Pulmonary Hypertension
the Other High Blood Pressure - Northeast Iowa Family Practice ...
the Other High Blood Pressure - Northeast Iowa Family Practice ...
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<strong>Pulmonary</strong> <strong>Hypertension</strong><br />
…the other high blood pressure<br />
Michael J. Krowka, MD<br />
Division of <strong>Pulmonary</strong> and Critical Care<br />
Mayo Clinic<br />
Jauch Symposium, Waterloo Iowa<br />
May 18, 2012
Current Issues to discuss<br />
► Diagnostics<br />
• New Classification<br />
• New Diagnostic Criteria<br />
• <strong>Pulmonary</strong> artery hypertension vs pulmonary venous hypertension<br />
► <strong>Pulmonary</strong> Artery <strong>Hypertension</strong> (PAH) Therapy update<br />
• The treatment options…$$$<br />
► Specific Family Practice considerations
<strong>Pulmonary</strong> <strong>Hypertension</strong> (PH)<br />
Concept…<br />
…increased blood pressure in the<br />
pulmonary vascular bed<br />
<strong>Pulmonary</strong> artery hypertension (PAH)? or<br />
….<strong>Pulmonary</strong> venous hypertension (PVH) or<br />
…both?
<strong>Pulmonary</strong> Artery <strong>Hypertension</strong>:<br />
The overall concept…<br />
A pulmonary arterial vascular obstructive process*… with<br />
variable presence of vasoconstriction…<br />
* endothelial proliferation<br />
smooth muscle proliferation<br />
in-situ thrombosis<br />
fibrosis<br />
platelet aggregates<br />
plexogenic change
<strong>Pulmonary</strong> <strong>Hypertension</strong> Classification<br />
►1973 Geneva<br />
• Primary pulmonary hypertension (PPH) vs secondary pulmonary hypertension<br />
►1998 Evian<br />
• 5 Subgroups of pulmonary hypertension<br />
• <strong>Pulmonary</strong> artery vs pulmonary venous hypertension<br />
►2003 Venice<br />
• Abandon term PPH – use “idiopathic <strong>Pulmonary</strong> Artery <strong>Hypertension</strong>” (IPAH)<br />
►2008 Dana Point<br />
• Add hematologic diagnoses (hemolytic anemias/splenectomy)<br />
►2013 Nice<br />
• Revisions??
<strong>Pulmonary</strong> Artery <strong>Hypertension</strong> (PAH)<br />
Consequence…<br />
…right heart failure and death
Classification of<br />
<strong>Pulmonary</strong><br />
<strong>Hypertension</strong> (PH)<br />
Summer, 2009<br />
I. <strong>Pulmonary</strong> artery hypertension<br />
(PAH)<br />
II. PH owing to left heart disease<br />
III. PH owing to lung<br />
disease/hypoxemia<br />
IV. PH due to chronic pulmonary<br />
emboli<br />
V. PH with unclear/multifactorial<br />
etiology
Group I - <strong>Pulmonary</strong> Artery <strong>Hypertension</strong><br />
Dana Point 2008<br />
I. <strong>Pulmonary</strong> artery hypertension - PAH<br />
1.1 Idiopathic<br />
1.2 Heritable<br />
a.BMPR2<br />
b. ALK1<br />
1.3 Drugs and Toxins<br />
1.4 Associated with<br />
1.4.1 connective tissue disease<br />
1.4.2 HIV infection<br />
1.4.3 portal hypertension<br />
1.4.4 congenital heart disease<br />
1.4.5 schistosomiasis<br />
1.4.6 chronic hemolytic anemia<br />
Importance?...<br />
…insurers will pay for Group I
The Diagnostic Criteria for <strong>Pulmonary</strong> Artery<br />
<strong>Hypertension</strong><br />
Right heart catheterization...<br />
• Mean <strong>Pulmonary</strong> Artery Pressure (MPAP) > 25 mm Hg<br />
• <strong>Pulmonary</strong> Artery Occlusion Pressure (PAOP) < 15 mm Hg<br />
• <strong>Pulmonary</strong> Vascular Resistance (PVR) > 240 dynes.s.cm -5<br />
where PVR = (MPAP-PAOP) * 80<br />
CO
Common <strong>Pulmonary</strong> Hemodynamics<br />
Associated with <strong>Pulmonary</strong> <strong>Hypertension</strong><br />
MPAP PVR CO PAOP<br />
► Hyperdynamic<br />
Circulatory State<br />
(Anemia, Liver disease)<br />
► Excess Volume<br />
(Left heart dysfunction)<br />
► Vasoconstriction<br />
with vasoproliferation<br />
(IPAH, POPH)
Screening for <strong>Pulmonary</strong> <strong>Hypertension</strong><br />
Procedure of choice…<br />
• Transthoracic Doppler Echocardiography<br />
• Goals:<br />
– estimate right ventricular systolic pressure (RVSP)<br />
– determine RV size/function
Echocardiographic Features of <strong>Pulmonary</strong> <strong>Hypertension</strong><br />
TR=Tricuspid Regurgitant<br />
Peak Velocity (m/sec)<br />
Dp= 4(TR) 2<br />
RVSP = RA est + Dp<br />
Barnett, C. F. et al. JAMA 2008;299:324-331.<br />
Copyright restrictions may apply.
