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Dr. Taufik Indrajaya, dr.,SpPD.,K-KV,FINASIM

pons (apneuistic and pneumotaxic)

High BP in the arteries that supply the lungs is called
Pulmonary hypertension (PH) or Pulmonary arterial hypertension (PAH).

The BP measured on arm isnt directly related to the pressure in lungs.

The blood vessels constrict and the walls thicken, cant carry much blood, then pressure builds up and backs up. The heart works harder, trying to force the blood through. If the pressure is high enough, the heart cant keep up, and less blood can circulate through the lungs to pick up oxygen. Pts tired, dizzy and short of breath.

Secondary PH -- If there is a pre-existing disease 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.

So, Its important to repair congenital heart problems (when possible) before permanent PH develop. Intracardiac L-to-R shunts (VSD or ASD) can cause too much blood flow through the lungs, sometimes called Eisenmenger complex.

In the past, the holes cant be repaired due 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.

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 (secondary polycythemia).

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.

Treating PH
Once PH has been diagnosed, often more medical therapy is needed.
These will require regular follow-up with a cardiologist or pulmonologist trained in caring for patients with this condition.

As long as the underlying disease exists it will keep causing PAH. Once have PAH, curing the disease that caused it may not make the high BP go away. In that case, PAH usually need special treatment. While PH is a serious illness, treatments are available.

The treatment are:

oxygen, agents to help heart pump better, diuretics, anticoagulants and medications to lower PAH and reverse the scarring in the blood vessels in the lung.

Sometimes lung transplants also are done for PAH, but in congenital heart disease usually heart and lung transplantation.

A diagnosis of PAH doesnt mean pts cant 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 high altitude.

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.

Most medications often used to treat coexistent 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 with pregnancy and delivery produce changes that can seriously endanger the life of the mother and baby.

Important for women with PH to use a more permanent but safe contraception. Because estrogen can aggravate PH, its important to avoid any contraception containing estrogen. Progesterone forms of contraception are preferable.

Recommended that women with PH have tubal ligation or use the Mirena IUD.

Additional precautions are often taken with PAH 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.

Finally, if has begun medical treatment for PAH, stopping any medicines without physicians approval can be extremely dangerous. Medical therapy has significantly improved the outlook for most patients with PH, but it doesnt cure it. Dont stop medical therapies unless the physician recommends doing so.

Preventing Endocarditis
People with PH and certain underlying congenital heart defects may need AB before certain dental procedures to prevent endocarditis.

Primary or Unexplained PH
AHA Recommendation There is extremely wide variability in the severity of PH among various patients. Evaluating, accurately diagnosing and treating the condition is also very complex. Pts with symptoms suggest PH require thorough evaluation at a medical center with expertise and experience in PH.

Primary PH
Primary or unexplained PH (PPH) is a rare lung disorder in which the BP in the pulmonary artery rises far above normal levels for no apparent reason. The pulmonary artery is a blood vessel carrying oxygen-poor blood from the RV to the lungs. In the lungs, the blood picks up oxygen, then flows to the heart's left side, where the LV pumps it to the rest of the body through the aorta.

HTN is a medical term for abnormally high BP. Normal average ("mean") pulmonary artery pressure is about 14 mm Hg at rest. In PPH, the average is > 25 mmHg at rest and > 30 mmHg during exercise. This abnormally high pressure (PH) is linked with changes in the small blood vessels in the lungs. These changes increase resistance to blood flowing through the vessels. This increased resistance puts a strain on the RV, which now must work harder than usual to move enough blood through the lungs.

Causes of PPH
Don't know the cause. The low incidence makes learning more about the disease extremely difficult. Studies of PPH also have been difficult because a good animal model of the disease hasn't been available.

Thougt that in most people who develop PPH, the blood vessels are very sensitive to certain factors that trigger this disease to develop. For examples :
People with Raynaud's syndrome seem more likely than others to develop PPH. In addition, appetite suppressants, cocaine and HIV are some factors believed to trigger the constriction, or narrowing, of the pulmonary artery.

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, bluish discoloration of the lips and skin, and chest pain more often occur later in the disease. One of the great difficulties in treating PPH is that the diagnosis is often delayed due to the slowly progressive and insidious onset of the symptoms.

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 depends upon a thorough evaluation at a medical center with expertise in PH.

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 is appropriate for a given patient.

