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Vascular Disorders of the Lung - Coggle Diagram
Vascular Disorders of the Lung
Pulmonary Oedema
Excessive liquid accumulation in the tissue and air spaces of the lungs
Pathophysiology
Movement of fluid between vascular + interstitial + alveolar compartments is in response to net hydrostatic and osmotic forces acting across vessel walls, modulated by permeability of the membrane
Haemodynamic Pulmonary Oedema Aetiology
Increased Hydrostatic pressure
Left heart failure
Decreased oncotic pressure
Low protein states
Cytotoxic Oedema
Injury to the capillaries of the alveolar septa
Capillary permeability
https://www.youtube.com/watch?v=oRDOUv6dEpE
Pulmonary Emboli
Venous Thromboembolism (VTE)
DVT and PE are known as VTE - a dangerous, potentially deadly medical condition
Types of Thrombosis
Deep vein thrombosis DVT
A blood clot that forms in a deep vein, usually the leg, groin or arm
Pulmonary embolism
A blood clot that occurs when DVT clot breaks free from a vein wall and travels to the lungs, blocking some or all of the blood supply
Can be fatal
Embolus
Detatched intravascular solid, liquid of gaseous mass carried by the blood to a site distant from the origin
Types
99% are thromboemboli (blood clot)
Bone marrow
Atheroma
Fat
Tumour
Foreign bodies (catheter tip, talc (ivdu))
Pulmonary embolus
Occulsion of the pulmonary artery/ branch by blood clot
Most common preventable cause of death in hospitalised patients
Contributing factors
Age
Female
Obesity
Congestive heart failure
Malignancy
Surgery
Childbirth
Polycythaemia
Oral contraceptive pill
Primary hypercoagulable states
Antithrombin III
Protein C deficiency
Mechanism
Promoting blood stasis
Damaging vascular endothelium
Activating coagulation factors
Clinically significant factors
Size of emboli and vessel
Number of emboli
Overall status of CVS
Release of vasoactive factors from platelets
Thomboxane A
Outcomes
Wost case scenario
Sudden death due to acute right heart failure due to embolus in main pulmonary artery - saddle embolus
Respiratory comprmise
Non prefused but ventilated segment of lung
Haemodynamic compromise
Increase resistance to blood flow owing toembolic obstruction
Infarct
Infarction
Small emboli may preceed larger one
10% cases result in infarction
3/4 affect lower lobes
Common in already inadequate circulation
Heart and lung disease
Chronically unresolves multiple small emboli may lead to pulmoanry hypertension + right heart strain / failure
Not usually excised unless clinically unsuspected
eg Unresolved infiltrate or nodular opacity
Classically a wedge shape with base on pleural surface
Central band necrosis with ghosts of lung architecture, haemorrhage, active fibroblasts at edge, squamous metaplasia, reactive atypia
Eventually a fibrous scar
Histopathology
Acute thromoembolus - RBCs enmeshed in fibrin, platelets,neutrophils arranged in alternating linear zones - Lines of Zahn
2-3 days - Organisation, ingrowth of fibroblasts + capillaries from vessel wall
Thrombus is replaced by fibrosis and recanalisation where original vessel lumen is divided into multiple canals
Pulmonary Hypertension
WHO Classification
Group I
Pulmonary arterial Hypertension PAH :lower_left_paintbrush:
Idiopathic IPAH
Familial FPAH
Associated with Other Diseases (APAH)
Scleroderma
Congenital shunts
Infection (HIV)
Drugs
Toxins
Portal hypertension
https://www.youtube.com/watch?v=fvMEoc7Voa0
Group II
Pulmonary hypertension associated with Left Heart Disease :<3:
Atrial valvular disease
Ventricular valvular disease
Group III
Pulmonary hypertension associated with Lung disease and/or hypoxia :wind_blowing_face:
COPD
ILD
Sleep apnoea
