AM Report Lauren Galpin, MD MA

 “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.”

    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

 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.

 Recurrent thromboembolism  In situ pulmonary artery thrombosis • From underlying arteriopathy?

 Pulmonary vascular remodeling • • Genetic susceptibility?

Inflammatory mechanism?

   Low correlation between degree of obstruction and degree of pulmonary hypertension Hemodynamic progression is seen in absence of recurrent events Histopathologic evidence

 “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

      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?

  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

      LE Doppler V/Q scan CT angiogram Right heart catheterization Angioscopy MRA with contrast

 Of segmental perfusion deficits on V/Q scan • • • Pulmonary veno-occlusive disease Pulmonary capillary hemangiomatosis Fibrosing mediastinitis • • Pulmonary vasculitis Sarcomas of pulmonary arteries

 Without treatment • • • Pulmonary Arterial Pressure >40mmHg: 30% 5y survival PAP>50mmHg: 10% 5y survival In another study, >30mmHg: 10% survival

 With Pulmonary Endarterectomy (+lifelong anticoagulation and IVC filter) • •   5-24% perioperative mortality overall, <5% at experienced centers In experienced centers, well-selected patients Near normalization of hemodynamics Substantial improvement in clinical symptoms

 “… there is no degree of embolic occlusion within the pulmonary vascular tree that is inaccessible and no degree of right ventricular impairment or any level of pulmonary vascular resistance that is inoperable.” -- Jamieson et al, UCSD

   Medicine • • Epoprostenol – prostacyclin infusion Sildenafil – phosphodiesterase-5 inhibitor • Bosentan – endothelin receptor antagonist Balloon angioplasty • Has been done successfully • Still considered experimental Lung transplant • For people with advanced small vessel disease without comorbidities that preclude surgery

     Review of systems and careful physical exam are important!

Chronic thromboembolic disease is uncommon, but not rare Chronic thromboembolism is a treatable cause of pulmonary hypertension V/Q scan still an important diagnostic tool “No 21 year-old should ever be short of breath.”