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HIPERTENSI PULMONAL
& EDEMA PARU
Dr. Taufik Indrajaya, dr.,SpPD.,K-KV,FINASIM
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pons (apneuistic and pneumotaxic)
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Definition
High BP in the arteries that supply the
lungs is called
Pulmonary hypertension (PH) orPulmonary arterial hypertension
(PAH).
The BP measured on arm isnt directly
related to the pressure in lungs.
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The blood vessels constrict and the wallsthicken, cant carry much blood, then
pressure builds up and backs up.
The heart works harder, trying to force theblood through.
If the pressure is high enough, the heart
cant keep up, and less blood can circulatethrough the lungs to pick up oxygen.
Pts tired, dizzy and short of breath.
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Secondary PH -- If there is a pre-existingdisease triggered the PH - a left heart or
lung disorder.
Congenital Heart Disease (VSD or ASD)can cause PH thats similar to PH when
the cause isnt known (idiopathic PAH).
The problem is due to scarring in the
small arteries in the lung.
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So, Its important to repair congenitalheart problems (when possible)
before permanent PH develop.
Intracardiac L-to-R shunts (VSD or
ASD) can cause too much blood flowthrough the lungs, sometimes called
Eisenmenger complex.
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In the past, the holes cant be repaireddue to increased stress on the heart
produced by the high pressures in the
scarred lung blood vessels.
Now, this situation is changing with the
availability of new medicines.
Heart valve conditions, such as MS, can
also cause secondary PH. Fixing the valve
usually reverses the PH.
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Cyanosis due to PH with Congenital HD
In ASD, VSD, AVSD or PDA, blood goes
from the low oxygen chamber or vessel to
the high one. The pts who was once
acyanotic becomes cyanotic.
One way the body compensates is to
increase the ability of the blood to carry
oxygen by increasing the number of RBC
(secondarypolycythemia).
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Symptoms that may prompt to recommend
phlebotomy: headaches, excessive
fatigue and worsening exercise tolerance.
Before this is performed, it is important to
test for iron deficiency.
Phlebotomy is preferably performed in a
center with experience in adult congenital
disease.
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Treating PH
Once PH has been diagnosed, often
more medical therapy is needed.
These will require regular follow-up
with a cardiologist or pulmonologisttrained in caring for patients with this
condition.
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As long as the underlying disease exists -it will keep causing PAH.
Once have PAH, curing the disease thatcaused it may not make the high BP goaway.
In that case, PAH usually need specialtreatment.
While PH is a serious illness, treatmentsare available.
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The treatment are: oxygen,
agents to help heart pump better,
diuretics,
anticoagulants and
medications to lower PAH and reverse thescarring in the blood vessels in the lung.
Sometimes lung transplants also are donefor PAH, but in congenital heart disease -usually heart and lung transplantation.
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A diagnosis of PAH doesnt mean ptscant have an active, fulfilling life.
PAH is a lifelong illness that can be
made worse by a variety of factors,
such as smoking or traveling to highaltitude.
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Once PAH has been diagnosed -- advice
about physical activity, should be as active
as physically possible.
Strenuous physical activity -- is associated
with serious increases in PA pressure.
Avoid isometric exercises and activities like
heavy lifting, or bench press that can
produce dangerous symptoms, such as
chest pain or dizziness.
A supervised cardiopulmonary rehabilitation
program may help promote conditioning.
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Most medications often used to treatcoexistent illnesses are safe for people
with PAH.
Anesthetics or sedatives -- hazardous.Discuss -- decongestant cold medicines.
Pregnancy is not recommended in women
with PH. The changes associated withpregnancy and delivery produce changes
that can seriously endanger the life of the
mother and baby.
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Important for women with PH to use amore permanent but safe contraception.
Because estrogencan aggravate PH, its
important to avoid any contraceptioncontaining estrogen. Progesterone forms
of contraception are preferable.
Recommended that women with PH have
tubal ligation or use the Mirena IUD.
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Additional precautions are often taken withPAH pts. These include :
Supplemental oxygen during air travel,
AB for significant respiratory tract infections, pneumococcal pneumonia vaccine and flu
vaccines.
Also avoid conditions in which the ambient
oxygen concentration may be decreased,
such as high altitude and travel in
unpressurized airplane cabins.
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Finally, if has begun medical treatment forPAH, stopping any medicines withoutphysicians approval can be extremely
dangerous. Medical therapy has significantly improved
the outlook for most patients with PH, butit doesnt cure it.
Dont stop medical therapies unless thephysician recommends doing so.
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Preventing Endocarditis
People with PH and certain
underlying congenital heart defectsmay need AB before certain dental
procedures to prevent endocarditis.
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Primary or Unexplained PH
AHA Recommendation There is extremely wide variability in the
severity of PH among various patients.
