Pulmonary Hypertension & Pulmonary Embolisms Flashcards
What is Cor pulmonale
Impairment of RV function due to respiratory disease
Primary disease of pulmonary arterial vessels [4]
Recurrent PE
Pulmonary vasculitis
Sickle cell anaemia
Altitude sickness
Secondary pulmonary arterial compromise [8]
COPD Parenchymal - pulmonary fibrosis, pneumoconiosis, CF Chronic hypoventilation OSAS Thoracic deformity Alveolar capillary dysplasia Bronchopulmonary dysplasia
What are miscellaneous causes? [3]
Elaborate on conditions that cause chronic hypoventilation > HTN [3]
Systemic sclerosis - cause fibrosis
SLE
Hepatic cirrhosis
Chronic hypoventilation
- Polio
- MG
- MND
Symptoms of pulmonary hypertension [5]
Worsening SOB at rest/exertional, fatigue, syncope
Peripheral edema
Angina type chest discomfort unresponsive to GTN
Haemoptysis
Hepatic congestion
Pulmonary hypertension complications [3]
Right heart failure, SCD
HSM, ascites, peripheral oedema
Pulmonary regurgitation
Problems during childbirth
How do you investigate Cor Pulmonale [5]
CXR ECHO - RV enlarged and dilated pulmonary artery ECG - RVH / RAD / RBBB ECHO to estimate pulmonary pressure V/Q Scan CT pulmonary angiogram (diagnostic)
A1AT deficiency
Autoab (CTD)
Thrombophilia (chronic VTE)
Spirometry (COPD)
Pathophysiology of cor pulmonale [4]
- VQ mismatch
- Chronic hypercapnia and respiratory acidosis
- Anatomical disruption of pulmonary vascular bed
- Increased blood viscosity
Systemic Examination: cor pulmonale [8]
Cyanosis, plethora Markedly hyper-expanded chest Laboured respiratory effort Systolic bruits over lung fields Marked hepatojugular reflux Hepatomegaly Liver pulsaltility Peripheral pitting edema
Precordial examination: cor pulmonale signs
Left systolic parasternal heave
Elevated JVP
S3 or S4 pan systolic murmur of tricuspid regurgitation
Split S2 with loud pulmonary component
Ejection murmur, sharp ejection click over pulmonary artery
Diastolic pulm regurg murmur over pulmonary artery
Causes of pulmonary arterial hypertension [4]
Heritable
Drug, toxin induced
Secondary to other diseases
Connective Tissue disorders
Causes:
What is an inherited cause?
What diseases can cause secondary pulmonary hypertension [5]
What CTDs can cause? [5]
Genetic: rare autosomal dominant BMPR2 mutation or other mutations
Secondary to:
- HIV
- portal HTN
- congenital heart disease
- schistosomiasis
- chronic haemolytic anaemia
CTD:
- CREST, systemic sclerosis
- Sjogren’s, RA, SLE
What are 3 important investigations in pulmonary hypertension
Right heart catheterisation
ECHO (RV fx, estimate pressure)
Vasoreactivity testing (responsiveness to CCB)
BNP
Who is high risk for PAH?
- Rapid disease progression
- RHF
- Functional class IV
- 6 min walk distance <400m
- Very elevated BNP
- Pericardial effusion and/or significant RV dysfunction on echo, high right atrial pressure and low cardiac index on right heart catheterisation
Management of PAH is dependent on vasoreactivity testing and risk of PAH:
Positive response [2]
Negative response [2]
Low risk PAH [2]
Positive response: CCB eg amlodipine or diltiazem
Negative response but high risk: Prostacyclin analogue given via long term indwelling CVC
Low risk PAH:
- endothelin receptor antagonist eg BOSENTAN
- or PDEi eg SILDENAFIL
Pharmacological adjuncts for PAH [5]
Surgical interventions [2]
Anticoagulation in PE
LTOT
Diuretics
Digoxin (if SVTs and/or RHF)
Annual influenza vaccination
Pulmonary endarterectomy
Transplantation
Side effect
Endothelin receptor antagonist BOSENTAN
Deranged LFTs
Investigation
Blood tests in Pulmonary hypertension [7]
Aim to identify underlying pathology:
◆ clotting studies;
◆ auto-antibodies (e.g. anti-nuclear antibodies [ANA], anti-dsDNA);
◆ iron studies;
◆ thyroid function tests (TFT);
◆ sickle screen;
◆ HIV testing;
◆ brain natriuretic peptide (BNP)/N-terminal brain natriuretic peptide (nt-BNP).
