Non-pharmacological measures

Pacemaker

Sequential pacemaker implantation is an interesting therapeutic alternative

refractory to medical treatment in symptomatic patients. There is sufficient
evidence that, in selected patients with significant intraventricular gradients,
pacemakers are effective, improving gradients (50%) and symptoms in most
cases.

Septal ablation

This revolutionary technique is to create a

"controlled septal infarction" that could be
compared to a "myectomy percutaneous
chemistry." With this procedure a stroke
(with standard enzyme curve) with
consequent reduction septal akinesia and
intraventricular gradient is induced.

implantable defibrillators

Following studies in patients with ischemic heart disease, the use of implantable
defibrillators as alternative to conventional antiarrhythmic therapy is increasing
exponentially. Thus, patients at risk of sudden death which is able to identify an
arrhythmogenic mechanism are clear candidates for this device.
Surgery

Surgery is an effective alternative and

consolidated over time in patients with
refractory MH (Class III and IV NYHA)
medical treatment (class I
recommendation). These cases probably
represent <5% of patients with MH.
Myotomy-myectomy (Morrow technique) is
the most used and allows reducing or
abolishing intraventricular gradients and reduce the degree of mitral
regurgitation technique. It has been shown that these effects are maintained
long-term and that patients obtained clinical improvement maintained.

Surgical risk increases in elderly patients and those requiring coronary

revascularization associated. On the other hand, the effectiveness of surgery to
prevent sudden death has not been established.

Restrictive cardiomyopathy

Restrictive cardiomyopathy (MR) is defined as a disease of the myocardium /

endocardium producing diastolic dysfunction secondary to increased ventricular
stiffness, with normal or decreased diastolic volumes in one or both ventricles.
Systolic function is often preserved, and the wall thickness conserved or
increased depending on the aetiology.
As a result of these hemodynamic changes, symptoms and signs of left and
right ventricular failure and / or affected by cavities they occur. Often it
dominates the right involvement with increased venous pressure, ascites and
edemas.

syndromic diagnosis

The diagnosis of MR may be suspected when at least two of the following three
criteria:

a) heart failure unexplained

b) echocardiogram suggestive but would essentially track with slightly dilated

ventricles, with preserved systolic function (or only slightly decreased) and signs
of diastolic dysfunction.

c) systemic manifestations that may suggest any entity capable of giving rise to
MR.

Clinical manifestations

Most patients are diagnosed when the disease is well advanced and there are
clear symptoms of ventricular failure. Symptoms include shortness of breath,
paroxysmal nocturnal dyspnea, orthopnea, edema, ascites, tiredness and
fatigue.

Physical exploration

On physical examination patients have the

consequences of elevated filling pressures.
The right side, assessed by the venous
pressure which is remarkably high, with
pronounced xey collapses. Ascites and e
demas are present in advanced cases and
sometimes with painful hepatomegaly and
pulsatile manifest. A auscultation may be
atrioventricular regurgitation murmurs and
third noise.
Supplementary tests

The ECG is almost always abnormal, but very unspecific, describing

repolarization abnormalities, left bundle branch block dominance, AV block,
atrial arrhythmias and low voltage. Chest radiography in the MR is not
characterized, unlike the MD, by a large growth in the size of the cardiac
silhouette. Doppler echocardiography is the fundamental element Bloodless
diagnosis and often provide very useful information from anatomically and
hemodynamically.

Cardiac catheterization in MR provides valuable information to document the

diagnosis, assess the severity, if possible distinguish it from the PC and, in
selected cases, establish the etiology by endomyocardial biopsy.

specific forms

restrictive cardiomyopathy familiar, there are cases of genetic basis, as

described family affectations, sometimes associated with skeletal myopathy
with autosomal dominant inheritance and development in the third or fourth
decade of life, with AV block and muscle weakness.

amyloidosis

Cardiac involvement is most common in

the primary form, caused by the
production of light chain immunoglobulins
by plasma and prob ably related to
multiple myeloma cells.

