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.

October 7, 2004

Mr. CORNYN introduced the following bill; which was read twice and referred to the Committee on Health, Education, Labor, and Pensions

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.

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

SECTION 1. SHORT TITLE.

This Act may be cited as the `Pulmonary Hypertension Research Act of 2004'.

SEC. 2. FINDINGS.

The Congress finds as follows:

(1) In order to take full advantage of the tremendous potential for finding a cure or effective treatment, the Federal investment in pulmonary hypertension must be expanded, and coordination among the national research institutes of the National Institutes of Health must be strengthened.

(2) Primary, or unexplained, pulmonary hypertension (`PPH') is a rare lung disorder which occurs for no apparent reason. It has been historically chronic and incurable with a poor survival rate.

(3) In the United States it has been estimated that 300 new cases of PPH are diagnosed each year, or about two persons per million population per year; the greatest number are reported in women between the ages of 21 and 40. While at one time the disease was thought to occur among young women almost exclusively; we now know, however, that men and women in all age ranges, from very young children to elderly people, can develop PPH. It also affects people of all racial and ethnic origins equally.

(4) The low prevalence of PPH makes learning more about the disease extremely difficult. Studies of PPH also have been difficult because a good animal model of the disease has not been available.

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

(6) In the more advanced stages of PPH, the patient is able to perform only minimal activity and has symptoms even when resting. The disease may worsen to the point where the patient is completely bedridden.

(7) PPH remains a diagnosis of exclusion and is rarely picked up in a routine medical examination. Even in its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs.

(8) In 1981, the National Heart, Lung, and Blood Institute established the first PPH-patient registry in the world. The registry followed 194 people with PPH over a period of at least 1 year and, in some cases, for as long as 7.5 years. Much of what we know about the illness today stems from this study.

(9) Because we still do not understand the cause or have a cure for PPH, basic research studies are focusing on the possible involvement of immunologic and genetic factors in the cause and progression of PPH, looking at agents that cause narrowing of the pulmonary blood vessels, and identifying factors that cause growth of smooth muscle and formation of scar tissue in the vessel walls.

(10) Secondary pulmonary hypertension (`SPH') means the cause is known. Common causes of SPH are the breathing disorders emphysema and bronchitis. Other less frequent causes are the inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome or systemic lupus erythematosus (`SLE'). Other causes include congenital heart diseases that cause shunting of extra blood through the lungs like ventricular and atrial septal defects, chronic pulmonary thromboembolism, HIV infection, liver disease and certain diet drugs.

SEC. 3. EXPANSION, INTENSIFICATION, AND COORDINATION OF ACTIVITIES OF NATIONAL HEART, LUNG, AND BLOOD INSTITUTE WITH RESPECT TO RESEARCH ON PULMONARY HYPERTENSION.

Subpart 2 of part C of title IV of the Public Health Service Act (42 U.S.C. 285b et seq.) is amended by inserting after section 424B the following:

`PULMONARY HYPERTENSION

`SEC. 424C. (a) IN GENERAL-

`(1) EXPANSION OF ACTIVITIES- The Director of the Institute shall expand, intensify, and coordinate the activities of the Institute with respect to research on pulmonary hypertension.

`(2) COORDINATION WITH OTHER INSTITUTES- The Director of the Institute shall coordinate the activities of the Director under paragraph (1) with similar activities conducted by other national research institutes and agencies of the National Institutes of Health to the extent that such Institutes and agencies have responsibilities that are related to pulmonary hypertension.

`(b) CENTERS OF EXCELLENCE-

`(1) IN GENERAL- In carrying out subsection (a), the Director of the Institute shall make grants to, or enter into contracts with, public or nonprofit

private entities for the development and operation of centers to conduct research on pulmonary hypertension.

`(2) RESEARCH, TRAINING, AND INFORMATION AND EDUCATION-

`(A) IN GENERAL- With respect to pulmonary hypertension, each center assisted under paragraph (1) shall--

`(i) conduct basic and clinical research into the cause, diagnosis, early detection, prevention, control, and treatment of such disease;

`(ii) conduct training programs for scientists and health professionals;

`(iii) conduct programs to provide information and continuing education to health professionals; and

`(iv) conduct programs for the dissemination of information to the public.

`(B) STIPENDS FOR TRAINING OF HEALTH PROFESSIONALS- A center under paragraph (1) may use funds under such paragraph to provide stipends for scientists and health professionals enrolled in programs described in subparagraph (A)(ii).

`(3) COORDINATION OF CENTERS; REPORTS- The Director shall, as appropriate, provide for the coordination of information among centers under paragraph (1) and ensure regular communication between such centers, and may require the periodic preparation of reports on the activities of the centers and the submission of the reports to the Director.

`(4) ORGANIZATION OF CENTERS- Each center under paragraph (1) shall use the facilities of a single institution, or be formed from a consortium of cooperating institutions, meeting such requirements as may be prescribed by the Director.

`(5) NUMBER OF CENTERS; DURATION OF SUPPORT- The Director shall, subject to the extent of amounts made available in appropriations Acts, provide for the establishment of not less than three centers under paragraph (1). Support of such a center may be for a period not exceeding 5 years. Such period may be extended for one or more additional periods not exceeding 5 years if the operations of such center have been reviewed by an appropriate technical and scientific peer review group established by the Director and if such group has recommended to the Director that such period should be extended.

`(c) DATA SYSTEM; CLEARINGHOUSE-

`(1) DATA SYSTEM- The Director of the Institute shall establish a data system for the collection, storage, analysis, retrieval, and dissemination of data derived from patient populations with pulmonary hypertension, including where possible, data involving general populations for the purpose of identifying individuals at risk of developing such condition.

`(2) CLEARINGHOUSE- The Director of the Institute shall establish an information clearinghouse to facilitate and enhance, through the effective dissemination of information, knowledge and understanding of pulmonary hypertension by health professionals, patients, industry, and the public.

`(d) PUBLIC INPUT- In carrying out subsection (a), the Director of the Institute shall provide for means through which the public can obtain information on the existing and planned programs and activities of the National Institutes of Health with respect to primary hypertension and through which the Director can receive comments from the public regarding such programs and activities.

`(e) REPORTS- The Director of the Institute shall prepare biennial reports on the activities conducted and supported under this section, and shall include such reports in the biennial reports prepared by the Director under section 407.

`(f) AUTHORIZATION OF APPROPRIATIONS- For the purpose of carrying out this section, there are authorized to be appropriated such sums as may be necessary up to $25,000,000 for each of the fiscal years 2005 through 2009. Such authorizations of appropriations are in addition to any other authorization of appropriations that is available for such purpose.'.

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