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PULMONARY HYPERTENSION INTRODUCTION BRIEF HISTORY WHAT IS PH? WHAT CAUSES PH? SIGNS AND SYMPTOMS DIAGNOSIS OF PH TREATMENT OF PH INTRODUCTION In the human body, there are two types of circulation that enable distribution of blood throughout the body. The portion that pumps oxygenated blood from the left side of the heart via the left ventricle to all parts of the body is known as the SYSTEMIC CIRCULATION. On the other hand, the portion that pumps deoxygenated blood from the right side of the heart via the right ventricle into the lungs to obtain oxygen is referred to as the PULMONARY CIRCULATION. INTRODUCTION Millions of people are affected by a condition known as high blood pressure (hypertension) whereby the blood travels through the body’s arteries at a pressure higher than normal. PULMONARY HYPERTENSION is a far less common type of high blood pressure that affects specifically the arteries in the lungs. Pressures in the lung arteries are normally significantly lower than the pressures in the systemic circulation. Pulmonary hypertension occurs when the pressure in the pulmonary circulation becomes abnormally elevated. It is a serious condition that becomes progressively worse and eventually proves fatal. INTRODUCTION An estimated of 500 – 1000 new cases are diagnosed annually. There is an incidence of about 2-3 per million per year and a prevalence of 15 per million. This disease can occur in men, women and children of all ages. However, it is most common in females between 20 and 40 years old, with twice as many cases reported in women then men. The condition is rare in children but is sometimes seen in infants born with heart defects. Pulmonary hypertension may be a primary or secondary cause of hypoxia in neonates. H The first reported case – 1891 • E. Romberg, German doctor I S T • published description of a patient who, at autopsy, showed thickening of the pulmonary artery but no heart or lung disease In 1951, 39 cases were reported by Dr. D.T. Dresdale in the United States. The illness received its name. O R Y Between 1967 and 1973, a 10-fold increase in unexplained pulmonary hypertension was reported in central Europe. The rise was subsequently traced to aminorex fumarate, an amphetaminelike drug introduced in Europe in 1965 to control appetite. It was later removed from the market. WHAT IS PULMONARY HYPERTENSION? Ordinarily, blood vessels in the lungs provide less resistance to blood flow than blood vessels in the rest of the body do. Hence, blood pressure is usually much lower in the lungs. While pressure in general circulation is about 120/80 mm Hg, in the pulmonary arteries, it is only around 25/15 mm Hg. Mean (average) pulmonary artery pressure = number between highest and lowest pressures Normal at rest : 14 mm Hg Pulmonary hypertension at rest : 25 mm Hg during exercise : 30 mm Hg Primary Pulmonary Hypertension • no underlying cause for the high blood pressure in lungs • likely to begin with spasm of the muscle layer in pulmonary arteries • patients are rather sensitive to substances that cause blood vessels to constrict • may have an inherited predisposition for the disease Secondary Pulmonary Hypertension • results directly from another medical problem • most probable from diseases that impedes flow of blood through lungs or that causes periods of low oxygen in blood • eg. Chronic Obstructive Pulmonary Disease, scleroderma, sleep apnea, pulmonary fibrosis, lung diseases such as asbestosis Classification I. Classification of PH based on etiology Classification… Abnormally high BP in pulmonary arteries Increased pressure damages large and small pulmonary arteries Blood vessel walls thicken Cannot transfer oxygen and carbon dioxide normally Levels of oxygen in blood fall Constriction of pulmonary arteries Further increase in pressure in pulmonary circulation Pulmonary Hypertension right side of heart must work harder cor pulmonale push blood through pulmonary arteries to lungs right ventricle thickens and enlarges Heart Failure In some people, the bone marrow will produce more red blood cells to compensate for less of oxygen in blood leading Polycythemia Extra RBCs cause the blood to become thicker and stickier, further increasing the load on the heart Pulmonary Embolism Functional Classification A. Class I- Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain, or near