Amyloidosis, a disease that is rarely

considered in the differential diagnosis of
heart failure with normal systolic function,
may cause conduction disturbances,
ventricular arrhythmias or supra, heart
failure, or a combination of both. The familial form is autosomal dominant
heterozygote, with a risk of transmission to the affected child 50%, and possible
late-onset presentation without systemic manifestations.
sarcoidosis

granulomatous infiltrative disease with

multisystem involvement, lung, skin and the
reticuloendothelial system, preferably. The
almost constant pulmonary infiltration leads
to fibrosis, pulmonary hypertension and
right heart failu re.

The diagnosis should be considered in

relatively young patients with any of the
above clinical manifestations and bilateral hilar lymphadenopathy on chest
radiography.

hemochromatosis

It is characterized by excessive deposition of Fe in several parenchymal (liver,

heart, gonads and pancreas) in relation to:

a) idiopathic or familial involvement

b) hemoglobin synthesis defect

c) chronic liver disease

d) by excessive intake of oral Fe for years.

endomyocardial disease

endomyocardial fibrosis

Preferably occurs in tropical Africa, although

cases have also been reported in India, Brazil,
Colombia and Sri Lanka. It affects both sexes,
more common in children, young adults,
especially black individuals. 50% will have
biventricular heart failure, left 40% pure and
10% pure right. When it affects the right ventricle and a thick dense fibrosis
input tract and apical occurs, with
involvement of chordae and papillary
muscles, with possible th rombosis and
fibrosis obliterative apex distorting the
tricuspid valve.

Löffler endocarditis (hypereosinophilic

syndrome). Hypereosinophilia of any
origin may be associated with
endomyocardial disease. Its pathogenesis
is that more severe eosinophilia 1,500
eosinophils / .mu.l for 6 or more months, allergic conditions, autoimmune (eg,
Churg Strauss syndrome), parasitic, idiopathic or malignant disease (eg
leukosis), cause tissue damage attributable to cytotoxic proteins. These
proteins could be deposited in the myocardium for an extended period of time
with continued tissue damage (necrotic phase) that can affect pericardium,
myocardium and endocardium; It would be continued with another phase
characterized by thrombotic formation of mural thrombi and eventually
supervene, either localized or diffuse fibrotic phase.

Clinical manifestations include weight loss, fever, cough, skin rash and
congestive heart failure in advanced stages (more than half), both right and left.
The cardiomegaly, systolic murmur of mitral and systemic embolism are
common.

Finally, treatment of heart failure and arrhythmias and preventing

thromboembolism, might be considered the standard with few hues, while the
specific will be the medical treatment of each specific entity, with the possibility
of surgery in the case of fibrosis and culminating endomyocardial all heart
transplantation, mainly in cases of refractory heart failure, provided that no
extracardiac systemic disease that compromises the result.

Myocarditis
It is the inflammation of the heart muscle. The condition is calledpediatric
myocarditiswhen it occurs in children.

Causes

Myocarditis is a rare disorder. Almost always it caused by an infection that

affects the heart.

When you have an infection, the immune system produces special cells to fight
disease. If the infection affects the heart, the disease-fighting cells enter the
heart. However, the chemicals produced by these cells can also damage the
heart muscle. As a result, the heart becomes thick, inflamed and weak.

Many cases are caused by a virus that reaches the heart. These may include
influenza virus (flu), coxsackievirus, the parovirus, thecytomegalovirus,
Adenovirus and others.
It can also be caused by bacterial infections such as Lyme disease,
streptococci, mycoplasmas andchlamydia.

Other causes of myocarditis may include:

 Allergic reactions to certain medications, such as certain chemotherapy

drugs

 Exposure to environmental chemicals, such as heavy metals

 Infections due to fungi or parasites

 Radiation

 Autoimmune disorders that cause inflammation throughout the body

symptom

There may be no symptoms. Symptoms may be similar to those of flu. If

present, symptoms may include:

 chest pain that can be likened to aheart attack

  Fatigue and lightheadedness
 Feverand other signs of infection, including headache, muscle aches,
sore throat, diarrhea or rashes
 swelling orJoint pain
 Swelling of the legs
 Pallor, cold hands and feet (poor circulation signal)
 Fast breathing
 rapid heartbeat

Other symptoms that may occur with this disease:

 FaintingOften related to irregular heart rhythms

 Little urine output

Diagnosis

Early diagnosis is key to preventing long-term heart damage. After a physical

examination, your doctor might order one or more tests to confirm that you have
myocarditis and determine its severity. Tests might include:

 Electrocardiogram (ECG). This noninvasive test shows your heart's

electrical patterns and can detect abnormal rhythms.

