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Pulmonary Hypertension-Pathways, Diagnostic

Содержание

PH - HistoryHistory of smokingETOH/recreational drug useSystemic hypertensionCyanosis/murmur as a childJoint/musculoskeletal painRaynaud’s SyndromeFH of unexplained early cardiopulmonary diseaseUse of appetite suppressant drugs

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Слайд 1 Pulmonary Hypertension-Pathways, Diagnostic.


Made by,
RISHABH GURU,
3rd year, PSMU
Pulmonary Hypertension-Pathways, Diagnostic.

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

PH - HistoryHistory of smokingETOH/recreational drug useSystemic hypertensionCyanosis/murmur as a childJoint/musculoskeletal painRaynaud’s SyndromeFH of unexplained early cardiopulmonary

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
Pulmonary circulationLow resistance, high compliance vascular bedOnly organ to receive entire cardiac output (CO)Changes in CO as

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



OutlineReview classification of pulmonary hypertension (PH)Pulmonary arterial hypertension (PAH)Evaluation of PH and how to differentiate PAH from

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
Classification of Pulmonary Hypertension (PH)1) Pulmonary arterial hypertension2) Pulmonary hypertension due to left heart disease3) Pulmonary hypertension

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
Vascular Pressure in Systemic and Pulmonary Circulations (mm Hg)PulmonaryCirculationSystemic CirculationArteriesArteriesVeinsVeins120/80, mean 9325/8, mean 14LeftAtriumMean 5RightAtriumMean >6RightVentricle25/2-5LeftVentricle120/5-10LungBodySVR= 17.6

Слайд 7PH- Symptoms
DOE
Fatigue, weakness
Chest pain
LE or abdominal swelling
Syncope
Not typical of

PAH: orthopnea

PH- SymptomsDOEFatigue, weaknessChest painLE or abdominal swellingSyncope 	Not typical of PAH: orthopnea

Слайд 43Algorithm illustrating general diagnostic workup for pediatric pulmonary arterial hypertension

Algorithm 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
Classification of PAH, Group 1Idiopathic PAH (formerly primary pulmonary hypertension, PPH)Heritable		Drug/toxin inducedAssociated with:Connective tissue diseasesHIV infectionPortal hypertensionSystemic

Слайд 45Group 2 PH
Pulmonary hypertension owing to left heart disease
Systolic dysfunction
Diastolic

dysfunction
Valvular disease
Pulmonary venous obstruction

Group 2 PHPulmonary hypertension owing to left heart diseaseSystolic dysfunctionDiastolic dysfunctionValvular diseasePulmonary 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

PH with unclear or multifactorial mechanisms: Group 51.Hematologic disorders2.Systemic disorders: vasculitis 3.Metabolic disorders4.Others: chronic renal failure on

Слайд 47Vascular
Remodeling
Pathogenesis : An Integrated View
Inflammation

VascularRemodelingPathogenesis : 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

Evaluation for PHECGChest x-rayV/Q scan or contrasted spiral CT (+/- angiogram)PFTsExercise oximetryEchocardiogramRight heart catheterization w/vasodilator testingLabs: CBC,

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

PH - Radiographic studiesCXR: 		-large proximal PA with peripheral 			tapering (pruning)		-cardiomegaly due to enlarged RA, RV 		-pleural

Слайд 51CXR in PAH

CXR in PAH

Слайд 52CXR in Eisenmenger Syndrome

CXR in Eisenmenger Syndrome

Слайд 53Mitral Stenosis

Mitral Stenosis

Слайд 54PA
A
Enlarged main PA on CT Standard view

Coronal view

PAA	 Enlarged main PA on CT  Standard view		   Coronal view

Слайд 57Ventilation Perfusion Lung Scan

Ventilation Perfusion Lung Scan

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

CTEPH: Pulmonary AngiographyConfirms diagnosis of CTEPH in patients with PHAssess thrombus accessibilityDistinct angiographic patterns“Web” narrowingPoststenotic dilatationProximal occlusion“Pouch”

Слайд 59Organized Clot Removed at Surgery

Organized 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%)
Pulmonary Function testsNo characteristic changesMandatory to screen for significant restrictive or obstructive lung diseaseDiffusing capacity often significantly

Слайд 61RV, RA Enlargement on Echocardiogram
RV
LV
RA
LA
Normal

RV, RA Enlargement on EchocardiogramRVLVRALANormal			       PH

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
Other Helpful Diagnostic Tests (Determined by patient’s history)High resolution chest CTCardiopulmonary exercise studyPolysomnographyArterial blood GasHepatitis serologiesLeft heart

Слайд 63Echocardiographic findings in ESRD patients undergoing transplant

Echocardiographic 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

Treatment of non PAH-pulmonary hypertensionPulmonary Venous Hypertension:Treat heart failure with afterload reductionSystolic or diastolicMV or AV diseaseReplace

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
Treatment of non PAH-pulmonary hypertensionPH associated with disorders of the respiratory system and/or hypoxemia:Rx of hypoxemia is

Слайд 66Adjunctive treatments of PAH
Anticoagulation
Diuretics
Digoxin
Oxygen
Calcium channel blockers
Exercise
Salt restriction

Adjunctive treatments of PAHAnticoagulationDiuretics DigoxinOxygenCalcium channel blockersExerciseSalt 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

Specific PAH TreatmentEpoprostenol (generic and Flolan®)Treprostinil (Remodulin®)Iloprost (Ventavis®)Bosentan (Tracleer®)Ambrisentan (Letairis®)Tadalifil (Adcirca®)Sildenafil (Revatio®)Endothelin receptor antagonists (ERAs)Phosphodiesterase 5 inhibitors

Слайд 68PAH Determinants of Risk
.

PAH Determinants of Risk.

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

Take Home PointsPH can not be diagnosed by Echo alone, need a thorough evaluation for all patients

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