Pulmonary Hypertension


the Other High Blood Pressure - Northeast Iowa Family Practice ...

Pulmonary Hypertension

…the other high blood pressure

Michael J. Krowka, MD

Division of Pulmonary and Critical Care

Mayo Clinic

Jauch Symposium, Waterloo Iowa

May 18, 2012

Current Issues to discuss

► Diagnostics

• New Classification

• New Diagnostic Criteria

Pulmonary artery hypertension vs pulmonary venous hypertension

Pulmonary Artery Hypertension (PAH) Therapy update

• The treatment options…$$$

► Specific Family Practice considerations

Pulmonary Hypertension (PH)


…increased blood pressure in the

pulmonary vascular bed

Pulmonary artery hypertension (PAH)? or

….Pulmonary venous hypertension (PVH) or


Pulmonary Artery Hypertension:

The overall concept…

A pulmonary arterial vascular obstructive process*… with

variable presence of vasoconstriction…

* endothelial proliferation

smooth muscle proliferation

in-situ thrombosis


platelet aggregates

plexogenic change

Pulmonary Hypertension Classification

►1973 Geneva

• Primary pulmonary hypertension (PPH) vs secondary pulmonary hypertension

►1998 Evian

• 5 Subgroups of pulmonary hypertension

Pulmonary artery vs pulmonary venous hypertension

►2003 Venice

• Abandon term PPH – use “idiopathic Pulmonary Artery Hypertension” (IPAH)

►2008 Dana Point

• Add hematologic diagnoses (hemolytic anemias/splenectomy)

►2013 Nice

• Revisions??

Pulmonary Artery Hypertension (PAH)


…right heart failure and death

Classification of


Hypertension (PH)

Summer, 2009

I. Pulmonary artery hypertension


II. PH owing to left heart disease

III. PH owing to lung


IV. PH due to chronic pulmonary


V. PH with unclear/multifactorial


Group I - Pulmonary Artery Hypertension

Dana Point 2008

I. Pulmonary artery hypertension - PAH

1.1 Idiopathic

1.2 Heritable


b. ALK1

1.3 Drugs and Toxins

1.4 Associated with

1.4.1 connective tissue disease

1.4.2 HIV infection

1.4.3 portal hypertension

1.4.4 congenital heart disease

1.4.5 schistosomiasis

1.4.6 chronic hemolytic anemia


…insurers will pay for Group I

The Diagnostic Criteria for Pulmonary Artery


Right heart catheterization...

• Mean Pulmonary Artery Pressure (MPAP) > 25 mm Hg

Pulmonary Artery Occlusion Pressure (PAOP) < 15 mm Hg

Pulmonary Vascular Resistance (PVR) > 240 dynes.s.cm -5

where PVR = (MPAP-PAOP) * 80


Common Pulmonary Hemodynamics

Associated with Pulmonary Hypertension


► Hyperdynamic

Circulatory State

(Anemia, Liver disease)

► Excess Volume

(Left heart dysfunction)

► Vasoconstriction

with vasoproliferation


Screening for Pulmonary Hypertension

Procedure of choice…

• Transthoracic Doppler Echocardiography

• Goals:

– estimate right ventricular systolic pressure (RVSP)

– determine RV size/function

Echocardiographic Features of Pulmonary Hypertension

TR=Tricuspid Regurgitant

Peak Velocity (m/sec)

Dp= 4(TR) 2

RVSP = RA est + Dp

Barnett, C. F. et al. JAMA 2008;299:324-331.

Copyright restrictions may apply.

