II 

109TH CONGRESS 

1ST SESSION S. 1774 

To amend the Public Health Service Act to provide for the expansion, intensification, and coordination of the activities of the National Heart, Lung, 

and Blood Institute with respect to research on pulmonary hypertension. 

IN THE SENATE OF THE UNITED STATES 

SEPTEMBER 27, 2005 

Mr. CORNYN (for himself and Ms. MIKULSKI) introduced the following bill; 

which was read twice and referred to the Committee on Health, Education, Labor, and Pensions 

A BILL 

To amend the Public Health Service Act to provide for 

the expansion, intensification, and coordination of the 

activities of the National Heart, Lung, and Blood Institute with respect to research on pulmonary hypertension. 

1 Be it enacted by the Senate and House of Representa2 tives of the United States of America in Congress assembled, 

3 SECTION 1. SHORT TITLE. 

4 This Act may be cited as the ‘‘Pulmonary Hyper5 tension Research Act of 2005’’. 

6 SEC. 2. FINDINGS. 

7 Congress finds the following: 
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1 (1) In order to take full advantage of the tre2 mendous potential for finding a cure or effective 

3 treatment, the Federal investment in pulmonary hy4 pertension must be expanded, and coordination 

5 among the national research institutes of the Na6 tional Institutes of Health must be strengthened. 

7 (2) Pulmonary hypertension (‘‘PH’’) is a seri8 ous and often fatal condition where the blood pres9 sure in the lungs rises to dangerously high levels. In 

10 PH patients, the walls of the arteries that take 

11 blood from the right side of the heart to the lungs 

12 thicken and constrict. As a result, the right side of 

13 the heart has to pump harder to move blood into the 

14 lungs, causing it to enlarge and ultimately fail. 

15 (3) In the United States it has been estimated 

16 that 300 new cases of PPH are diagnosed each year, 

17 or about 2 persons per million population per year; 

18 the greatest number are reported in women between 

19 the ages of 21 and 40. While at one time the disease 

20 was thought to occur among young women almost 

21 exclusively, we now know, however, that men and 

22 women in all age ranges, from very young children 

23 to elderly people, can develop PPH. It also affects 

24 people of all racial and ethnic origins, with African 
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1 Americans suffering from a mortality rate twice as 

2 high as that affecting Caucasians. 

3 (4) The low prevalence of PPH makes learning 

4 more about the disease extremely difficult. Studies 

5 of PPH also have been difficult because a good ani6 mal model of the disease has not been available. 

7 (5) In about 6 to 10 percent of cases, PPH is 

8 familial. The familial PPH gene is located on chro9 mosome 2 and was discovered in July 2000. This 

10 discovery provided new insights for determining the 

11 molecular basis of PPH and opened new avenues of 

12 study for understanding the fundamental nature of 

13 the disease. 

14 (6) In the more advanced stages of PPH, the 

15 patient is able to perform only minimal activity and 

16 has symptoms even when resting. The disease may 

17 worsen to the point where the patient is completely 

18 bedridden. 

19 (7) PPH remains a diagnosis of exclusion and 

20 is rarely picked up in a routine medical examination. 

21 Even in its later stages, the signs of the disease can 

22 be confused with other conditions affecting the heart 

23 and lungs. The use of new diagnostic standards has 

24 been positively related to the rates of diagnosis. 
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1 (8) In 1981, the National Heart, Lung, and 

2 Blood Institute established the first PPH-patient 

3 registry in the world. The registry followed 194 peo4 ple with PPH over a period of at least 1 year and, 

5 in some cases, for as long as 7.5 years. Much of 

6 what we know about the illness today stems from 

7 this study. 

8 (9) As research progresses, so do treatments for 

9 PH. Currently, there are 4 FDA-approved medica10 tions for PH and 3 more in trials. However, all 

11 medications are not effective on all patients. Lung 

12 transplantation is often considered a treatment of 

13 last resort for PH. 

14 (10) Because we still do not understand the 

15 cause or have a cure for PPH, basic research studies 

16 are focusing on the possible involvement of 

17 immunologic and genetic factors in the cause and 

18 progression of PPH, looking at agents that cause 

19 narrowing of the pulmonary blood vessels, and iden20 tifying factors that cause growth of smooth muscle 

21 and formation of scar tissue in the vessel walls. 

22 (11) Secondary pulmonary hypertension 

23 (‘‘SPH’’) means the cause is known. Common 

24 causes of SPH are the breathing disorders emphy25 sema and bronchitis. Other less frequent causes are 
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1 the inflammatory or collagen vascular diseases such 

2 as scleroderma, CREST syndrome, or systemic lupus 

3 erythematosus (‘‘SLE’’). Other causes include con4 genital heart diseases that cause shunting of extra 

5 blood through the lungs like ventricular and atrial 

6 septal defects, chronic pulmonary thromboembolism, 

7 HIV infection, and liver disease. Sickle cell anemia 

8 is also linked to SPH, with preliminary studies sug9 gesting that approximately one third of sickle cell 

10 patients develop SPH. 