syncope. B. Class II- patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnoea or fatigue, chest pain, or near syncope. C. Class III- patients with pulmonary hypertension resulting in marked. Limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea, fatigue, and chest pain or near syncope. D. Class IV -patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. these patients manifest signs of right heart failure. Dyspnoea and /or fatigue may be present even at rest. Discomfort is increased by any physical activity WHAT CAUSES PULMONARY HYPERTENSION? Although the exact cause of primary pulmonary hypertension is unknown, scientists believe that most people who develop the disorder are especially sensitive to substances that cause blood vessels to constrict. Cocaine and fenfluramine (fen-phen), which was withdrawn from the market in 1997, are two of the substances that may contribute to PH in many people. Other people with PH have an inherited predisposition for the disease. In these people, PH is triggered by another medical condition such as chronic liver disease (cirrhosis), AIDS, sickle cell anemia, scleroderma and lupus. Pulmonary hypertension resulting directly from another medical problem is called secondary pulmonary hypertension. Medical conditions that may lead to secondary PH include : blood clots in the lungs (pulmonary emboli) chronic obstructive pulmonary disease such as emphysema connective tissue disorders, such as scleroderma sleep apnea – upper airway obstructed during sleep congenital heart disease obesity with reduced ability to breathe (Pickwickian syndrome) neuromuscular diseases involving respiratory muscles HIV infection lung diseases such as pulmonary fibrosis (causes scarring in the tissue between the lungs’ air sacs) Left-sided heart failure • heart’s left ventricle weakens and cannot pump out enough blood • increase in pressure backs up through pulmonary veins to arteries in lungs High Altitude • above altitude of 8000 feet - may develop PH • low blood oxygen (hypoxemia) • constricts small pulmonary arteries • climb to high elevations without first becoming acclimated • risk of pulmonary edema too – air sacs filled with fluid instead of with air, always associated with PH The overall rise in blood pressure in PH is the end result of a process which begins with changes in the endothelial cells that line the lungs’ arteries. Changes → causes formation of extra tissue → blockage in vessels Scarring (fibrosis) usually also occurs → arteries stiff and narrow These causes increased resistance to blood flow which raises pressure in the pulmonary arteries. Less often, PH is caused by extensive loss of lung tissue from surgery/trauma. Injury to endothelial cells leads to overproduction of endothelin – key cause of blood vessel scarring and spasm & to reduced production of nitric oxide and prostacyclins – 2 key body chemicals which keep blood vessels relaxed and open. The ‘Two-Hit’ Hypothesis According to the hypothesis, vascular abnormalities characteristic of PPH are triggered by accumulation of genetic and/or environmental insults in a susceptible individual. A combination of germline BMPR2 mutation (‘first hit’) and the ingestion of appetite suppressants (‘second hit’) were used to generate the clinical disease. A genetic cause of the familial form of primary PH has been discovered. It is caused by mutations in a gene called BMPR2, as used in the ‘Two-Hit’ Hypothesis. BMPR2 encodes a receptor (transforming growth factor beta type II receptor) that sits on the surface of cells and binds molecules of the TGFbeta superfamily. Binding triggers conformational changes → series of biochemical reactions ↓ affect cell’s behaviour The mutations block this process. Hence, this discovery provides means of genetic diagnosis and a potential target for the therapy with familial and possibly, sporadic primary pulmonary hypertension. SIGNS AND SYMPTOMS Like other forms of high blood pressure (hypertension), the signs and symptoms of pulmonary hypertension are subtle in the early stages of the disease and may not be apparent for months or even years. As the disease progresses, these signs become more noticeable. Also, the symptoms of PH are often hidden by the underlying condition causing the disease. Symptoms, however, tend to vary from patient to patient. The diagnosis of PH are often overlooked by physicians. It is sufficiently common and of such