Download Pulmonary Hypertension

Katie DePlatchett M.D.
AM Report
June 29, 2010
Elevated Pulmonary Artery pressure
 Secondary R Ventricular failure
 Mean Pulm Artery Pressure of
>25mmHg at rest
 Pulmonary capillary wedge pressure
(PCWP) <15mmHG

◦ Diagnosed by Right Heart Catherization
(RHC)
Ohm’s Law:
P=QxR
Pa- Pv = CO (right sided) x PVR
Pa = (CO x PVR) + PCWP
 PVR: occlusion of small arterioles,
hypoxic vasoconstriction
 CO: congenital defects (shunts),
cirrhosis
 PCWP: systolic HF, valvular dx
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intimal hyperplasia and fibrosis, medial
hypertrophy, and in situ thrombi of the
small pulmonary arteries and arterioles
Previously idiopathic PAH vs secondary
PAH
 Amended to groups (Group 1-5)
based on etiology

Idiopathic
 Heritable
 Disease which localize to small pulm
arterioles

◦ connective tissue disease, HIV, portal htn, chronic
hemolytic anemia, congenital

Drug or toxin induced
◦ amphetamines, cocaine, appetite suppressants
(fen-fen), chemotherapies, St. John’s wort, SSRIs

PH secondary to L heart disease (G2)

PH secondary to lung dx or hypoxemia
(Group 3)
◦ systolic, diastolic, valvular dx
◦ COPD, ILD, OSA, Hypoventilation
Chronic thromboembolic PH (Group 4)
 PH due to “unclear multifactorial
mechanisms” (Group 5)

◦ Heme, systemic (sarcoidosis), metabolic
(glycogen storage dx)
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Increased intensity of pulmonic
component of 2nd heart sound or split P2
Systolic ejection murmur over LSB
Diastolic murmur over LSB (d/t pulm
regurg)
R-sided S3 or S4
Elevated JVP
Peripheral edema
Hepatomegaly, ascites
CXR: enlargement of the central
pulmonary arteries with attenuation of
the peripheral vessels
 ECG R heart strain, RAD
 Echo estimate the pulmonary artery
systolic pressure and to assess right
ventricular size, thickness, and
function

◦ PH is likely if the PASP is >50 and the TRV is >3.4
Labs: HIV, LFTs, ANA, RF, ANCA, ?
evidence of chronic hemolytic anemia
 PFTs to identify and characterize
underlying lung disease that may be
contributing
 Overnight oximetry to assess
nocturnal oxyhemoglobin desaturation
 Sleep study for eval of OSA
 V/Q scan for chronic thromboemboli

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Right Heart Catherization
◦ necessary to confirm the diagnosis of PH and
accurately determine the severity of the
hemodynamic derangements

6 min walk
◦ To determine functional status
◦ Assist with prognosis
◦ Some cases are exercised induced
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Class: I Ordinary physical activity does not cause
undue fatigue or dyspnea, chest pain, or heart
syncope.
Class II: Slight limitation of physical activity.
Ordinary physical activity results in undue fatigue
or dyspnea, chest pain, or heart syncope.
Class III: Less than ordinary physical activity
causes undue fatigue or dyspnea, chest pain, or
heart syncope.
Class IV: Inability to carry on any physical activity
without symptoms. Usually manifest signs of
right heart failure. Dyspnea and/or fatigue may
be present even at rest.
Oxygen
 Diuretics
 Anticoagulation for groups 1 & 4

◦ intrapulmonary thrombosis & thromboembolism,
due to sluggish pulmonary blood flow, dilated right
heart chambers, venous stasis, and a sedentary
lifestyle
◦ Warfarin therapy w/ INR target of 2.0
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Exercise…get off that couch!
◦ Improved functional status
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Phosphodiesterase 5 inhibitors (sildenafil)
◦ prolong the vasodilatory effect of nitric oxide
◦ SUPER trial, improved HD & exercise capacity, no change
in mortality
◦ Class III
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Endothelian receptor antagonist (Tracleer)
◦ Endothelin-1 is a potent vasoconstrictor and smooth
muscle mitogen
◦ BREATHE-1 trial, improved symptoms, the six-minute
walking distance, and the WHO functional class
◦ Class II & III
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CCB
◦ Class II & III
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Vasoreactivity testing during RHC
◦ Measures HD response to nitric oxide or Flolan
◦ If Pulm artery pressure decreases by 10mm Hg & is
<40mm Hg w/o a significant change in CO, then
trial of CCB (Diltiazem 120mg daily & titrate)
◦ If negative (no response) Prostanoids (Flolan,
Remodulin)
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Prostanoids = prostacyclins
◦ Potent vasodialator & inhibitor of platet aggregation
Symptomatic IPAH w/o treatment have
a median survival of ~ 3 years
 Symptomatic w/ PAH that is
associated with another disease
generally have a worse prognosis
 Severe PAH or right CHF median
survival of 1 year w/o treatment
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