Evaluating, accurately diagnosing andtreating the condition is also very complex.
Pts with symptoms suggest PH requirethorough evaluation at a medical center
with expertise and experience in PH.
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Primary PH
Primary or unexplained PH (PPH) is a rarelung disorder in which the BP in thepulmonary artery rises far above normallevels for no apparent reason.
The pulmonary artery is a blood vesselcarrying oxygen-poor blood from the RV to
the lungs. In the lungs, the blood picks upoxygen, then flows to the heart's left side,where the LV pumps it to the rest of thebody through the aorta.
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HTN is a medical term for abnormally high BP. Normal average ("mean") pulmonary artery
pressure is about 14 mm Hgat rest.
In PPH, the average is > 25 mmHg at rest and >
30 mmHg during exercise. This abnormally highpressure (PH) is linked with changes in the smallblood vessels in the lungs.
These changes increase resistance to blood
flowing through the vessels. This increasedresistance puts a strain on the RV, which nowmust work harder than usual to move enoughblood through the lungs.
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Causes of PPH
Don't know the cause.
The low incidence makes learning moreabout the disease extremely difficult.
Studies of PPH also have been difficult
because a good animal model of the
disease hasn't been available.
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Thougt that in most people who developPPH, the blood vessels are very sensitiveto certain factors that trigger this diseaseto develop.
For examples :
People with Raynaud's syndrome seem morelikely than others to develop PPH.
In addition, appetite suppressants, cocaineand HIV are some factors believed to triggerthe constriction, or narrowing, of thepulmonary artery.
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Symptoms of PPH
The first symptom is often fatigue or tiredness. Difficulty in breathing, dizziness and even
fainting spells can occur.
Swelling in the ankles or legs, bluishdiscoloration of the lips and skin, and chest
pain more often occur later in the disease.
One of the great difficulties in treating PPH isthat the diagnosis is often delayed due to the
slowly progressive and insidious onset of the
symptoms.
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Evaluation a patient with PPH
Significant advances in PPH therapy --markedly affected the survival and
quality of life for people with PPH.
The optimal medical and/or surgical
treatment for pts with PPH dependsupon a thorough evaluation at a
medical center with expertise in PH.
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The evaluation includes a right heart
cardiac catheterization.
This is the only way to measure the
pressure in the pulmonary artery and
find out what medical therapy isappropriate for a given patient.
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Therapy for PPH
Choose from a variety of drugs that help
lower BP in the lungs and improve heart
performance in many patients.
Physicians now know that patients with
PPH respond differently to the different
medications that dilate or relax bloodvessels and that no one drug is
consistently effective in all patients.
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Because individual reactions vary,different drugs have to be tried beforechronic or long-term treatment begins.
During the course of the disease, theamount and type of medicine alsomay have to be changed.
To find out which medicine works best fora particular patient, doctors evaluate thedrugs during cardiac catheterization.
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At present, about one-quarter of patients can betreated with calcium channel-blocking drugsgiven orally.
Prostacyclin IV Is a vasodilator, helps pts who don't respond to
CCB given by mouth.
Improves PH and permits increased physical
activity. This improves the quality of life for pts ofall ages.
Is sometimes used as a bridge to help ptswaiting for a transplant. In other cases it's usedfor long-term treatment.
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Besides oral CCB and chronic IVprostacyclin, clinical trials are under way toevaluate new drugs to improve thetreatment of PPH.
Some patients also do well by takingmedicines that make the right ventricle'swork easier. Anticoagulants - decrease the tendency of the blood
to clot, thus permitting the blood to flow more freely.
Diuretics - reduce the amount of fluid in the body,reducing the amount of work the heart has to do.
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Some patients also require supplemental
oxygen delivered through nasal prongs ora mask if breathing becomes difficult.Some need oxygen around the clock.
Transplantation (heart-lung or lung) isreserved for pts who don't respond tomedical therapy. The decision whether a
patient requires heart-lung transplantationor lung transplantation is made after athorough evaluation at a lungtransplantation center.
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The long-term prospects for ps with PPH
Despite the complexity of some of thevarious medical therapies, accurate, early
diagnosis and initiation of treatment have
saved the lives of many pts with PPH. With optimal medical and/or surgical
therapy, pts can often return to a virtually
normal lifestyle, including running ahousehold, returning to school and
participating in many physical activities.
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PULMONARY EDEMA
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Is fluid accumulation in the lungs.
Impaired gas exchange and may causerespiratory failure.
It is due to either failure of the heart to removefluid from the lung circulation ("cardiogenicpulmonary edema") or a direct injury to the lungparenchyma ("noncardiogenic pulmonaryedema").
Treatment depends on the cause, but focuseson maximizing respiratory function and removingthe cause.
