Pulmonary Hypertension

Kelly Latimer, MD, MPH
Michael Layne, MD
Maya Payne, MD

American Family Physician. 2024;110(2):183-191.

Author disclosure: No relevant financial relationships.

This clinical content conforms to AAFP criteria for CME.

Pulmonary hypertension includes a diverse set of conditions defined by a mean pulmonary artery pressure greater than 20 mm Hg found during right heart catheterization that can lead to right-sided heart failure and death if untreated. The most common cause of pulmonary hypertension is left-sided heart failure, followed by chronic obstructive lung disease. Pulmonary hypertension presents as unexplained dyspnea on exertion and possible findings of right-sided heart failure. The diagnosis is commonly delayed because the symptoms are often attributed to underlying heart or lung disease. Echocardiography is the initial study of choice, and findings can suggest a low, intermediate, or high risk of pulmonary hypertension. Right heart catheterization is the standard of care for diagnosing and classifying pulmonary hypertension, and the results may inform treatment. Patients with pulmonary hypertension should be referred to a center specializing in treatment. Patients with pulmonary hypertension have a high risk of perioperative complications, and detailed specialty preoperative evaluation is recommended. Physicians should counsel patients of childbearing age with pulmonary hypertension to prevent pregnancy to avoid worsening the severity of pulmonary hypertension and fetal loss. Pulmonary hypertension is severe, chronic, progressive, and challenging to treat; therefore, family physicians should update the patient’s immunization status, screen for and address mental health conditions, and discuss goals of care and advance directives with patients.

Pulmonary hypertension includes conditions that result in elevated pressures in the lung vasculature. Pulmonary hypertension is defined as a mean pulmonary artery pressure of greater than 20 mm Hg at rest found during right heart catheterization.1 Pulmonary hypertension affects an estimated 1% of the global population and approximately 10% of people older than 65 years.1,2 Pulmonary arterial hypertension, a subset of pulmonary hypertension, is a relatively rare diagnosis with a high mortality rate if untreated.1 Family physicians most often encounter pulmonary hypertension as a downstream consequence of cardiac or pulmonary disease.

WHAT’S NEW ON THIS TOPIC

Pulmonary Hypertension
The presence of pulmonary hypertension is a poor prognostic sign, with a 5-year estimated survival rate of 67% or less. The prognosis is slightly worse when pulmonary hypertension is secondary to lung disease and slightly better in chronic thromboembolic pulmonary hypertension.
A 2020 systematic review demonstrated a 12% risk of maternal mortality and 23% risk of pregnancy loss in patients with pulmonary hypertension.
Patients with persistent dyspnea after recovery from COVID-19 are at increased risk of pulmonary hypertension. In one meta-analysis, 22% of patients developed pulmonary hypertension after intensive care unit admission due to COVID-19.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendation Evidence rating Comments
Patients with suspected pulmonary hypertension should undergo echocardiography as the initial test.1,10 C Expert consensus
Right heart catheterization is the test of choice for confirmation of pulmonary hypertension (especially if pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension is suspected) and can guide treatment decisions.1 C Expert consensus
Patients with pulmonary arterial hypertension should be referred to a specialty center.1 C Expert consensus
Patients with pulmonary arterial hypertension whose arterial blood oxygen pressure is less than 60 mm Hg should be on long-term oxygen therapy with some recommendations suggesting oxygen use to maintain oxygen saturation greater than 90% at sea level.1,20 C Expert consensus; no data to demonstrate improved patient-oriented outcomes
Patients with chronic thromboembolic pulmonary hypertension (group 4) should be on lifelong therapeutic doses of anticoagulation.1 C Expert consensus and inconsistent randomized controlled trials showing improved survival
Patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension who are optimized and stable on medical therapy should participate in a supervised exercise training program.1,19,25,26 A Consistent evidence from randomized controlled trials showing improvement in a 6-minute walking test and quality-of-life scores
Patients of childbearing potential who have pulmonary hypertension should be counseled to avoid pregnancy and consider two forms of contraception, particularly those treated with bosentan.1,28 C Expert consensus and clinical review article

A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.