Radiological signs of PH on CXR
◆ pulmonary oedema;
◆ cardiomegaly or ‘prominent’ pulmonary vessels;
◆ ‘pruning’ (rapidly tapering diameter) of the pulmonary vessels.
What is the gold standard investigation for pulmonary hypertension?
Gold standard investigation (mPAP ≥ 25 mmHg is diagnostic).
Pulmonary pressures, cardiac output/index, right atrial pressure, pulmonary vascular resistance/
pulmonary artery saturations and pulmonary artery wedge pressure are assessed.
What is Virchows triad?
- Venous stasis (e.g. immobility [hospitalisation, long haul flights], heart failure, malignancy).
- Hypercoagulable state (e.g. thrombophilia, dehydration, pregnancy).
- Vessel wall damage (e.g. surgery, trauma, inflammatory conditions).
What are ECG changes in massive PE?
Evidence of right heart strain (massive PE) – classical S wave in lead , Q and T waves in lead 3 ‘S1Q3T3’ pattern, tall R waves V1, right axis deviation, right bundle branch block (RBBB).
What is the use of D-dimers in diagnosis of PE
- degradation products of cross-linked fibrin.
- Raised levels are linked with thrombotic activity.
- High sensitivity (and so a useful rule-out test) in patients with a low pretest probability
- specificity can be < 50% with false positives in infection, inflammation, cancer, surgery and pregnancy.
What is the gold standard radiological imaging for suspected PE
Identify other modalities of radiological imaging
CXR shows specific signs: pulmonary edema, cardiomegaly, prominent pulmonary vessels
CTPA remains gold standard test
VQ scanning is indicated in pregnancy, low risk young pt with normal lungs, nephropathy so unable to have CTPA
Stratify low risk, intermediate and high risk PE’s
PE: risk stratified according to haemodynamic status, presence of RV dysfunction (RVD) or myocardial injury.
◆ High risk (or massive) PE: acute PE with sustained hypotension (SBP < 90 mmHg or drop
≥40 mmHg >5minutes).
◆ Intermediate risk (or sub-massive) PE: presence of RVD or injury (e.g. troponin) in absence of
hypotension.
◆ Low risk PE: absence of hypotension, and absence of RVD or injury.
Management of PE
3 ways
- Heparin
- Oral anticoagulants
- Thrombolysis, alteplase (IV) - in confirmed, life-threatening PE with the following findings the use of thrombolysis
Use of heparin in management of PE
LMWH, dalteparin
- Initiate treatment before diagnostic scan if high clinical suspicion
- Contine for at least 5d until INR>2 for 2 consecutive days
- Oncology patients: consider long term as prophylaxic
- Monitor for HIIT
When would clot retrieval or surgical embolectomy be considered?
When would IVC be indicated?
Follow up for PE
- Cardiac arrest with suspected PE. Intra-vessel thrombolysis/clot retrieval or surgical embolectomy
are used in some centres. - Inferior vena cava (IVC) filters used for primary or secondary prevention (usually where
anticoagulation is contraindicated). - Patients should be monitored for chronic thromboembolic pulmonary hypertension, with an
echocardiogram 1 year after PE (looking for RVD); may result in lifelong anticoagulation.
Duration of anticoagulation treatment
Provoked PE with temporary cause, e.g. fracture, long flight
3 months of warfarin (INR 2–3) or rivaroxaban.
Provoked PE with temporary cause, e.g. fracture, long flight
Duration of anticoagulation
Unprovoked PE
Usually longer duration (e.g. 6–12 months +) of anticoagulation (warfarin or rivaroxaban) after risk–benefit assessment.
Unprovoked PE
Duration of anticoagulation
Secondary to cancer
Low molecular weight heparin continued until cancer considered cured or at least 6 months.
Secondary to cancer
Duration of anticoagulation
Two or more episodes of PE regardless of underlying risk factors
Extended anticoagulation (no scheduled stop date).
Two or more episodes of PE regardless of underlying risk factors
