II. Epidemiology

  1. Prevalence increases with age (Prevalence may approach 10-20%)

III. Definitions

  1. Pulmonary Hypertension
    1. Pulmonary artery systolic pressure >30 mmHg
    2. Pulmonary artery mean pressure >25 mmHg (by cardiac catheterization)
  2. Pulmonary Arterial Hypertension (Previously known as Primary Pulmonary Hypertension)
    1. Idiopathic Pulmonary Hypertension
  3. Secondary Pulmonary Hypertension
    1. Secondary to one of Pulmonary Hypertension Causes
  4. Cor Pulmonale
    1. Right ventricular failure
    2. Secondary to respiratory cause of Pulmonary Hypertension

IV. Pathophysiology

  1. Pulmonary vascular bed pressures (25/10) are typically much lower than systemic pressures (120/80)
  2. Pulmonary vasculature changes in response to increased pressure
    1. Pulmonary artery medial hypertrophy
    2. Intimal fibrosis
    3. Fibrinoid necrosis
    4. Intravascular thrombus formation

V. Types: Acute Pulmonary Hypertension

  1. Acute increase in main pulmonary artery pressures
  2. May result from many acute insults (e.g. Hypoxia, hypercapnia, acidosis, left Heart Failure)

VI. Types: Chronic Pulmonary Hypertension (replaces old system of primary versus secondary Pulmonary Hypertension)

  1. Pulmonary artery Hypertension (WHO Group 1, rare)
    1. Least common Pulmonary Hypertension type, but most specific management options
    2. Mechanism
      1. Progressive distal pulmonary artery narrowing
    3. Causes
      1. Idiopathic or familial
      2. Includes Persistent Pulmonary Hypertension of the Newborn
      3. Risk factors include collagen vascular disease including Systemic Sclerosis, as well as HIV Infection
  2. Pulmonary Hypertension associated with left heart disease (WHO Group 2, most common)
    1. Mechanism
      1. Pulmonary venous congestion with Vasoconstriction and venous remodeling
    2. Causes
      1. Left-sided valvular heart disease
      2. Left-sided atrial or ventricular heart disease
      3. Left Heart Failure (>25% have Pulmonary Hypertension)
  3. Pulmonary Hypertension associated with lung disease, Hypoxemia or both (WHO Group 3, common)
    1. Mechanism
      1. Alveolar capillary bed destruction or chronic hypoxic Vasoconstriction
    2. Causes
      1. Chronic Obstructive Lung Disease
        1. Pulmonary Hypertension in 20% of hospitalized COPD and 50% of end-stage COPD
      2. Interstitial Lung Disease
      3. Sleep Apnea
      4. Chronic high altitude exposure
  4. Pulmonary Hypertension associated with chronic thromboembolic disease (WHO Group 4)
    1. Mechanism
      1. Vasoconstriction and pulmonary arterial bed remodeling in response to large vessel obstruction
    2. Causes
      1. Thromboembolism of proximal or distal pulmonary arteries (3.8% with PH at 2 years after PE)
      2. Thromboembolism not due to thrombi (e.g. tumor, Parasites)
  5. Miscellaneous Pulmonary Hypertension (WHO Group 5)
    1. Sarcoidosis
    2. Pulmonary vessel compression

IX. Symptoms

  1. Common
    1. Progressive Dyspnea on exertion (Exercise intolerance)
    2. Fatigue (or generalized weakness)
    3. Syncope (especially Syncope on exertion)
  2. Less Common
    1. Hoarseness (Oertner Syndrome)
      1. Pulmonary artery compress left recurrent laryngeal
    2. Angina-type exertional Chest Pain
  3. Rare
    1. Cough
    2. Hemoptysis
    3. Raynaud's Phenomenon (2%)

X. Signs

  1. Jugular Vein distention
  2. Prominent right ventricular impulse
  3. Fixed Split S2 Heart Sound
    1. Accentuated second pulmonic valve component (P2)
      1. Louder than the aortic second sound (A2)
    2. A2 remains louder as stethoscope moved to apex
  4. Right Ventricular Fourth Heart Sound (S4 Heart Sound)
  5. Right-sided Third Heart Sound (S3 Heart Sound)
    1. Indicates advanced disease
    2. Associated with poor prognosis
  6. Tricuspid insufficiency murmur
    1. More prominent as right ventricle dilates
  7. Hepatomegaly
  8. Peripheral Edema

