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Pulmonary hypertension

Pulmonary hypertension is a biology topic covered in the lgStudy science library. This page brings together a partial reference excerpt, illustrations, worked examples, real-world applications and a short study plan, so you can understand Pulmonary hypertension rather than just read about it. In short: Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure in the arteries of the lungs. Symptoms include shortness of breath, fainting, tiredness, chest pain, swelling of the legs, and a fast heartbeat.

Pulmonary hypertension — main illustration
Pulmonary hypertension — illustration

Key takeaways

  • Pulmonary hypertension belongs to biology; place it in that map before memorising details.
  • Learn the definition first, then one example that makes the definition concrete.
  • Connect Pulmonary hypertension to a quantity you can measure, compute or draw — that is where exam questions come from.
  • Reproduce the core statement of Pulmonary hypertension from memory before moving on to harder problems.

Reference excerpt

Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure in the arteries of the lungs. Symptoms include shortness of breath, fainting, tiredness, chest pain, swelling of the legs, and a fast heartbeat. The condition may make it difficult to exercise. Onset is typically gradual. According to the definition at the 6th World Symposium of Pulmonary Hypertension in 2018, a patient is deemed to have pulmonary hypertension if the pulmonary mean arterial pressure is greater than 20mmHg at rest, revised down from a purely arbitrary 25mmHg, and pulmonary vascular resistance (PVR) greater than 3 Wood units. The cause is often unknown. Risk factors include a family history, prior pulmonary embolism (blood clots in the lungs), HIV/AIDS, sickle cell disease, cocaine use, chronic obstructive pulmonary disease, sleep apnea, living at high altitudes, and problems with the mitral valve. The underlying mechanism typically involves inflammation and subsequent remodeling of the arteries in the lungs. Diagnosis involves first ruling out other potential causes. High cardiac output states, such as advanced liver disease or the presence of large arteriovenous fistulas, may lead to an elevated mean pulmonary artery pressure (mPAP) greater than 20 mm Hg despite a pulmonary vascular resistance (PVR) less than 2 Wood units, which does not necessarily indicate pulmonary vascular disease. As of 2022 there was no cure for pulmonary hypertension, although research to find a cure is ongoing. Treatment depends on the type of disease. A number of supportive measures such as oxygen therapy, diuretics, and medications to inhibit blood clotting may be used. Medications specifically used to treat pulmonary hypertension include epoprostenol, treprostinil, iloprost, bosentan, ambrisentan, macitentan, and sildenafil, tadalafil, selexipag, riociguat. Lung transplantation may be an option in severe cases.

The frequency of occurrence is estimated at 1,000 new cases per year in the United States. Females are more often affected than males. Onset is typically between 20 and 60 years of age. Pulmonary hypertension was identified by Ernst von Romberg in 1891.

Classification According to the WHO classification, there are 5 groups of PH. Group I (pulmonary arterial hypertension) is further subdivided into Group I' and Group I'' classes. The WHO classification system in 2022 (with adaptations from the more recent ESC/ERS guidelines shown in italics) can be summarized as follows: WHO Group I – Pulmonary arterial hypertension (PAH)

Caused by narrowing and thickening of tiny arteries of the lung Idiopathic in most cases (heritable in some cases) Heritable (BMPR2, ALK1, SMAD9, caveolin 1, KCNK3 mutations) Drug- and toxin-induced (e.g., methamphetamine, amphetamine, or cocaine use ) Associated conditions:Connective tissue disease, HIV infection, Portal hypertension, Congenital heart diseases, Schistosomiasis WHO Group I' – Pulmonary veno-occlusive disease (PVOD), pulmonary capillary hemangiomatosis (PCH)

Idiopathic Heritable (EIF2AK4 mutations) Drugs, toxins, and radiation-induced Associated conditions: connective tissue disease, HIV infection WHO Group I" – Persistent pulmonary hypertension of the newborn WHO Group II – Pulmonary hypertension secondary to left heart disease

Left ventricular systolic dysfunction Left ventricular diastolic dysfunction Valvular heart disease Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathy Congenital/acquired pulmonary venous stenosis WHO Group III – Pulmonary hypertension due to lung disease, chronic hypoxia

Chronic obstructive pulmonary disease (COPD) Interstitial lung disease Mixed restrictive and obstructive pattern pulmonary diseases Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure to high altitude Developmental abnormalities WHO Group IV – Chronic arterial obstruction

Chronic thromboembolic pulmonary hypertension (CTEPH) Other pulmonary artery obstructions Angiosarcoma or other tumor within the blood vessels Arteritis Congenital pulmonary artery stenosis Parasitic infection (hydatidosis) WHO Group V – Pulmonary hypertension with unclear or multifactorial mechanisms

Hematologic diseases: chronic hemolytic anemia (including sickle cell disease) Systemic diseases: sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid diseases Others: pulmonary tumoral thrombotic microangiopathy, fibrosing mediastinitis, chronic kidney failure, segmental pulmonary hypertension (pulmonary hypertension restricted to one or more lobes of the lungs)

Signs and symptoms The symptoms of pulmonary hypertension include the following:

