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