Compare conditions

Put any two — or three — conditions side by side, adult or pediatric, to spot the look-alike differences fast, row by row.

PH · Pulmonary Hypertension

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In one line

·Five types by cause: blood vessel wall thickening (type 1), left heart backup (type 2), lung disease (type 3), clot blockage (type 4), or mixed (type 5) — all raise pressure in the lung arteries and wear out the right heart.

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Normal physiology

·Normally, the right side of your heart pumps blood gently through wide, soft arteries in your lungs, where the blood picks up oxygen and drops off carbon dioxide, then returns to the left heart to be pumped out to your body.

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What goes wrong

·In pulmonary high blood pressure (hypertension), pressure inside the lung arteries climbs above 20 mmHg at rest (measured by right heart catheter), forcing the right ventricle to work much harder; the root cause splits into five types.

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Hallmark signs

·Shortness of breath during activity
·Feeling tired or weak all the time
·Chest discomfort or pressure
·Swelling in your ankles, legs, or belly
·Your neck veins bulge or stand out
·Bluish lips or skin (cyanosis)
·Feeling dizzy or fainting (syncope)
·A loud second heart sound (P2) when the doctor listens with a stethoscope

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Red flags · escalate now

·Sudden fainting or near-fainting during exercise—this can mean your heart is at its limit and cannot send enough blood to your brain.
·New or worsening leg swelling, bulging neck veins, or a swollen belly—these are signs that the right heart is failing and blood is backing up into your body.
·Blue lips, fingernails, or skin—this means your blood oxygen is dangerously low.
·Chest pain that feels like pressure or squeezing, especially with activity—the struggling right heart may not be getting enough oxygen itself.
·Coughing up blood (hemoptysis)—high lung pressure can burst small blood vessels in the airways.

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Workup

·Transthoracic echocardiogram (ultrasound of the heart)
·Right heart catheterization (a thin tube threaded through a vein into the right heart and pulmonary artery to measure pressures directly)
·Six-minute walk test
·Ventilation-blood flow (perfusion) (V/Q) scan (a nuclear medicine scan that shows air flow and blood flow in the lungs)
·High-resolution chest CT with contrast (a detailed X-ray scan of the lungs)
·Pulmonary function tests (spirometry and diffusing capacity for carbon monoxide, DLCO)
·BNP or NT-proBNP (blood test for heart strain)
·Connective tissue disease antibody panel (ANA, anti-Scl-70, anti-centromere, anti-U1-RNP)

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Treatment

·Right heart catheterization to confirm the diagnosis and measure exact pressures
·PDE5 inhibitor (sildenafil 20 mg three times daily or tadalafil 40 mg once daily) or endothelin receptor antagonist (ambrisentan 5–10 mg daily, bosentan 125 mg twice daily, or macitentan 10 mg daily) for Group 1 pulmonary arterial high blood pressure (hypertension)
·Treat the upstream cause: diuretics and GDMT for left heart failure (Group 2), oxygen and inhalers for COPD or interstitial lung disease (Group 3), anticoagulation and surgery for chronic clots (Group 4)
·Loop diuretic (furosemide 20–80 mg daily, titrated to symptoms) for fluid overload and right heart failure
·Supplemental oxygen (target oxygen saturation 90 percent or higher) for patients with low oxygen in the blood (hypoxemia)
·Pulmonary thromboendarterectomy (PTE surgery to cut out organized clots from the pulmonary arteries) for chronic thromboembolic pulmonary high blood pressure (hypertension) (Group 4 CTEPH)
·Riociguat (a soluble guanylate cyclase stimulator, 0.5–2.5 mg three times daily) for inoperable or persistent CTEPH, or for Group 1 PAH

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NCLEX trap

·CCBs only help a tiny group of PAH patients — about 1 in 20 — whose lung vessels actually relax and open up during a special breathing test (the vasoreactivity test, where the patient breathes nitric oxide or another medicine while the doctor measures pressures). Most PH patients do NOT respond, and giving CCBs to the wrong patient can drop blood pressure dangerously and make the right heart fail faster. Always test first; only use a CCB if the test shows it works.
·You must first prove the diagnosis with a right-heart catheterization (RHC, a thin tube threaded into the heart through a vein to measure pressures directly) showing mean pulmonary artery pressure above 20 mmHg, AND figure out which of the five types of PH the patient has. Different types need different treatments — for example, type 2 (from a failing left heart) gets better when you fix the left heart, not when you give pulmonary vasodilators. Never treat without the diagnosis and the type.
·In pulmonary high blood pressure (hypertension), the right heart depends on blood flowing back from the body (venous return) to fill it and pump forward. Too much diuretic yanks out so much fluid that blood pressure drops and the right heart suddenly has nothing to pump — it collapses. Use diuretics gently, watch blood pressure closely, and stop if systemic pressure falls too low or the patient feels faint.
·Pulmonary high blood pressure (hypertension) is high pressure inside the pulmonary arteries (the blood vessels carrying blood from the right heart to the lungs). Pulmonary edema (fluid flooding the lungs) is fluid leaking out of vessels into the tiny air sacs, making it hard to breathe. Type 2 PH (from a weak or stiff left heart) can cause pulmonary edema because back-pressure floods the lungs, but types 1, 3, 4, and 5 usually do NOT flood the air sacs — the problem is in the vessels themselves or farther upstream. Know the difference.
·Pulmonary high blood pressure (hypertension) is a progressive disease — the pressures tend to creep up over time even on treatment. Patients need repeat right-heart catheterization, 6-minute walk tests, and blood tests (like BNP, a hormone the heart releases when it is stretched and struggling) every few months to catch worsening early. Most patients eventually need a second or third medicine added (combination therapy) to keep the right heart from failing. The goal is to prevent sudden collapse and death.
·Oxygen helps in types 3 (from lung disease like COPD or scarred lungs) and 5 (from other causes) where low oxygen in the blood tells the lung vessels to squeeze tight (hypoxic vasoconstriction), raising pressure. In ALL types, keeping oxygen levels normal helps the right heart work less hard and prevents dangerous heart rhythms. Oxygen is part of the safety net — it does not cure PH, but it supports the body while the specific medicines do their job.

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