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Small nonrandomized studies have suggested that sildenafil added to subcutaneous treprostinil 95 and sildenafil added to bosentan 96 may improve exercise tolerance in patients with PAH. A placebo-controlled study of the addition of an investigational oral treprostinil to either sildenafil or an endothelin receptor antagonist or both FREEDOM-C did not show a significant benefit of the study drug over placebo in the primary end point of 6-minute walk distance.

Because pharmacologic regimens can be revised or supplemented, patients should be carefully monitored to assess whether outcome objectives are being achieved. One study adopted a systematic approach to modifying treatment on the basis of whether prognostically improved end points had been achieved. This strategy yielded a better outcome for survival, lung transplant, and need for intravenous prostacyclin analogues than was expected given historical controls and predictive formulae. The need for systematic reevaluation of patients is a compelling argument for managing these patients in a higher-volume referral center with experienced physician and nursing staffs.

A complex and demoralizing aspect of treating patients with PH is the great expense of the required medications. Although PH is relatively rare compared with such diseases as coronary artery disease and chronic heart failure, expenses of this magnitude pose a burden not only for individual patients but also for medical insurers and for society as a whole.

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Negotiating the medical reimbursement obstacles represents a major part of the management of patients with PH. Use of sildenafil presents uniquely challenging scenarios for the clinician. Although sildenafil is approved for use at a dosage of 20 mg 3 times daily, higher doses not infrequently have more beneficial effects.

Interestingly, higher doses can be provided at the same cost if sildenafil is prescribed as Viagra as much as mg 3 times daily , which is approved only for erectile dysfunction, rather than as Revatio, which is approved for use in PAH. Even though the drugs are the same and are manufactured by the same pharmaceutical company Pfizer, New York, NY , medical coverage agencies, including Medicare, nevertheless generally will not cover Viagra for this purpose.

This insurance policy leads to the awkward situation in which men with erectile dysfunction are at an advantage with respect to medical coverage, compared with the typical young woman with life-threatening PAH. Although this inequality can occasionally be rectified for individual patients, such case-by-case corrections do not mitigate the essential injustice of this policy. Efforts are ongoing to correct this policy. Pulmonary arterial hypertension is a complex disease with correspondingly complex treatment.

In many ways, the pharmacologic treatment of patients with PAH can be likened to the treatment of patients with cancer using chemotherapeutic agents. Indeed, because PAH may represent a disorder of angiogenesis, some investigators consider it to be a neoplastic process. In general, the management of cancer is orchestrated by teams of specialists in centers of expertise where carefully tailored therapy, follow-up, and clinical research are conducted.

The management of PAH should arguably be provided in analogous centers of experience, where dedicated multispecialty nursing staff and clinicians can bring to bear their experience and efficiencies to ensure optimal treatment of patients. The clinical understanding of PAH is imperfect and its recognition and diagnosis are frequently delayed. Treatment of patients with PAH is, at best, palliative, and assessment of outcomes and modification of treatment is an ambiguous process. Large populations of patients with PH are without resources for management, including patients in developing countries who have PH secondary to schistosomiasis.

Efforts are under way to improve the medical approach to each of these issues. Effective diagnosis requires that both physicians and patients be aware of PH as a potential explanation for observed symptoms. Substantial resources have been deployed to enhance the understanding of PH. A lynchpin in this effort has been the Pulmonary Hypertension Association, a patient-initiated coalition of scientists, clinicians, patients, pharmaceutical companies, and specialty pharmacies that has organized educational programs, research funding, support groups, Web sites, and conferences in an effort to advance our understanding of this disease.

As information has been disseminated to general internists, pulmonologists, cardiologists, and rheumatologists, it has become increasingly apparent that, although recognition and diagnosis reside in these first-line disciplines, treatment and follow-up need to be focused in dedicated centers of specialization. Centers with dedicated clinics and experienced personnel are best equipped to efficiently address the complex logistical and clinical problems associated with the long-term care of patients with PAH, which is analogous to the management of patients with life-threatening cancers.

Identifying the best and most clinically appropriate way to conduct follow-up management of patients with PAH remains the focus of considerable effort. To date, parameters of symptomatic status and exercise capacity have been the primary variables of therapeutic efficacy and progression of disease. Hemodynamic assessment, although central to the pathophysiologic understanding and diagnosis of the disease, has shown an uncertain relationship to PAH symptoms and outcome. This uncertainty may in part be the result of inherent ambiguities of noninvasive hemodynamic assessment eg, echocardiography and the infrequency of invasive evaluation eg, hemodynamic right heart catheterization.

