Pulmonary hypertension
Pulmonary hypertension is a resting mean pulmonary artery pressure of 20 mmHg or greater on right heart catheterization, classified into five WHO groups by mechanism. Diagnosis requires screening echocardiography followed by confirmatory right heart catheterization, and management is group-specific, with PAH-targeted vasodilator therapy reserved for group 1 disease.
- Topic reviewThe full article, organized the way the exam asks it.
- ABIM-format MCQsVignettes with an explanation for every wrong answer.
- Key factsThe numbers and thresholds worth memorizing, up front.
- AI tutorMedBrain answers the question you're stuck on.
What this article covers
- Overview
A hemodynamic diagnosis, not a single disease: pulmonary hypertension (PH) is a resting mean pulmonary artery pressure (mPAP) of 20 mmHg or greater on right heart catheterization (RHC); normal resting mPAP is around 14 mmHg.
- Epidemiology
Diagnosis is delayed: PH symptoms are nonspecific, so average time from symptom onset to diagnosis exceeds 2 years.
- Risk factors
Left-sided heart disease (heart failure with reduced or preserved ejection fraction, mitral or aortic valvular disease including mitral-stenosis): the most common overall cause of PH (group 2).
- Pathophysiology
Group 1 (PAH): intrinsic small-vessel arteriopathy.
- Clinical features
Exertional dyspnea and lightheadedness are the earliest and most common symptoms, reflecting an RV that cannot augment output with exercise; their nonspecificity drives the long diagnostic delay.
- Differential diagnosis
Chronic thromboembolic disease without overt PH (pulmonary-embolism): dyspnea and hypoxemia from unresolved thrombus not yet meeting the mPAP threshold; V/Q scan and follow-up hemodynamics confirm.
- Diagnosis
PH is confirmed by right heart catheterization (RHC) showing a resting mPAP of 20 mmHg or greater; RHC also gives wedge pressure, cardiac output, and pulmonary vascular resistance, which assign the…
- Management
First, treat the underlying disease when PH is group 2 or group 3: for left heart disease, use cautious diuresis (furosemide 20-40 mg PO/IV daily, titrated to euvolemia) and arrhythmia control; for…
- Complications
Right ventricular failure (cor-pulmonale): the dominant driver of symptoms, functional decline, and death across all PH groups.
- Prognosis
Survival tracks WHO functional class and hemodynamic severity at diagnosis: worse functional impairment and higher pulmonary vascular resistance predict worse outcomes.
- Prevention
Screen systemic sclerosis patients for PAH periodically, since roughly 10% develop PAH, particularly with limited cutaneous disease.
Read the whole article
Subscribing to Internal Medicine opens every article, the full question bank and MedBrain, on the web, iPhone and Android.
Related articles
- Cor pulmonale
Cor pulmonale is right ventricular structural and functional impairment caused by group 3 pulmonary hypertension from chronic lung disease or hypoxemia. Diagnosis rests on echocardiographic screening confirmed by right heart catheterization, and management centers on treating the underlying lung disease, correcting hypoxemia, and cautious diuresis.
- Chronic obstructive pulmonary disease (COPD)
Chronic obstructive pulmonary disease is a persistent, usually progressive airflow obstruction driven mainly by cigarette smoke, confirmed by a postbronchodilator FEV1/forced vital capacity (FVC) ratio below 0.70 and staged by GOLD spirometric grade plus symptom/exacerbation group. Maintenance management is a stepwise ladder of smoking cessation, long-acting bronchodilators, add-on inhaled glucocorticoid, roflumilast, or azithromycin for frequent exacerbators, long-term oxygen for resting hypoxemia, and vaccination, with acute exacerbations managed as a separate protocol.
- Interstitial lung disease
Interstitial lung disease (ILD), also called diffuse parenchymal lung disease, is a diverse group of nonmalignant disorders causing inflammation and scarring of the alveolar interstitium, presenting as subacute to chronic exertional dyspnea and nonproductive cough. Diagnosis rests on pulmonary function testing plus high-resolution CT pattern recognition; management is exposure removal, glucocorticoids for inflammatory disease, antifibrotic therapy for progressive fibrotic disease, oxygen for hypoxemia, and early transplant referral.
- Pulmonary embolism
Pulmonary embolism is thrombus, usually from a lower-extremity deep vein thrombosis, obstructing the pulmonary arterial circulation and causing V/Q mismatch, right ventricular strain, and hypoxemia. Diagnosis follows Wells criteria (with PERC in low-risk patients) to sequence D-dimer, CT pulmonary angiography, and V/Q scanning, and management is risk-stratified anticoagulation escalating to thrombolysis or embolectomy for hemodynamic instability.
- Eisenmenger syndrome
Eisenmenger syndrome is the end stage of a large uncorrected left-to-right cardiac shunt, in which chronic pulmonary overcirculation causes irreversible pulmonary vascular disease and shunt reversal to right-to-left with cyanosis. The defect can no longer be closed; management is specialized supportive care, pulmonary vasodilators, and avoidance of destabilizing insults.
- Portopulmonary hypertension
Portopulmonary hypertension is pulmonary arterial hypertension arising in a patient with portal hypertension, confirmed by right heart catheterization showing an elevated mean pulmonary artery pressure and pulmonary vascular resistance with a normal or low wedge pressure. It carries a high mortality rate, and severity by mean pulmonary artery pressure and right ventricular function determines candidacy for liver transplantation, which is not curative on its own.
- Sleep apnea
Obstructive sleep apnea is repetitive upper airway collapse during sleep, diagnosed by an apnea-hypopnea index of 5 or more per hour with symptoms, or 15 or more per hour regardless of symptoms, and treated first with weight loss plus positive airway pressure.
- Mitral stenosis
Mitral stenosis is obstruction of left atrial emptying from a narrowed mitral valve, overwhelmingly rheumatic in origin. It causes left atrial hypertension, atrial fibrillation, and pulmonary congestion, with a diastolic rumble and opening snap; severe symptomatic disease is treated with percutaneous balloon valvuloplasty or valve surgery.