricular walls and a slitlike ventricle (star). The mitral and aortic valves may be completely atretic. In fetal life, a PDA allows retrograde filling of the ascending aorta, the brachiocephalic vessels, and coronary vessels, whereas anterograde flow fills and supplies the descending aorta because left ventricular outflow is not adequate. At birth, children can be at risk for coronary or cerebral ischemia if retrograde flow through the PDA is not sufficient to properly perfuse the myocardium and cerebral tissue.



A 26-month-old child is brought to the pediatrician by his mother, who states that he has episodes after crying or eating when his fingertips, toes, and lips become blue (shown). He has passed out after crying spells, and she notes that he is constantly squatting. When he was in the nursery after birth, the mother was told that the baby had a "heart problem with a murmur" but she never followed up. The child has delayed growth and a systolic murmur.
What is the most likely diagnosis?
A. Coarctation of the aorta
B. Tetralogy of Fallot
C. Congenital mitral valve stenosis
D. Hypoplastic left heart



Answer: B. Tetralogy of Fallot
Tetralogy of Fallot consists of 4 anatomic anomalies: (1) right ventricular outflow tract obstruction, (2) right ventricular hypertrophy (demarcated in yellow), (3) a VSD (red arrow), and (4) an anterior shifted aorta "overriding" the VSD (blue arrow). The right ventricular outflow obstruction can vary from mild stenosis of the pulmonary outflow tract to complete pulmonary atresia. All forms of right ventricular outflow obstruction decrease blood flow to the pulmonary system; increase resistance in the right heart during systole, yielding right ventricular hypertrophy; and increase passage of deoxygenated blood across the VSD to the left heart, aorta, and systemic circulation.



Tetralogy often presents with cyanosis in the neonatal period as the PDA closes. However, some children with either less severe right heart outflow obstruction or sufficient aortopulmonary collaterals tolerate tetralogy for the first few years of life. As these children outgrow the collateral blood supply, they are susceptible to cyanotic episodes. They present with "tet spells" or periods of cyanosis (fingertips, toes, and lips) associated with periods of agitation, as described in the previous case. Children may squat to increase peripheral vascular resistance (afterload), which will reduce the amount of blood freely moving through the VSD and force more blood into the pulmonary vasculature.



A 1-month-old infant presents to the emergency department with peripheral cyanosis, diaphoresis, and tachypnea. On exam, the respiratory rate is 56 breaths per minute and rales are heard throughout the lung fields. High-flow oxygen provides some improvement. ECG shows left atrial and ventricular enlargement, and the chest radiograph shows cardiomegaly; increased pulmonary vascular markings; and a large, right-sided aortic arch. The child is stabilized and admitted where a cardiac MR image is obtained (shown; A, ascending aorta; P, pulmonary trunk).
What is the lesion causing this child's symptoms?
A. Aortic stenosis
B. Hypoplastic right heart
C. Truncus arteriosus
D. Patent foramen ovale



Answer: C. Truncus arteriosus
Truncus arteriosus is a rare congenital heart disease occurring in 5-10 of 100,000 births in the United States. A single great artery (TA, truncus arteriosus) leaves the base of the heart giving rise to the ascending aorta, pulmonary trunk, and coronary arteries. Because of mixing of the outflows of the right (venous) and left heart (oxygenated) blood within the truncus, systemic hypoxia and cyanosis paired with pulmonary vasculature overload and congestive heart failure develop. Early operative repair is the mainstay of treatment. Prenatal diagnosis by ultrasound has allowed for better perinatal surgical planning and increased infant survival (A, aorta; PA, pulmonary artery).



An infant born 10 hours ago suddenly develops profound cyanosis. The prenatal course was significant only for maternal diabetes. The physician suspects a cardiac anomaly, but the cardiac echocadiographic lab is down and instead orders a STAT angiogram (shown). The contrast is injected directly into the right ventricle and the outflow tract is highlighted as above.
What is the most likely cardiac anomaly?
A. Hypoplastic left heart
B. Transposition of the great arteries, with the aorta arising from the right heart and the pulmonary trunk from the left heart
C. Atrial septal defect
D. Coarctation of the aorta



Answer: B. Transposition of the great arteries, with the aorta arising from the right heart and the pulmonary trunk from the left heart
Oxygenated blood enters the left heart and is pumped to the lungs via the abnormal pulmonary trunk, whereas venous blood entering the right heart is pumped back into the systemic circulation via the aberrant aorta, bypassing the lungs. This pattern of flow is incompatible with life and requires immediate surgical repair at birth. In the angiogram shown, contrast injected into the RV travels directly to the ascending aorta (AAo) and then to the subclavian (Sc) and carotid (Car) arteries. Note the paucity of contrast in the pulmonary circulation, as there is no mixing of oxygenated and deoxygenated blood.



