2/4/2011. Nathan Kerner, M.D.

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1 Nathan Kerner, M.D. Definition Elevated pressures - cut off usually >40 mmhg pulmonary artery systolic pressure (PASP) Usually associated with elevated pulmonary vascular resistance (PVR) measured in dynessec/cm -5 or Wood units - Normal WU (1-4 in table 67-1) Causes and WHO Classification 1. Pulmonary arterial hypertension Idiopathic Familial Portal hypertension, HIV, drugs and toxins Pulmonary venous hypertension 2. Pulmonary hypertension (PHT) with left heart disease 3. PHT from respiratory system disease or hypoxemia 4. PHT from pulmonary thromboembolic disease 5. PHT due to systemic diseases affecting the pulmonary vasculature 1

2 WHO Functional Classification Class I Patients with pulmonary HTN but without limitation of physical activity Class II Pulmonary HTN resulting in slight limitation of physical activity. Comfortable at rest, although ordinary activity causes symptoms. Class III Marked limitation of activity even mild (less than ordinary activity) Class IV Symptoms may be present at rest Signs and/or symptoms of right heart failure Clinical Manifestations Dyspnea Signs/Symptoms of right ventricular failure Edema Angina usually signifies more advanced disease Syncope or near syncope Signs/symptoms related to underlying disease e.g. CHF or COPD symptoms Diagnostic Testing - Causes Chest x-ray EKG Ventilation/Perfusion lung scan (Chest CT) for pulmonary embolism Echocardiography TTE (TEE for possible shunts) Pulmonary function testing Sleep apnea studies Connective tissue disease workup (SLE, polyarteritis nodosa, scleroderma) Liver function tests, U/S, CT 2

3 Screening for presence of pulmonary HTN Assessment of cause of pulmonary HTN Quantitation of pulmonary pressure (RVSP) and pulmonary vascular resistance (PVR) Assessment of RV/RA size and function Evaluation of response to medical +/or surgical therapy Research in pulmonary HTN Screening for PHTN measurement of RVSP Measurement of RV-RA Gradient by TV velocity profile Estimation of Right Atrial Pressure IVC Change with Respiration or "Sniff" Estimated RA Pressure Small (<1.5cm) Collapse 0-5 mmhg Normal ( cm) Decrease by >50% 5-10 mmhg Normal Decrease by <50% mmhg Dilated (>2.5cm) Decrease by <50% mmhg Dilated w/systolic flow reversal in hepatic veins No Change >20 mmhg IVC Subcostal view Record and measure to look for respiratory variation) 3

4 IVC M-Mode Record strip assess (and measure where necessary to look for respiratory variation) LV systolic dysfunction LV diastolic dysfunction Valvular heart disease Native or prosthetic Congenital heart disease ASD, VSD, other shunts Pulmonary embolus Obstructive lesions Coarctation Sub- or supravalvular aortic stenosis Short axis Mid LV level Septal flattening 4

5 Note difficulty in assessing RV contractility?normal or Abnormal Subcostal view in same patient?normal or Abnormal Measure RVOT flow Look for rapid acceleration of flow Notching of flow pattern Notching Normal Rapid Acceleration 5

6 Measurement TR jet in multiple views and use the highest value RV inflow Short Axis 4-Chamber Subcostal Make sure you have holosystolic flow pattern In irregular rhythm need average of 5 beats Note Must add the RA pressure to these values to get the RVSP Badesh, D. et. al. (2009)Diagnosis and Assessment of Pulmonary Hypertension. JACC 54(1): Suppl.S Note that PA pressure (RV systolic pressure) may be relatively normal at rest and may rise abnormally with exercise Normal response is little or no rise 6

7 Assessment of Right ventricular systolic function 3-Dimensional reconstruction of RVEF Assessment of RA and RV chamber size Best done with other modalities (e.g. MRI) Echocardiography is the mainstay in the screening for patients with PHTN Echo extremely useful in identifying the cause(s) of PHTN Monitoring of therapy is aided by echo 7

8 We can and do play a central role in the assessment, treatment and outcome of these patients Meticulous detail is therefore required to Accurately report RVSP Assess the hemodynamic consequence to any individual patient 8