Слайд 1
Pulmonary Hypertension-Pathways, Diagnostic.
Made by,
RISHABH GURU,
3rd year, PSMU
Слайд 2PH - History
History of smoking
ETOH/recreational drug use
Systemic hypertension
Cyanosis/murmur as a
child
Joint/musculoskeletal pain
Raynaud’s Syndrome
FH of unexplained early cardiopulmonary disease
Use of appetite
suppressant drugs
Слайд 3Pulmonary circulation
Low resistance, high compliance vascular bed
Only organ to receive
entire cardiac output (CO)
Changes in CO as well as pleural/alveolar
pressure affect pulmonary blood flow
Different reactions compared to the systemic circulation
Normally in a state of mild vasodilation
Слайд 4Outline
Review classification of pulmonary hypertension (PH)
Pulmonary arterial hypertension (PAH)
Evaluation of
PH and how to differentiate PAH from other forms of
PH
PH and cardiac, renal and hepatic transplantation
Review PAH-approved therapy and treatment of non-Group 1 PH
Слайд 5Classification of Pulmonary Hypertension (PH)
1) Pulmonary arterial hypertension
2) Pulmonary hypertension
due to left heart disease
3) Pulmonary hypertension due to lung
diseases and/or hypoxia
4) Chronic Thromboembolic pulmonary hypertension (CTEPH)
5) Pulmonary Hypertension with unclear and/or multifactorial mechanism
Слайд 6Vascular Pressure in Systemic and Pulmonary Circulations (mm Hg)
Pulmonary
Circulation
Systemic
Circulation
Arteries
Arteries
Veins
Veins
120/80,
mean 93
25/8, mean 14
Left
Atrium
Mean 5
Right
Atrium
Mean >6
Right
Ventricle
25/2-5
Left
Ventricle
120/5-10
Lung
Body
SVR= 17.6 PVR=
1.8
Слайд 7PH- Symptoms
DOE
Fatigue, weakness
Chest pain
LE or abdominal swelling
Syncope
Not typical of
PAH: orthopnea
Слайд 43Algorithm illustrating general diagnostic workup for pediatric pulmonary arterial hypertension
Слайд 44Classification of PAH, Group 1
Idiopathic PAH (formerly primary pulmonary hypertension,
PPH)
Heritable
Drug/toxin induced
Associated with:
Connective tissue diseases
HIV infection
Portal hypertension
Systemic to pulmonary shunts
Schistosomiasis
Chronic
hemolytic anemia
Слайд 45Group 2 PH
Pulmonary hypertension owing to left heart disease
Systolic dysfunction
Diastolic
dysfunction
Valvular disease
Pulmonary venous obstruction
Слайд 46PH with unclear or multifactorial mechanisms: Group 5
1.Hematologic disorders
2.Systemic disorders:
vasculitis
3.Metabolic disorders
4.Others: chronic renal failure on dialysis
Слайд 47Vascular
Remodeling
Pathogenesis :
An Integrated View
Inflammation
Слайд 48Evaluation for PH
ECG
Chest x-ray
V/Q scan or contrasted spiral CT (+/-
angiogram)
PFTs
Exercise oximetry
Echocardiogram
Right heart catheterization w/vasodilator testing
Labs: CBC, CMP, INR, ANA,
HIV, TFTs
Слайд 50PH - Radiographic studies
CXR:
-large proximal PA with peripheral tapering
(pruning)
-cardiomegaly due to enlarged RA, RV
-pleural effusion is uncommon
CT:
-PA
>aorta
-cardiomegaly, enlarged RV
-pericardial effusion
Слайд 54PA
A
Enlarged main PA on CT
Standard view
Coronal view
Слайд 58CTEPH: Pulmonary Angiography
Confirms diagnosis of CTEPH in patients with PH
Assess
thrombus accessibility
Distinct angiographic patterns
“Web” narrowing
Poststenotic dilatation
Proximal occlusion
“Pouch” defects
Слайд 59Organized Clot Removed at Surgery
Слайд 60Pulmonary Function tests
No characteristic changes
Mandatory to screen for significant restrictive
or obstructive lung disease
Diffusing capacity often significantly reduced in patients
with scleroderma (<50%)
Слайд 61RV, RA Enlargement on Echocardiogram
RV
LV
RA
LA
Normal
Слайд 62Other Helpful Diagnostic Tests
(Determined by patient’s history)
High resolution chest CT
Cardiopulmonary
exercise study
Polysomnography
Arterial blood Gas
Hepatitis serologies
Left heart catheterization, evaluation of coronary
arteries
Слайд 63Echocardiographic findings in ESRD patients undergoing transplant
Слайд 64Treatment of non PAH-pulmonary hypertension
Pulmonary Venous Hypertension:
Treat heart failure with
afterload reduction
Systolic or diastolic
MV or AV disease
Replace the valve
Pulmonary vein
stenosis
Pulmonary vein stenting
Слайд 65Treatment of non PAH-pulmonary hypertension
PH associated with disorders of the
respiratory system and/or hypoxemia:
Rx of hypoxemia is often the main
therapy
PH due to chronic thromboembolic disease:
Thromboendarterectomy for proximal disease
Can consider PAH therapy for distal disease
Слайд 66Adjunctive treatments of PAH
Anticoagulation
Diuretics
Digoxin
Oxygen
Calcium channel blockers
Exercise
Salt restriction
Слайд 67Specific PAH Treatment
Epoprostenol (generic and Flolan®)
Treprostinil (Remodulin®)
Iloprost (Ventavis®)
Bosentan (Tracleer®)
Ambrisentan (Letairis®)
Tadalifil
(Adcirca®)
Sildenafil (Revatio®)
Endothelin receptor
antagonists (ERAs)
Phosphodiesterase 5
inhibitors (PDE5 inhibitors)
Prostaglandins
Слайд 69Take Home Points
PH can not be diagnosed by Echo alone,
need a thorough evaluation for all patients
Right heart catheterization
is necessary in ALL patients to accurately diagnose PH
PAH is a progressive disease, even with Rx
Make sure the patient has PAH before treating
Despite multiple therapies, lung transplantation is the only curative treatment for PAH
PH negatively impacts outcome of all solid organ transplants