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 blood vessels and that no one drug is consistently effective in all patients.

Because individual reactions vary, different drugs have to be tried before chronic or long-term treatment begins. During the course of the disease, the amount and type of medicine also may have to be changed.

To find out which medicine works best for a particular patient, doctors evaluate the drugs during cardiac catheterization.

At present, about one-quarter of patients can be treated with calcium channel-blocking drugs given 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 of all ages. Is sometimes used as a bridge to help pts waiting for a transplant. In other cases it's used for long-term treatment.

Besides oral CCB and chronic IV prostacyclin, clinical trials are under way to evaluate new drugs to improve the treatment of PPH.

Some patients also do well by taking medicines that make the right ventricle's work 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.

Some patients also require supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult. Some need oxygen around the clock. Transplantation (heart-lung or lung) is reserved for pts who don't respond to medical therapy. The decision whether a patient requires heart-lung transplantation or lung transplantation is made after a thorough evaluation at a lung transplantation center.

The long-term prospects for ps with PPH

Despite the complexity of some of the various 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 a household, returning to school and participating in many physical activities.


Is fluid accumulation in the lungs. Impaired gas exchange and may cause respiratory failure. It is due to either failure of the heart to remove fluid from the lung circulation ("cardiogenic pulmonary edema") or a direct injury to the lung parenchyma ("noncardiogenic pulmonary edema"). Treatment depends on the cause, but focuses on maximizing respiratory function and removing the cause.

Acute pulmonary edema

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 main complication of hypoxia.

If pulmonary edema has been developing gradually, symptoms of fluid overload may be elicited. These include nocturia, ankle edema (swelling of 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 paroxysmal nocturnal dyspnea (episodes of severe sudden breathlessness at night).

Is suspected due to findings in the medical history, such as previous cardiovascular disease. Physical examination: End-inspiratory crackles (sounds heard at the end of a deep breath) on auscultation are characteristic for pulmonary edema. The presence of a third heart sound (S3) is predictive of cardiogenic PE. Blood tests are performed for electrolytes (sodium, potassium) and markers of renal function (creatinine, urea). Liver enzymes, inflammatory markers (usually CRP) and a complete blood count as well as coagulation studies (PT, aPTT) are typically requested. B-type natriuretic peptide (BNP) ,low levels of BNP (<100 pg/ml) make a cardiac cause very unlikely.

The diagnosis is confirmed on X-ray of the lungs, which shows increased fluid in the alveolar walls. Kerley B lines, increased vascular filling, pleural effusions, upper lobe diversion (increased blood flow to the higher parts of 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 provide grounds for various forms of treatment. If urgent echocardiography is available, this may strengthen the diagnosis, as well as identify valvular heart disease. In rare occasions, insertion of a SwanGanz catheter may be required to distinguish between the two main forms of pulmonary edema.

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.

Pulmonary edema is either due to direct damage to the tissue or a result of inadequate functioning of the heart or circulatory system. When directly or indirectly caused by increased pulmonary blood pressure, pulmonary edema may appear when this pressure increases 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/slow heartbeat) Hypertensive crisis Pericardial effusion with tamponade Fluid overload, e.g., from kidney failure or intravenous therapy

May occur after upper airway obstruction, intravenous fluid overload, neurogenic causes (seizures, head trauma, strangulation, electrocution). Can also be seen with ARDS (adult respiratory distress syndrome):

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 or lung transplantation Immersion pulmonary edema[5][6] Multiple blood transfusions Severe infection

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]

Therapy Focus is initially on maintaining adequate oxygenation. The patient is given high-flow oxygen, noninvasive ventilation (either continuous positive airway pressure (CPAP) or variable positive airway pressure (VPAP)[11][12]) or mechanical ventilation in extreme cases. When circulatory causes have led to pulmonary edema, treatment with intravenous nitrates (glyceryl trinitrate), and loop diuretics, such as furosemide or bumetanide, is the mainstay of therapy. These improve both preload and afterload, and aid in improving cardiac function. Viagra (Sildenafil) is used as a preventative treatment for altitude-induced pulmonary edema[13][14], although the mechanism of action is not known.[citation needed] While this effect has only recently been discovered, sildenafil is already becoming an accepted treatment for this condition, in particular in situations where the standard treatment of rapid descent has been delayed for some reason.[15]