STRATEGY OF TREATMENT OF Presented by : Niraj Maheshwari & Shweta

Burange
Group: 16-36
Dept. of Propaedeutic of Internal Diseases,
Teacher; Dr.

Olga Georgievna Goryacheva

blood pressure that affects the arteries in your lungs and

the right side of your heart.
The disease is defined hemodynamically as a mean pulmonary artery pressure ≥ 25 mmHg at rest.
Some forms of pulmonary hypertension are serious conditions that become progressively worse and are sometimes fatal.
Although some forms of pulmonary hypertension aren't curable, treatment can help lessen symptoms and improve your quality of life.

increased pulmonary vascular resistance which leads to progressive elevations in

pulmonary artery pressure, resulting in compensatory right ventricular hypertrophy and, ultimately, heart failure.
Common pathogenic features of pulmonary hypertension include sustained pulmonary vasoconstriction, vascular remodeling of the small pulmonary arteries, in situ thrombosis, and increased vascular wall stiffness, resulting in increased pulmonary arterial pressure due to increased pulmonary vascular resistance.

Hypertension (PVH) are included in the category of Pulmonary Hypertension

(PH)

PVH

PH

classify the severity of your disease into one of several

classes, including:
Class I. No strong symptoms with normal activity.
Class II. No symptoms at rest but with little physical activity arises symptoms like fatigue, dyspnoea, and chest pain.
Class III. No symptoms at rest but presented with strong symptoms during physical activity.
Class IV. Symptoms present during physical activity and while at rest.

common type of Pulmonary Hypertension (PH) which raises the blood

pressure in your lungs.
PH is defined as an increase in mean pulmonary arterial pressure (PAPm) ≥25 mmHg at rest as assessed by right heart catheterization (RHC).
Data analysis have shown that the normal PAPm at rest is 14 ± 3 mmHg with an upper limit of normal of approximately 20 mmHg.

into 5 groups:

that the permanent treatment of PAH is not possible.
So mostly

only palliative care treatment is provided for the expected result of better life expectancy for suffering individuals.
That is why, we will be looking towards the treatment strategies based upon the well defined criteria of WHO of groups.
It often takes some time to find the most appropriate treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care.
When pulmonary hypertension is caused by another condition, it is important to treat the underlying cause whenever possible.

category of diseases with the shared features of progressively increased

Pulmonary Vascular Resistance (PVR) due to obstructive changes within the pulmonary vasculature.
The treatment specifically include the treatment of the underlying causes, that is treating the primary diseases which causes the secondary effect of Pulmonary Hypertension.
The treatment process of PAH patients cannot be considered as a mere prescription of drugs, but is characterised by a complex strategy that includes the initial evaluation of severity and the subsequent response to treatment.

can be divided into three main steps:
The initial approach includes

general measures, and supportive therapy (oral anticoagulants, diuretics, O2, digoxin)
The second step includes initial therapy with high-dose CCB in vaso-reactive patients or drugs approved for PAH in non-vaso-reactive patients according to the prognostic risk of the patient and the grade of recommendation and level of evidence for each individual compound or combination of compounds.
The third part is related to the response to the initial treatment strategy; in the case of an inadequate response, the role of combinations of approved drugs and lung transplantation are proposed.

goal of therapy in PH-LHD must be to improve global

management of the underlying condition prior to considering specific measures to treat PH.
Systolic HF
Use: ACE inhibitors; β-blockers; diuretics
Possibly: cardiac resynchronization, implantable cardioverter defibrillator placement.
PH secondary to left valvular heart disease
Evaluation for correction of valvular disease

due to lung diseases and/or hypoxaemia):
Mild PH is common

in both severe interstitial lung disease and severe chronic obstructive pulmonary disease (COPD).
Whereas, severe PH is uncommon, and severe PH can be seen in the combined emphysema/fibrosis syndrome, where the prevalence of PH is high.

is no specific therapy for PH associated with lung diseases,

but these measures can be followed:
Optimize treatment of underlying cause
Sleep apnea
COPD (Long term O2 Therapy)
Idiopathic lung disease
Use supplemental oxygen to avoid hypoxia
Enroll in pulmonary rehabilitation
Don’t use PAH therapy in PH due to lung disease

of recurrent clot / embolism (anticoagulation)
Antiplatelet drugs; such as aspirin,

clopidogrel, dipyridamole.
Pulmonary thromboendarterectomy (PTE)
Refer to center experienced in procedure and postop
Improves symptoms in most patients
Surgical mortality at experienced centers: <5%

unclear and/or multifactorial mechanisms includes several disorders with multiple patho-aetiologies.

A common feature of these diseases is that the mechanisms of PH are poorly understood.
These patients need careful diagnosis. Treatment is tailored for that diagnosis, ie. treatment of PH is secondary. 