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Acute pulmonary edema
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Signs and symptoms
Include difficulty breathing, coughing up
blood, excessive sweating, anxiety, and
pale skin.
A classic sign of pulmonary edema is the
production of pink frothy sputum. If left
untreated, it can lead to coma and even
death, in general, due to its maincomplication ofhypoxia.
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If pulmonary edema has been developing
gradually, symptoms of fluid overload may be
elicited.
These include nocturia, ankle edema (swellingof the legs, in general, of the "pitting" variety,
wherein the skin is slow to return to normal when
pressed upon), orthopnea (inability to lie down
flat due to breathlessness), and paroxysmalnocturnal dyspnea (episodes of severe sudden
breathlessness at night).
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Diagnosis
Is suspected due to findings in the medical history, suchas previous cardiovascular disease.
Physical examination: End-inspiratory crackles (soundsheard at the end of a deep breath) on auscultation arecharacteristic for pulmonary edema. The presence of a
third heart sound (S3) is predictive of cardiogenic PE. Blood tests are performed forelectrolytes (sodium,
potassium) and markers ofrenal function (creatinine,urea). Liver enzymes, inflammatory markers (usually
CRP) and a complete blood count as well as coagulationstudies (PT, aPTT) are typically requested. B-typenatriuretic peptide (BNP) ,low levels of BNP (
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The diagnosis is confirmed on X-ray of the lungs, which
shows increased fluid in the alveolar walls. Kerley Blines, increased vascular filling, pleural effusions, upperlobe diversion (increased blood flow to the higher partsof the lung) may be indicative of cardiogenic PE,whereas patchy alveolar infiltrates with air bronchograms
are more indicative of noncardiogenic edema. Low oxygen saturation and disturbed arterial blood gas
readings may strengthen the diagnosis and providegrounds for various forms of treatment.
If urgent echocardiography is available, this maystrengthen the diagnosis, as well as identify valvularheart disease. In rare occasions, insertion of a Swan-Ganz cathetermay be required to distinguish betweenthe two main forms of pulmonary edema.
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Pulmonary Alveolar Edema. There is extensive, bilateral
airspaces disease with fluid in the
minor fissure (blue arrow) and bilateral pleural effusions
(ref arrows). Although the heart is not
enlarged, the cause was still on a cardiogenic basis.
C
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Causes
Pulmonary edema is either due to direct damage to the tissue or aresult of inadequate functioning of the heart or circulatory system.
When directly or indirectly caused by increased pulmonary bloodpressure, pulmonary edema may appear when this pressureincreases from the normal 15 mmHg[3] to above 25 mmHg.[4]
Cardiogenic:
Congestive heart failure Severe heart attack with left ventricular failure
Severe arrhythmias (tachycardia/fast heartbeat or bradycardia/slowheartbeat)
Hypertensive crisis
Pericardial effusion with tamponade
Fluid overload, e.g., from kidney failure orintravenous therapy
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Non-cardiogenic May occur after upper airway obstruction, intravenous fluid overload,
neurogenic causes (seizures, head trauma, strangulation,electrocution). Can also be seen with ARDS (adult respiratory distresssyndrome):
Alveolar
Inhalation of toxic gases Pulmonary contusion, i.e., high-energy trauma
Aspiration, e.g., gastric fluid or in case of drowning
Reexpansion, i.e. post pneumonectomy or large volume thoracentesis
Reperfusion injury, i.e. postpulmonary thromboendartectomy orlungtransplantation
Immersion pulmonary edema[5][6] Multiple blood transfusions
Severe infection
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Other/unknown
Multitrauma, e.g., severe car accident
Neurogenic, e.g., subarachnoid hemorrhage
Certain types of medication, illicit drug use Upper airway obstruction, i.e. negative pressure
pulmonary edema[7][8]
Arteriovenous malformation
Hantavirus pulmonary syndrome Ascent to high altitude occasionally causes high
altitude pulmonary edema (HAPE)[9][10]
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Therapy Focus is initially on maintaining adequate oxygenation. The patient is given
high-flow oxygen, noninvasive ventilation (eithercontinuous positive airwaypressure (CPAP) orvariable positive airway pressure (VPAP)[11][12]) ormechanical ventilationin extreme cases.
When circulatory causes have led to pulmonary edema, treatment withintravenous nitrates (glyceryl trinitrate), and loop diuretics, such asfurosemide orbumetanide, is the mainstay of therapy. These improve bothpreload and afterload, and aid in improving cardiac function.
Viagra (Sildenafil) is used as a preventative treatment for altitude-inducedpulmonary edema[13][14], although the mechanism of action is notknown.[citation needed] While this effect has only recently been discovered,sildenafil is already becoming an accepted treatment for this condition, inparticular in situations where the standard treatment of rapid descent hasbeen delayed for some reason.[15]
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