PH diagnosis via right heart

catheterization (RHC)


MPAP- mean pulmonary artery pressure

CO - cardiac output

PAOP - pulmonary capillary wedge pressure


PVR - pulmonary vascular resistance

Key points regarding Doppler


►RVSP is an estimate of the pulmonary artery

systolic pressure*

►Normal RVSP < 35- 40 mmHg

►But remember…the definition of PH is based

upon mean pulmonary artery pressure

*Assumes pulmonary valve is normal

Signs and symptoms of PH

►Nothing is “pathognomonic”

►Early - exertional dyspnea


• chest pain

• chest pressure

• leg edema

• abnormal ECG/CXR

• syncope

ECG findings of Importance

► RV and RA enlargement

► Tall P waves II, III, aVF

► Right axis

► ST depression and T wave

inversion V1-V4 suggests severe


Chest 1997;111:537-43


►An open lung biopsy to diagnose the

“cause” of pulmonary hypertension?

…risky business

…rarely done

…don’t do it

3 Treatment Pathways




Current PAH Medication Options

• Ca ++ Channel Blocker X

Mild Moderate Severe


IV Prostacyclins

► Prostacyclin (PGI 2 )

► pulmonary vasodilator

• inotropic effect

• inhibits platelet


• antiproliferation?

► continuous 24 hour


► most experience

…other therapeutic options

►If all meds fail…

• atrial septostomy

• off loads RV

…but creates R→L shunt (and hypoxemia)

• organ transplantation

►heart-double lung

►double lung

Treatment Success?

► Hemodynamics

• Doppler Echo

► ↓ RVSP

• Right heart cath

► ↓ MPAP

► ↓ PVR

► ↑ CO

• Right ventricular


► Survival

► Surrogate Markers

• Pro-BNP (B-type natriuretic


• Uric Acid

• 6 Minute walk

• Quality of life

• Symptoms

Family practice considerations…

Survival data…REVEAL Registry publicatiions

…a few comments

►Elderly…PAH vs PVH


►DVT (and what may follow)


►“Lets stop at McDonalds”

►The neighborhood alcoholic

►“Good night Irene”

N = 3,500

DVT x2…5 years later…


2011; 183: 1605-13

►CTEPH frequency?


► .57- 3.8% of those surviving acute PE

►~ 66% have no previous PE history

• Recurrent DVT

• RVSP > 50 mmHg at time of PE

• Myeloproliferative disorders

• Indwelling catheters/ventriculoatrial shunts

• Splenectomy

• Hypercoagulable states (Antiphospholipid antibody syndrome)

Pregnancy and PAH

►A fatal combination

• 30-56% maternal mortality over the years

• Mortality usually within 4 weeks of delivery

►Early counseling of PAH women

►Use Referral Centers

►IV prostacyclin or oral phosphodiesterase



IPAH =29

CHD = 29

Other = 15

Maternal death 18/73 (25%)

Primigravidae highest risk

ERAs teratogenic

All C-section

Sildenafil use

2 maternal deaths


2 weeks postop

Obesity and PH

…massive ascites

due to alcoholic cirrhosis

Portopulmonary hypertension

► PAH as a consequence of portal hypertension

• 3 rd most common reason for referral to PH Clinics

REVEAL Registry 5-year survivals

Chest 2012; 139:1285-1293

IPAH: 64%

POPH: 40%

Overnight Pulse Oximetry

Newest ideas in PAH Treatment

►Identify genetically susceptible individuals

►Initiate “preventive” therapies

• Anti-platelet aggregation

• Inhibition of growth factors

► Prostacyclin receptor agonists (oral)

► Tyrosine kinase inhibitors (Imatinib)

► Rapamycin

Mayo PH Clinic

Daily appts (MD/Self referral)

6 Cardiologists

2 Pulmonologists


In Summary...

► New onset exertional dyspnea…think PH

• Not uncommon in FP setting

► Screen by Transthoracic Doppler Echo

► Definitive diagnosis by right heart cath

• PAH versus PVH

► In PAH… oral vs inhaled vs SQ vs IV?

► selection a function of PAH severity (and $$$)

► Referral PH centers can be very helpful … “co-primary”

Pulmonary Hypertension Association (PHA) Website

• excellent resource

... and sometimes we all are just plain “stuck” and have to ask for help…

Thank you

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