Pulmonary hypertension

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Pulmonary hypertension


Apnea and hypoventilation

Apnea of prematurity

Sleep apnea

Pleura and pleural space disorders


Pleural effusion



Pulmonary hypertension


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USMLE® Step 1 questions

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High Yield Notes

11 pages


Pulmonary hypertension

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USMLE® Step 1 style questions USMLE

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A 6-year-old girl is brought to her primary pediatrician for evaluation of dyspnea. The patient feels tired throughout the day, despite sleeping for nine hours every night. She additionally describes intermittent trouble breathing with exertion. The patient’s mother and grandmother have suffered from similar symptoms throughout their lives and have a “problem with the vessels in our lungs.” An echocardiogram is performed, which is within normal limits. Cardiac catheterization is performed, and the patient’s mean pulmonary artery pressure is 32 mmHg (normal <25 mmHg). A mutation in which of the following genes is the most likely etiology of this patient's symptoms?  

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Amphetamines p. 243

pulmonary arterial hypertension p. 703

Arteriosclerosis p. 307

pulmonary hypertension p. 703

Chronic thromboembolic pulmonary hypertension p. 703

Cocaine p. 571

pulmonary arterial hypertension p. 703

Congenital heart disease p. 304-306

pulmonary arterial hypertension p. 703

Connective tissue diseases

pulmonary arterial hypertension p. 703

Cor pulmonale p. 318, 690, 731

pulmonary hypertension p. 703


pulmonary hypertension p. 703


pulmonary hypertension p. 690


pulmonary hypertension p. 711

Heart failure p. 318

pulmonary hypertension p. 703


pulmonary hypertension p. 690

HIV (human immunodeficiency virus) p. 172

pulmonary arterial hypertension p. 703


for pulmonary hypertension p. 711

Multifactorial pulmonary hypertension p. 703

Portal hypertension p. 398

pulmonary arterial hypertension p. 703

Pulmonary arterial hypertension (PAH) p. 703

high altitude and p. 692

Pulmonary hypertension p. 703

cor pulmonale p. 690

drug therapy p. 711

PDE-5 inhibitors for p. 681

Schistosoma p. , 157

sleep apnea p. 703

Right ventricular hypertrophy (RVH)

pulmonary hypertension p. 703


pulmonary arterial hypertension p. 703


Pulmonary hypertension refers to increased blood pressure in the pulmonary circulation, more specifically a mean pulmonary arterial pressure that is greater than 25 mmHg.

The pulmonary circulation starts with the right ventricle.

From there - blood is pumped into the large pulmonary trunk, which splits to form the two pulmonary arteries – one for each lung.

The pulmonary arteries divide into smaller arteries known as pulmonary arterioles and then eventually into pulmonary capillaries which surround the alveoli - which are the millions of tiny air sacs where gas exchange happens.

At that point, oxygen enters the blood and carbon dioxide enters the alveoli.

The pulmonary capillaries drain into small veins that join to form the two pulmonary veins exiting each lung, and these pulmonary veins complete the circuit by delivering oxygen-rich blood into the left atrium.

The blood pressure in the pulmonary circulation is normally much lower than the systemic blood pressure.

The normal pulmonary artery pressure is about 25/10 mmHg with a mean arterial pressure of 15 mmHg.

Pulmonary hypertension most commonly develops as a result of left heart disease.

Here the pulmonary blood vessels are normal and undamaged, but the left side of the heart is unable to pump efficiently – for example because of heart failure or valvular dysfunction.


Pulmonary hypertension (PH) is a condition characterized by an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries. It can be due to left heart disease, chronic lung disease, or other conditions that specifically cause pulmonary arterial hypertension. Regardless of the cause, it can complicate right-sided heart failure, what's referred to as cor pulmonale. Symptoms of PH include shortness of breath, dizziness, fainting, and leg swelling. Treatment focuses on the underlying cause and may include supplemental oxygen, and medications like endothelin receptor antagonists and prostacyclins.


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  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Pathogenesis of Pulmonary Arterial Hypertension" Circulation (2005)
  6. "ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension" Journal of the American College of Cardiology (2009)
  7. "Updated Clinical Classification of Pulmonary Hypertension" Journal of the American College of Cardiology (2013)

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