Avenues for a more definitive understanding of the hemodynamics of PH are being explored. Implantable hemodynamic monitors may advance our understanding of the hemodynamic natural history of PH and the response of patients to treatment. With the advent of multiple therapeutic options, the treatment strategies that may be optimal for particular subpopulations of patients with PAH are unclear. Large-scale database registries are being developed to investigate both the demographics of PAH and the outcomes associated with various combinations of pharmacologic treatment.

Convergence of these many investigational efforts may substantially advance our understanding of PAH within the next several years. Current treatment is directed at 3 recognized pathobiologic pathways involved in the initiation and maintenance of PH Figure 1. However, other mechanisms have been implicated in this condition and may have therapeutic ramifications. Many of these mechanisms are either under consideration for clinical studies or are under active investigation Table 3.

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During the past decade, the clinical concept of PAH has evolved from that of a rare and incurable fatal disease to that of a not uncommonly encountered syndrome that is a component of numerous clinical scenarios and that can be managed effectively to reduce symptoms and prolong life. Advances in understanding the mechanisms, clinical presentations, and natural history of PAH, as well as treatments for patients with PAH, continue to evolve.

Keys to promoting this progress rely on the prompt recognition and evaluation of suspected PAH cases by physicians and the application of effective treatment and clinical study protocols in dedicated PAH centers. National Center for Biotechnology Information , U. Journal List Mayo Clin Proc v. Kane , MD, PhD. Address correspondence to Michael D. Individual reprints of this article and a bound reprint of the entire Symposium on Cardiovascular Diseases will be available for purchase from our Web site www.

This article has been corrected. See Mayo Clin Proc. This article has been cited by other articles in PMC. Abstract Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. M olecular and C ellular M echanisms Regardless of underlying causes or associations, several interrelated mechanisms play a role in the development and maintenance of PAH Figure 1. Open in a separate window. DIAGNOSIS Although pulmonary hypertension refers to the presence of a mean pulmonary arterial pressure mPAP of greater than 25 mm Hg regardless of mechanism, pulmonary hypertensive disorders are classified into groups on the basis of underlying mechanism, presentation, clinical context, histopathology, and response to treatment Table 1.

Current Clinical Classification of Pulmonary Hypertension a. C alcium C hannel B lockers Before the availability of the current therapeutic agents, patients with pulmonary hypertensive conditions were treated with numerous vasodilator agents, with little demonstrable benefit. P rostacyclin A nalogues Prostacyclin, a metabolite of arachidonic acid that is produced in the vascular endothelium, inhibits platelet aggregation and is a potent pulmonary vasodilator.

E ndothelin R eceptor A ntagonists Endothelin-1 causes vasoconstriction of pulmonary vessels and stimulates smooth muscle and fibroblast proliferation by acting at endothelin receptor subtypes A and B. P hosphodiesterase -5 I nhibitors Sildenafil, which was originally used for treatment of patients with erectile dysfunction, is a potent, highly specific inhibitor of PDE5 that improves symptoms and functional capacity in patients with PAH.

A ngina The predominant symptom of PAH, and the one with which patients typically present, is dyspnea on exertion. E xercise The clinical importance of PH that develops only with exercise remains enigmatic. A ccuracy of H emodynamic E valuation A criterion of therapeutic success is the degree to which an indicator of disease severity changes during treatment. D isproportionate PH Pulmonary hypertension is often associated with other diseases, such as left-sided heart disease, various lung diseases, and sleep disorders. P regnancy Pregnancy poses extremely high risks for the woman with PAH and her fetus.

C ombinations of M edications Many patients with PAH do not achieve treatment objectives through monotherapy. A ssessment of T herapeutic E fficacy Because pharmacologic regimens can be revised or supplemented, patients should be carefully monitored to assess whether outcome objectives are being achieved. M edication C ost and I nsurance C overage A complex and demoralizing aspect of treating patients with PH is the great expense of the required medications.

Pulmonary hypertension - Symptoms and causes - Mayo Clinic

T reatment D irected at O ther P athobiologic M echanisms Current treatment is directed at 3 recognized pathobiologic pathways involved in the initiation and maintenance of PH Figure 1. Update in pulmonary arterial hypertension Pulmonary arterial hypertension [published correction appears in J Am Coll Cardiol. Taichman DB, Mandel J. Epidemiology of pulmonary arterial hypertension. Pathobiology of pulmonary hypertension. Circulation ; Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines.