Alveolar hypoventilation
Chronic exposure to high altitude
Group IV
Pulmonary hypertension due to chronic thrombotic and/or embolic disease (PE) :red_circle:
Group V
Miscellaneous Disease
Mean artery pressure > 25mmHg at rest
35mmHg during exercise
Factors contributing to Small pulmonary Artery Obstruction
Vasoconstriction
Cellular proliferation and Fibrosis
Thrombosis
Pathogenesis
Dysfunction of endothelial cells
Decreased elaboration of substances that normally promote vasodilation:
Prostacyclin
Nitic acid
Vasoconstriction and platelet adhesion / adhesion
Production and release of GFs and Cytokines that induce cellular proliferation
Heath and Edwards Grades
Devised for patients with pulmonary hypertension secondary to congenital heart disease w/ left to right shunting
I) Medial hypertrophy
II) Intimal proliferation - mild
III) Intimal fibrosis - moderate
IV) Plexiform or dilation lesions, necrotising arterisis - severe
Clinical
Dyspnoea
Fatigue
Chest pain
Severe resp distress
Cyanosis
Right ventricular hypertrophy and strain
Death from cor pulmonale
Primary Pulmoanry Hyerptension
Primary plexifrom arteriopathy
Young women 20-40yrs
Dyspnoea, fatigue, some chest pain
Progression to resp distress, right ventricular hypertrophy and cor pulmonale
Rx
Vasodilators
Anticoagulants
Prostacyclins
Viagra
Pulmonary Haemorrhage and Vasculitis
Alveolar Haemorrhage Syndromes
Secondary alveolar haemorrhage
Venous congestions (mital valve disease)
Renal failure with volume overload
Thrombocytopenia
Other coagulopathies
Localised haemorrhage
Neoplasm
Fungas ball
Cavities
Granulomatosis with polyangiitis (GPA)
Wegner's granulomatosis (WG)
Automimmune disease effecting small-medium blood vessels
Triad
Granulomatous inflammation of upper and lower resp tract
Generalised vasculitis
Glomerulonephritis
Epidemiology
Lung most frequently affected
Middle aged adults
Symptoms
Chough
Chest pain
Haemoptysis
Systemic symptoms
Fever
Malaise
Weight loss
Co-morbidities
Renal failure
Anaemia
Sinusitis
Radiology
Multiple lung nodules
Maybe cavitating suggesting metastasis or cavitating abcesses
Serology
Antineutrophil cytoplasmic antibodies (ANCA)
C-ANCA diffuse granular cytoplasmic staining in WG
P-ANCA perinuclear staining pattern in microscopic polyangitis
Histopathology
Necrotising granulomatous inflammation
Dirty blue / basophilic necrosis
Large geographic areas of necrosis
Microabscesses - neutrophils and eosinophils too
Multinucleated giant cells
Necrotising vasculitis
Differential diagnosis
TB
Fungal infx
https://www.youtube.com/watch?v=3IwmGV_LJCw
Goodpasture's Syndrome
Anti Glomerular basement membrane disease
Autoimmune attacking collagen in basement membranes of lungs/kidneys
Pathogenesis
Anti-GBM antibodies bind to capillary basement membrane
Attract and activate WBCs (neutrophils)
WBCs attack vessel walls
Vessel wall inflammation
Glomerulonephritis and alveolar capillaritis
Short-lived production of circulating autoantibodies, directed against an antigen intrinsic to the glomerular basement membrane (GBM), in response to an unknown inciting stimulus
Epidemiology
Pulmonary involvement - alveolar haemorrage in 30-40%
PTs have underlying pulmonary involvement - injury
Smoking
Infx
Cocaine inhalation
Presentation
Shortness of breath
Cough
Hemoptysis (airway bleeding)
Pulmonary infiltrates on CXR due to haemoglobin in alveoli
Iron deficiency anaemia - possibly due to prolonged pulmonary bleeding
https://www.youtube.com/watch?v=Vko4lYWgwrs
Idiopathic pulmonary haemosiderosis IPH
Recurrent lung bleeding causing iron accumulation in lungs
Collagen Vascular Disease
Group of Autoimmune disorders affecting connective tissues
Especially lupus
Drugs / Inhalants