PH diagnosis via right heart<br />
catheterization (RHC)<br />
Measure<br />
MPAP- mean pulmonary artery pressure<br />
CO - cardiac output<br />
PAOP - pulmonary capillary wedge pressure<br />
Calculate<br />
PVR - pulmonary vascular resistance
Key points regarding Doppler<br />
echo...<br />
►RVSP is an estimate of the pulmonary artery<br />
systolic pressure*<br />
►Normal RVSP < 35- 40 mmHg<br />
►But remember…the definition of PH is based<br />
upon mean pulmonary artery pressure<br />
*Assumes pulmonary valve is normal
Signs and symptoms of PH<br />
►Nothing is “pathognomonic”<br />
►Early - exertional dyspnea<br />
►Late<br />
• chest pain<br />
• chest pressure<br />
• leg edema<br />
• abnormal ECG/CXR<br />
• syncope
ECG findings of Importance<br />
► RV and RA enlargement<br />
► Tall P waves II, III, aVF<br />
► Right axis<br />
► ST depression and T wave<br />
inversion V1-V4 suggests severe<br />
PAH<br />
Chest 1997;111:537-43
Caveat<br />
►An open lung biopsy to diagnose the<br />
“cause” of pulmonary hypertension?<br />
…risky business<br />
…rarely done<br />
…don’t do it
3 Treatment Pathways<br />
Block<br />
Enhance<br />
Enhance
Current PAH Medication Options<br />
• Ca ++ Channel Blocker X<br />
Mild Moderate Severe<br />
25
IV Prostacyclins<br />
► Prostacyclin (PGI 2 )<br />
► pulmonary vasodilator<br />
• inotropic effect<br />
• inhibits platelet<br />
aggregation?<br />
• antiproliferation?<br />
► continuous 24 hour<br />
infusion<br />
► most experience
…other therapeutic options<br />
►If all meds fail…<br />
• atrial septostomy<br />
• off loads RV<br />
…but creates R→L shunt (and hypoxemia)<br />
• organ transplantation<br />
►heart-double lung<br />
►double lung
Treatment Success?<br />
► Hemodynamics<br />
• Doppler Echo<br />
► ↓ RVSP<br />
• Right heart cath<br />
► ↓ MPAP<br />
► ↓ PVR<br />
► ↑ CO<br />
• Right ventricular<br />
size/function<br />
► Survival<br />
► Surrogate Markers<br />
• Pro-BNP (B-type natriuretic<br />
peptide)<br />
• Uric Acid<br />
• 6 Minute walk<br />
• Quality of life<br />
• Symptoms
Family practice considerations…<br />
Survival data…REVEAL Registry publicatiions<br />
…a few comments<br />
►Elderly…PAH vs PVH<br />
►Syncope<br />
►DVT (and what may follow)<br />
►Pregnancy<br />
►“Lets stop at McDonalds”<br />
►The neighborhood alcoholic<br />
►“Good night Irene”
N = 3,500
DVT x2…5 years later…
AJRCCM<br />
2011; 183: 1605-13<br />
►CTEPH frequency?<br />
►Risk?<br />
► .57- 3.8% of those surviving acute PE<br />
►~ 66% have no previous PE history<br />
• Recurrent DVT<br />
• RVSP > 50 mmHg at time of PE<br />
• Myeloproliferative disorders<br />
• Indwelling catheters/ventriculoatrial shunts<br />
• Splenectomy<br />
• Hypercoagulable states (Antiphospholipid antibody syndrome)
Pregnancy and PAH<br />
►A fatal combination<br />
• 30-56% maternal mortality over the years<br />
• Mortality usually within 4 weeks of delivery<br />
►Early counseling of PAH women<br />
►Use Referral Centers<br />
►IV prostacyclin or oral phosphodiesterase<br />
inhibitors
N=73<br />
IPAH =29<br />
CHD = 29<br />
Other = 15<br />
Maternal death 18/73 (25%)<br />
Primigravidae highest risk<br />
ERAs teratogenic
All C-section<br />
Sildenafil use<br />
2 maternal deaths<br />
intraop<br />
2 weeks postop
Obesity and PH
…massive ascites<br />
due to alcoholic cirrhosis
Portopulmonary hypertension<br />
► PAH as a consequence of portal hypertension<br />
• 3 rd most common reason for referral to PH Clinics
REVEAL Registry 5-year survivals<br />
Chest 2012; 139:1285-1293<br />
IPAH: 64%<br />
POPH: 40%
Overnight Pulse Oximetry
Newest ideas in PAH Treatment<br />
►Identify genetically susceptible individuals<br />
►Initiate “preventive” therapies<br />
• Anti-platelet aggregation<br />
• Inhibition of growth factors<br />
► Prostacyclin receptor agonists (oral)<br />
► Tyrosine kinase inhibitors (Imatinib)<br />
► Rapamycin
Mayo PH Clinic<br />
Daily appts (MD/Self referral)<br />
6 Cardiologists<br />
2 Pulmonologists
PHAssociation.org
In Summary...<br />
► New onset exertional dyspnea…think PH<br />
• Not uncommon in FP setting<br />
► Screen by Transthoracic Doppler Echo<br />
► Definitive diagnosis by right heart cath<br />
• PAH versus PVH<br />
► In PAH… oral vs inhaled vs SQ vs IV?<br />
► selection a function of PAH severity (and $$$)<br />
► Referral PH centers can be very helpful … “co-primary”<br />
► <strong>Pulmonary</strong> <strong>Hypertension</strong> Association (PHA) Website<br />
• excellent resource<br />
... and sometimes we all are just plain “stuck” and have to ask for help…
Thank you
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