11/1/2014 how does ph come to our attention?...pulmonary vascular resistance (pvr) •the analog of...
TRANSCRIPT
11/1/2014
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PULMONARY HYPERTENSION
Dr. Bradley Weinberg 10/31/14
How Does PH Come to Our Attention?
• Evaluation of symptoms such as Dyspnea
• Echocardiogram done for other reasons
• Screening test done for connective tissue disease of for family history of PAH
Pulmonary HypertensionClinical Classification
• GROUP 1 Pulmonary Arterial Hypertension
• Idiopathic Formerly Primary Pulmonary HTN
• Familial (BMPR2)• Associated with Connective Tissue Disorders• Congenital Left to Right Shunt• Portal HTN• Drugs or Toxins• HIV• Hemoglobinopathies• Schistosomiasis• Heriditary Hemorrhagic Telangiectasia
GROUP 2 Left Heart Disease
• Systolic Heart Failure
• Valvular Heart Disease
• Diastolic Heart Failure
Pulmonary HypertensionClinical Classification
• GROUP 3 Hypoxemia or Lung Disease Interstitial Pulmonary Fibrosis, COPD, ?Disordered Sleep Breathing
• GROUP 4 Chronic Thombolembolic PH (CTEPH)
• GROUP 5 Miscellaneous Sarcoidoisis, Histiocytosis Lymphangiomatosis
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Post-capillary vs. Precapillary PHNormal value of mean pulmonary
artery pressure
• Normal mean PA pressure 15 +/- 5 mm Hg
• So a typical PA pressure might be 25/10
• Pulmonary HTN is a mean PA > or = 25 mm Hg
Pulmonary vascular resistance (PVR)
• The analog of Ohm’s law V= I x R
• Pressure drop across pulmonary vasculature is = Cardiac output x PVR
• Pressure drop across pulmonary vasculature is the mean PA – mean PCWP
• PVR = (mean PA-mean PCWP)/Cardiac output
• Normal PVR about 1 to 2 Wood units so mean PA of 15 – PCWP of 10 =5 divided by CO of 5
Post-capillary PH or Group 2 PH
• Elevated LVEDP leads to elevated left atrial pressure, that is greater than 15 mm Hg
• This leads to increased pressure in the pulmonary veins and PA pressure passively increases
• Over time, reactive pulmonary vasoconstriction may occur further elevating the pulmonary artery pressure
• Ultimately, fixed pulmonary hypertension may occur.
Patient 1
• Mean RA 17 mm Hg
• RV 66/20
• PA 65/27 Mean 39
• PCWP Mean 22
• Fick CO 5.9 liters/min (Index 2.7 L/m/sqm BSA)
• Transpulmonary gradient 17 mm Hg
• PVR 2.9 Wood units (17/5.9)
Patient 1
• This is Group 2 PH or post-capillary PH
• Also called pulmonary venous hypertension
• LVEF is 25%
• Five prior myocardial infarctions
• Chronic atrial fibrillation
• Ended up doing well with an LVAD or left ventricular assist device.
• (Finally quit smoking!)
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Patient 2
• Mean RA 22 mm Hg
• RV 95/26
• PA 98/38 Mean 58
• PCWP Mean 10
• Fick CO 3.1 liters/min (index 2.04 l/min/sq m)
• Transpulmonary gradient 48 mm Hg
• PVR 15.5 Wood units (48/3.1)
Patient 2
• This is precapillary PH
• It is due to Group 1 pulmonary hypertension but without more clinical information or testing it could alternatively be due to Group 3 or Group 4 or Group 5 that is pulmonary disease or Chronic thromboembolic pulmonary hypertension or “other”
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Patient 3 Right heart cath findings
• Mean RA pressure 13 mm Hg
• RV pressure 92/14
• PA pressure 99/32 with PA mean 52
• Mean PCWP 18 mm Hg
• Fick Cardiac output 5.13 l/min (Index 3.04)
• Transpulmonary pressure gradient 34 mm Hg
• PVR 6.6 Wood units
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