XI. Diagnosis

  1. Challenging diagnosis
    1. Diagnosis is often delayed 2-4 years after symptom onset
      1. Despite multiple primary care and specialist visits
    2. More significant cases may present with right Heart Failure
      1. Lower extremity edema
      2. Jugular Venous Distention
  2. See Pulmonary Hypertension Diagnosis
  3. Mean Pulmonary Artery Pressure (PAP)
    1. Normal: <15 mmHg
    2. Pulmonary Hypertension
      1. Rest: 25 mmHg or higher
      2. Exercise: 30 mmHg or higher
  4. Pulmonary capillary wedge pressure (PCWP)
    1. PCWP <15 mmHg: Pre-capillary Pulmonary Hypertension
      1. All Causes of Pulmonary Hypertension
      2. EXCEPT those due to left heart disease (WHO Groups 1,3,4,5)
    2. PCWP >15 mmHg: Post-capillary Pulmonary Hypertension
      1. Left heart disease related causes (WHO Group 2 Pulmonary Hypertension)

XII. Labs: Initial Dyspnea Evaluation

  1. Complete Blood Count (CBC)
    1. Evaluate for Anemia (high output Heart Failure)
  2. Comprehensive Metabolic Panel (electrolytes, Renal Function tests, Liver Function Tests)
  3. B-Type Natriuretic Peptide (BNP)
  4. Serum Troponin
  5. Thyroid Stimulating Hormone (TSH)
    1. Evaluate for Hyperthyroidism (high output Heart Failure)
  6. Other tests to consider at initial presentation
    1. HIV Test
    2. Oximetry (6 Minute Walk Test)

XIII. Imaging

  1. Chest XRay
    1. Cardiomegaly
    2. Right atrial enlargement
    3. Mediastinal narrowing (lateral view)
      1. Right Ventricular Hypertrophy
      2. Pulmonary vasculature pruning (vessels taper off quickly at hilum)

XIV. Diagnostics

  1. See Pulmonary Hypertension Diagnosis
  2. Echocardiogram
    1. First-line testing for suspected cases
    2. Estimated pulmonary pressure >35 to 40 mmHg is consistent with Pulmonary Hypertension
    3. Also evaluates right ventricular function
    4. Right Ventricular Hypertrophy
      1. Right ventricular wall thickening (suspicious if >5mm, RVH if >10mm)
      2. Right ventricle pushes into left ventricle on PSAX View (D Sign)
  3. Electrocardiogram (EKG)
    1. See Right Ventricular Strain EKG Pattern
    2. Right Ventricular Hypertrophy
    3. Right Bundle Branch Block
    4. Right strain pattern (S1-Q3-T3 pattern)
    5. T Wave Inversion V1-V4
    6. ST Elevation in aVR
    7. Sinus Tachycardia
    8. Atrial Fibrillation
      1. New onset rate control may be challenging in Pulmonary Hypertension and risk decompensation
  4. Pulmonary Function Tests
    1. Evaluate for other Dyspnea Causes
  5. Evaluate WHO functional class status
    1. Six minute walk test (with oximetry)

XV. Evaluation: Screening of high risk groups

  1. Protocol
    1. Annual Echocardiogram
    2. Reflex to right heart catheterization if positve Echocardiogram for pulmonary artery Hypertension
  2. Indications
    1. BMPR2 gene positive (screen first degree relatives for gene)
    2. HIV Infection
    3. Portal Hypertension (if considering liver transplantation)
    4. Prior appetite suppressant medication such as Fenfluramine if symptoms
    5. Sickle Cell Disease
    6. Systemic Sclerosis
    7. Congenital Heart Disease with shunt
    8. Recent Acute Pulmonary Embolism with persistent symptoms at 3 months
      1. Consider ventilation-perfusion scan with reflex to Pulmonary Angiography if positive

XVI. Differential Diagnosis

XVII. Management

XVIII. Complications

  1. Increased mortality
    1. Five year mortality: 36%
    2. In comorbid conditions (e.g. COPD), Pulmonary Hypertension is among greatest risks for increased mortality
  2. Right Ventricular Failure
    1. Common outcome of persistently increased pulmonary artery pressures regardless of cause
      1. Thin walled right ventricle responds poorly to high pressures and leads to right Heart Failure
    2. Secondary to persistent Pulmonary Hypertension
    3. Cor Pulmonale: Subtype of right ventricular failure
      1. Second to respiratory cause of Pulmonary Hypertension

XIX. Resources

  1. Pulmonary Hypertension Association
    1. https://www.phassociation.org/

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Ontology: Pulmonary Hypertension (C0020542)

Definition (MEDLINEPLUS)

Pulmonary hypertension (PH) is high blood pressure in the arteries to your lungs. It is a serious condition. If you have it, the blood vessels that carry blood from your heart to your lungs become hard and narrow. Your heart has to work harder to pump the blood through. Over time, your heart weakens and cannot do its job and you can develop heart failure.

