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Shah SJ. Pulmonary hypertension. JAMA. doi:10.1001/jama.2012.12347.
eTable. Clinical Characteristics of Three Women with Dyspnea, Exercise Intolerance, and
Increased Pulmonary Artery Systolic Pressure on Echocardiography*
eFigure. Calculation of Estimated Pulmonary Artery Systolic Pressure: The Simplified Bernoulli
Equation
This supplementary material has been provided by the author to give readers additional
information about the work.
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eTable. Clinical Characteristics of Three Women with Dyspnea, Exercise Intolerance, and
Increased Pulmonary Artery Systolic Pressure on Echocardiography
Patient A
77-year-old female
Dyspnea, exercise
intolerance, leg swelling
III
Systemic hypertension,
diabetes, chronic kidney
disease,
atrial fibrillation
Age / Sex
Symptoms
WHO functional classa
Comorbidities
Physical examination
Echocardiography
BP 172/65 mmHg, JVP,
bibasilar crackles, irregularly
irregular rhythm, loud P2, 2/6
holosystolic murmur at left
sternal border, 2+ leg edema







Invasive
hemodynamicsb


LV ejection fraction 65%
Indeterminate diastolic
function
Mild RV enlargement
Normal RV systolic
function
PASP 60 mmHg
RA pressure 10 mmHg
Mild TR
Patient Bc
75-year-old female
Dyspnea, exercise
intolerance, leg swelling
III
End-stage renal disease,
systemic hypertension,
diabetes, rheumatoid
arthritis, interstitial lung
disease
BP 165/94 mmHg, JVP,
bibasilar crackles, regular
rate and rhythm, loud P2,
3/6 holosystolic murmur at
left sternal border, 2+ leg
edema
 LV ejection fraction 70%
 Moderate diastolic
dysfunction
 Moderate RV
enlargement
 Mild RV systolic
dysfunction
 PASP 93 mmHg
 RA pressure 15 mmHg
 Moderate-to-severe TR
 RA pressure: 24 mmHg
 PA pressure 68/28 (41)
mmHg
 PCWP: 28 mmHg
 Cardiac index: 2.1
L/min/m2
 PVR: 300 dynes/s/cm5
 Systemic BP: 165/94
mmHg
 WHO Group II PH
(pulmonary venous
hypertension)
 Carvedilol for systemic
hypertension
 Intensive hemodialysis
(4x/week) for fluid
overload
Patient C
71-year-old female
Dyspnea, exercise
intolerance, leg swelling
III
Connective tissue disease,
interstitial lung disease,
chronic obstructive
pulmonary disease
BP 93/69 mmHg, JVP,
bibasilar crackles, regular
rate and rhythm, loud P2, 2/6
holosystolic murmur at left
sternal border, 1+ leg edema







LV ejection fraction 58%
Mild diastolic dysfunction
Severe RV enlargement
Severe RV systolic
dysfunction
PASP 76 mmHg
RA pressure 10 mmHg
Moderate TR


RA pressure: 12 mmHg
PA pressure 62/30 (41)
mmHg
 PCWP: 10 mmHg

 Cardiac index: 1.8

L/min/m2

 PVR: 944 dynes/s/cm5

 Systemic BP: 93/69
mmHg
Final diagnosis

 WHO Group I PH
(pulmonary arterial
hypertension)
Treatment

 Diuretics, digoxin,
warfarin, supplemental
oxygen

 Bosentan and
subcutaneous treprostinil
for pulmonary
vasodilation
WHO = World Health Organization; BP = blood pressure; JVP = jugular venous pulsations; LV = left ventricle; RV = right
ventricle; PASP = pulmonary artery systolic pressure; RA = right atrial; TR = tricuspid regurgitation; PA = pulmonary
arterial; PCWP = pulmonary capillary wedge pressure; PVR = pulmonary vascular resistance
a
WHO functional class is a modified form of the New York Heart Association functional classification, which was designed
specifically for patients with pulmonary hypertension
b
Normal values: RA pressure: 0-6 mmHg; PA pressure: < 24/12 mmHg; mean PA pressure < 20 mmHg; pulmonary
vascular resistance: < 150 dynes/s/cm5; cardiac index: 2.5-4 L/min/m2; PA pressure is displayed as systolic/diastolic
(mean).
c
Patient B is the patient whose case narrative is described in the text of the main article.
RA pressure: 15 mmHg
PA pressure 67/29 (42)
mmHg
PCWP: 29 mmHg
Cardiac index: 3.4
L/min/m2
PVR: 145 dynes/s/cm5
Systemic BP: 172/65
mmHg
WHO Group II PH
(pulmonary venous
hypertension)
Carvedilol for systemic
hypertension
Furosemide for fluid
overload
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eFigure. Calculation of Estimated Pulmonary Artery Systolic Pressure:
The Simplified Bernoulli Equation
Bernoulli equation: P1-P2 = ½  (22-12) +  (d/dt)·ds + R()
CONVECTIVE ACCELERATION FLOW ACCELERATION VISCOUS FRICTION Modified Bernoulli equation: ΔP = 4(TR)2
PASP = RV-RA gradient (ΔP) + RA pressure = 4(TR)2 + RA pressure
The simplified (modified) Bernoulli equation assumes that the contributions of flow
acceleration and viscous friction to the pressure gradient between the right ventricle and
right atrium are negligible. In addition, the modified Bernoulli equation assumes that the
proximal velocity (ν1) is also negligible, and it uses an approximation for the constant ρ
that accounts for the mass density of blood and a conversion factor for measurement
units. The right atrial pressure is most commonly estimated on echocardiography by
examining the size and collapsibility of the inferior vena cava during inspiration.
Abbreviations: PASP, pulmonary artery systolic pressure; RV, right ventricle; RA, right
atrium; TR, tricuspid regurgitation
© 2012 American Medical Association. All rights reserved. Downloaded From: http://jamanetwork.com/pdfaccess.ashx?url=/data/journals/jama/24997/ on 06/15/2017