PULMONARY HYPERTENSION INDIA

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PULMONARY HYPERTENSION

PULMONARY HYPERTENSIONPULMONARY HYPERTENSIONPULMONARY HYPERTENSION
KNOW MORE

PULMONARY HYPERTENSION

PULMONARY HYPERTENSIONPULMONARY HYPERTENSIONPULMONARY HYPERTENSION
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1. WHAT IS PULMONARY HYPERTENSION?

EXPERT: DR. VIKAS KOHLI (PEDIATRIC CARDIOLOGIST & PH SPECIALIST)

a. DEFINITION

  1. Pulmonary hypertension (PH), is a complex and often misunderstood disease. The term PH means high blood pressure in the lungs. In “regular” hypertension the pressure in the arteries throughout the body is higher than it should be. This can be measured with a blood pressure cuff. 
  2. In PH, the blood vessels specifically in the lungs are affected. They can become stiff, damaged or narrow, and the right side of the heart must work harder to pump blood through.

b. CLASSIFICATION/CAUSES

  1. Pulmonary Arterial Hypertension (PAH)
  2. Pulmonary Hypertension due to Left Heart Disease
  3. Pulmonary Hypertension due to Lung Diseases and/ or Hypoxia
  4. Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
  5. Pulmonary Hypertension with unclear and / or multi-factorial mechanisms

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2. HOW IS IT ASSESSED?

Pulmonary hypertension (PH) can be difficult to diagnose in a routine medical exam because the most common symptoms of PH, such as breathlessness, fatigue and dizziness, are also associated with many other conditions.  

Preliminary Tests

1. Echocardiography

Echocardiogram/ Echocardiography in this procedure, electrodes are placed on the patient’s skin, and a sonogram of the heart is taken. This painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and assessing how well the heart is functioning. Other heart conditions that produce symptoms similar to PH may be diagnosed with an echocardiogram. In addition, an echocardiogram may be used to monitor a patient’s condition.

2. Chest X-Rays

Chest X-rays can reveal an enlarged right ventricle or pulmonary arteries. Chest X-rays can also show signs of emphysema or scarring (interstitial fibrosis) of the lungs.

3. Pulmonary Function Tests

These tests measure how much air your lungs can hold, how much air moves in and out of them and the lungs’ ability to exchange oxygen. These tests may be performed to potentially identify its cause.  

4. Nuclear Scan (Ventilation/Perfusion Scan or V/Q Scan)

This diagnostic tool tests for blood clots in the lungs by producing a picture of air and blood flow to the lungs. A small dose of radioactive material is breathed in and another small dose is injected via a blood vessel into the lungs. The doctor will review the images that are produced to evaluate the health of the lungs.

5. Right-Heart Catheterisation

Right-heart catheterization is one of the most accurate and useful tests to get a definitive diagnosis for pulmonary hypertension. This is the only test that directly measures the pressure inside the pulmonary arteries, and it should be done in all patients at least once to confirm a patient’s diagnosis of PH. During the test, doctors insert a catheter (a thin rubber tube) through a large vein in the patient’s groin or neck. They then pass the catheter up into the patient’s heart to measure the blood pressure in the right side of the heart and lungs.

3. HOW DO MEDS WORK?

Although there is currently no cure for Pulmonary Hypertension, there are treatment options available and more are on the horizon. Treatments include what health care providers call conventional medical therapies as well as oral, inhaled, subcutaneous (into the skin), and intravenous (into the vein) options. Depending on the severity of PH, heart or lung transplant may also be an option.

Efficacy of drugs for Pulmonary Arterial Hypertension

Efficacy of drugs for Pulmonary Arterial Hypertension

Reference: PULMONARY HYPERTENSION | N. GALIÈ ET AL.

Table: Recommendations for efficacy of drug monotherapy for pulmonary arterial hypertension (group 1) according to World Health Organization functional class. The sequence is by pharmacological group, by rating and by alphabetical order.

4. SYMPTOMS OF PULMONARY HYPERTENSION

The signs and symptoms of pulmonary hypertension develop slowly. You may not notice them for months or even years. Symptoms get worse as the disease progresses.  

Pulmonary hypertension signs and symptoms include:  

  1. Blue lips and skin (cyanosis) 
  2. Chest pressure or pain 
  3. Dizziness or fainting spells (syncope) 
  4. Fast pulse or pounding heartbeat (palpitations) 
  5. Fatigue Shortness of breath (dyspnea), initially while exercising and eventually while at rest 
  6. Swelling (edema) in the ankles, legs and eventually the belly area (abdomen)

5. ECHO: ROLE IN DIAGNOSIS AND MANAGEMENT

Transthoracic Echocardiography is used to image the effects of PH on the heart and estimate PAP from continuous wave Doppler measurements. Echocardiography should always be performed when PH is suspected and may be used to infer a diagnosis of PH in patients in whom multiple different echocardiographic measurements are consistent with this diagnosis.  


