Pulmonary Hypertension
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Transcript of 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 = Q x R 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
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)◦systolic, diastolic, valvular dx
PH secondary to lung dx or hypoxemia (Group 3)◦COPD, ILD, OSA, Hypoventilation
Chronic thromboembolic PH (Group 4) PH due to “unclear multifactorial mechanisms” (Group 5)◦Heme, systemic (sarcoidosis), metabolic
(glycogen storage dx)
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
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
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 Exercise…get off that couch!
◦ Improved functional status
Phosphodiesterase 5 inhibitors (sildenafil)◦ prolong the vasodilatory effect of nitric oxide◦ SUPER trial, improved HD & exercise capacity, no
change in mortality◦ Class III
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
CCB◦ Class II & III
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)
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