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Pulmonary Hypertension: Overview
 Pulmonary Hypertension (PH) is an under-
recognized disease
 PH is estimated to be the third most common
cardiovascular syndrome
 Is the result of pulmonary, cardiac, infectious,
and connective tissue disease
 Treatment varies based on etiology
Pulmonary Arterial Hypertension:
Definition
 Pulmonary arterial hypertension (PAH) is a
progressive, incurable disease of the small
pulmonary arteries characterized by vascular cell
proliferation, aberrant remodeling, and thrombosis in
situ
 PH is defined as a combination of:
 Mean pulmonary artery pressure > or =25mmHg
at rest
 Mean pulmonary artery pressure > or =30mmHg
with exercise
Vascular Pressure in Systemic and
Pulmonary Circulations (mm Hg)
Systemic
Circulation
Body
SVR= 17.6
PVR= 1.8
120/80, mean 90
25/10, mean 15
Arteries
Arteries
Right
Atrium
Mean >6
Left
Atrium
Mean 5
Veins
Right
Left
Ventricle Ventricle
25/5
120/5
Veins
Pulmonary
Circulation
Lung
WHO Clinical Classification of PH
 Group 1. Pulmonary arterial hypertension
 Group 2. Pulmonary venous hypertension
 Group 3. PH associated with disorders of the
respiratory system and/or hypoxemia
 Group 4. PH due to chronic thromboembolic
disease
 Group 5. Miscellaneous
KDMC Statistics FY 08
415.0-415.9
(Acute Pulm
Heart Disease –
includes Cor
Pulmonale, Pulm
Embolism)
416
450
400
350
314
297
300
250
200
150
162
130
115
100
50
416.8 (PH,
Secondary)
41
41
4
0
415.0-415.19
416.8
Inpatient
416.0-416.9
Outpatient
ED
416.0 – 416.9
(Chronic Pulm
Heart Disease –
includes all types
of PH, Cor
Pulmonale)
KDMC Statistics FY07 – FY08
Outpatient
350
314
300
250
200
162
115
150
100
84
FY07
F08
122
79
Inpatient
450
400
50
350
0
300
415.0-415.19
416
416.8
416.0-416.9
297
233
250
200
Emergency Department
45
41
150
41
130
35
50
30
0
415.0-415.19
25
FY07
F08
20
15
11
8
5
4
5
0
415.0-415.19
416.8
416.0-416.9
126
100
40
10
113
FY07
F08
416.8
416.0-416.9
Appropriate Patient for Referral
 Echo revealing:
 Pulmonary Artery (PA) Systolic Pressure >
40mmHg
 PA Systolic pressures between 35-40
mmHg when patient is symptomatic
 Right Heart Cath revealing:
 Sustained Mean PA Pressure >25mmHg at
rest or >30 mmHg with exercise
 Mean pulmonary-capillary wedge pressure
and left ventricular end-diastolic pressure
<15 mmHg
Appropriate Patient for Referral
 CXR revealing pulmonary artery
enlargement or pruning
 EKG showing Right Ventricular
strain
Required
Documentation/Testing
 Detailed Physician H&P
 Complete PFT
 VQ Scan
 CXR
 Echo
Initial Clinic Visit
 Assessment
 12 lead EKG
 6 Minute Walk Test
 Patient Education
 Additional Testing ordered as indicated
 Testing will be coordinated for minimal
patient inconvenience
Possible Additional Testing
 Radiology
 HRCT of Thorax
 Pulmonary Angiogram

 Labs:
 CBC with platelets
 BMP
 LFTs
 BNPEP
 Thyroid Function Panel
 ANA screen
 RF






Sed Rate
CRP
Scl-70
PT/INR
Hypercoagulability
Panel
HIV
Antiphospholipid
Antibodies
 Sleep Study
 Right Heart Cath
Follow up Clinic Visit
 All testing will be evaluated
 Patient follow up evaluation
 Diagnosis including WHO Group and
Functional Class
 Treatment Plan initiated
 Continual patient education
 Letter to referring physician detailing
findings and treatment plan
Multi-Departmental Support
Echo Lab
 Things to obtain and focus on when performing
and interpreting ALL Echocardiograms

A global evaluation of right heart structure and
function


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


Right Ventricular systolic pressures
Right Atrial and/or Ventricular enlargement
Diastolic Dysfunction
Valvular Heart Disease, e.g. TR
Ejection fraction
Pericardial disease / Pericardial effusion
Multi-Departmental Support
 Cath Lab
 Right
Heart Cath protocols must be
followed in order to diagnose and obtain
treatment
Right Heart Catheterization in
PAH: Indications
 Confirm presence of PH
 Aid to determining etiology
 Prognostication
 Guide therapeutic choices, e.g. CCBs
 Follow up therapeutic interventions
RHC in PAH:
Key Findings
 PH: mean PAP ≥ 25mmHg at rest or ≥
30 mmHg with exercise
 PAWP <15 mmHg
 PVR >3 Wood Units
 Evidence of valvular heart disease
 Evidence of left-sided dysfunction
 Evidence of congenital heart disease
RHC in PAH
Special Procedures
 Vasodilator administration
 Fluid challenge
 Exercise
 Shunt evaluation
Multi-Departmental Support
 All Departments need to provide the
best possible testing and timely
reporting
 These two aspects are crucial to
treating these patients quickly and
effectively