 Chest X-ray. An X-ray image shows the size and shape of your heart, as
well as whether you have fluid in or around the heart that might indicate
heart failure.

 MRI. Cardiac MRI will show your heart's size, shape and structure. This
test can show signs of inflammation of the heart muscle.

 Echocardiogram. Sound waves create moving images of the beating

heart. An echocardiogram might detect enlargement of your heart, poor
pumping function, valve problems, a clot within the heart or fluid around
your heart.

 Blood tests. These measure white and red blood cell counts, as well as
levels of certain enzymes that indicate damage to your heart muscle.
Blood tests can also detect antibodies against viruses and other organisms
that might indicate a myocarditis-related infection.
  Cardiac catheterization and endomyocardial biopsy. A small tube
(catheter) is inserted into a vein in your leg or neck and threaded into your
heart. In some cases, doctors use a special instrument to remove a tiny
sample of heart muscle tissue (biopsy) for analysis in the lab to check for
inflammation or infection.

Treatment

In many cases, myocarditis improves on its own or with treatment, leading to a

complete recovery. Myocarditis treatment focuses on the cause and the
symptoms, such as heart failure.

In mild cases, persons should avoid competitive sports for at least three to six
months. Rest and medication to help your body fight off the infection causing
myocarditis might be all you need. Although antiviral medications are available,
they haven't proved effective in the treatment of most cases of myocarditis.

Certain rare types of viral myocarditis, such as giant cell and eosinophilic
myocarditis, respond to corticosteroids or other medications to suppress your
immune system. In some cases caused by chronic illnesses, such as lupus,
treatment is directed at the underlying disease.

Drugs to help your heart

If myocarditis is causing heart failure or arrhythmias, your doctor might

hospitalize you and prescribe drugs or other treatments. For certain abnormal
heart rhythms or severe heart failure, you may be given medications to reduce
the risk of blood clots forming in your heart.

If your heart is weak, your doctor might prescribe medications to reduce your
heart's workload or help you eliminate excess fluid, including:

 Angiotensin-converting enzyme (ACE) inhibitors. These medications,

such as enalapril (Vasotec), captopril (Capoten), lisinopril (Zestril, Prinivil)
and ramipril (Altace), relax the blood vessels in your heart and help blood
flow more easily.
  Angiotensin II receptor blockers (ARBs). These medications, such as
losartan (Cozaar) and valsartan (Diovan), relax the blood vessels in your
heart and help blood flow more easily.

 Beta blockers. Beta blockers, such as metoprolol (Lopressor, Toprol-

XL), bisoprolol and carvedilol (Coreg), work in multiple ways to treat heart
failure and help control arrhythmias.

 Diuretics. These medications, such as furosemide (Lasix), relieve

sodium and fluid retention.
Treating severe cases

In some severe cases of myocarditis, aggressive treatment might include:

 Intravenous (IV) medications. These might improve the heart-pumping

function more quickly.

 Ventricular assist devices. Ventricular assist devices (VADs) are

mechanical pumps that help pump blood from the lower chambers of your
heart (the ventricles) to the rest of your body. VADs are used in people
who have weakened hearts or heart failure. This treatment may be used to
allow the heart to recover or while waiting for other treatments, such as a
heart transplant.

 Intra-aortic balloon pump. Doctors insert a thin tube (catheter) in a

blood vessel in your leg and guide it to your heart using X-ray imaging.
Doctors place a balloon attached to the end of the catheter in the main
artery leading out to the body from the heart (aorta). As the balloon inflates
and deflates, it helps to increase blood flow and decrease the workload on
the heart.

 Extracorporeal membrane oxygenation (ECMO). With severe heart

failure, this device can provide oxygen to the body. When blood is
removed from the body, it passes through a special membrane in the
ECMO machine that removes carbon dioxide and adds oxygen to the
blood. The newly oxygenated blood is then returned to the body.

Possible complications

Complications may include:

 cardiomyopathy
 Heart failure
 pericarditis