The pulmonary vascular system comprises many thin-walled vessels that enable efficient gas diffusion with little blood flow resistance, allowing the thin-walled right ventricle to maintain the same cardiac output as the left ventricle. Destruction, remodeling, vasoconstriction, or blockage of the pulmonary vasculature can increase the resistance to blood flow and lead to pulmonary hypertension and increased strain on the right ventricle, causing progressive right-sided heart failure (HF). The pathologic vascular changes can originate in the pulmonary arteries, capillaries, or veins, alone or in combination.3

PRESENTATION

The presentation of pulmonary hypertension is nonspecific and similar to HF or valvular disease (Table 11). Symptoms of pulmonary hypertension are chronic, progressive dyspnea with minor exertion (60%), fatigue (19%), and presyncope or syncope (13%).4 Patients with advanced disease may have evidence of right-sided HF, including jugular venous distention, ascites, lower extremity edema, and, in very advanced cases, hemoptysis.1 For most patients, there are no specific examination findings distinct from those of the cause of the pulmonary hypertension. Recognizing undiagnosed pulmonary hypertension is challenging in the primary care setting, with a mean time to diagnosis of 2 years, because insidious symptoms and signs may initially be attributed to underlying chronic heart or lung disease.4

TABLE 1. Presentation of Pulmonary Hypertension

Symptoms of pulmonary hypertension
Most common
Dyspnea on exertion
Fatigue
Lightheadedness
Presyncope or syncope
Other symptoms
Chest pain
Palpitations
Weight gain due to fluid retention
Medical history associated with risk of pulmonary hypertension
Family history of pulmonary hypertension in a first-degree relative
Personal history of high-risk sexual behavior (e.g., patients with HIV), methamphetamine or cocaine use, or pulmonary embolism
Physical examination findings of pulmonary hypertension
Diastolic decrescendo murmur (pulmonic insufficiency)
High-pitched holosystolic murmur (tricuspid regurgitation)
Loud P2 heart sound (pulmonic closure)
Low pulse oximetry
Right ventricular third heart sound
Physical examination findings of pulmonary hypertension with right-sided heart failure
Ascites
Hepatojugular reflux
Hepatomegaly
Jugular venous distention
Lower extremity edema
Prolonged capillary refill

Information from reference 1.

The differential diagnosis for pulmonary hypertension is broad and includes HF, coronary artery disease, pulmonary fibrosis, chronic obstructive pulmonary disease, valvular heart disease, pulmonary embolism (PE), liver disease, and Budd-Chiari syndrome. Clinical suspicion for pulmonary hypertension should be increased in younger, symptomatic patients with comorbid diseases such as HIV, sickle cell disease, connective tissue disease, and congenital heart disease or a history of methamphetamine use. Delayed diagnosis of pulmonary arterial hypertension by more than 2 years is associated with an 11% increase in the mortality rate.5

DIAGNOSTIC EVALUATION

When a patient’s history suggests possible pulmonary hypertension, electrocardiography, chest radiography, echocardiography, pulmonary function testing, and basic laboratory testing should be performed. Table 2 summarizes suggested tests and findings for pulmonary hypertension.1,68 Although the role of brain natriuretic peptide (BNP) in diagnosis is uncertain, normal BNP results with normal electrocardiography findings suggest that pulmonary hypertension is unlikely. Chest radiography is often the first imaging test performed and has a high sensitivity and specificity for detecting moderate to severe pulmonary hypertension. Findings on chest radiography may include enlargement of the pulmonary artery, right atrium, or right ventricle.9

TABLE 2. Initial Evaluation of Suspected Pulmonary Hypertension

TestFindings suggestive of pulmonary hypertension
ElectrocardiographyFrequent premature atrial contractions, left ventricular hypertrophy, multifocal atrial tachycardia, right axis deviation, right ventricular hypertrophy
Chest radiographyLeft atrium enlargement, left ventricle enlargement, pleural effusion, pulmonary artery enlargement, right atrium dilation, right ventricle enlargement, septal thickening (Kerley lines)
Laboratory tests: complete blood count, complete metabolic panel, thyroid-stimulating hormone, BNP/N-terminal pro-BNPThe role of BNP in the diagnosis is not established; however, normal BNP results with normal electrocardiography findings are reassuring
Additional laboratory tests based on medical historyAntinuclear antibodies, coagulation studies, hepatitis antibodies, HIV, iron studies
Pulmonary function testingNormal spirometry, normal lung volumes, low diffusing capacity of lungs for carbon monoxide
Transthoracic echocardiographyFlattening of the interventricular septum in systole, increased inferior vena cava diameter, pulmonary artery enlargement, pulmonary regurgitation, right atrium enlargement, right ventricle enlargement or dysfunction, right ventricle/left ventricle ratio > 1, tricuspid regurgitation

BNP = brain natriuretic peptide.