Chest ; 1 suppl: Screening, early detection, and diagnosis of pulmonary arterial hypertension: Chest ; suppl 1: Guidelines on diagnosis and treatment of pulmonary arterial hypertension: Medical therapy for pulmonary arterial hypertension: Voelkel NF, Cool C. Pathology of pulmonary hypertension. N Engl J Med. Chest ; 6: Familial primary pulmonary hypertension gene PPH1 is caused by mutations in the bone morphogenetic protein receptor-II gene.

Am J Hum Genet. Genetic basis of pulmonary arterial hypertension: J Am Coll Cardiol. Genetic anticipation and abnormal gender ratio at birth in familial primary pulmonary hypertension. Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia [published correction appears in J Med Genet.

Hyperplasia of pulmonary artery smooth muscle cells is causally related to overexpression of the serotonin transporter in primary pulmonary hypertension. Chest ; 3 suppl: Serotonin-induced smooth muscle hyperplasia in various forms of human pulmonary hypertension. Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension.

An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. Endothelin-1 in primary pulmonary hypertension and the Eisenmenger syndrome. Pulmonary endothelin-1 clearance in human pulmonary arterial hypertension. Chest ; 6 suppl: Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension.

Decreased whole body endogenous nitric oxide production in patients with primary pulmonary hypertension. Increased plasma serotonin in primary pulmonary hypertension. Circulation ; 98 Anorexic agents aminorex, fenfluramine, and dexfenfluramine inhibit potassium current in rat pulmonary vascular smooth muscle and cause pulmonary vasoconstriction. Circulation ; 94 9: Inflammation in pulmonary arterial hypertension. Exuberant endothelial cell growth and elements of inflammation are present in plexiform lesions of pulmonary hypertension.

Coagulation and fibrinolytic parameters in patients with pulmonary hypertension. Altered hemostasis in pulmonary hypertension. Blood Coag Fibrinolysis ; 9 2: Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Coagulation and fibrinolytic profiles in patients with severe pulmonary hypertension.

Chest ; 3: Clinical classification of pulmonary hypertension. Diagnosis and differential assessment of pulmonary arterial hypertension. Pulmonary arterial hypertension associated with fenfluramine exposure: International Primary Pulmonary Hypertension Study Group Appetite-suppressant drugs and the risk of primary pulmonary hypertension. Is methamphetamine use associated with idiopathic pulmonary arterial hypertension? Circulation ; 70 4: The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension.

Thrombotic arteriopathy and anticoagulation in pulmonary hypertension. Chest ; 2: Bjornsson J, Edwards WD. Rich S, Brundage BH. High-dose calcium channel-blocking therapy for primary pulmonary hypertension: Circulation ; 76 1: Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation June14; Primary Pulmonary Hypertension Study Group A comparison of continuous intravenous epoprostenol prostacyclin with conventional therapy for primary pulmonary hypertension. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: Survival in primary pulmonary hypertension: Repeat hospitalizations were identified through ongoing surveillance of hospitalization, while deaths were ascertained through linkage to the provincial vital statistics registry.

This ensured comparable follow-up for the entire HF population. Patient accrual for the present study began on October 15, and concluded on December 31, to allow for days of follow-up. The primary end point — combined all-cause mortality and hospitalization — was censored at the one-year follow-up. Secondary outcomes included the one-year total mortality and all-cause hospital readmission rate. From July to January , HF clinics HF clinic group were established in four geographically distinct locations within the province.

Two of the four clinics were located within a large teaching hospital. The other two were within hospitals Patients were accepted by referral if there was a previous hospitalization for HF within the past three months. The structure of these clinics was very similar and consisted of a nurse specialist working in collaboration with three to six physician specialists with experience in the management of HF.

In addition to assessment and provision of evidence-based therapies, patients and their caregivers underwent detailed and repeated educational sessions in which information relating to the diagnosis and causes of HF, an explanation of their treatment regimen, causes of decompensation, dietary advice and suggestions for actions should evidence of early decompensation occur were provided.

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  • Patients tended to be seen frequently every one to two weeks until clinical stabilization and successful negotiation of educational sessions, and then every one to three months. Patients were given a telephone number to call and clinics were conducted five times weekly; there was no weekend or after-hours coverage.