Less common signs/symptoms include a non-productive cough and exercise-induced nausea and vomiting. Coughing up of blood may occur in some patients, particularly those with specific subtypes of pulmonary hypertension such as heritable pulmonary arterial hypertension, Eisenmenger syndrome, and chronic thromboembolic pulmonary hypertension. Pulmonary venous hypertension typically presents with shortness of breath while lying flat or sleeping (orthopnea or paroxysmal nocturnal dyspnea), while pulmonary arterial hypertension (PAH) typically does not. Other typical signs of pulmonary hypertension include an accentuated pulmonary component of the second heart sound, a right ventricular third heart sound, and parasternal heave indicating a hypertrophied right ventricle. Signs of systemic congestion resulting from right-sided heart failure include jugular venous distension, ascites, and hepatojugular reflux. Evidence of tricuspid insufficiency and pulmonic regurgitation is also sought and, if present, is consistent with the presence of pulmonary hypertension.

… excerpt ends here. Continue reading the full article.

Illustrations

Pulmonary hypertension illustration
Pulmonary hypertension: Right ventricle (on left side)
Right ventricle (on left side)
Pulmonary hypertension: Micrograph showing arteries in pulmonary hypertensive with marked thickening of the walls
Micrograph showing arteries in pulmonary hypertensive with marked thickening of the walls
Pulmonary hypertension: Three major signaling pathways involved in the pathogenesis of pulmonary arterial hypertension
Three major signaling pathways involved in the pathogenesis of pulmonary arterial hypertension
Pulmonary hypertension: Phonocardiogram and jugular venous pulse tracing from a middle-aged man with pulmonary hypertension caused by cardiomyopathy. The jugular venous pulse tracing demonstrates a prominent a wave without a c or v wave being observed. The phonocardiograms (fourth left interspace and cardiac apex) show a murmur of tricuspid insufficiency and ventricular and atrial gallops.
Phonocardiogram and jugular venous pulse tracing from a middle-aged man with pulmonary hypertension caused by cardiomyopathy. The jugular venous pulse tracing demonstrates a prominent a wave without a c or v wave being observed. The phonocardiograms (fourth left interspace and cardiac apex) show a murmur of tricuspid insufficiency and ventricular and atrial gallops.

Worked examples

Example 1 — a first encounter with Pulmonary hypertension

Start with the simplest possible case. Write down what Pulmonary hypertension claims or describes in one sentence, then invent the smallest concrete situation in which that sentence is true. In biology, the smallest case is usually a single object, a single equation or a single measurement. Check that every symbol or term in your sentence has a meaning in that case.

Example 2 — changing one variable

Take the situation from Example 1 and change exactly one quantity: double it, halve it, or set it to zero. Predict what should happen to Pulmonary hypertension before you calculate. Comparing your prediction with the result is the fastest way to find out whether you understand the idea or only the words.

Example 3 — an exam-style question

Typical questions about Pulmonary hypertension ask you to (a) state it precisely, (b) apply it to given data, and (c) explain a limitation. Practise writing all three answers in under five minutes; the third part is what separates a full-mark answer from an average one.

Applications of Pulmonary hypertension

In research
Pulmonary hypertension appears in biology research whenever the underlying quantities have to be modelled precisely. Papers usually cite it as a starting assumption and then explore where it breaks down.
In technology and industry
Engineering practice reuses Pulmonary hypertension in design rules, simulations and safety margins. Knowing the idea lets you read a specification sheet and understand why the numbers look the way they do.
In the classroom
Pulmonary hypertension is common in secondary-school and first-year university syllabi. It links to neighbouring topics Hypertension, Pulmonary heart disease and diseases of pulmonary circulation, Rare diseases, so understanding it makes those chapters shorter.
In everyday life
Look for Pulmonary hypertension outside the textbook — in sport, cooking, traffic, electronics or the sky above you. An example you found yourself is remembered far longer than one you were given.
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How to study Pulmonary hypertension in 20 minutes

  1. Read the reference excerpt below once, without taking notes.
  2. Close the page and write down what Pulmonary hypertension means in your own words.
  3. Compare your version with the excerpt and mark what you missed.
  4. Work through the three examples above with pen and paper.
  5. Explain Pulmonary hypertension out loud to somebody else — or to Teacher Smith in the lgStudy chat.

Frequently asked questions

What is Pulmonary hypertension in simple terms?

Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure in the arteries of the lungs. Symptoms include shortness of breath, fainting, tiredness, chest pain, swelling of the legs, and a fast heartbeat.

Why does Pulmonary hypertension matter?

Because it connects several biology ideas at once: it gives you a definition you can apply, a quantity you can calculate, and a way to check whether a result is plausible.

How should I study Pulmonary hypertension?

Read the excerpt, restate it from memory, then work through the examples and applications listed on this page. The five-step study plan above takes about twenty minutes.

What does this page cover?

It gives you a compact reference excerpt plus original lgStudy explanations, examples, applications and study material on Pulmonary hypertension.

Tags

  • Hypertension
  • Pulmonary heart disease and diseases of pulmonary circulation
  • Rare diseases

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