The probability of PH may then be judged as high, intermediate or low. When interpreted in a clinical context, the echocardiographic result is required to decide the need for Cardiac Catheterization in individual patients. To facilitate and standardize assignment to the level of probability of PH, several additional echocardiographic signs are proposed in addition to criteria based on TRV. 


These signs provide an assessment of the RV size and pressure overload, the pattern of blood flow velocity out of the RV, the diameter of the PA and an estimate of RAP. Their measurement has been defined in recommendations endorsed by the EACVI.  Echocardiography can help detect the cause of suspected or confirmed PH. Two-dimensional, Doppler and contrast examinations can be used to identify CHD. 


High pulmonary blood flow found on pulsed wave Doppler in the absence of a detectable shunt or significant dilatation of proximal PA despite only moderate PH may warrant transoesophageal examination with contrast or cardiac magnetic resonance (CMR) imaging to exclude sinus venous atrial septal defect and/or anomalous pulmonary venous return. 


In cases of suspicion of LV diastolic dysfunction, Doppler echocardiographic signs should be assessed even if their reliability is considered low. RHC should be considered when the diagnosis remains uncertain after non-invasive investigations. 


Reference: PULMONARY HYPERTENSION | N. GALIÈ ET AL

TABLE: Diagnostic management is suggested according to the echocardiographic probability of pulmonary hypertension in patients with symptoms compatible with pulmonary hypertension, with or without risk factors for pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.

6. MANAGEMENT AND FOLLOW UP

  1. Learning that you have pulmonary hypertension can turn your whole world upside down. Every new patient, when learning that they have PH, responds differently. It’s important to remember that there are no right or wrong reactions. Give yourself the space to feel and respond to your diagnosis without judgment so you can begin to move forward. Before they were diagnosed, most newly diagnosed patients experienced breathlessness and other symptoms of PH. While your diagnosis marks an ending, it also marks a beginning. Finding a PH-treating doctor, getting on PH-specific treatment, making accommodations for PH in your everyday life, and acknowledging your fears and feelings are all parts of a journey that begins now.


Appropriate diagnosis and analysis of the problem are necessary before starting any treatment. Treatment varies by patient, based on the different underlying causes, but generally includes:  

  1. Taking medications 
  2. Making lifestyle and dietary changes 
  3. Having surgery, if necessary 
  4. Regular follow up with your PH specialist doctor.


Many different types of medications are available to treat pulmonary hypertension. Treatment choices, such as those listed below, depending on the underlying cause of pulmonary hypertension, how severe the pulmonary hypertension is, how likely it is to progress, and a patient’s drug tolerance.


  • Know the names of your medications and how they work. Keep a list with you. 
  • Take your medications at the same time every day. If you forget a dose, do not take two doses to make up for the dose you missed. 
  • Do not take any over-the-counter drugs unless you ask your doctor or pharmacist first.
  • Also, avoid any over-the-counter medications whose labels state that caution is to be used if you have high blood pressure.
  • Do not stop taking or change your medications unless you first talk with your doctor.


Dietary changes:

  1. Eat foods that are high in nutrients like potassium (e.g., dried fruits, bananas, oranges) and magnesium (e.g., peanuts, tofu, broccoli) and vitamins. 
  2. Limit your total daily calories, if necessary, to achieve your ideal body weight. 
  3. Limit foods that contain refined sugar, saturated fats, and cholesterol. Eat foods that are high in fibre, including whole grains, beans, fruits, and vegetables. 
  4. Reduce your sodium intake: Buy foods that are low in sodium. Choose foods with labels that read "low salt" and "low sodium." Avoid table salt and seasoning salt. 
  5. Avoid smoked, cured, salted, and canned meat products. 
  6. Avoid fast food and limit prepared foods; they usually have a high sodium content. 
  7. Monitor your fluid intake. Cut down the intake of fluids if you have become more short of breath or if you notice swelling.

7. HOW WE MAKE A DIFFERENCE

  • Detail explanation on What is PH?  
  • Regular  follow up are planned and communicated with the patients.
  • Regular follow up through calls.
  • Effects & doses of medicine are explained in detail
  • Register underprivileged children for free medicine.

8. RISK MANAGEMENT

Call us if you are having problems with:


  • Weight gain — 2 pounds in one day or 5 pounds in one week 
  • Swelling in your ankles, legs, or abdomen that has become worse 
  • Shortness of breath that has become worse, especially if you wake up short of breath Extreme fatigue or decreased tolerance for activity 
  • A respiratory infection or a cough that has become worse 
  • Fast heart rate (around 120 beats per minute) 
  • Episodes of chest pain or discomfort with exertion that are not relieved with rest 
  • Difficulty breathing with regular activities or at rest 
  • Restlessness, confusion Constant dizziness or lightheadedness 
  • Nausea or poor appetite 

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