Pulmonary Hypertension-Pathways, Diagnostic

Июль 23, 2022

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Pulmonary Hypertension-Pathways, Diagnostic

Содержание

2. PH - History History of smoking ETOH/recreational drug use Systemic hypertension Cyanosis/murmur as a child Joint/musculoskeletal
3. Pulmonary circulation Low resistance, high compliance vascular bed Only organ to receive entire cardiac output (CO)
4. Outline Review classification of pulmonary hypertension (PH) Pulmonary arterial hypertension (PAH) Evaluation of PH and how
5. Classification of Pulmonary Hypertension (PH) 1) Pulmonary arterial hypertension 2) Pulmonary hypertension due to left heart
6. Vascular Pressure in Systemic and Pulmonary Circulations (mm Hg) Pulmonary Circulation Systemic Circulation Arteries Arteries Veins
7. PH- Symptoms DOE Fatigue, weakness Chest pain LE or abdominal swelling Syncope Not typical of PAH:
43. Algorithm illustrating general diagnostic workup for pediatric pulmonary arterial hypertension
44. Classification of PAH, Group 1 Idiopathic PAH (formerly primary pulmonary hypertension, PPH) Heritable Drug/toxin induced Associated
45. Group 2 PH Pulmonary hypertension owing to left heart disease Systolic dysfunction Diastolic dysfunction Valvular disease
46. PH with unclear or multifactorial mechanisms: Group 5 1.Hematologic disorders 2.Systemic disorders: vasculitis 3.Metabolic disorders 4.Others:
47. Vascular Remodeling Pathogenesis : An Integrated View Inflammation
48. Evaluation for PH ECG Chest x-ray V/Q scan or contrasted spiral CT (+/- angiogram) PFTs Exercise
50. PH - Radiographic studies CXR: -large proximal PA with peripheral tapering (pruning) -cardiomegaly due to enlarged
51. CXR in PAH
52. CXR in Eisenmenger Syndrome
53. Mitral Stenosis
54. PA A Enlarged main PA on CT Standard view Coronal view
57. Ventilation Perfusion Lung Scan
58. CTEPH: Pulmonary Angiography Confirms diagnosis of CTEPH in patients with PH Assess thrombus accessibility Distinct angiographic
59. Organized Clot Removed at Surgery
60. Pulmonary Function tests No characteristic changes Mandatory to screen for significant restrictive or obstructive lung disease
61. RV, RA Enlargement on Echocardiogram RV LV RA LA Normal PH
62. Other Helpful Diagnostic Tests (Determined by patient’s history) High resolution chest CT Cardiopulmonary exercise study Polysomnography
63. Echocardiographic findings in ESRD patients undergoing transplant
64. Treatment of non PAH-pulmonary hypertension Pulmonary Venous Hypertension: Treat heart failure with afterload reduction Systolic or
65. Treatment of non PAH-pulmonary hypertension PH associated with disorders of the respiratory system and/or hypoxemia: Rx
66. Adjunctive treatments of PAH Anticoagulation Diuretics Digoxin Oxygen Calcium channel blockers Exercise Salt restriction
67. Specific PAH Treatment Epoprostenol (generic and Flolan®) Treprostinil (Remodulin®) Iloprost (Ventavis®) Bosentan (Tracleer®) Ambrisentan (Letairis®) Tadalifil
68. PAH Determinants of Risk .
69. Take Home Points PH can not be diagnosed by Echo alone, need a thorough evaluation for
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PH - 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

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Pulmonary 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

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Outline
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

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Classification 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

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Vascular Pressure in Systemic and Pulmonary Circulations (mm Hg)
Pulmonary
Circulation
Systemic
Circulation
Arteries
Arteries
Veins
Veins
120/80, mean

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

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

orthopnea

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Algorithm illustrating general diagnostic workup for pediatric pulmonary arterial hypertension

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Classification 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 45

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

disease
Pulmonary venous obstruction

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PH with unclear or multifactorial mechanisms: Group 5
1.Hematologic disorders
2.Systemic disorders: vasculitis

3.Metabolic disorders
4.Others: chronic renal failure on dialysis

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Vascular
Remodeling
Pathogenesis : An Integrated View
Inflammation

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Evaluation 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

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PH - 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

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CXR in PAH

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CXR in Eisenmenger Syndrome

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Mitral Stenosis

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PA
A
Enlarged main PA on CT Standard view Coronal view

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Ventilation Perfusion Lung Scan

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CTEPH: Pulmonary Angiography
Confirms diagnosis of CTEPH in patients with PH
Assess thrombus

accessibility
Distinct angiographic patterns
“Web” narrowing
Poststenotic dilatation
Proximal occlusion
“Pouch” defects

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Organized Clot Removed at Surgery

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Pulmonary 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%)

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RV, RA Enlargement on Echocardiogram
RV
LV
RA
LA
Normal PH

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Other 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

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Echocardiographic findings in ESRD patients undergoing transplant

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Treatment 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

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Treatment 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

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

Слайд 67

Specific 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

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PAH Determinants of Risk
.

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Take 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

Pulmonary Hypertension-Pathways, Diagnostic

Содержание

PH - HistoryHistory of smokingETOH/recreational drug useSystemic hypertensionCyanosis/murmur as a childJoint/musculoskeletal

Pulmonary circulationLow resistance, high compliance vascular bedOnly organ to receive entire

OutlineReview classification of pulmonary hypertension (PH)Pulmonary arterial hypertension (PAH)Evaluation of PH

Classification of Pulmonary Hypertension (PH)1) Pulmonary arterial hypertension2) Pulmonary hypertension due

Vascular Pressure in Systemic and Pulmonary Circulations (mm Hg)PulmonaryCirculationSystemic CirculationArteriesArteriesVeinsVeins120/80, mean

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

Algorithm illustrating general diagnostic workup for pediatric pulmonary arterial hypertension

Classification of PAH, Group 1Idiopathic PAH (formerly primary pulmonary hypertension, PPH)Heritable Drug/toxin

Group 2 PHPulmonary hypertension owing to left heart diseaseSystolic dysfunctionDiastolic dysfunctionValvular

PH with unclear or multifactorial mechanisms: Group 51.Hematologic disorders2.Systemic disorders: vasculitis

VascularRemodelingPathogenesis : An Integrated View Inflammation

Evaluation for PHECGChest x-rayV/Q scan or contrasted spiral CT (+/- angiogram)PFTsExercise

PH - Radiographic studiesCXR: -large proximal PA with peripheral tapering (pruning) -cardiomegaly