high impact that all patients with scleroderma should be screened for its presence on a regular basis. shortness of breath (dyspnea) Initially, only short of breath when exert oneself physically but eventually may be short of breath most of the time, even when at rest. fatigue or light-headed upon exertion dizziness or fainting spells (syncope) swelling (edema) in ankles, legs and eventually in abdomen (ascites) – fluid leak out of veins and into tissues bluish colour to lips and skin (cyanosis) coughing (sometimes with blood) and wheezing distended neck veins enlarged liver racing pulse or heart palpitations angina-like chest pain feel weak – body tissues not receiving enough oxygen achy joints (often developed years before apparent onset of disorder) Signs and Symptoms in Children The symptoms of PH for children are similar to that of an adult, though children are more likely to experience tiredness, dizziness and breathlessness and for many, fainting is common. fail to put on weight like a normal child slowed growth Children tend to be diagnosed earlier than adults, but just like adults, they are often misdiagnosed several times before a correct diagnosis is made. The commonest misdiagnosis is asthma. Untreated PH in children worsens quicker than the same condition in adults. However, with treatment, children appear to have an overall better prognosis than adults. DIAGNOSIS OF PH It is often very difficult to initially diagnose PH. In fact, there is often a lengthy delay between the time when patients first visit their doctor and the time they receive specialist care at a hospital. Since there is no single test that will tell the healthcare team if a patient has PH, it is important to consider all associated diseases as well as other causes of breathlessness, such as certain lung and heart diseases and blood clots. The ruling out of different diseases that are possible causes of particular symptoms is called the differential diagnosis. A definite diagnosis of PH usually requires passing a tube through a vein in an arm or a leg into the right side of the heart to measure the blood pressure in the right ventricle and the pulmonary artery. For patients with suspected PH, there are several initial steps that are commonly taken to confirm the diagnosis. These are first discussed between the patient and the healthcare team : • history of present illnesses • past medical history • family history • any past or present medications that the patient may have taken A thorough physical examination will also take place. After this, a number of tests may be ordered to aid in assessment and diagnosis of PH. Chest X-Ray Based on the symptoms, a doctor may suspect PH in people who have an underlying lung disorder. A chest x-ray may show that the pulmonary arteries are enlarged. This imaging test offers the physician a picture of the general size, shape and structure of the heart and lungs. One of the things the physician will check is whether the right side of the heart is enlarged. Echocardiography (Ultrasonic Cardiography) This test uses sound waves to track the structure and function of the heart. It can compose images of a beating heart on a monitor and detects: • heart’s thickness, size and function • motion pattern and structure of the four heart valves → revealing any potential leakage (regurgitation) • thickening of right ventricle, enlarged right heart • reversal of blood through tricuspid valve • extent of lung damage A specific echocardiogram, Doppler ultrasound, is sometimes used to determine pulmonary artery pressure. Exercise echocardiogram – determine how well heart works under stress Electrocardiogram (ECG) ECG is a record of the electrical activity produced by the heart. Abnormal rhythms (arrhythmias) may indicate that the heart or part of the heart is undergoing unusual stress. Exercise ECG helps evaluation of performance of the heart during exercise, for example, walking on a treadmill in the examination room. Pulmonary Angiogram Used to measure circulation in the lungs and to visualize clots in the lung on x-rays. The test involves insertion of a thin catheter into the pulmonary artery through which an iodine dye is injected. Image of any blood clots present in the lung can be observed and circulation of blood through lung’s blood vessels can be tracked. Perfusion Lung Scan Uses small amounts of radioactive tracers (radioisotopes) to study blood