    Adverse outcomes that occurred in patients removed from active follow-up were attributed to the HF clinic group by an analogy to the intention-to-treat principle. No patient was counted twice within or between clinics. Individuals were included in the present study if they were a resident in the province and were admitted to a Nova Scotia hospital with a primary diagnosis of HF during the study period HF population group.

    They were further included in the HF clinic group if they attended an HF clinic at least once during the study period. The primary outcome was a composite of total mortality and hospitalization for any cause within days following enrollment. Thus, any rehospitalization event that occurred before the first clinic visit was not included in the analysis of either the HF clinic or nonclinic groups.

    Secondary outcomes included the separate outcomes of mortality and hospitalization. Descriptive statistics were calculated for each group HF clinic versus the overall HF population and raw outcomes reported. Medication usage at hospital discharge was reported but data regarding subsequent changes to medication were not available. A univariate regression analysis was performed using all 55 variables for the primary outcome Appendix. No variable was forced into the analysis. This process was repeated while excluding patients who were not taking ACE inhibitors, and was repeated with other variables.

    The number needed to treat for one year to prevent the occurrence of one event NNT was based on the adjusted HR for outcomes compared with the overall population adverse event rates. A test for heterogeneity was performed to determine whether the results were influenced by any of the clinics. A cohort of HF clinic patients older than 65 years of age was also extracted and compared for occurrence of the primary end point with nonclinic patients matched for age, sex, renal function, hemoglobin and ACE inhibitor use at baseline.


    During the study, patients were identified; in the nonclinic group and in the HF clinic group followed for one year. The four HF clinics each followed between 37 and patients. The average number of patients followed in the HF clinic group increased from 44 in to in Table 1. The number of non-clinic visits was not available for either group. The baseline characteristics of both groups are provided in Table 2 and indicate several differences.

    The patients in the HF clinic group were younger, more likely to be men and had higher body mass, plasma hemoglobin and rates of previous myocardial infarction. They also demonstrated a lower ejection fraction EF , serum creatinine and blood pressure at study entry, and had a decreased likelihood of receiving ACE inhibitor therapy at hospital discharge. There was no difference in baseline previous New York Heart Association functional class median 3. Data regarding patient no-show or request for discontinuation of HF clinic care were available from the largest HF clinic.

    Of patients, three 0.

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    • Selected baseline characteristics of the heart failure clinic care versus usual care groups. Variables documented at index hospital admission unless otherwise indicated.

      Pulmonary Hypertension: Diagnosis and Management

      After correction for all covariates, except performance of cardiac catheterization or measurement of EF it was believed that these variables would have been affected by clinic care , there was still a significant reduction in rehospitalization Table 3. The adjusted NNT for treatment for one year in an HF clinic to avoid one hospitalization or death the primary outcome was 5. Other important multivariate predictors of mortality and hospitalization are listed in Table 4.

      The logistic regression was repeated for the primary end point including only patients receiving ACE inhibitors, yielding a similar hazard reduction of outcomes in association with HF clinic care. This analysis was also performed for several other variables for which there was a difference between the HF clinic and non-clinic groups such as EF, blood pressure, age, etc , all with similar results.

      This analysis was also performed to exclude EF because there were many missing values for this variable; results similar to those described above were obtained. Finally, HF clinic patients older than 65 years of age were case-matched with nonclinic patients of the same age, sex and renal function there was no significant difference in hemoglobin. Kaplan-Meier survival function estimate of one-year total mortality in heart failure clinic care red line versus usual care black line groups.

      Adjusted survival was significantly better for those who underwent care in a heart failure clinic than for those followed by usual care. Kaplan-Meier survival function estimate of one-year total mortality or all-cause hospitalization in heart failure clinic care red line versus usual care black line groups. Adjusted freedom from mortality or all-cause rehospitalization was significantly better for those who underwent care in a heart failure clinic than for those followed by usual care.

      NNT Number needed to treat for one year to prevent one event in the target population. Follow-up data on medication usage were unavailable in three of the HF clinics and in the nonclinic group. These data attest to the high level of compliance to a complicated medical regimen that can be achieved in a disease management program such as an HF clinic. Our data show that implementation of a multidisciplinary HF clinic is associated with a significant reduction of hospitalization and mortality in a real-world setting of HF care. We calculated that for every five patients followed over the course of one year in an HF clinic, one less death or rehospitalization occurred.