The anomaly pictured (large white arrow) is a congenital heart disease almost always accompanied by an atrial septal defect (ASD) (usually a patent foramen ovale), ventricular septal defect, hypertrophied right atrium (RA), and a hypoplastic right ventricle (RV). The left ventricle (LV) is responsible for pumping blood through the pulmonary system (made capable by VSD) and systemic circulation (through the normal aorta). However, blood is chronically desaturated secondary to mixing of venous and oxygenated blood.
What is the most likely defect?
A. Transposition of the great arteries
B. Pulmonary atresia
C. Mitral valve atresia
D. Tricuspid atresia



Answer: D. Tricuspid atresia
The term tricuspid atresia describes several anomalies of the tricuspid valve, including the muscular or fibrous form of valve or the fused leaflet form of valve. Blood returning to the right atrium cannot flow to the right ventricle, and all blood must pass through an ASD (white dotted arrow above) to the left atrium, where it flows through the normal mitral valve and into the left ventricle. In order to reach the lungs, blood must be pumped through a PDA or from the left ventricle across a VSD (pictured) into the right ventricle outflow tract and through the pulmonary valve into the pulmonary circulation. Image courtesy of Wikimedia Commons.



The altered circulation in patients with tricuspid atresia not only creates a mixing of deoxygenated and oxygenated blood within the left heart, but precipitates right atrial hypertrophy in order to pump blood across the ASD (red arrow) into the left heart circulation with resulting right ventricular hypoplasticity (black arrow). Clinical findings include cyanosis, heart failure, and a murmur associated with ASD and VSD (holosystolic crescendo/decrescendo murmur). ECG will show signs of right atrial enlargement (tall P waves) and left-axis deviation secondary to the hypoplasticity of the right ventricle. Treatment is surgical, with a common approach connecting the right atrium to the pulmonary trunk to circulate deoxygenated blood directly to the lungs.



An 8-year-old boy is brought to the emergency department by emergency medical services (EMS) after experiencing syncope during gym class at school. He was found unresponsive and asystolic by EMS. Despite the best resuscitative efforts of the emergency department staff, they were not able to regain a spontaneous pulse. Upon autopsy, the valvular defect pictured above is discovered.
What was the most likely cause of this child's death?
A. Aortic valve stenosis
B. Mitral valve prolapse
C. Pulmonary atresia
D. Tricuspid insufficiency



Answer: A. Aortic valve stenosis
Pediatric aortic stenosis causes varying degrees of obstruction of left ventricular outflow. Most commonly, the normal tricuspid aortic valve arises as a bicuspid valve with a fused junction between leaflets and a displaced/stenotic opening (outlined in yellow). Left ventricular hypertrophy develops, placing children at risk for cardiac ischemia because increases in myocardial oxygen demand during growth spurts or exercise cannot be met by the hypertrophied left heart. Failure to meet oxygen needs results in symptoms of easy fatigability, anginal chest pain, and syncope during exercise. Children are at risk for sudden cardiac death. On exam, a systolic ejection murmur and palpable thrill at the suprasternal notch are common.



Two other related congenital defects are subaortic and supra-aortic valvular stenosis. Subaortic stenosis results from an anatomic obstruction to the left ventricular outflow tract causing turbulence to flow. Repeated trauma against the valve damages leaflets and causes regurgitation. Left ventricular hypertrophy develops to overcome turbulence and the resulting aortic insufficiency. Supravalvular aortic stenosis (red arrows) is a narrowing of the ascending aorta distal to the valve. Stenosis results in increased pressures and dilation within the coronary system, increasing the risk for atherosclerotic disease. Also common is elevated blood pressure in the right upper extremity due to preferential direction of the blood flow through the stenotic portion of aorta into the brachiocephalic artery.


26.