Pulmonary Hypertension and Right Heart Failure

Pulmonary venous hypertension (Cardiac)• LVF-ischaemic • Mitral Regurgitation / Stenosis• Cardiomyopathy-eg alchohol ,viralPulmonary arterial hypertension• Hypoxic – COPD , OSA , Fibr Alveolitis• Multiple Po Emboli• Po vasculitis –eg SLE , PAN ,Systemic Sclerosis• Drugs –eg appetite suppressants• Cardiac Left to right shunt – ASD , VSD • Primary pulmonary hypertension (only after excluding all of above)

Clinical Signs of Pulmonary Hypertension

and Right Heart Failure

• Central cyanosis if hypoxic • Dependent oedema• Raised JVP with V waves (due to secondary

tricuspid regurg)• Right ventricular heave at left parasternal edge • Murmur of tricuspid regurgitation • Load P2 • Enlarged liver (pulsatile )

Investigation of Pulmonary Hypertension

• ECG• CXR• SaO2 and arterial blood gases• Pulmonary function • Echocardiogram / Cardiac Catheterisation • D dimers and VQ scan if PE suspected • CT Pulmonary Angiogram • Auto-antibodies if vasculitis suspected

Primary pulmonary hypertension

• Diagnosis by exclusion of other secondary causes • Progressive SOBOE and signs of right heart failure • Pharmacologic Treatment

-prophylactic anticoagulation [warfarin] -O2 if hypoxic -Po Vasodilators :Endothelin antagonist (Oral Bosentan) , PDE5-inhibitor (Oral Sildenafil), iv Epoprostenol

THROMBOEMBOLIC LUNG DISEASE

• Pulmonary infarction-in situ

-venous emboli

• Virchow’s Triad-Stasis

-Vessel wall damage-Hypercoagulablity

RISK FACTORS FOR DVT AND PE #1

•Thrombophilia- FH,freq,site,age

•Contraceptive pill ,HRT

•Pregnancy

•Pelvic obstruction-eg uterus,ovary,lymph nodes

•Trauma-eg RTA

RISK FACTORS FOR DVT AND PE #2

• Surgery- eg pelvic,hip,knee

• Immobility-eg bed rest,long haul flights

• Malignancy

• Myocardial infarction

• Po hypertension/vasculitis

DVT

• Proximal (Ileofemoral)-most likely to embolise

-most likely to lead to chronic venous insufficiency and venous leg ulcers

• Distal (Polpiteal)-least likely to embolise

Clinical presentation of DVT

• Whole leg or calf involved depending on site

• Swollen,hot,red,tender

• Differential:Popliteal synovial rupture[Bakers cyst],Superficial thrombophlebitis,Calf cellulitis

Investigation of DVT

• Ultrasound Doppler leg scan(1st line)-Non invasive -Exclude popliteal cyst, pelvic mass

• CT scan of ileofemoral veins,IVC and pelvis

• Constrast venography -Invasive,contrast(irritant,allergy)-Rarely indicated

Pulmonary Emboli

• Predisposing DVT may be silent

• Clinical presentation depends on size:

• Large-cardiovascular shock,low BP,central cyanosis,sudden death

• Medium-pleuritic pain,haemoptysis,breathless

• Small recurrent-progressive dyspnoea, pulmonary hypertension and right heart failure

Diagnosis of PE #1

• Clinical Signs-Tachycardia,Tachypnoea,Cyanosis,Fever Low BP,Crackles, Rub, Pleural effusion

• Arterial blood gases-PaO2,Sao2 (Type 1 resp failure:PaCO2 normal or low)

• CXR-Normal early on before infarction -Basal atelectesis,Consolidation , Pleural effusion after infarction

Diagnosis of PE #2

Investigations• ECG :Acute Rt heart strain pattern

(S1,Q3,T3 , T inv in V1-3) • D-dimers usually raised • Isotope lung scan (Ventilation/Perfusion) • Perfusion defect before infarction• Perfusion+Ventilation matched defect after

infarction

V/Q isotope scan in Recurrent Po emboli Multiple filling defects (arrows) on perfusion (Q) scanMismatched to ventilation (V) scanDyspnoea ,Hypoxia,Cardiomegaly ,Po Hypertension and Large RV on Echo , Restrictive Lung Vols with Low DLCO ,Hypoxia

Diagnosis of PE #3

• CT pulmonary angiogram to image pulmonary artery filling defect

• Leg and pelvic ultrasound to detect silent DVT

• Echocardiogram to measure pulmonary artery pressure and RV size

• Gas transfer factor (TLCO) to measure perfusion defect

CT Po Angiogram in Acute Massive PEOccluded Rt main Po Artery (arrow ) and filling defect Lt Po artery Acute Dyspnoea ,Hypoxia ,Low BP , Acute Rt Heart Strain on ECG Raised D dimers .No clot seen in IVC or ileofemoral veins Treated with Thrombolysis and Low MW Heparin

Investigation of underlying cause of PE

• If no obvious underlying cause –eg surgery /pregnancy /malignancy /immobility

• Look for underlying Ca – Clin exam ,CXR,PSA,CA125,CEA,Pelvic USS

• Autoantibodies (SLE) – Antinuclear ,Anti-Cardiolipin

• Coagulation factor screen – Antithrombin-3,Protein C/S, Factor 5/8

Prevention of DVT

• Early post-op mobilisation• TED compression stockings• Calf muscle exercises • Subcutaneous low dose low mol wt heparin

perioperatively• Dabigatran - direct thrombin inhibitor

Rivaroxaban - direct inhibitor of activated factor X- both given orally for prophylaxis of venous thromboembolism in adults after hip or knee replacement surgery

Treatment of DVT/PE #1

• Anticoagulation prevents clot propagation-tips balance to thrombolysis-body dissolves clot

• Initiate with parenteral heparin-fast acting-via antithrombin-3

• Usually therapeutic dose of s/c low mol wt heparin ( Dalteparin “Fragmin”)

Treatment of DVT/PE #2

• Low mol wt heparin –once daily injection ,no monitoring –no hassle

• IV infusion unfractionated heparin -more hassle-need to monitor clotting, increased bleeding risk- rarely used nowadays

Treatment of DVT/PE #3

• Start concurrent oral warfarin-takes 3 days-antagonises vit K1 dependent prothrombin

• After 3-5 days stop heparin-when INR>2

• Need to monitor APTT with unfractionated -but not with low mol wt heparin

Treatment of DVT/PE #4

• Continue Warfarin for 3-6 months

• Monitor Warfarin with INR-Target range 2.5-3.5

• Interactions which increase anticoagulation -Alcohol,Antibiotics ,Aspirin,NSAIDs,

Amiodarone, Cimetidine,Omeprazole ,etc etc

• Look in BNF for possible interactions

Treatment of DVT/PE #5

• Thrombolysis-Streptokinase or TPA• Only for large life threatening PE-ie low BP

and severe hypoxaemia due to main pulmonary artery occlusion

• IVC filter to prevent embolisation from large ileofemoral/IVC clot - for recurrent PE’s

• Thrombo-embolectomy –rarely indicated • Aspirin –no role – anti-platelet

Overanticoagulation

• Address underlying cause-eg drug interaction,chronic liver disease,CHF

• If bleeding then stop anticoagulant and reverse effect• Low MW Heparin has a long half life • Warfarin has a long half life• May need cover with prothrombin complex concentrate

or fresh frozen plasma• Reverse warfarin with vitamin K1(especially if chronic

liver disease)• Reverse heparin with protamine