II. Epidemiology

  1. Incidence: 1-2 cases per 1 million (rare)
  2. Female predominance after Puberty in ratio of 1.7 to 1
  3. Most prevalent in ages 20 to 40 years old

III. Causes

  1. See Pulmonary Arterial Hypertension Causes
  2. Idiopathic or familial
  3. Genetic associations
    1. BMPR2 gene
    2. ALK1 gene
    3. Endoglin (with or without Hereditary Hemorrhagic Telangiectasia)

IV. Associated Conditions

  1. Collagen Vascular Disease (e.g.` Systemic Sclerosis)
  2. HIV Infection
  3. Includes Persistent Pulmonary Hypertension of the Newborn
  4. Predisposing medication use (see Pulmonary Arterial Hypertension Causes)

V. Pathophysiology

  1. Progressive narrowing of distal pulmonary arteries
  2. Processes
    1. Inflammation
    2. Hypercoagulability
    3. Pre-capillary pulmonary arteriole disorder
      1. Endothelial dysfunction
      2. Excess cell proliferation and dysfunctional apoptosis
      3. Vasconstriction
  3. Mediators
    1. Nitric oxide
    2. Prostacyclin
      1. Reduces platelet aggregation and causes vasodilation
      2. Reduced in PAH
    3. Cyclic guanosine monophosphate (cGMP)
      1. Vasodilator
      2. cGMP is rapidly degraded by PDE5 which present in right ventricle of PAH patients
    4. Endothelin-1
      1. Vasoconstricor and smooth muscle mitogen
      2. Increased in PAH
  4. Pulmonary circulation obstruction results from underlying factors
    1. Increased pulmonary vascular resistance
      1. Contrast with the normal low-resistance, high compliance pulmonary circulation
    2. Right Ventricular Afterload
      1. Right ventricle gradually compensates for increasing right Ventricular Afterload increases
      2. Right ventricular increased pressure and volume force interventricular septum to bow into left ventricle
        1. Results in decreased left ventricular filling and ejection fraction
      3. Ultimately compensation fails, resulting in right ventricular failure
      4. Right ventricle is unable to compensate for acute increases to Afterload resulting in PAH Crisis

VII. Signs

  1. See Pulmonary Hypertension
  2. Signs of underlying cause (e.g. Portal Hypertension, connective tissue disease)
  3. Signs of right ventricular Heart Failure
    1. Jugular Venous Distention
    2. Hepatomegaly
    3. Ascites
    4. Peripheral Edema
    5. Tricuspid regurgitation murmur

VIII. Diagnosis

  1. See Pulmonary Hypertension Diagnosis for labs, imaging and diagnostics

XI. Prevention

  1. Avoid predisposing factors (e.g. weight loss drugs)
  2. Avoid pregnancy (mortality risk approaches 50% in some studies)
  3. Vaccinations
    1. Influenza Vaccine
    2. Pneumococcal Vaccine
  4. Advanced Directives: Severe Pulmonary Arterial Hypertension
    1. Advise Do-Not-Resuscitate (very poor outcomes for ROSC)
    2. Discuss end-of-life wishes

XII. Prognosis

  1. Median survival from diagnosis
    1. Prior medications: 2.8 years
    2. New medications: may exceed 10 years

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Ontology: Idiopathic pulmonary hypertension (C0152171)

Definition (NCI) Increased blood pressure in the arteries of the lungs; the etiology is unknown.
Concepts Disease or Syndrome (T047)
ICD9 416.0
ICD10 I27.0
SnomedCT 155328008, 266293003, 26174007
English PPHT - Primary pulm hyperten, PPHT - Primary pulmonary hypertension, PULMONARY HYPERTENSION PRIMARY, Pulmonary hypertension primary, Primary pulmonary hypertension, primary pulmonary hypertension, primary pulmonary hypertension (diagnosis), Prim pulm hypertension, hypertension primary pulmonary, Hypertension;pulmonar;primary, pulmonary primary hypertension, Idiopathic pulmonary hypertension, IPAH, Idiopathic Pulmonary Hypertension, Idiopathic Pulmonary Arterial Hypertension, Pulmonary arterial hypertension, Primary Pulmonary Hypertension, Essential pulmonary hypertension, Primary pulmonary hypertension (disorder), Pulmonary hypertension, essential, Pulmonary hypertension, idiopathic, Pulmonary hypertension, primary, Hypertension;pulmonar;idiopath, idiopathic pulmonary hypertension
Spanish Hipertensión pulmonar primaria, hipertensión pulmonar primaria, hipertensión pulmonar primaria (trastorno), hipertensión pulmonar esencial, hipertensión pulmonar idiopática
Italian Ipertensione polmonare primitiva
French Hypertension pulmonaire primitive
Dutch pulmonaire hypertensie primair, primaire pulmonaire hypertensie, Primaire pulmonale hypertensie
German primaere pulmonale Hypertonie, pulmonale Hypertonie primaer, Primaere pulmonale Hypertonie
Portuguese Hipertensão pulmonar primária
Japanese ゲンパツセイハイコウケツアツショウ, 原発性肺高血圧症
Czech Primární plicní hypertenze, Plicní hypertenze primární
Korean 원발성 폐성 고혈압
Hungarian Primaer pulmonalis hypertonia