Information from references 1, and 68.

Transthoracic echocardiography is the recommended initial test for patients with suspected pulmonary hypertension.10,11 Although echocardiography findings cannot confirm the diagnosis, abnormal findings can be categorized as low, intermediate, or high risk1,6 (Table 31). Echocardiography findings suggestive of pulmonary hypertension include the quantitative findings of a peak tricuspid regurgitation velocity of more than 2.8 meters per second or an estimated systolic pulmonary artery pressure greater than 35 mm Hg, with at least two anatomic abnormalities (e.g., abnormal right or left ventricle size, wall thickness, function, increased right atrium size, increased inferior vena cava and pulmonary artery diameter).1,7,8,11

TABLE 3. Echocardiography Findings and Probability of Pulmonary Hypertension

Peak tricuspid regurgitation velocity (m/s)Any other suggestive echocardiography finding?* Probability
≤ 2.8NoLow
≤ 2.8YesIntermediate
2.9 to 3.4NoIntermediate
2.9 to 3.4YesHigh
> 3.4Yes or noHigh

m/s = meters per second.

*—Basal diameter of right ventricle/basal diameter of left ventricle > 1, flattened interventricular septum, tricuspid annular plane systolic excursion/systolic pulmonary arterial pressure ratio < 0.55, right ventricular outflow tract acceleration time < 105 m/s, midsystolic notching of pulmonary artery, early diastolic pulmonary regurgitation velocity > 2.2 m/s, pulmonary artery diameter > 25 mm, inferior vena cava diameter > 21 mm, or right atrium area > 18 cm2.

Adapted with permission from Humbert M, Kovacs G, Hoeper MM, et al.; ESC/ERS Scientific Document Group. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3645.

Pulmonary hypertension found incidentally on echocardiography performed for other reasons should be taken seriously. Even mild elevations in pulmonary pressures increase mortality risk.12 Patients should be evaluated by the standard clinical pathway. If any combination of the workup suggests pulmonary hypertension, referral to a specialist is indicated. Right heart catheterization is the standard for diagnosing and classifying pulmonary hypertension and informs the treatment of pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Right heart catheterization may not be indicated when left-sided heart disease or lung disease is a sufficient explanation for clinical and echocardiography findings in the absence of suspicion for pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.1

Current guidelines recommend measuring pulmonary artery wedge pressure and pulmonary vascular resistance (PVR) during catheterization to determine if the source of hypertension is precapillary, postcapillary, or both because this affects the clinical classification group, prognosis, and treatment options.10,13 Pulmonary arterial wedge pressure is an indirect estimate of the pressure in the left atrium; therefore, it is normal when the source of the pulmonary hypertension originates in the precapillary pulmonary vascular bed, such as in pulmonary arterial hypertension, and is elevated when the source originates in the postcapillary vascular bed such as in left-sided HF. PVR is a calculated value of the difference between the mean pulmonary artery pressure and the left atrial pressure and is elevated in pulmonary arterial hypertension but normal in pulmonary hypertension caused by HF.

TREATMENT

Treatment of pulmonary hypertension focuses on the optimization of cardiopulmonary comorbidities, assessment of the risk of disease progression, and use of combination therapies. Patients are risk-stratified based on World Health Organization functional classification (Table 414), a 6-minute walking test, and BNP/N-terminal pro-BNP level, which further guide the treatment strategy.1,14 Medications targeting pulmonary hypertension are only available for pulmonary arterial hypertension and select patients with chronic thromboembolic pulmonary hypertension; treatment for other types focuses on the underlying etiology.