flow in the lungs. Radioisotopes are attached to radiopharmaceuticals which are then injected into a vein the arm. A gamma camera takes pictures of blood flow in the lungs’ blood vessels. It is generally used to determine whether blood clots may be causing symptoms of PH. Pulmonary Function Tests Non-invasive tests to measure how much air your lungs can hold and the airflow in and out of your lungs. They can also measure the amount of gases exchanged across the membrane between the lung wall and capillary membrane. During the tests, the patient will be asked to blow into a spirometer. An abnormality here may be amongst the first indication of PH. Computerized Tomography (CT) Organs can be scanned in two-dimensional ‘slices’. Split-second computer processing creates images as a series of very thin x-ray beams pass through the body. A contrast medium is used to help visualization. The fast CT machine can scan arteries in less than 20 seconds as opposed to 20 minutes for a standard CT. Speed is important because it allows the dye to be visualized while still in the arteries. Magnetic Resonance Imaging (MRI) Uses no x-rays but instead, a computer creates tissue ‘slices’ from data generated by a powerful magnetic field and radio waves. Although not yet routinely used to diagnose PH, it is showing great value in assessing the pulmonary arteries. It cannot, however, measure artery pressure. Other screening or diagnostic methods exercise testing ventilation-perfusion (V/O) scanning arterial blood gas studies central hematocrit count serum glucose and calcium levels count platelet count hyperoxia (100% oxygen) challenge test TREATMENT OF PH 6 Minute walk Testing In patients with PAH, serial determinations of functional class and exercise capacity assessed by the 6 minute walk test provide benchmarks for disease severity, response to therapy, and progression. ACCP: QOE: good; Benefit: intermediate; SOR: A Treatment Goals in PAH Improved exercise capacity Improved function Improved hemodynamics Improved survival Prevention of clinical worsening Therapy for PAH Functional class II / III / IV General Care Oral anticoagulants ;B for IPAH, E/C for other PAH] diuretics O2 [E/A] digoxin Acute Vasoreactivity Testing [A for IPAH, E/C for other PAH] YES NO Oral CCB [B for IPAH], E/B for other PAH] Sustained Response YES NO Functional Class III Functional Class IV Endothelin Receptor Antagonists (Bosentan) [A] or Chronic IV Epoprostenol [A] or Prostanoid Analogues Chronic IV Epoprostenol [A] Bosentan [B] Treprostinil [B] Chronic IV Iloprost [C] SQ Treprostinil [B], Inh Iloprost [B], Beraprost [I] No improvement or deterioration Continue CCB PDE-5 Inhibitors (Sildenafil) [C] Atrioseptostomy Lung Transplantation Therapy Targets for PAH Humbert M, Sitbon O, Simonneau G. N Engl J Med 2004;351:1425-36 Targeting the Mechanism of PAH Calcium Channel Blockers For patients (20%) with meaningful reduction of PAP associated with reduction of PVR on acute vasoreactivity tests. Nifedipine 240mg/day, 30 mg BID Diltiazem 900mg/day, 60mg TID Limiting factors for dose increase: systemic hypotension and lower limb peripheral edema Pathophysiology in PAH Deficiency of prostaglandin and an excess of thromboxane in PAH patients Christman et al, NEJM 1992 Decreased expression of prostacyclin synthase in lungs from patients with severe PAH Tuder et al, AJRCCM 1999 Epoprostenol PGI2 Potent vasodilator in both the pulmonary and systemic circulation Has antiplatelet aggregatory activity Exogenously administered prostanoid analogues Unstable at acidic pH, not taken orally Very short half life,<6 mins For moderately severe to severe PAH Epoprostenol Requires constant IV administration Kept cold prior to infusion Initial dose: 1 – 2 ng/kg/min Gradually titrating in increments of 1- 2 ng/kg/min, based upon side effects and tolerance to reach a “plateau” between 20 – 40 ng/kg/min Epoprostenol Side effects: Flushing, headache, jaw pain with first bite of food, diarrhea, nausea, blotchy erythematous rash and musculoskeletal pain (legs and feet ) Chronic IV therapy: Line related infections, catheter associated venous thrombosis, thrombocytopenia Treprostinil Stable prostacyclin analogue Half life of 3 hours Stable at room temperature Undergoing research: Inhaled treprostinil Treprostinil Developed for SC delivery Initially 1.25 ng/kg/min up to maximum of 22.5 ng/kg/min Side effects: Headache, diarrhea, nausea, rash, jaw pain, infusion site pain, erythema or induration Iloprost Prostacyclin analogue Stable at room temperature, at pH 7.4 and normal light Serum half-life of 20 – 25 mins For functional class 3 – 4 Iloprost Administered via nebulized aerosol Administered 6 – 9 times a day, each inhalation requires 10 – 15 mins Dose: 2.5 – 5 ug, median inhaled dose of 30 ug/day Side effects: Cough, headache and flushing Beraprost First chemically stable and orally active prostacyclin analogue Peak concentration is reached after 30 minutes and elimination half-life is 35 – 40 minutes after oral administration Median dose of 80 ug PO daily Approved therapy for PAH in Japan Endothelin Leu Met Ser Ser Cys Ser Cys NH2 Asp Lys Glu Cys Val Tyr Phe Cys His Leu Asp Ile Ile Trp CO2 A 21-amino acid peptide released primarily from the endothelium Pathophysiology in PAH Endothelin 1 is a potent vasoconstrictor and SM mitogen Increase in vascular tone and pulmonary vascular hypertrophy 2 distinct endothelin- receptor isoforms: ETA – facilitates vasoconstriction and proliferation of vascular SM cells ETB – involved in clearance of endothelin and cause vasodilatation and NO release Expression of Endothelin in the Lungs of PH Patients Giaid A et al. N Engl J Med. 1993 Jun 17;328(24):1732-9. Bosentan Orally active nonpeptide antagonist of both endothelin receptor subtypes Prevents and even reverses the development of PH, pulmonary vascular remodelling and right ventricular hypertrophy Initial dose of 62.5 mg bid for first 4 weeks and followed by target dose of 125 mg bid Side effects: Hepatotoxicity and teratogenicity Sitaxsentan Selective ETA antagonist Has oral bioavailability and a long duration of action (t 1/2, 5-7h) . Approximately 6,500 fold more selective as antagonist for ETA compared with ETB receptors. Side effects: ↑ INR and prothrombin time due to inhibition of CYP2C9 P450 enzyme Ambrisentan ETA selective antagonist Under research Phosphodiesterase Inhibitors Sildenafil Potent and highly specific PDE5 inhibitor Inhibits degradation of cGMP by phosphodiesterases Prevent rebound pulmonary vasoconstriction after acute withdrawal of inhaled NO Revatio – contains sildenafil same active ingredient used in Viagra blocks the enzyme phosphodiesterase accentuates actions of nitric oxide opens blood vessels in the lungs - dilation side effect - vision problems Anticoagulant warfarin (Coumadin) prevent formation of blood clots within pulmonary arteries risk of bleeding complications – prevent normal blood coagulation periodic blood tests – check how well the drug is working more than 100 drugs can interact with anticoagulants Diuretics water pills eliminate excess fluid from body reduces amount of work heart has to do limit fluid buildup in the lungs improve exchange of gases in lungs Oxygen oxygen therapy especially for those who live in high altitude or have sleep apnea continuous use of oxygen through nasal prongs/oxygen mask relieve shortness of breath Cardiotonics strengthen the contractions of the heart heart does not need to beat as often to circulate adequate blood for body Transplants surgical interventions – considered only in extreme cases treatment for severe secondary PH if treatment of the underlying disorder fails surgically replace damage organs with healthy donated organs lung and/or heart transplantation most common : single-lung transplant, fewer complications than double-lung or heart-lung transplant lung transplant - improvement in structure and functioning of right ventricle major risks : rejection of transplanted organ, serious infection take immunosuppressant drugs for life – help reduce chance of rejection survival rate is about 60% per year and 37% per 5 years Nitric Oxide Inhaled form Acts as direct smooth muscle relaxant via activation of the guanylate cyclase system Short therapeutic half life Ameliorates hypoxemia and lowers PVR by direct pulmonary vasodilatation Anticoagulation Rationale for use is based on the presence of traditional risk factors for VTE, such as heart failure and sedentary lifestyle, thrombophilic predisposition, thrombotic changes in the pulmonary microcirculation and in the elastic pulmonary arteries. Dose adjusted according to INR 2 - 3 Supplemental Oxygen Low mixed venous oxygen saturation caused by low cardiac output and low minimally altered ventilation/ perfusion matching. Improves quality of life and ↓ mortality