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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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