TABLE 4. World Health Organization Functional Classification

Class I
No limitation of physical activity
Ordinary activity does not cause undue dyspnea or fatigue, chest pain, or near syncope
Class III
Marked limitation of physical activity
Comfortable at rest, but minimal activity can cause dyspnea or fatigue, chest pain, or near syncope
Class II
Slight limitation of physical activity
Comfortable at rest, but ordinary activity can cause undue dyspnea or fatigue, chest pain, or near syncope
Class IV
Complete limitation of physical activity
Any activity causes symptoms
Dyspnea and fatigue may be present at rest
Manifest signs of right-sided heart failure

Note: Based on clinician assessment of patient’s symptoms. Treatments are tailored to functional class.

Information from reference 14.

PROGNOSIS

Pulmonary hypertension is a poor prognostic sign, with a 5-year estimated survival rate of 67% or less. The prognosis is slightly worse when pulmonary hypertension is secondary to lung disease and slightly better in chronic thromboembolic pulmonary hypertension.15 Although numerous prognostic factors exist for each etiology of pulmonary hypertension, functional capacity measured by a 6-minute walking test has predictive value across all groups.15 Regardless of the etiology, the development of pulmonary hypertension is associated with worsening symptoms and increased mortality.1,16

CLINICAL CLASSIFICATION GROUPS

Pulmonary hypertension is divided into five clinical groups based on etiology (Table 5).1,17

TABLE 5. Clinical Classification of Pulmonary Hypertension

Group 1: Pulmonary arterial hypertension
1.1 Idiopathic
1.1.1 Nonresponders at vasoreactivity testing
1.1.2 Acute responders at vasoreactivity testing
1.2 Heritable
1.3 Associated with drugs or toxins (e.g., methamphetamine, fenfluramine [Fintepla])
1.4 Associated with:
1.4.1 Connective tissue disease
1.4.2 HIV
1.4.3 Portal hypertension
1.4.4 Congenital heart disease
1.4.5 Schistosomiasis
1.5 Pulmonary arterial hypertension with features of venous/capillary involvement
1.6 Persistent pulmonary hypertension of the newborn
Group 2: Pulmonary hypertension associated with left-sided heart disease
2.1 Heart failure
2.1.1 Heart failure with preserved ejection fraction
2.1.2 Heart failure with reduced ejection fraction
2.2 Valvular heart disease
2.3 Congenital/acquired cardiovascular conditions leading to postcapillary pulmonary hypertension
Group 3: Pulmonary hypertension associated with lung disease or hypoxia
3.1 Obstructive lung disease or emphysema
3.2 Restrictive lung disease
3.3 Lung disease with mixed restrictive/obstructive pattern
3.4 Hypoventilation syndromes
3.5 Hypoxia without lung disease (e.g., high altitude)
3.6 Developmental lung disorders
Group 4: Pulmonary hypertension associated with pulmonary artery obstruction
4.1 Chronic thromboembolic pulmonary hypertension
4.2 Other pulmonary artery obstructions
Group 5: Pulmonary hypertension with unclear/multifactorial etiologies
5.1 Hematologic disorders
5.2 Systemic disorders
5.3 Metabolic disorders
5.4 Chronic renal failure
5.5 Pulmonary tumor thrombotic microangiopathy
5.6 Fibrosing mediastinitis

Note: Hypoxia and hypoventilation with hypercapnia contribute to the pathogenesis of pulmonary hypertension in obstructive sleep apnea, which may be associated with conditions in category 3.4 or 3.5.

Adapted with permission from Humbert M, Kovacs G, Hoeper MM, et al.; ESC/ERS Scientific Document Group. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3638, with additional information from reference 17.

Group 1: Pulmonary Arterial Hypertension

In pulmonary arterial hypertension, the intima, media, and adventitia of small pulmonary arteries and arterioles undergo remodeling, hyperplasia, and vasoconstriction, narrowing the lumen and increasing vascular resistance. Certain conditions increase the risk of pulmonary arterial hypertension; therefore, expert consensus recommends annual screening for pulmonary arterial hypertension with transthoracic echocardiography for patients at high risk1,11,18 (Table 61,18).

TABLE 6. Indications for Annual Screening With Echocardiography for Pulmonary Arterial Hypertension

Asymptomatic patients at high risk
Carrier of the bone morphogenetic protein receptor type II gene mutation (pulmonary arterial hypertension lifetime risk of 20%)
First-degree relative with heritable pulmonary arterial
hypertension
Systemic scleroderma
Undergoing assessment for a liver transplant
Symptomatic patients at high risk
Congenital heart disease
HIV
Methamphetamine use
Nonsystemic sclerosis connective tissue disease
Portal hypertension
Sickle cell disease

Information from references 1 and 18.