in patients with PH due to chronic respiratory insufficiency. Surgical Interventions Atrial Septostomy Allow R L shunting to increase systemic output that, in spite of fall in the systemic arterial oxygen saturation, will produce an increase in systemic oxygen transport. Shunt at the atrial level would allow decompression of the RA and RV, alleviating s/s of right heart failure. Considered after short term failure of maximal medical therapy. Heart / Lung Transplantation 1 year survival of 70% 5 year survival of 50% International Society for Heart and Lung Transplantation Registry Effective therapy for patients with end stage pulmonary vascular disease. WHO Classification of Functional Status of Patients with PH Class Description I Patients with PH in whom there is no limitation of usual physical activity; ordinary physical activity does not cause increased dyspnea, fatigue, chest pain, or presyncope. II Patients with PH who have mild limitation of physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnea, fatigue, chest pain, or presyncope. III Patients with PH who have a marked limitation of physical activity. There is no discomfort at rest, but less than ordinary activity causes increased dyspnea, fatigue, chest pain, or presyncope. IV Patients with PH who are unable to perform any physical activity at rest and who may have signs of right ventricular failure. Dyspnea and/or fatigues may be present at rest, and symptoms are increased by almost any physical activity. Chest 2004:126 (Suppl), JACC 2004:43 (Suppl) Thank You Until recently, nothing much could be done for people with pulmonary hypertension. Before 1990, there were very few treatments available for PH and the survival rate was approximately two to four years. Since then, a number of exciting new treatments that are able to slow progression of the disease and may even reverse some of the damage to lungs and heart are gradually becoming available. Some people do well on drugs; others may need a transplant. Some patients might also require supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult whereas some need oxygen around the clock. In severely affected cases, a heart-lung, single lung of double lung transplantation may be appropriate. PH patients respond differently to different medicines that are prescribed to dilate or relax blood vessels and no one drug can be said to be consistently effective in all patients. Because individual reactions vary, different drug have to be tried before chronic or long-term treatment begins. During the course of disease, the amount and type of medicine may also have to be changed. To find out which medicine works best for a particular patient, the drugs should be evaluated via cardiac catheterization. This way, they can see the effect of the medicine on the patient’s heart and lungs. They can also adjust the dose to reduce the side effects such as systemic low BP, nausea, angina, headache etc that may occur. To determine whether a drug is improving a patient’s condition, both the pulmonary pressure and the amount of blood being pumped by the heart (cardiac output) must be evaluated. Calcium Channel Blockers blocks entry of calcium into muscle cells of heart & arteries improve ability of heart to pump blood relaxes smooth muscle in walls of heart and blood vessels amlodipine (Norvasc), diltiazem (Cardizem, Tiazac), nifedipine (Adalat, Procardia), nicardipine (Cardene) etc. only small number of people with PH respond to them side effects – constipation, nausea, headache, rash, edema, drowsiness, dizziness, low blood pressure Blood Vessel Dilators Prostacyclin substance that acts like a hormone (prostaglandin) imitates behaviour of natural prostacyclin powerful vasodilator and anti-clotting agent prevent blood clots from forming given intravenously through catheter bridge to help those waiting for transplant Epoprostenol (Flolan) - 1st vasodilator approved by FDA Ilopost - inhaled through nebulizer Treprostinil - injected under skin side effects - jaw pain, nausea, leg cramps etc need comprehensive follow-up care Endothelin Receptor Antagonists available in pill form reverse effect of endothelin (blood vessels constriction) Bosentan (Tracleer) - may improve stamina of people with PH not for pregnant women need monthly liver monitoring - risk of liver complications Phosphodiesterase Inhibitors Revatio – contains sildenafil same active ingredient