High altitude pulmonary edema

What it is

From: Orphanet

A rare pulmonary condition characterized by non-cardiogenic pulmonary edema occurring in otherwise healthy individuals within days of an ascent above 2500-3000 m. Early symptoms include exertional dyspnea, non-productive cough, chest tightness, and reduced exercise performance, followed by dyspnea at rest and possibly orthopnea, as well as gurgling in the chest and pink frothy sputum in advanced cases. Clinical signs are cyanosis, tachypnea, tachycardia, crackles or wheezing, and elevated body temperature (generally not exceeding 38.5°C). Signs of concomitant high-altitude cerebral edema may also be observed. Chest x-rays typically show patchy opacities predominantly in the right middle lobe.

Reported clinical features and what the terms mean

The following findings are associated with this condition in Orphanet. They are not a checklist for diagnosing yourself, and they do not all occur in every affected person. Some are examination, imaging or laboratory findings that cannot be recognised at home.

The frequency labels describe how often a finding was reported among people with the condition in the source. They do not give the chance that a person with that symptom has the condition. Definitions below reproduce HPO terminology; they explain the term, not the likely severity in an individual.

Dyspnea · Very frequent (99-80%)

Difficult or labored breathing. Dyspnea is a subjective feeling only the patient can rate, e.g., on a Borg scale.

Hypoxemia · Obligate (100%)

An abnormally low level of blood oxygen.

Pulmonary edema · Obligate (100%)

Fluid accumulation in the lungs.

Anorexia · Frequent (79-30%)

Lack of desire to eat (loss of appetite).

Cough · Frequent (79-30%)

A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation.

Crackles · Frequent (79-30%)

Crackles are discontinuous, explosive, and nonmusical adventitious lung sounds normally heard in inspiration and sometimes during expiration. Crackles are usually classified as fine and coarse crackles based on their duration, loudness, pitch, timing in the respiratory cycle, and relationship to coughing and changing body position.

Cyanosis · Frequent (79-30%)

Bluish discoloration of the skin and mucosa due to poor circulation or inadequate oxygenation of arterial or capillary blood.

Exercise intolerance · Frequent (79-30%)

A functional motor deficit where individuals whose responses to the challenges of exercise fail to achieve levels considered normal for their age and gender.

Headache · Frequent (79-30%)

Cephalgia, or pain sensed in various parts of the head, not confined to the area of distribution of any nerve.

Increased total leukocyte count · Frequent (79-30%)

An abnormal increase in the number of leukocytes in the blood.

Nausea and vomiting · Frequent (79-30%)

Nausea is a commonly encountered symptom that has been defined as an unpleasant painless subjective feeling that one will imminently vomit. Vomiting has been defined as the forceful expulsion of the contents of the stomach, duodenum, or jejunum through the oral cavity. While nausea and vomiting are often thought to exist on a temporal continuum, this is not always the case. There are situations when severe nausea may be present without emesis and less frequently, when emesis may be present without preceding nausea.

Pulmonary opacity · Frequent (79-30%)

Opacity refers to any area that preferentially attenuates the x-ray beam and therefore appears more opaque than the surrounding area. It is a nonspecific term that does not indicate the size or pathologic nature of the abnormality.

Sleep abnormality · Frequent (79-30%)

An abnormal pattern in the quality, quantity, or characteristics of sleep.

Tachycardia · Frequent (79-30%)

A rapid heartrate that exceeds the range of the normal resting heartrate for age.

Other findings in the same source

From: Orphanet

Additional reported features include Abnormal sputum (Occasional (29-5%)); Chest pain (Occasional (29-5%)); Fever (Occasional (29-5%)); Orthopnea (Occasional (29-5%)); Tachypnea (Occasional (29-5%)); Vertigo (Occasional (29-5%)); Hemoptysis (Very rare (<4-1%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

All ages

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Point prevalence: 1-9 / 1 000 000; Europe; Class only.

Which doctor should you see?

The suggested department for discussing High altitude pulmonary edema is Pulmonology, with a pulmonologist / chest physician as the relevant type of clinician. General physician / Family Medicine; paediatrician for children. Referral depends on symptoms.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• What is the likely explanation for the breathing symptoms?
• Would a breathing test or another investigation change management?
• If symptoms worsen, what written action plan should be followed?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for High altitude pulmonary edema. That gap does not mean that treatment is unavailable. A clinician needs to establish the diagnosis and review current guidance before recommending medicines, procedures, rehabilitation or other support.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.