Pulmonary arterial hypertension requires right heart catheterization because pulmonary arterial wedge pressure and PVR are essential to a diagnosis. Patients should be referred to a specialized center for targeted treatment because therapy for pulmonary arterial hypertension is complex.1

Pulmonary arterial hypertension is treated with vasoactive medications that decrease the PVR (Table 71). Patients with idiopathic pulmonary arterial hypertension who have positive findings on vasoreactivity testing during right heart catheterization (acute vasodilation in response to short-acting pulmonary vasodilators) benefit from high-dose calcium channel blockers.1 Long-term oxygen therapy, including in-flight oxygen, is recommended in patients with pulmonary arterial hypertension with arterial blood oxygen pressure less than 60 mm Hg or oxygen saturation less than 90% at sea level.1 These patients may require an increase in the flow rate of supplemental oxygen if they travel to a high altitude.1,19,20

TABLE 7. Medications for Treatment of Pulmonary Arterial Hypertension

Medication and maximum target dosageComments and adverse effects
High-dose calcium channel blockers
Amlodipine, 15 to 30 mg once daily
Diltiazem, 120 to 360 mg twice daily
Felodipine, 15 to 30 mg once daily
Nifedipine, 20 to 60 mg twice or three times daily
Only for patients with positive findings on vasoreactive testing on right heart catheterization
Adverse effects: hypotension, edema
Endothelin-1 receptor antagonists
Ambrisentan (Letairis), once 10 mg once daily
Bosentan, 125 mg twice daily
Macitentan (Opsumit), 10 mg once daily
All are teratogenic
Other adverse effects
Ambrisentan: edema
Bosentan: multiple drug interactions; increased liver enzymes
Macitentan: anemia
Nitric oxide pathway activity:
Phosphodiesterase-5 inhibitors
Sildenafil, 20 mg three times daily
Tadalafil, 40 mg once daily
Soluble guanylate cyclase stimulator
Riociguat (Adempas), 2.5 mg three times daily
Improves exercise capacity, symptoms, hemodynamics, World Health Organization functional classification
Adverse effects: flushing, headache
Prostacyclin analogues
Epoprostenol (Veletri), 30 ng per kg per minute intravenously by infusion pump
Iloprost (Ventavis), 5 mcg 6 to 9 times daily, inhaled
Treprostinil, 0.25 mg twice daily, inhaled (Tyvaso), or intravenously or subcutaneously by infusion pump
Improves symptoms, exercise capacity, hemodynamics, mortality
Adverse effects of intravenous or subcutaneous medications are related to complications of the catheter or pump
Prostacyclin receptor agonist
Selexipag (Uptravi), 1,600 mcg twice daily
Reduces pulmonary vascular resistance
Reduced morbidity and mortality

Note: Initiate therapy at a low dosage and increase as tolerated to the target dosage. These medications are often prescribed in combination and always with expert consultation.

Adapted with permission from Humbert M, Kovacs G, Hoeper MM, et al.; ESC/ERS Scientific Document Group. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3664.

Targeted therapies and support strategies for pulmonary arterial hypertension have significantly improved survival for group 1. The 5-year survival rate for pulmonary arterial hypertension is estimated at 59%.15 Patients who do not respond to optimal medical therapy or have an elevated risk of mortality should be referred for a lung transplant. Patients who survive 1 year after a lung transplant have a median survival of 10 years.1,19

Group 2: Pulmonary Hypertension Associated With Left-Sided Heart Disease

Heart disease is the most common cause of pulmonary hypertension. Up to 72% of patients who have HF with reduced ejection fraction and 83% of patients who have HF with preserved ejection fraction have pulmonary hypertension.16 For patients with left-sided heart disease, elevated pressure in the left atrium can cause pulmonary venous congestion, leading to remodeling and vasoconstriction of pulmonary veins and pulmonary hypertension.

