//fpnotebook.com/
Pulmonary Hypertension
Aka: Pulmonary Hypertension
- See Also
- Pulmonary Arterial Hypertension
- Pulmonary Hypertension Causes
- Pulmonary Hypertension Diagnosis
- Pulmonary Hypertension Management
- Cor Pulmonale
- Epidemiology
- Prevalence increases with age (Prevalence may approach 10-20%)
- Definitions
- Pulmonary Hypertension
- Pulmonary artery systolic pressure >30 mmHg
- Pulmonary artery mean pressure >25 mmHg (by cardiac catheterization)
- Pulmonary Arterial Hypertension (Previously known as Primary Pulmonary Hypertension)
- Idiopathic Pulmonary Hypertension
- Secondary Pulmonary Hypertension
- Secondary to one of Pulmonary Hypertension Causes
- Cor Pulmonale
- Right ventricular failure
- Secondary to respiratory cause of Pulmonary Hypertension
- Pathophysiology
- Pulmonary vascular bed pressures (25/10) are typically much lower than systemic pressures (120/80)
- Pulmonary vasculature changes in response to increased pressure
- Pulmonary artery medial hypertrophy
- Intimal fibrosis
- Fibrinoid necrosis
- Intravascular thrombus formation
- Types: Acute Pulmonary Hypertension
- Acute increase in main pulmonary artery pressures
- May result from many acute insults (e.g. Hypoxia, hypercapnia, acidosis, left Heart Failure)
- Types: Chronic Pulmonary Hypertension (replaces old system of primary versus secondary Pulmonary Hypertension)
- Pulmonary artery Hypertension (WHO Group 1, rare)
- Least common Pulmonary Hypertension type, but most specific management options
- Mechanism
- Progressive distal pulmonary artery narrowing
- Causes
- Idiopathic or familial
- Includes Persistent Pulmonary Hypertension of the Newborn
- Risk factors include collagen vascular disease including Systemic Sclerosis, as well as HIV Infection
- Pulmonary Hypertension associated with left heart disease (WHO Group 2, most common)
- Mechanism
- Pulmonary venous congestion with Vasoconstriction and venous remodeling
- Causes
- Left-sided valvular heart disease
- Left-sided atrial or ventricular heart disease
- Left Heart Failure (>25% have Pulmonary Hypertension)
- Pulmonary Hypertension associated with lung disease, Hypoxemia or both (WHO Group 3, common)
- Mechanism
- Alveolar capillary bed destruction or chronic hypoxic Vasoconstriction
- Causes
- Chronic Obstructive Lung Disease
- Pulmonary Hypertension in 20% of hospitalized COPD and 50% of end-stage COPD
- Interstitial Lung Disease
- Sleep Apnea
- Chronic high altitude exposure
- Pulmonary Hypertension associated with chronic thromboembolic disease (WHO Group 4)
- Mechanism
- Vasoconstriction and pulmonary arterial bed remodeling in response to large vessel obstruction
- Causes
- Thromboembolism of proximal or distal pulmonary arteries (3.8% with PH at 2 years after PE)
- Thromboembolism not due to thrombi (e.g. tumor, Parasites)
- Miscellaneous Pulmonary Hypertension (WHO Group 5)
- Sarcoidosis
- Pulmonary vessel compression
- Causes
- See Pulmonary Hypertension Causes
- Associated Conditions
- Chronic Hemolytic Anemia
- Congenital Heart Disease
- Connective Tissue Disease
- Human Immunodeficiency Virus (HIV Infection)
- Portal Hypertension
- Persistent Pulmonary Hypertension of the Newborn
- Symptoms
- Common
- Progressive Dyspnea on exertion (Exercise intolerance)
- Fatigue (or generalized weakness)
- Syncope (especially Syncope on exertion)
- Less Common
- Hoarseness (Oertner Syndrome)
- Pulmonary artery compress left recurrent laryngeal
- Angina-type exertional Chest Pain
- Rare
- Cough
- Hemoptysis
- Raynaud's Phenomenon (2%)
- Signs
- Jugular Vein distention
- Prominent right ventricular impulse
- Fixed Split S2 Heart Sound
- Accentuated second pulmonic valve component (P2)
- Louder than the aortic second sound (A2)
- A2 remains louder as stethoscope moved to apex
- Right Ventricular Fourth Heart Sound (S4 Heart Sound)
- Right-sided Third Heart Sound (S3 Heart Sound)
- Indicates advanced disease
- Associated with poor prognosis
- Tricuspid insufficiency murmur
- More prominent as right ventricle dilates
- Hepatomegaly
- Peripheral Edema
- Diagnosis
- Challenging diagnosis
- Diagnosis is often delayed 2-4 years after symptom onset
