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Churg-Strauss Syndrome
Churg-Strauss
syndrome is a rare disease characterised by generalised inflammation of
small to medium blood vessels in the body. The syndrome was described by 2
doctors ‘Churg and Strauss’ in 1951.
Age at onset - varies from 15-70 years, with mean age being third to
fourth decade.
Sex - Males are affected slightly more frequently than females
Increased levels of certain cells in the blood, which are known as
eosinophils are seen. The etiology of the syndrome remains unknown and
thought to be autoimmune in nature (meaning that antibodies are produced
against ones own body cells). The organs that are usually affected are
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Lungs
- asthma, sinusitis and rhinitis
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Joints
- Arthritis
-
Skin -
Nodules and rash
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Heart
- Myocarditis
(inflammation of hear muscle), Pericarditis (inflammation of covering of
the heart), involvement of coronary arteries (arteries which supply blood
to heart muscle)
Symptoms related to heart are
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Shortness of breath
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Chest pain
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Palpitations (awareness of
ones own heart beat)
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Syncope
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Swelling of legs due to
collection of water
-
Very rarely can present as
sudden cardiac death due to rhythm disturbances or even myocardial
infarction (heart attack)
The most common cause of
death is from heart involvement in the form of myocardial infarction or
heart failure.
Treatment
The main stay of treatment is with use of
steroids either orally as tablets or as injections into veins depending on
the severity of the condition. In most of the cases steroids are adequate,
in complicated case and in patients who do not respond to steroids more
powerful and toxic drugs are used like Cyclophosphamide.
Patients should be under the
care of specialists depending on the organs involved like cardiologists when
heart is involves etc.
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Right
Bundle Branch Block (RBBB)
What
is Right Bundle Branch Block?
Every normal heart is gifted with a natural in-built
electrical system. The electrical impulse generated from this system results
in beating of the heart. The electrical system divides into 2 branches
(called right and left bundle) at the level of the ventricle (bottom
chambers of the heart).
The right bundle stimulates the right ventricle into action
and the left bundle stimulates the left ventricle. If there is blockage in
any of the branches, it results in the delayed activation of the supplied
ventricle. This is reflected on the 12-lead ECG (electrical tracing of the
heart), which is interpreted as right or left bundle branch block.
How common
is the right bundle branch block?
Right
bundle branch block (RBBB) is not an uncommon finding in the general
population. The prevalence increases with age, more commonly seen in elderly
individuals. RBBB is also not an uncommon finding in young people especially
athletes. The prevalence of the condition in young, middle age and elderly
individuals is believed to be 0.2%, 0.7% and 11.3% respectively
What is
the significance of RBBB on an ECG?
RBBB is
usually incidental finding on the ECG, which would have been carried out for
another reason. However in the presence of symptoms like chest pain or
shortness of breath or syncope, it might signify underlying heart or lung
disorders such as
- Long
standing right heart failure
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Myocardial infarction (heart attack)
-
Occasionally congenital heart conditions (hole in the heart)
- Long
standing lung conditions affecting right side of the heart
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Pulmonary embolism (clot in the lung)
RBBB seen in elderly
individuals without heart problem could be due to the degenerative changes
of the right bundle as a part of normal ageing process.
Does RBBB
need further investigations and treatment?
As said
before, RBBB can be an incidental finding. If there are no symptoms
associated, there is no need for further investigations and treatment. If
associated with any of the above-mentioned symptoms, then needs further
evaluation in the form of echocardiography (ultrasound scan of the heart)
and treatment of the conditions diagnosed.
So we
suggest that without any of the above symptoms, RBBB is not routinely
investigated.
On the other hand left
bundle branch block (LBBB) on the ECG almost always suggest underlying heart
conditions, which needs to be further investigated.
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Kawasaki Disease
What is Kawasaki disease?