Symptoms of PH include

  • Shortness of breath during routine activity, such as climbing two flights of stairs
  • Tiredness
  • Chest pain
  • A racing heartbeat
  • Pain on the upper right side of the abdomen
  • Decreased appetite

As PH worsens, you may find it hard to do any physical activities.

There are two main kinds of PH. One runs in families or appears for no known reason. The other kind is related to another condition, usually heart or lung disease.

There is no cure for PH. Treatments can control symptoms. They involve treating the heart or lung disease, medicines, oxygen, and sometimes lung transplantation.

NIH: National Heart, Lung, and Blood Institute

Definition (NCI_CTCAE) A disorder characterized by an increase in pressure within the pulmonary circulation due to lung or heart disorder.
Definition (NCI) Increased pressure within the pulmonary circulation due to lung or heart disorder.
Definition (MSH) Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.
Definition (CSP) increased pressure with the the pulmonary circulation, usually secondary to cardiac or pulmonary disease.
Concepts Disease or Syndrome (T047)
MSH D006976
ICD10 I27.2
SnomedCT 155328008, 266293003, 70995007
LNC LP128702-0, MTHU040643
English Hypertension, Pulmonary, HYPERTENSION PULMONARY, Pulmonary Hypertension, HYPERTENSION PULM, PULM HYPERTENSION, pulmonary hypertension (diagnosis), pulmonary hypertension, Hypertension pulmonary, Pulmonary hypertension NOS, Hypertension, Pulmonary [Disease/Finding], pulmonary hypertension disorder, hypertension caused by lung disease, Pulmonary hypertensions, Pulmonary hypertension, PHT - Pulmonary hypertension, Pulmonary hypertension (disorder), hypertension; pulmonary, pulmonary; hypertension, Pulmonary hypertension, NOS
Portuguese HIPERTENSAO PULMONAR, Hipertensão pulmonar NE, Hipertensão pulmonar, Hipertensões pulmonares, Hipertensão Pulmonar
Spanish HIPERTENSION PULMONAR, Hipertensión pulmonar NEOM, hipertensión pulmonar (trastorno), hipertensión pulmonar, Hipertensiones pulmonares, Hipertensión pulmonar, Hipertensión Pulmonar
Italian Ipertensioni polmonari, Ipertensione polmonare NAS, Ipertensione polmonare
Dutch hypertensie pulmonaal, pulmonaire hypertensie NAO, hypertensie; pulmonaal, pulmonaal; hypertensie, pulmonaire hypertensie, pulmonale hypertensies, Hypertensie, pulmonale, Pulmonale hypertensie
French Hypertension pulmonaire SAI, HTAP, HYPERTENSION PULMONAIRE, Hypertension artérielle pulmonaire, Hypertension pulmonaire, Hypertensions pulmonaires
German pulmonale Hypertonie NNB, Hypertonie pulmonal, HYPERTONIE PULMONAL, Pulmonale Hypertonien, pulmonale Hypertonie, Hypertension, pulmonale, Hypertonie, pulmonale
Japanese 肺高血圧症NOS, ハイコウケツアツショウNOS, ハイコウケツアツショウ, 肺高血圧, 高血圧症-肺, 肺高血圧症, Ayerza症候群
Swedish Lunghypertoni
Finnish Keuhkoverenpainetauti
Russian SINDROM AIERSY, GIPERTENZIIA LEGOCHNAIA, ГИПЕРТЕНЗИЯ ЛЕГОЧНАЯ, СИНДРОМ АЙЕРСЫ
Czech Plicní hypertenze, Plicní hypertenze NOS, Hypertenze plicní, Pulmonální hypertenze, plicní hypertenze, hypertenze plicní
Croatian HIPERTENZIJA, PLUĆNA
Polish Nadciśnienie płucne, Zespół Ayerzy
Hungarian Pulmonalis hypertonia k.m.n., Pulmonalis hypertonia, Pulmonális hypertoniák, Pulmonalis hipertonia
Norwegian Pulmonal hypertensjon, Hypertoni, pulmonal, Hypertensjon, pulmonal