- Despite multiple primary care and specialist visits
- More significant cases may present with right Heart Failure
- Lower extremity edema
- Jugular Venous Distention
- See Pulmonary Hypertension Diagnosis
- Mean Pulmonary Artery Pressure (PAP)
- Normal: <15 mmHg
- Pulmonary Hypertension
- Rest: 25 mmHg or higher
- Exercise: 30 mmHg or higher
- Pulmonary capillary wedge pressure (PCWP)
- PCWP <15 mmHg: Pre-capillary Pulmonary Hypertension
- All Causes of Pulmonary Hypertension
- EXCEPT those due to left heart disease (WHO Groups 1,3,4,5)
- PCWP >15 mmHg: Post-capillary Pulmonary Hypertension
- Left heart disease related causes (WHO Group 2 Pulmonary Hypertension)
- Labs: Initial Dyspnea Evaluation
- Complete Blood Count (CBC)
- Evaluate for Anemia (high output Heart Failure)
- Comprehensive Metabolic Panel (electrolytes, Renal Function tests, Liver Function Tests)
- B-Type Natriuretic Peptide (BNP)
- Serum Troponin
- Thyroid Stimulating Hormone (TSH)
- Evaluate for Hyperthyroidism (high output Heart Failure)
- Other tests to consider at initial presentation
- HIV Test
- Oximetry (6 Minute Walk Test)
- Imaging
- Chest XRay
- Cardiomegaly
- Right atrial enlargement
- Mediastinal narrowing (lateral view)
- Right Ventricular Hypertrophy
- Pulmonary vasculature pruning (vessels taper off quickly at hilum)
- Diagnostics
- See Pulmonary Hypertension Diagnosis
- Echocardiogram
- First-line testing for suspected cases
- Estimated pulmonary pressure >35 to 40 mmHg is consistent with Pulmonary Hypertension
- Also evaluates right ventricular function
- Right Ventricular Hypertrophy
- Right ventricular wall thickening (suspicious if >5mm, RVH if >10mm)
- Right ventricle pushes into left ventricle on PSAX View (D Sign)
- Electrocardiogram (EKG)
- See Right Ventricular Strain EKG Pattern
- Right Ventricular Hypertrophy
- Right Bundle Branch Block
- Right strain pattern (S1-Q3-T3 pattern)
- T Wave Inversion V1-V4
- ST Elevation in aVR
- Sinus Tachycardia
- Atrial Fibrillation
- New onset rate control may be challenging in Pulmonary Hypertension and risk decompensation
- Pulmonary Function Tests
- Evaluate for other Dyspnea Causes
- Evaluate WHO functional class status
- Six minute walk test (with oximetry)
- Evaluation: Screening of high risk groups
- Protocol
- Annual Echocardiogram
- Reflex to right heart catheterization if positve Echocardiogram for pulmonary artery Hypertension
- Indications
- BMPR2 gene positive (screen first degree relatives for gene)
- HIV Infection
- Portal Hypertension (if considering liver transplantation)
- Prior appetite suppressant medication such as Fenfluramine if symptoms
- Sickle Cell Disease
- Systemic Sclerosis
- Congenital Heart Disease with shunt
- Recent Acute Pulmonary Embolism with persistent symptoms at 3 months
- Consider ventilation-perfusion scan with reflex to Pulmonary Angiography if positive
- Differential Diagnosis
- See Causes of Dyspnea with Clear Lung Sounds
- Management
- See Pulmonary Hypertension Management
- Complications
- Increased mortality
- Five year mortality: 36%
- In comorbid conditions (e.g. COPD), Pulmonary Hypertension is among greatest risks for increased mortality
- Right Ventricular Failure
- Common outcome of persistently increased pulmonary artery pressures regardless of cause
- Thin walled right ventricle responds poorly to high pressures and leads to right Heart Failure
- Secondary to persistent Pulmonary Hypertension
- Cor Pulmonale: Subtype of right ventricular failure
- Second to respiratory cause of Pulmonary Hypertension
- Resources
- Pulmonary Hypertension Association
- https://www.phassociation.org/
- References
- Orman, Greenwood and Swaminathan in Herbert (2016) EM:Rap 16(10): 9-11
- Dunlap (2016) Am Fam Physician 94(6):463-9 [PubMed]
- Gaine (2000) JAMA 284:3160-8 [PubMed]
- Greenwold (2015) Emerg Med Clin North Am 33(3): 623-43 +PMID:26226870 [PubMed]
- Nauser (2001) Am Fam Physician 63(9):1789-98 [PubMed]
- Rubin (1997) N Engl J Med 336:111-7 [PubMed]
- Rubin (1993) Chest 104:236-50 [PubMed]
- McLaughlin (2009) Circulation 119(16): 2250-94 [PubMed]
- Stringham (2010) Am Fam Physician 82(4): 370-7 [PubMed]