11 SEC. 3. EXPANSION, INTENSIFICATION, AND COORDINA12 TION OF ACTIVITIES OF NATIONAL HEART, 

13 LUNG, AND BLOOD INSTITUTE WITH RESPECT 

14 TO RESEARCH ON PULMONARY HYPER15 TENSION. 

16 Subpart 2 of part C of title IV of the Public Health 

17 Service Act (42 U.S.C. 285b et seq.) is amended by insert18 ing after section 424B the following section: 

19 ‘‘PULMONARY HYPERTENSION

20 ‘‘SEC. 424C. (a) IN GENERAL.— 

21 ‘‘(1) EXPANSION OF ACTIVITIES.—The Director 

22 of the Institute shall expand, intensify, and coordi23 nate the activities of the Institute with respect to re24 search on pulmonary hypertension. 

25 ‘‘(2) COORDINATION WITH OTHER INSTI26 TUTES.—The Director of the Institute shall coordi-
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1 nate the activities of the Director under paragraph 

2 (1) with similar activities conducted by other na3 tional research institutes and agencies of the Na4 tional Institutes of Health to the extent that such 

5 Institutes and agencies have responsibilities that are 

6 related to pulmonary hypertension. 

7 ‘‘(b) CENTERS OF EXCELLENCE.— 

8 ‘‘(1) IN GENERAL.—In carrying out subsection 

9 (a), the Director of the Institute shall make grants 

10 to, or enter into contracts with, public or nonprofit 

11 private entities for the development and operation of 

12 centers to conduct research on pulmonary hyper13 tension. 

14 ‘‘(2) RESEARCH, TRAINING, AND INFORMATION

15 AND EDUCATION.— 

16 ‘‘(A) IN GENERAL.—With respect to pul17 monary hypertension, each center assisted 

18 under paragraph (1) shall— 

19 ‘‘(i) conduct basic and clinical re20 search into the cause, diagnosis, early de21 tection, prevention, control, and treatment 

22 of such disease; 

23 ‘‘(ii) conduct training programs for 

24 scientists and health professionals; 
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1 ‘‘(iii) conduct programs to provide in2 formation and continuing education to 

3 health professionals; and 

4 ‘‘(iv) conduct programs for the dis5 semination of information to the public. 

6 ‘‘(B) STIPENDS FOR TRAINING OF HEALTH

7 PROFESSIONALS.—A center under paragraph 

8 (1) may use funds provided under such para9 graph to provide stipends for scientists and 

10 health professionals enrolled in the programs 

11 described in subparagraph (A)(ii). 

12 ‘‘(3) COORDINATION OF CENTERS; REPORTS.— 

13 The Director shall, as appropriate, provide for the 

14 coordination of information among centers under 

15 paragraph (1) and ensure regular communication 

16 between such centers, and may require the periodic 

17 preparation of reports on the activities of the centers 

18 and the submission of the reports to the Director. 

19 ‘‘(4) ORGANIZATION OF CENTERS.—Each cen20 ter under paragraph (1) shall use the facilities of a 

21 single institution, or be formed from a consortium of 

22 cooperating institutions, meeting such requirements 

23 as may be prescribed by the Director. 

24 ‘‘(5) NUMBER OF CENTERS; DURATION OF SUP25 PORT.—The Director shall, subject to the extent of 
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1 amounts made available in appropriations Acts, pro2 vide for the establishment of not less than 3 centers 

3 under paragraph (1). Support of such a center may 

4 be for a period not exceeding 5 years. Such period 

5 may be extended for 1 or more additional periods 

6 not exceeding 5 years if— 

7 ‘‘(A) the operations of such center have 

8 been reviewed by an appropriate technical and 

9 scientific peer review group established by the 

10 Director; and 

11 ‘‘(B) such group has recommended to the 

12 Director that such period should be extended. 

13 ‘‘(c) DATA SYSTEM; CLEARINGHOUSE.— 

14 ‘‘(1) DATA SYSTEM.—The Director of the Insti15 tute shall establish a data system for the collection, 

16 storage, analysis, retrieval, and dissemination of 

17 data derived from patient populations with pul18 monary hypertension, including, where possible, data 

19 involving general populations for the purpose of 

20 identifying individuals at risk of developing such 

21 condition. 

22 ‘‘(2) CLEARINGHOUSE.—The Director of the 

23 Institute shall establish an information clearinghouse 

24 to facilitate and enhance, through the effective dis25 semination of information, knowledge and under-
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1 standing of pulmonary hypertension by health pro2 fessionals, patients, industry, and the public. 

3 ‘‘(d) PUBLIC INPUT.—In carrying out subsection (a), 

4 the Director of the Institute shall provide for means 

5 through which the public can obtain information on the 

6 existing and planned programs and activities of the Na7 tional Institutes of Health with respect to primary hyper8 tension and through which the Director can receive com9 ments from the public regarding such programs and ac10 tivities. 

11 ‘‘(e) REPORTS.—The Director of the Institute shall 

12 prepare biennial reports on the activities conducted and 

13 supported under this section, and shall include such re14 ports in the biennial reports prepared by the Director 

15 under section 407. 

16 ‘‘(f) AUTHORIZATION OF APPROPRIATIONS.—For the 

17 purpose of carrying out this section, there is authorized 

18 to be appropriated $50,000,000 for each of the fiscal years 

19 2006 through 2010.’’. 

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