Ontology: Familial pulmonary arterial hypertension (C1701939)

Concepts Disease or Syndrome (T047)
English Familial pulmonary arterial hypertension
Spanish Hipertensión pulmonar arterial primaria familiar
Italian Ipertensione arteriosa polmonare familiare
French Hypertension artérielle pulmonaire familiale
Dutch familiaire pulmonale arteriële hypertensie
German familiaere Pulmonalarterienhypertonie
Portuguese Hipertensão arterial pulmonar familiar
Czech Familiální pulmonální arteriální hypertenze
Hungarian Familiaris arterialis pulmonalis hypertonia
Japanese 家族性肺動脈性肺高血圧症, カゾクセイハイドウミャクセイハイコウケツアツショウ

Ontology: Pulmonary hypertensive crisis (C2363966)

Concepts Disease or Syndrome (T047)
English Pulmonary hypertensive crisis
Spanish Crisis hipertensiva pulmonar
Dutch pulmonale hypertensieve crisis
Italian Crisi ipertensiva polmonare
German pulmonale Hypertonie-Krise
French Poussée hypertensive pulmonaire
Portuguese Crise hipertensiva pulmonar
Czech Pulmonární hypertenzní krize
Japanese ハイコウケツアツクリーゼ, 肺高血圧クリーゼ
Hungarian Pulmonális hypertensiv krizis

Ontology: Pulmonary arterial hypertension (C2973725)

Concepts Disease or Syndrome (T047)
SnomedCT 11399002
English Pulmonary arterial hypertension, Pulmonary hypertensive arterial disease, Hypertensive pulmonary arterial disease, Pulmonary hypertensive arterial disease (disorder), hypertension; lesser circulation, lesser circulation; hypertension
Spanish Hipertensión arterial pulmonar, hipertensión arterial pulmonar, enfermedad hipertensiva de arteria pulmonar (trastorno), hipertensión arterial pulmonar (trastorno), enfermedad hipertensiva de arteria pulmonar
Italian Ipertensione arteriosa polmonare
French Hypertension artérielle pulmonaire
Dutch pulmonale arteriële hypertensie, hypertensie; kleine circulatie, kleine circulatie; hypertensie
German pulmonale arterielle Hypertonie
Portuguese Hipertensão arterial pulmonar
Czech Pulmonální arteriální hypertenze
Hungarian Pulmonalis arterialis hypertonia
Japanese 肺動脈性肺高血圧症, ハイドウミャクセイハイコウケツアツショウ

Ontology: Idiopathic pulmonary arterial hypertension (C3203102)

Concepts Disease or Syndrome (T047)
MSH D065627
SnomedCT 697898008
Spanish Hipertensión pulmonar arterial primaria idiopática, hipertensión de arteria pulmonar idiopática, hipertensión de arteria pulmonar idiopática (trastorno)
Italian Ipertensione arteriosa polmonare idiopatica
French Hypertension artérielle pulmonaire idiopathique
Dutch idiopathische pulmonale arteriële hypertensie
German idiopathische Pulmonalerterienhypertonie
Portuguese Hipertensão arterial pulmonar idiopática
Czech Idiopatická pulmonální arteriální hypertenze
Hungarian Idiopathiás arterialis pulmonalis hypertonia
English Idiopathic pulmonary arterial hypertension, Idiopathic pulmonary arterial hypertension (disorder), Idiopathic Pulmonary Arterial Hypertension, Idiopathic Pulmonary Hypertension, Hypertensions, Idiopathic Pulmonary, Primary Pulmonary Hypertension, Hypertensions, Primary Pulmonary, Primary Pulmonary Hypertensions, Hypertension, Primary Pulmonary, Pulmonary Hypertensions, Primary, Idiopathic Pulmonary Hypertensions, Pulmonary Hypertensions, Idiopathic, Pulmonary Hypertension, Primary, 1, Hypertension, Idiopathic Pulmonary, Pulmonary Hypertension, Idiopathic, Pulmonary Hypertension, Primary
Japanese 特発性肺動脈性肺高血圧症, トクハツセイハイドウミャクセイハイコウケツアツショウ