Kawasaki disease is a
children's illness. It's also known as Kawasaki syndrome or mucocutaneous (mu"ko-ku-TA'ne-us)
lymph node syndrome. It and acute rheumatic (roo-MAT'ik) fever are the two
leading causes of acquired heart disease in children in the United States.
Who gets Kawasaki disease?
About 80 percent of the
people with Kawasaki disease are under age five. Children over age eight are
rarely affected. The disease occurs more often among boys (over 60 percent)
and among those of Asian ancestry. But it can occur in every racial and
ethnic group. Over 4,000 cases of Kawasaki disease are being diagnosed
annually in the United States. Less than 1 percent of those who get it die.
What happens to those with
Kawasaki disease?
The symptoms of Kawasaki disease include...
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fever
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rash
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swollen hands and feet
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irritation and redness
of the whites of the eyes
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swollen lymph glands in
the neck
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irritation and
inflammation of the mouth, lips and throat
What Causes Kawasaki
disease?
Doctors don't know what
causes Kawasaki disease, but it doesn't seem to be hereditary or contagious.
Scientists who've studied it think the evidence strongly suggests it's
caused by an infectious agent such as a virus. It's very rare for more than
one child in a family to develop Kawasaki disease. Less than 2 percent of
children have another attack of Kawasaki disease.
In as many as 15 to
25 percent of the children with Kawasaki disease, the heart is affected. The
coronary arteries or the heart muscle itself can be damaged.
How does Kawasaki
disease affect the heart?
The coronary arteries are
most often affected. Part of a coronary wall can be weakened and balloon
(bulge out) in an aneurysm. A blood clot can form in this weakened area and
block the artery, sometimes leading to a heart attack. The aneurysm can also
burst, but this rarely happens.
Other changes include
inflammation of the heart muscle (myocarditis) or the sac surrounding the
heart (pericarditis). Arrhythmias (abnormal heart rhythms) or abnormal
functioning of some heart valves also can occur.
Usually all the heart
problems go away in five or six weeks, and there's no lasting damage.
Sometimes coronary artery damage persists, however.
An arrhythmia or damaged
heart muscle can be detected using an electrocardiogram (ECG). An
echocardiogram (or "echo") is used to look for possible damage to the heart
or coronary arteries.
How is Kawasaki disease
treated?
Even though the cause of
Kawasaki disease is unknown, certain medicines are known to help. Aspirin is
often used to reduce fever, rash, joint inflammation and pain, and to help
prevent blood clots from forming. Another medicine, intravenous gamma
globulin, can decrease the risk of developing coronary artery abnormalities
when given early in the illness.
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Patent Foramen
Ovale (PFO)
What is a Patent Foramen
Ovale (PFO) and what causes it?
A Patent Foramen Ovale
(or PFO for short) is a flap between the top 2 chambers of the
heart which has not closed the way it should do at birth. During a baby’s
development in the womb, this flap is fully open as a hole called the
Foramen Ovale, and is found in everyone. It is necessary to allow blood
containing oxygen from the placenta to get the rest of the body, bypassing
the lungs which are not yet working. However when a baby is born, the lungs
start working and providing oxygen-rich blood to the heart; pressures inside
the heart change; and the Foramen Ovale (which is now no longer required),
usually closes within the first 2 years of life in 70-80% of people.
However in the remainder of
people, the Foramen Ovale does not close, and it is then known as a
Patent Foramen Ovale (PFO). There is some evidence that genetics
may play a role in keeping a Foramen Ovale open, but generally PFOs
are not thought to be inherited unless they are associated with other heart
conditions.
How common is a PFO?
Studies have shown that a PFO
is a relatively common finding in adult populations, being present in up to
30% of individuals. However, most people with a PFO do not even know they
have it, and it is usually found by chance on investigations for other
problems or during cardiac screening.
What symptoms can you get
with a PFO?
