Description
Pulmonary arterial hypertension (PAH) is a progressive and debilitating disease that affects the arteries in the lungs, leading to increased pulmonary arterial pressure and right heart failure. Despite significant advances in the management of PAH in recent years, it remains a challenging disease to treat, and many patients continue to experience significant symptoms and reduced quality of life. However, there are several innovative approaches to PAH management that are currently being explored and show promise in improving outcomes for patients. One such approach is the use of combination therapy. While single-agent therapy has traditionally been the first-line treatment for PAH, recent studies have shown that combining different classes of drugs can lead to better outcomes. For example, combining an endothelin receptor antagonist with a phosphodiesterase type 5 inhibitor has been shown to improve exercise capacity and reduce clinical worsening in patients with PAH. Another promising approach is the use of novel drugs that target specific pathways involved in the development of PAH. For example, selexipag, a prostacyclin receptor agonist, has been shown to improve exercise capacity and reduce hospitalization rates in patients with PAH. Similarly, macitentan, a dual endothelin receptor antagonist, has been shown to improve exercise capacity and reduce clinical worsening in patients with PAH.
Résumé
- Pulmonary hypertension (PH) is a common cause of breathlessness, often confused with asthma, COPD, or cardiac failure. Misdiagnosis is frequent, with pulmonary hypertension being a missed underlying cause of breathlessness. A case study of a 40-year-old female presenting with progressive dyspnea on exertion and initially diagnosed with asthma underscores the importance of considering PH.
- Specific patient populations warrant screening for PH. A family history of pulmonary arterial hypertension or syncope is very crucial. Systemic sclerosis, a connective tissue disorder, also frequently presents with PH. Unexplained high pulmonary pressures during routine echo assessments should also raise suspicion for the condition.
- Pulmonary hypertension is categorized into five groups. Arterial hypertension, thromboembolic hypertension, hypertension due to heart or lung disease, and multifactorial causes are included. The primary focus should be on pulmonary arterial hypertension which requires early diagnosis and treatment. Guidelines stress the importance of treating the primary disease rather than automatically prescribing PH medication for high pulmonary pressures.
- The new diagnostic criteria for pulmonary arterial hypertension has a reduced pressure threshold, from 25 mm of mercury to 20 mm of mercury. Undiagnosed PH can delay diagnosis, potentially being initially misattributed to asthma or COPD. Objective Eco cardiography focuses on pressure measurement is important for exact determination.
- PH symptoms include dyspnea on exertion, lethargy, fatigue, exertional chest pain, and abdominal pain. Risk assessment classifies patients into low, intermediate, and high-risk categories, influencing treatment strategies. Progression of symptoms indicates risk and determines aggressive treatment.
- PH treatment algorithms include pharmacotherapy and other measures. Pregnancy is typically not advisable for women with PH. Vaccinations, psychological counseling, and oxygen therapy are also important. Regional anesthesia over general anesthesia is often preferred for patients undergoing surgery. Diuretics and anti-coagulation therapy can also be part of the treatment.
- Pharmacological interventions target the endothelin, nitric oxide, and prostacycline pathways. Monotherapy, sequential addition, or upfront combination therapy is prescribed. Clinical trials highlight the benefits of combination therapy over monotherapy, demonstrating improved patient outcomes and longer walking distances.
- Combination therapy shows manageable side effects. The American College of Chest Physicians recommends initial combination therapy to improve six-minute walk tests and stabilize symptomatic patients. Prognosis for untreated PH is poor. The one-year survival rate is 85%, while the seven-year survival rate is 49%.
- Regular follow-up and monitoring are critical. Medical assessments, ECG, six-minute walk tests, CPT, Eco, and basic labs should occur at baseline and be repeated at intervals to monitor treatment response and disease progression. Exertional dyspnea should raise suspicion for PH.
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