Chronic Thromboembolic Pulmonary Hypertension

AM Report
Lauren Galpin, MD MA
 “Thromboembolic
obstruction of the major
pulmonary arteries due to unresolved
pulmonary embolism [with pulmonary
 “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
 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
 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
 Hemodynamic progression is seen in
absence of recurrent events
 Histopathologic evidence
 “Two
compartment pulmonary
vasculature bed” – Braunwald and Moser,
• 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
• 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
 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
• Pulmonary Arterial Pressure >40mmHg: 30% 5y
• 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
 “No 21 year-old should ever be short of

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