Therapy for patients with pulmonary hypertension secondary to cardiac disease emphasizes goal-directed treatment of the underlying disease process.1 Group 2 pulmonary hypertension has a 5-year survival rate comparable to pulmonary arterial hypertension.15

Group 3: Pulmonary Hypertension Associated With Lung Disease

The second most common cause of pulmonary hypertension is chronic obstructive lung disease. Group 3 pulmonary hypertension affects up to 5% of patients with severe chronic obstructive lung disease.1,2 In this group, destruction of lung tissue is accompanied by loss of pulmonary capillaries to increase PVR, while hypoxia causes pulmonary arteriole vasoconstriction.

Therapy for patients with pulmonary hypertension secondary to lung disease emphasizes goal-directed treatment of the underlying disease process.1 The 5-year survival rate is 38% for this group.15

Group 4: Pulmonary Hypertension Associated With Pulmonary Artery Obstruction

This is a rare but increasingly recognized cause of pulmonary hypertension in which pulmonary arteries become obstructed by chronic fibrotic clots, usually as a sequela of PE.1,3 Chronic thromboembolic pulmonary hypertension has a prevalence of 3 per 100,000 and occurs in up to 3% of patients who survive PE.21 These patients tend to be older and are more likely to have had recurrent, proximal PE.21 Chronic thromboembolic pulmonary hypertension should be suspected in patients with PE who are dyspneic after appropriate treatment. Diagnostic testing should include ventilation-perfusion imaging and pulmonary computed tomography angiography.

Surgical intervention via pulmonary endarterectomy is the treatment of choice for surgical candidates who have chronic thromboembolic pulmonary hypertension and accessible lesions. Balloon angioplasty may be an alternative for patients who cannot have surgery or for those with persistent pulmonary hypertension following pulmonary endarterectomy.22 Lifelong therapeutic anticoagulation is recommended for all patients who have chronic thromboembolic pulmonary hypertension.1,22

Group 5. Pulmonary Hypertension With Unclear or Multifactorial Etiologies

Group 5 includes pulmonary hypertension with unclear or multiple mechanisms and is related to conditions such as sickle cell anemia, glycogen storage disease, and chronic renal failure.1

OTHER CONSIDERATIONS

Exercise-Induced Pulmonary Hypertension

Some patients with mild pulmonary vascular disease or left-sided heart disease have normal pulmonary artery pressures at rest but develop pulmonary hypertension and exertional dyspnea with exercise. Although the clinical significance of this condition is unclear, exercise-induced pulmonary hypertension can lead to adverse outcomes and reduced survival in patients with systemic sclerosis or valvular heart disease.23,24

Exercise Rehabilitation

Patients with pulmonary hypertension who are stable should be referred for a supervised rehabilitation and exercise training program.1,19 Exercise training programs have demonstrated improvement in exercise capacity as measured by the 6-minute walking test, peak oxygen consumption, functional capacity, and quality-of-life scores, especially for patients in pulmonary hypertension groups 1 and 4.25,26

Advance Directives and Code Status

Discussions around goals of care are important in chronic diseases. Conversations about advance directive planning for patients with pulmonary hypertension should take place in a sensitive and caring manner, especially for patients with World Health Organization functional status class III or IV. Code status is important because in-hospital cardiopulmonary resuscitation has a low survival rate. Palliative care and hospice can help manage distressing symptoms as functional status worsens toward the end of life.1,19

Contraception and Pregnancy

Patients of childbearing potential who have pulmonary hypertension should be counseled to avoid pregnancy because of the high risk of worsening disease and maternal or fetal death.1 A 2020 systematic review demonstrated a 12% risk of maternal mortality and 23% risk of pregnancy loss in patients with pulmonary hypertension.27

There is no strong evidence or consensus to guide the best choice of contraception.28 It is prudent to use two forms of contraception (a primary mode plus a backup barrier of protection) because the implications of pregnancy are serious in patients with pulmonary hypertension. Low-dose combined oral contraceptives and emergency contraceptives are safe; however, bosentan, an endothelin receptor antagonist, reduces the effectiveness of hormonal contraception.1 Long-acting reversible contraceptives are another option with low failure rates.

