Slide 2 Slide 3 Slide 4 AM Report Lauren Galpin, MD MA Slide 5
Thromboembolic obstruction of the major pulmonary arteries due to
unresolved pulmonary embolism [with pulmonary hypertension]
intraluminal thrombus organization and fibrous stenosis or complete
obliteration of pulmonary arteries The consequence is an increased
pulmonary vascular resistance resulting in pulmonary hypertension
and progressive right heart failure. Slide 6 True incidence unknown
500-2500 pts per year, 0.1-0.5% acute PE survivors 2 prospective
follow up studies of patients with acute PE: 44% had persistent
pulmonary htn +/- RV dysfunction, 5.1% developed CTEPH 3.1% had
CTEPH at 1 year, 3.8% at 2 years Up to 2/3 of patients with CTEPH
have no history of acute PE Slide 7 Risk factors: 223 patient
study: Previous PE history large perfusion deficit younger age 78
patient study: Age>70 systolic pulmonary artery pressure
>50mmHg at time of diagnosis Echo at 6 weeks accurately
distinguished complete resolution from persistent pulmonary htn.
Slide 8 Recurrent thromboembolism In situ pulmonary artery
thrombosis From underlying arteriopathy? Pulmonary vascular
remodeling Genetic susceptibility? Inflammatory mechanism? Slide 9
Low correlation between degree of obstruction and degree of
pulmonary hypertension Hemodynamic progression is seen in absence
of recurrent events Histopathologic evidence Slide 10 Two
compartment pulmonary vasculature bed Braunwald and Moser, 1971
Non-obstructed arteries showed changes consistent with chronic
pumonary hypertension Arteries distal to occlusion were normal
Slide 11 10-20% have anticardiolipin antibody Elevated factor VIII
Chronic inflammatory disorders Myeloproliferative disorders
Venticulo-atrial shunt Splenectomy Abnormal RBCs interaction with
pulmonary arteries? Abnormal RBCs act as prothrombotic? Abnormal
platelet activation? Slide 12 May be asymptomatic months years
Physical exam findings Accentuated pulmonic component S2 Bruits
over anterior lung fields Dyspnea/exercise intolerance, chest pain,
presyncope, syncope late Slide 13 LE Doppler V/Q scan CT angiogram
Right heart catheterization Angioscopy MRA with contrast Slide 14
Of segmental perfusion deficits on V/Q scan Pulmonary
veno-occlusive disease Pulmonary capillary hemangiomatosis
Fibrosing mediastinitis Pulmonary vasculitis Sarcomas of pulmonary
arteries Slide 15 Without treatment Pulmonary Arterial Pressure
>40mmHg: 30% 5y survival PAP>50mmHg: 10% 5y survival In
another study, >30mmHg: 10% survival Slide 16 With Pulmonary
Endarterectomy (+lifelong anticoagulation and IVC filter) 5-24%
perioperative mortality overall,
