46. "Primary Pulmonary Hypertension" ("PPH") is defined as either or both of the following:

          a. For a diagnosis based on examinations and clinical findings prior to death:

               (1) Mean pulmonary artery pressure by cardiac catheterization of > 25 mm Hg at rest
               or > 30 mm Hg with exercise with a normal pulmonary artery wedge pressure < 15
               mm Hg ; or

                    (b) A peak systolic pulmonary artery pressure of > 60 mm Hg at rest
                    measured by Doppler echocardiogram utilizing standard procedures; or

                    (c) Administration of Flolan to the patient based on a diagnosis of PPH
                    with cardiac catheterization not done due to increased risk in the face of
                    severe right heart dysfunction; and

               (2) Medical records which demonstrate that the following conditions have been
               excluded by the following results :

                    (a) Echocardiogram demonstrating no primary cardiac disease including,
                    but not limited to, shunts, valvular disease (other than tricuspid or
                    pulmonary valvular insufficiency as a result of PPH or trivial, clinically
                    insignificant left-sided valvular regurgitation), and congenital heart disease
                    (other than patent foramen ovale); and

                    (b) Left ventricular dysfunction defined as LVEF < 40% defined by
                    MUGA, Echocardiogram or cardiac catheterization; and

                    (c) Pulmonary function tests demonstrating the absence of obstructive lung
                    disease (FEV1/FVC > 50% of predicted) and the absence of greater
                    than mild restrictive lung disease (total lung capacity > 60% of predicted
                    at rest); and

                    (d) Perfusion lung scan ruling out pulmonary embolism; and

                    (e) If, but only if, the lung scan is indeterminate or high probability, a
                    pulmonary angiogram or a high resolution angio computed tomography
                    scan demonstrating absence of thromboembolic disease; and

               (3) Conditions known to cause pulmonary hypertension , , including connective tissue
               disease known to be causally related to pulmonary hypertension, toxin induced lung
               disease known to be causally related to pulmonary hypertension, portal hypertension,
               significant obstructive sleep apnea, interstitial fibrosis (such as silicosis, asbestosis, and
               granulomatous disease) defined as greater than mild patchy interstitial lung disease, and
               familial causes, have been ruled out by a Board-Certified Cardiologist or
               Board-Certified Pulmonologist as the cause of the person's pulmonary hypertension.
 

                                              -OR-

          b. For a diagnosis made after the individual's death:

               (1) Autopsy demonstrating histopathologic changes in the lung consistent with primary
               pulmonary hypertension and no evidence of congenital heart disease (other than a
               patent foramen ovale) with left-to-right shunt, such as ventricular septal defect as
               documented by a Board-Certified Pathologist; and

               (2) Medical records which show no evidence of alternative causes as described above
               for living persons.

     This definition of PPH ("the PPH Definition") is intended solely for the purpose of describing claims excluded from
     the definition of Settled Claims and for purposes of Section VII.B.4 and 5, below. The Parties agree that the PPH
     Definition includes but is broader than the rare and serious medical condition suffered by the individuals described
     in L. Abenhaim, et al., Appetite-Suppressant Drugs and the Risk of Primary Pulmonary Hypertension,
     International Primary Pulmonary Hypertension Study Group, 335(9), New England Journal of Medicine, 609-16
     (1996) (the "IPPHS study"). The subjects in that study exhibited significantly elevated pulmonary artery pressures
     with an average systolic pulmonary artery pressure of 88 mm Hg and average mean pulmonary artery pressure of
     57 mm Hg. Two-thirds of the IPPHS patients demonstrated NYHA Class III or IV symptoms. While the IPPHS
     subjects would fall within the PPH Definition, the definition also includes persons with a milder, less serious
     medical condition.