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Login with your account to use your code. JavaScript must be enabled to use this site. Please enable JavaScript in your browser and refresh the page. Instructions to enable JavaScript. Leclerc is the first online comic bookstore that connects you to passionate retailers: Partenaires de Actialuna SAS: Leclerc, and select content tailored to your interests. Calcium channel blockers to treat pulmonary hypertension are not indicated in COPD. Available information on the use of specific medical treatment is anecdotal.

It should be taken into account that some specific drugs sildenafil, prostacyclin inhibit hypoxic pulmonary vasoconstriction and may worsen gas exchange in COPD. Pulmonary hypertension is usually mild in the context of interstitial lung disease, except when pulmonary fibrosis and hypertension occur alongside a connective tissue disease. Little information is available on the use of specific drugs in pulmonary hypertension associated with pulmonary fibrosis. What has been published is based only on small, open case series of patients followed for short periods.

Controlled clinical trials are currently under way. If a patient presents connective tissue disease and concurrent, severe pulmonary hypertension and moderate pulmonary fibrosis, it is recommended to follow the treatment protocol for PAH. The importance of this sleep disorder as a cause of pulmonary hypertension is uncertain. Patients who have sleep apnea and proven pulmonary hypertension improve with continuous positive airway pressure.

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Should that not be the case, concomitant PAH should be investigated. Two years after pulmonary thromboembolism, the incidence of related pulmonary hypertension is 3. This form should be investigated systematically in all patients with pulmonary hypertension, whether or not they have a history of pulmonary thromboemboli. Anticoagulant treatment is indispensable INR, 2. Thrombi that are organized and surgically accessible in the main, lobar, or segmental pulmonary arteries. Hospitals with the greatest volume of activity have the lowest mortality rates.

Patients with this diagnosis who are not eligible for surgical treatment are candidates for specific medical treatment with prostanoids, bosentan, or sildenafil. Small numbers of patients have been treated successfully, though the follow-up periods have been brief. The hemodynamic improvement achieved has been clearly inferior to that which is obtained through pulmonary thromboendarterectomy, however.

Medical treatment in these patients must be carried out in specialized referral units. The response to some drugs at 3 months has been shown to predict longer-term outcome. It is therefore recommended that the patient's clinical status functional class and exercise tolerance 6-minute walk test be assessed 3 months after starting treatment. Attaining functional class I or II, covering more than m in 6 minutes, a peak oxygen consumption greater than There is currently no information available from prospective studies that allow factors that can accurately predict response to treatment to be identified.

The timing of follow-up visits is dictated by the type of pulmonary hypertension, the patient's clinical status, the therapeutic regimen prescribed, and response. Table 7 shows guidelines for the follow-up scheduling. The dimensions and functioning of the right ventricle can be determined by TTE. This information is of prognostic value and useful during follow-up. PASP bears little relation to prognosis. TTE should be repeated every 6 to 12 months. The study of pulmonary hemodynamics 3 months after starting specific pulmonary hypertension treatment has prognostic relevance, particularly with regard to the results rather in accordance with the patient's clinical status, especially in cases of deterioration and when a change of treatment approach is being considered.

The prevalence of pulmonary hypertension is not high. PAH occurs at a rate of 15 cases per million inhabitants 18 and may certainly be termed a rare disease: Spain is estimated to have between and cases. Appropriate care of these patients demands complicated diagnostic and therapeutic procedures that are available only in specialized centers.

For the necessarily high level of specialization to be present, there must be experience, which can only be achieved and maintained if there is a certain volume of activity. The disease-specific drugs available at this time are very costly. The price of monotherapy ranges from? Therefore, the optimum cost-effectiveness ratio is served if the decision to treat is well founded and therapy is carefully monitored. The concentration of cases in specialized referral units should make it possible for there to be a critical number of patients so that multicenter clinical trials can be carried out.

Likewise, the health system thus benefits from external funding for very costly treatments. One of them should be the director or coordinator of the unit and take responsibility for it. The nurse should have experience with the disease and the various treatments used.

The care of patients with pulmonary hypertension is highly multidisciplinary. The unit should therefore act in concert with other specialists and departments in the hospital according to diagnostic and therapeutic requirements.

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Given the manner in which health care in Spain is organized, a pulmonary hypertension unit should expect to handle at least 5 new cases of PAH per year and follow patients for at least 3 years. A unit will be actively managing at least 30 patients at a time. Experience and quality of health care. Also important is experience with the various drug delivery systems, particularly continuous intravenous infusion. The specialized unit's staff should hold clinical review sessions regularly to discuss patient progress.

Certain outcomes should be designated as markers of success, specifically survival, consi stent with current standards. Specialized pulmonary hypertension referral units should be located in tertiary care hospitals with adequate facilities and resources for these patients. The minimum requirements for such units would include the ability to carry out echocardiography and assess cardiac hemodynamics.

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Also necessary would be a pulmonary function laboratory, a cardiopulmonary stress testing unit, a sleep laboratory, and a radiodiagnostic department able to perform spiral CT angiography and high resolution CT. Departments specialized in nuclear medicine, respiratory medicine and cardiology should also be available. In addition, the following services or facilities are highly desirable: The referral unit should have properly updated standard operating procedures for diagnosis and treatment. The following areas should be covered: The operating procedures should be revised annually.

The validity of the diagnostic tests should be analyzed, the level of appropriateness of previously established indications assessed, and the rates of procedural complications calculated. External auditing of compliance should be scheduled. Data storage and retrieval system. Electronic databases should be used so that information on actions taken and results found and their evaluation can be retrieved.

Patients should be listed in a database that incorporates their particulars, diagnosis, and results of follow-up tests. The unit should undertake research that contributes to the understanding of pulmonary hypertension. Original studies should be designed and conducted and the unit should also participate in national and international trials, contribute to case registries, and publish in scientific journals.

The visits for the initial evaluation of the patient's condition and follow-up should be specialized. To operate properly, the unit's office will require: The unit must have access to the resources needed to establish a diagnosis of class and type of pulmonary hypertension, including the patient's hemodynamic status, vasoreactivity, and exercise tolerance. The unit must have the resources to administer the various drug therapies available in their different modes of delivery.


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Specifically, the team must be capable of inserting a permanent central venous catheter to administer epoprostenol if necessary and be prepared to manage that treatment: It is useful for a referral center to have teams capable of performing nonmedical treatments interatrial septostomy, lung or heart-lung transplants, pulmonary thromboendarterectomy in coordination with the pulmonary hypertension unit. If that capability is not present, the pulmonary hypertension unit should establish protocols stipulating the criteria governing the transfer of a patient to other specialized units and should coordinate with such units.

A specific area of the hospital should be available to admit patients who need care that cannot be provided on an outpatient basis because integrated care is required or in case of serious deterioration of the patient's condition. In-hospital care may also be advisable in order to perform specific diagnostic or therapeutic. Admission to the day hospital may be sufficient for patients who are in stable condition.


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