      Follow-up in these studies has ranged from six months to more than four years. It is important to note that there does not seem to be an attenuation of the effects of HF clinic care over time Thus, at least in the setting of randomized clinical trials, HF clinics are efficacious. Despite the strengths of randomized trials, limitations do exist. Patients who participate in such studies are different from those in the general population.

      These differences exist across most variables, including age, demographics and clinical characteristics, and tend to limit the applicability of the results of randomized clinical trials to everyday practice. Evidence of efficacy does not necessarily translate into widespread effectiveness. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood out of your heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings.

      Your heart is composed of two upper and two lower chambers. The upper chambers the right and left atria receive incoming blood and pump it into the lower chambers. The lower chambers the more muscular right and left ventricles pump blood out of your heart. The heart valves — which keep blood flowing in the correct direction — are gates at the chamber openings.

      What is pulmonary hypertension?

      Normally, deoxygenated blood from all over your body enters the right atrium and flows into the right ventricle, where it's pumped through large blood vessels pulmonary arteries to your lungs. There, the blood releases carbon dioxide and picks up oxygen. The oxygen-rich blood then returns to the left atrium through the pulmonary veins, flows through the mitral valve into the left ventricle and finally leaves your heart through another large artery, the aorta. The aortic valve at the base of the aorta keeps the blood from flowing backward into your heart.

      From the aorta, the blood travels to the rest of your body. Cardiogenic pulmonary edema is a type of pulmonary edema caused by increased pressures in the heart. This condition usually occurs when the diseased or overworked left ventricle isn't able to pump out enough of the blood it receives from your lungs congestive heart failure. As a result, pressure increases inside the left atrium and then in the veins and capillaries in your lungs, causing fluid to be pushed through the capillary walls into the air sacs. Over time, the arteries that supply blood to your heart muscle can become narrow from fatty deposits plaques.

      A heart attack occurs when a blood clot forms in one of these narrowed arteries, blocking blood flow and damaging the portion of your heart muscle supplied by that artery. The result is that the damaged heart muscle can no longer pump as well as it should. Sometimes, a clot isn't the cause of the problem. Instead, gradual narrowing of the coronary arteries can lead to weakness of the left ventricular muscle.

      Although the rest of your heart tries to compensate for this loss, there are times when it's unable to do so effectively. The heart can also be weakened by the extra workload. When the pumping action of your heart is weakened, blood gradually backs up into your lungs, forcing fluid in your blood to pass through the capillary walls into the air sacs. This is chronic congestive heart failure. In mitral valve disease or aortic valve disease, the valves that regulate blood flow in the left side of your heart may not open wide enough stenosis. Or, they don't close completely, allowing blood to flow backward through the valve insufficiency or regurgitation.

      When the valves are narrowed, blood can't flow freely into your heart and pressure in the left ventricle builds up, causing the left ventricle to work harder and harder with each contraction. The left ventricle also dilates to allow greater blood flow, but this makes the left ventricle's pumping action less efficient.

      The increased pressure extends into the left atrium and then to the pulmonary veins, causing fluid to accumulate in your lungs.


      On the other hand, if the mitral valve leaks, some blood is backwashed toward your lung each time your heart pumps. If the leakage develops suddenly, you may develop sudden and severe pulmonary edema. Other conditions may lead to cardiogenic pulmonary edema, such as high blood pressure due to narrowed kidney arteries renal artery stenosis and fluid buildup due to kidney disease or heart problems.

      In normal lungs, air sacs alveoli take in oxygen and release carbon dioxide. In high-altitude pulmonary edema HAPE , it's theorized that vessels in the lungs constrict, causing increased pressure. This causes fluid to leak from the blood vessels to the lung tissues and eventually into the air sacs. Pulmonary edema that isn't caused by increased pressures in your heart is called noncardiogenic pulmonary edema.

      Pulmonary Hypertension

      In this condition, fluid may leak from the capillaries in your lungs' air sacs because the capillaries themselves become more permeable or leaky, even without the buildup of back pressure from your heart. Some factors that can cause noncardiogenic pulmonary edema include:. Mountain climbers and people who travel to high-altitude locations run the risk of developing high-altitude pulmonary edema HAPE.

      This condition — which generally occurs at elevations above 8, feet about 2, meters — can also affect hikers or skiers who start exercising at higher altitudes without first becoming acclimated, which can take from a few days to a week or so. But even people who have hiked or skied at high altitudes in the past aren't immune.