Pulmonary Hypertension Flashcards
is a severe, rare lung disease characterized by high blood pressure in the pulmonary arteries, which deliver blood from the heart to the lungs.
Pulmonary hypertension (PH)
Pulmonary blood pressure is normally a lot lower than
systemic blood pressure
Normal pulmonary artery pressure is — at rest
8-20 mmHg
If the pressure in the pulmonary artery is greater than —- at rest or — during physical activity, it is abnormally high and is called pulmonary hypertension.
25 mm Hg
30 mmHg
is a serious health condition that results when the arteries carrying blood from the right side of the heart to the lungs are constricted, disrupting blood flow.
Pulmonary hypertension (PH)
Pulmonary hypertension severity classifications
CLASS I, CLASS II, CLASS III, CLASS IV
Although you’ve been diagnosed with pulmonary hypertension, you have no symptoms with normal activity.
Class I
You don’t have symptoms at rest, but you experience symptoms such as fatigue, shortness of breath or chest pain with normal activity.
Class II
You’re comfortable at rest, but have symptoms when you’re physically active.
Class III
You have symptoms with physical activity and while at rest.
Class IV
if the cause of the disease was known
Primary PH
if the cause could not be identified
Secondary PH
Secondary pulmonary hypertension also was called
idiopathic pulmonary hypertension
Now the classification has been expanded to five groups, to emphasize the importance of the underlying cause of the disease
Group 1 PAH (Pulmonary arterial hypertension)
Group 2 PH (left heart disease)
Group 3 PH (lung disease)
Group 4 PH (thromboembolic disease)
Group 5 PH (multifactorial)
GROUP I—- is associated with the narrowing of the small blood vessels in the lungs. It also is called —
PH
PAH
There are multiple other subgroups in group 1, including
Familial, or heritable pulmonary hypertension (FPAH) or (HPH)
certain drugs or toxins (recreational drugs and diet medications)
scleroderma or lupus
congenital heart problems
high blood pressure in the liver
HIV
schistosomiasis
sickle cell anemia
liver disease
Group 1 also may be caused by rare blood conditions like:
pulmonary veno-occlusive disease (PVOD)
pulmonary capillary hemangiomatosis (PCH)
persistent pulmonary hypertension of the newborn (PPHN)
Group 2:
pulmonary hypertension due to left heart disease
Long-term problems with the left side of the heart can lead to changes in the pulmonary arteries and cause pulmonary hypertension.
Group 2: pulmonary hypertension due to left heart disease
Group 2 includes
Left ventricular systolic dysfunction
Left ventricular diastolic dysfunction
Valvular disease
Congenital heart defects
when the heart cannot pump blood effectively
Left ventricular systolic dysfunct
when the heart cannot properly relax to allow enough blood to flow in
Left ventricular diastolic dysfunction
when the valves of the left side of the heart are allowing blood to leak
Valvular disease
which can lead to problems with blood flowing in or out of the heart
Congenital heart defects (heart defects from birth)
Group 3
pulmonary hypertension due to lung disease and/or chronic hypoxia
The common diseases associated with group 3 pulmonary hypertension are:
Chronic obstructive pulmonary disease (COPD)
Interstitial lung disease (pulmonary fibrosis)
Sleep-disordered breathing (OSA) Chronic high-altitude exposure
Lung developmental abnormalities
Alveolar hypoventilation disorders
Group 4:
pulmonary hypertension due to blood clots in the lungs
Group 4 also can be referred to as
chronic thromboembolic pulmonary hypertension (CTEPH)
are blood clots that travel to the lungs
Pulmonary emboli
are clots that are formed in the lungs, which can block the pulmonary arteries.