Most people with a PFO don’t
even know that they have it as it causes very few symptoms. Rarely in a
child or young person, blue skin may develop, particularly when they strain
(e.g. during crying) due to blood without oxygen mixing with oxygen-rich
blood from the lungs. However, usually in this scenario, other heart
abnormalities are also present. In some cases, a PFO may be associated with
stroke or migraine; this is discussed below.
What problems can occur
with PFOs? Can PFOs cause Sudden Arrhythmic Death Syndrome (SADS)?
In most people, a PFO will
not cause any problems and they are not associated with sudden death or SADS.
There are some associations, however, with other conditions, and PFOs have
been linked with both stroke and migraine. Although an association of PFO
with stroke is well established, there is no conclusive evidence from
population-based studies that a PFO alone is associated with an increased
risk of a first or recurrent stroke. Therefore no preventive treatment is
recommended in individuals with an incidental finding of a
PFO. In young people who have had a stroke and
found to have a PFO but no other obvious cause (i.e. “cryptogenic” stroke),
treatment may be indicated and this is discussed below.
Similarly, despite an
association with migraines, evidence for this is conflicting and studies
have not shown any benefit in PFO closure for preventing migraine attacks.
PFOs have been associated
with decompression sickness from scuba diving given the pressure differences
that occur in the blood and chest wall with deep sea diving. Therefore for
this reason, the only lifestyle advice recommended to people with a PFO is
to avoid deep sea diving.
How is a PFO diagnosed?
A PFO is generally diagnosed
on an ultrasound scan of the heart called an echocardiogram, where a
probe with special gel on it is connected to an ultrasound machine and
passed along the chest wall. This shows the heart and its structures using
sound waves, and can detect blood flow through different parts of the heart.
However sometimes, this type of echocardiogram may be suggestive of a PFO
but not conclusive. One of the things a PFO can look like is an Atrial
Septal Defect (ASD), which is a hole between the top 2 chambers
of the heart. Therefore in order to clarify this and assess the heart
further, 2 special types of echocardiogram may be needed:
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Bubble Contrast
Echocardiography: this is exactly the same as an ordinary
echocardiogram, but in addition a small amount of agitated salt water is
injected into a vein. This water forms small micro-bubbles which
highlight the chambers of the heart, and can be seen crossing from one
chamber to the other if a PFO is present. During the study, you may be
asked to strain in order to increase the pressures in the heart and
determine whether a potential PFO is present which opens when pressures
in your chest increase (e.g. when coughing or sneezing).
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Sometimes, even a Bubble
Contrast Echocardiogram may be inconclusive, or may show a PFO which
needs further evaluation to determine its size and structure. In this
instance, another special echocardiogram called a Transoesophageal
Echocardiogram may be needed. This uses the same principles as a
transthoracic echocardiogram (i.e. sound waves) to visualise the heart,
but a special, smaller probe fixed to the end of a tube is passed into
the food pipe to visualise the heart from inside the body. This shows
the heart in much better detail, allowing the PFO to be assessed better.
Given that this test is a little more invasive and can be uncomfortable,
it is usually performed with sedation (a medication which makes you feel
drowsy and relaxed).
How is a PFO treated and
managed?
Generally, no specific
treatment is required for a PFO if found in isolation. However if found in
association with a stroke where no other cause for the stroke has been
established, treatment is recommended. In general, 2 options exist: medical
treatment with Aspirin, or closure of the PFO with a special device.
Current guidelines do not
advocate closure of the PFO over simple medical treatment, although in some
centres closure using a device inserted into the heart from the groin may be
offered to patients after they are given the pros and cons of each treatment
option. Trials comparing closure with a device to medical treatment with
aspirin are still on-going and in the UK, the National Institute of Clinical
Excellence have delayed updating their guidance until these have been
completed and the results from them assessed.
For a PFO found in isolation,
open heart surgery is almost never required.
Is long-term follow-up or
screening of other family members needed?
An incidentally detected PFO
generally requires no long-term follow-up or monitoring, unless the
individual develops symptoms suggestive of a stroke in which case
reassessment and treatment as described above may be indicated.
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