Vasodilators open narrowed blood vessels, and one of the most

commonly prescribed vasodilators for pulmonary hypertension is epoprostenol (Flolan)
Another form of the drug, iloprost (Ventavis), can be inhaled 6-9 times a day through a nebulizer, a machine that vaporizes your medication. Because it's inhaled, it goes directly to the lungs. Side effects associated with iloprost include chest pain which is often accompanied by a headache and nausea and breathlessness.
Treprostinil (Tyvaso, Remodulin, Orenitram), another form of the drug, can be given 4 times a day. It can be inhaled, taken as oral medication or administered by injection. It can cause side effects such as a headache, nausea and diarrhea.

reverse the effect of endothelin, a substance in the walls

of blood vessels that causes them to narrow.
These medications include bosentan (Tracleer), macitentan (Opsumit), and ambrisentan (Letairis).
Sildenafil and tadalafil (Phosphodiesterase 5 inhibitors (PDE5 inhibitors):
Sildenafil (Viagra) and tadalafil (Cialis) are sometimes used to treat pulmonary hypertension.
These drugs work by opening the blood vessels in the lungs to allow blood to flow through more easily. Side effects can include an upset stomach, headache and vision problems.

drugs help relax the muscles in the walls of the

blood vessels. They include medications such as amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others) and nifedipine (Procardia, others).
However, it is effective for the treatment of PH but only few number of people actually respond to them.
Soluble guanylate cyclase (SGC) stimulator. Soluble guanylate cyclase (SGC) stimulators (Adempas) interact with nitric oxide and help relax the pulmonary arteries and lower the pressure within the arteries. 
 They can sometimes cause dizziness or nausea.

to prescribe the anticoagulant warfarin (Coumadin, Jantoven) to help prevent

the formation of blood clots within the small pulmonary arteries. Because anticoagulants prevent normal blood coagulation, they increase your risk of bleeding complications.
Digoxin. Digoxin (Lanoxin) can help the heart beat stronger and pump more blood. It can help control the heart rate if you experience arrhythmias.
Diuretics. Commonly known as water pills, these medications help eliminate excess fluid from your body. This reduces the amount of work your heart has to do. They may also be used to limit fluid buildup in your lungs.

suggest that you sometimes breathe pure oxygen, a treatment known

as oxygen therapy, to help treat pulmonary hypertension, especially if you live at a high altitude or have sleep apnea.
Exercise: When medically supervised, exercise can help patients improve symptoms that include shortness of breath (dyspnea); fatigue; dizziness or fainting spells (syncope); pressure or pain in the chest.
Even patients with severe PH can see improvement from exercise because toned muscles use oxygen more efficiently than flaccid muscles.

lower risk patients)
Phosphodiesterase-5 Inhibitors (for lower risk patients)
Prostacyclins (for higher

risk patients)

of any type of left heart disease. One example is

as a complication of mitral valve disease.
Pulmonary venous hypertension (PVH) is associated with high blood pressure in the veins of the lungs (pulmonary vein) due to the lungs being unable to efficiently carry blood to the heart.

the underlying cause of the disease.
For example, surgery to repair

or replace a faulty mitral valve can be an option.
Medications such as beta blockers and angiotensin-converting enzyme (ACE) inhibitors may be used to prevent congestive heart failure.

addition of advanced therapies: as current model
Initial therapy: based on

illness severity + functional class
If PAH worsens: add therapies until treatment goals are met
Approach shown to improve 6-min walking distance test.
Combination therapy: Can be used
Optimum and safest approaches not yet established
Investigations ongoing

PH?
Atrial septostomy. If medications don't control the pulmonary hypertension, this open-heart

surgery might surely be considered as an option.
In an atrial septostomy, an opening is created between the left and right atrium to relieve the pressure on the right side of the heart.
Atrial septostomy can have serious complications, including heart rhythm abnormalities (arrhythmias), but it has proven to be helpful.

PH?
Transplantation. In some cases, a lung or heart-lung transplant might be

considered as an option, especially for younger people who have idiopathic pulmonary arterial hypertension.
Major risks of any type of transplantation include rejection of the transplanted organ and serious infection, and immunosuppressant drugs must be administered for life to help reduce the chance of rejection.

due to left heart or chronic hypoxemic lung disease
Treat underlying

disorders (not PH primarily)
Chronic thromboembolic PH
Use anticoagulants
PAH
Perform right heart catheterization with vasodilator test
Don’t treat empirically with calcium-channel blockers
Advanced therapies: prostacyclin's, endothelin-receptor antagonists,
Evaluate need for supplemental oxygen
Lung transplantation: In nonresponsive advanced disease