pulmonary thrombosis
Group 5:
pulmonary hypertension due to blood and other disorders
is the last category and includes other less-common causes that do not fit into any of the other four groups
Group 5
Group 5 are widely split into four categories:
Blood disorders
Systemic disorders
Metabolic disorders
Other: chronic kidney failure or tumors obstructing pulmonary arteries
Blood disorders
Anemia: polycythemia vera and thrombocythemia
Systemic disorders
sarcoidosis and histiocytosis
a condition that results in inflammation of different organs like the lungs and lymph nodes
Sarcoidosis
a rare disorder that causes scarring
Histiocystosis
Metabolic disorders
glycogen storage diseases and thyroid disorders
Predisposing factors
Fam hx and genetics : DS, CHD, gaucher
Age: 30-60
Sex: women
PRECIPITATING FACTORS
Lifestyle habits
Illegal drugs
Smoking
Overweight
Taking appetite suppressants
Clinical manifestations: early symptoms
SOB
Fatigue Chest pain (angina)
Racing heartbeat
Pain in upper right side of abdomen
Decreased appetite
Clinical manifestations: later symptoms
Feeling light-headed, especially during physical activity
Dizziness or fainting (syncope)
Swelling in the ankles or legs
Bluish color to lips or skin (cyanosis)
LABORATORY & DIAGNOSTIC TESTS
Echocardiogram.
exercise echocardiogram
Chest X-ray
Electrocardiogram (ECG)
Right heart catheterization
Blood tests
Cardiac MRI.
Complications
Right-sided heart enlargement and heart failure (cor pulmonale)
Blood clots
Arrhythmia.
Bleeding.
to check the size and functioning of the right ventricle, and the thickness of the right ventricle’s wall. can also show how well your heart chambers and valves are working
Echocardiogram.
to help determine how well your heart and lungs work under stress.
exercise echocardiogram
This test can show enlargement of the right ventricle of the heart or the pulmonary arteries, which can occur in pulmonary hypertension
Chest x-ray
This noninvasive test shows your heart’s electrical patterns and can detect abnormal rhythms. Doctors may also be able to see signs of right ventricle enlargement or strain.
Electrocardiogram
This test can often help confirm that you have pulmonary hypertension and determine the severity of your condition.
Right heart catheterization
a cardiologist places a thin, flexible tube (catheter) into a vein in your neck or groin. The catheter is then threaded into your right ventricle and pulmonary artery.
Right heart catheterization
allows your doctor to directly measure the pressure in the main pulmonary arteries and right ventricle. It’s also used to see what effect different medications may have on your pulmonary hypertension.
Right heart catheterization
to get detailed pictures of the structure and functioning of the heart and surrounding blood vessels
Cardiac MRI
Nrsg mgt
Get plenty of rest
Stay as active as possible
Don’t smoke
Avoid pregnancy and birth control pills
Avoid traveling to or living at high altitudes
Get vaccines
Get support
Heart-healthy eating
Physical rehabilitation
Watch your weight
MEDICAL MANAGEMENT
Blood vessel dilators (vasodilators)
calcium channel blockers
Anticoagulation or blood thinners
Digitalis, or digoxin
calcium channel blockers
nifedipine and diltiazem
endothelin receptor antagonists and phosphodiesterase type 5 inhibitors.
Blood vessel dilators (vasodilators)
epoprostenol (Flolan, Veletri)
Surgical mgt
Atrial septostomy.
Transplantation.
Balloon atrial septostomy
Balloon pulmonary angioplasty
Pulmonary endarterectomy surgery
Blood transfusions
Heart valve repair
a surgeon will create an opening between the upper left and right chambers of your heart (atria) to relieve the pressure on the right side of your heart.
Atrial septostomy
to decrease pressure in the right heart chambers and improve the
output of the left heart and oxygenation of the blood. In this procedure, a small hole is made in the wall between the right and left atria to allow blood to flow from the right to the left atrium.
Balloon atrial septostomy
to lower the blood pressure in your pulmonary artery and improve heart function in people who cannot have a pulmonary endarterectomy.
Balloon pulmonary angioplasty
to remove blood clots from the inside of the blood vessels of the lungs.
pulmonary endarterectomy
Blood pressure medicines such as— when left heart disease is the cause
angiotensin-converting enzymes inhibitors, beta blockers, or calcium channel blockers
treat sickle cell disease
Blood transfusions or hydroxyurea