used in Viagra blocks the enzyme phosphodiesterase accentuates actions of nitric oxide opens blood vessels in the lungs - dilation side effect - vision problems Lung and heart for transplant Other treatment procedures Dilation Atrial Septostomy experimental procedure use in patient with severe PH makes a small hole in the heart, slowly enlarging it to relieve some of the pressure in the heart’s right side shunts blood across the atrial septum and into the left side of the heart similar to balloon atrial septostomy – naturally occurring hole present at birth is enlarged to help those with congenital heart defects Other areas of research for treatment of PH includes gene therapy and stem cell research. Pregnancy and PH The consensus of medical opinion is that PH and pregnancy is very dangerous. The life of the mother and baby are put at great risk. Pregnancy can really take its toll on a woman’s body. For example, heart rate speeds up and the immune system does not work quite as well. For a woman whose body already has to deal with a severe illness, pregnancy can actually have catastrophic consequences. The risk of pregnancy-related death in women with PH is substantial – reported to be as high as 30-50%. Some drugs commonly used to treat PH can be harmful to the developing fetus (e.g. warfarin). Because of this twofold risk to both mother and baby, use of some form of birth control to avoid pregnancy is strongly advised in women of childbearing age with PH. Children and PH Unfortunately, there is no minimum age for PH sufferers. Newborn, young children, teenagers and even in early adulthood, PH may occur. Untreated PH in children worsens more quickly than the same condition in adults. Children also change more quickly than adults and have to be watched closely. However, with treatment, children appear to have an overall better prognosis than adults. Children are responsive to the same treatments as adults. Treatment for PH is lifelong. The treatment used has to be individualized and adjusted according to how the child is responding. Children and PH A child with PH can still go to school, dance, play sports or ride a bike. Just like other children, a child with PH can help themselves by eating a healthy and balanced diet and have good rest periods. It is really important to have a greater degree of caution in allowing other non-PH doctors prescribe medication for a child with PH. It is hard but children with PH should be treated as normally as possible. The child will be happier and feel safer if they are treated just like the rest of the family. News From Home KUALA LUMPUR : Siti Salmah Jasni is on a heartand-lung support machine and still in critical condition. A statement issued by the National Heart Institute (IJN) said Siti Salmah is awake and able to respond to doctors. Siti Salmah underwent a six-hour heart and lung transplant at 12.10am on Friday at IJN. She was suffering from pulmonary hypertension. The 18year-old is the first in IJN's history to receive a combined heart and lung transplant. The organs were harvested by a 10-member IJN team on Thursday night at the Sultanah Bahiyah Hospital in Kedah from a woman donor who became brain dead after a road accident IJN thanked the donor's family for their generosity during their hour of grief. New Straits Times 2007/11/04 Recent advances in genetics and newer understandings in cell biology are providing fresh insights into the nature of this disease. New therapies offer an improved quality of life and increased survival rates in both adults and children. References http://www.medicinenet.com/script/main/art.asp?articlekey=77244 http://www.medicinenet.com/pulmonary_hypertension/index.htm http://www.medicinenet.com/calcium_channel_blockers/article.htm www.merck.com/mmhe/sec04/ch054/ch054a.html www.mayoclinic.com/health/pulmonary-hypertension/DS00430 www.nlm.nih.gov/medlineplus/pulmonaryhypertension.html www.phassociation.org www.americanheart.org/presenter.jhtml?identifier=11076 www.nhlbi.nih.gov/health/dci/Diseases/pah/pah_what.html http://www.pha-uk.com/living_with_ph/ http://heart.health.ivillage.com/pulmonary/pulmonaryhypertension2.cfm www.mater.ie/pha-ireland/html/what.htm. http://www.medicalook.com/Lung_diseases/Pulmonary_hypertension.html www-ermm.cbcu.cam.ac.uk/04007513h.htm. home-2.tiscali.nl/~rsmid/index.html. http://www.nst.com.my/ THANK YOU FOR YOUR ATTENTION!! AND HAVE A PLEASANT DAY!!