If the patient is pregnant, referral to a pulmonary hypertension specialty center is indicated because outcomes are better when pulmonary hypertension is well controlled at pregnancy onset. Endothelin receptor antagonists, one of the primary treatments for pulmonary arterial hypertension, are teratogenic and not recommended.1

COVID-19

Patients with persistent dyspnea after recovery from COVID-19 are at increased risk of pulmonary hypertension. In one meta-analysis, 22% of patients developed pulmonary hypertension after intensive care unit admission due to COVID-19.29 Persistent pulmonary vasculopathy after COVID-19 may be a long-term sequela, regardless of the severity of the infection, that can lead to pulmonary arterial hypertension. Microthrombi in pulmonary vessels during COVID-19 may cause the development of chronic thromboembolic pulmonary hypertension.

More long-term studies are needed to assess the risk between COVID-19 and pulmonary hypertension. Pulmonary hypertension associated with COVID-19 has not yet been classified into one group, although it appears to be responsive to conventional pulmonary arterial hypertension treatment pathways.30

Immunizations

Patients who have pulmonary hypertension should receive immunizations for influenza, pneumococcus, and COVID-19.1,19

Mental Health

Patients with pulmonary hypertension often experience frustrating delays in diagnosis, uncomfortable and worsening symptoms that negatively affect lifestyle and income, complicated medical regimens, and the burden of having a serious disease. These factors influence emotional health. In 2021, a study of 217 patients found that 38.2% met the Diagnostic and Statistical Manual of Mental Disorders, 5th ed., criteria for a mental health disorder after receiving a diagnosis of pulmonary arterial hypertension, most commonly major depressive disorder (23%) and panic disorder (15.2%).31 Patients with pulmonary hypertension should be screened for depression and anxiety disorders using standard tools and encouraged to engage with support groups and counseling, and consider psychopharmacology.1,31

Perioperative Assessment

Pulmonary hypertension increases the risk of surgical complications, especially right-sided HF and death.1,32 The 2023 American Heart Association guideline for managing pulmonary hypertension in noncardiac surgery recommends a preoperative risk assessment to guide decision-making.32 For ideal intraoperative and postoperative management, the preoperative risk assessment and surgery should be performed at a center specializing in pulmonary hypertension. Preoperative evaluation includes repeat echocardiography, electrocardiography, laboratory tests, a 6-minute walking test, possible repeat right heart catheterization, and evaluation by anesthesia, a pulmonary hypertension specialist, or cardiology and pulmonology specialists.32 Pulmonary hypertension should be medically optimized for a minimum of 2 to 4 weeks before a planned surgery.19

CODING AND BILLING

Hierarchical condition category codes can be used when evaluating and caring for patients with pulmonary hypertension. Individual codes correspond to the clinical classification groups. Physicians should be specific in their documentation to ensure appropriate risk adjustment. Related International Classification of Diseases, Tenth Revision (ICD-10) codes include the following33:

  • Unspecified pulmonary hypertension: I27.20
  • Unspecified secondary pulmonary arterial hypertension: I27.21
  • Pulmonary hypertension groups 2 to 4: I27.22 to I27.24, respectively
  • Pulmonary hypertension group 5: I27.29.

This article updates previous articles on this topic by Dunlap and Weyer34; Stringham and Shah35; and Nauser and Stites.36

Data Sources: A PubMed search was completed using the key terms pulmonary hypertension and pulmonary arterial hypertension with diagnosis, pathophysiology, treatment, contraception, exercise, surgical assessment, COVID, and ESC/ERS. The search included meta-analyses, randomized controlled trials, clinical trials, and expert reviews. Essential Evidence Plus and the Cochrane database were also searched in addition to reference lists in retrieved articles. Also searched were the Library Services at the Naval Medical Center Portsmouth and Eastern Virginia Medical School. We critically reviewed studies that used patient categories such as race and/or gender but did not define how these categories were assigned, stating their limitations in the text. Search dates: June 2023, July 2023, January 2024, and June 2024.

The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the U.S. Navy, the U.S. Department of Defense, or the U.S. government.

KELLY LATIMER, MD, MPH, is an associate professor of family and community medicine at Eastern Virginia Medical School, Norfolk, Va.

MICHAEL LAYNE, MD, is an assistant professor of family and community medicine at Eastern Virginia Medical School.

MAYA PAYNE, MD, is a physician at MidAtlantic Long Term Care, Norfolk, Va. At the time this article was written, she was the ship physician for the USS John C. Stennis (CVN 74).

Address correspondence to Kelly Latimer, MD, MPH, at latimekm@evms.edu.

Author disclosure: No relevant financial relationships.

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