Chest x-ray. Arrow: prominent pulmonary artery. (Courtesy of Prof. T. Frauenfelder.)
The Diagnosis And Treatment Of Tricuspid Regurgitation
Background: It is estimated that 6% of persons over age 75 have clinically relevant tricuspid regurgitation (TR). This condition carries a high mortality and is of particular interest because of the recent development of new interventional treatments.
Methods: This review is based on publications that were retrieved by a selective search in the PubMed database for randomized controlled trials (RCTs), observational studies, registry studies, expert recommendations, and current international guidelines.
Results: The evidence reveals that TR is an independent cause of mortality. Mortality is correlated with the severity of TR: approximately 35% of patients with severe TR and right heart failure die within 1 year, and about 60% within 3 years. The clinical course varies depending on the etiology (primary TR, atrial/ventricular secondary TR, association with pacemaker systems). In the outpatient setting, timely diagnosis by transthoracic echocardiography is crucial. The options for pharmacotherapy are essentially limited to diuretic treatment (grade 2a recommendation). Early referral to a specialized heart valve center is essential for the prevention of irreversible damage of the right heart and secondary end-organ damage, including cardiohepatic and cardiorenal syndromes. In the heart valve center, an extended diagnostic evaluation with multimodal imaging is followed by a case discussion by the interdisciplinary cardiac team, with individual evaluation of the treatment options. The first randomized controlled trial of treatment for TR yielded a win ratio of 1.48 (95% confidence interval, [1.06; 2.13]) for interventional treatment (edge-to-edge repair) compared to optimal medical therapy.
Conclusion: As the understanding of tricuspid regurgitation improves, strategies for its interventional treatment are undergoing steady development, with the aim of lowering the mortality of this condition.
Tricuspid regurgitation (TR) is a serious, frequently occurring disease that is clinically underestimated. The prevalence of moderate to severe TR in the general population is 0.55% (1), rising in the over-75 age group to 6.6%, comparable with the prevalence of aortic valve stenosis (2). The pronounced symptoms are accompanied by increasing mortality associated with the severity of TR. Even mild TR is associated with increased mortality in the long run (hazard ratio 1.29, 95% confidence interval [1.27; 1.31]) (3). For patients with severe TR and right-sided heart failure, mortality rates of approximately 35% at 1 year and about 60% at 3 years after diagnosis have been described (4, 5). TR is a disease in its own right, regardless of associated conditions such as pulmonary hypertension, atrial fibrillation, left-sided heart failure, or mitral regurgitation (6).
Although the relevance of TR is undisputed, the disease is still frequently diagnosed too late, and diagnosis is followed by specific treatment in far fewer than 10% of cases (1, 7). Early diagnosis and evaluation of treatment options would be indicated in order to prevent manifest heart failure, despite the lack, to date, of evidence from randomized controlled trials to back up this recommendation.
This review article presents the current state of knowledge on the pathophysiology and etiology of TR, outlines strategies for timely outpatient management, and describes the diagnostic work-up and treatment at a specialized heart valve center.
Methods
The PubMed database was selectively searched (search terms: tricuspid regurgitation, right heart failure, transcatheter tricuspid valve intervention, TTVI, tricuspid edge-to-edge repair, T-TEER, transcatheter tricuspid valve replacement, TTVR, tricuspid surgery). The data analyzed came from two randomized controlled trials, from observational and registry studies, from expert recommendations, and from the recommendations of international guidelines on the diagnosis and treatment of TR.
Development and course of tricuspid regurgitation
Pathophysiology and clinical symptoms
Chronic TR is a progressive disease. In the early stages, compensation mechanisms result in an absence of symptoms, so that the TR often remains undetected. Chronic volume overload leads to increased myocardial wall tension and dilatation of the right heart. The resulting widening of the tricuspid valve annulus increases the coaptation defect (the gap between the leaflets of the valve). A vicious circle of growing TR and increasing right heart failure then begins (Figure 1). The clinical symptoms are initially non-specific. The reduced cardiac volume leads to a general decline in performance, fatigue, loss of appetite, and breathlessness on exercise. The decreasing cardiac output goes together with lower cerebral capacity. As the disease progresses there is increasing volumetric load, causing peripheral edema, signs of jugular vein distention, ascites, and anasarca. In the later stages of TR the patients complain of a considerably lower quality of life (8). There are frequent hospital admissions due to cardiac decompensation, and mortality is elevated (3, 4, 6). The TR induces potentially irreversible remodeling of the right heart. As right heart failure worsens, a prognostically unfavorable cardiohepatic and cardiorenal syndrome may arise (8).
Figure 1
Pathomechanism of tricuspid regurgitation
Etiological classification
Many factors contribute to the development of TR. A recently introduced classification takes account of leaflet morphology, atrial and ventricular anatomy, and the involvement of pacemaker systems (8) (Table 1). The etiology affects the prognosis of TR: for example, patients with ventricular secondary TR have a significantly poorer prognosis than those with atrial secondary TR (9) or primary TR (10).
Table 1
Etiological classification of tricuspid regurgitation
Primary tricuspid regurgitation
Primary TR, a rare occurrence, is characterized by structural defects of the leaflets and of the valvular apparatus in general. Primary tricuspid valve pathology can arise, for example, from fibroelastic degeneration, endocarditis, rheumatic fever, or carcinoid syndrome.
Atrial secondary tricuspid regurgitation
Atrial secondary TR is associated with chronic atrial fibrillation and is manifested by atrial expansion and annular widening with corresponding separation of the tricuspid leaflets. The size and function of the right ventricle are usually normal.
Ventricular secondary tricuspid regurgitation
Ventricular secondary TR arises from right ventricular dilatation, resulting in displacement of the papillary muscles and retraction of the tricuspid leaflets (tethering). Many different diseases may lead to right ventricular dilatation. The possible causes include left heart failure, left-sided heart valve pathology, pre- or postcapillary pulmonary hypertension, and primary right ventricular cardiomyopathy (8).
Tricuspid regurgitation associated with cardiac implantable electronic devices (CIED)
Pacemaker electrodes can lead to adhesion, perforation, laceration, or displacement of the tricuspid valve or the valvular apparatus. Moderate or severe TR is observed in approximately 5% of pacemaker implantations (11).
Outpatient management
Optimization of the entire treatment process is necessary if the symptoms and prognosis of patients with TR are to be improved. In the outpatient setting, early detection of the disease followed by initiation of medical treatment and timely referral to a specialized heart valve center is of crucial importance (12). The Box presents a code of practice for the diagnosis and treatment of TR including the indications for referral to a heart valve center. Transthoracic echocardiography is the method of choice for detection of the disease. Simple echocardiography with evaluation of leaflet morphology and function including color-coded Doppler sonography and assessment of the right ventricle suffices for initial diagnosis. The degree of TR severity may fluctuate depending on volume status and diuretic treatment. The goal of outpatient management is to achieve low-grade TR (grade 1) and then hold the disease at this level in the long term by means of medical treatment.
Box
Code of practice for the diagnosis and treatment of atients with tricuspid regurgitation
Conservative medical treatment
The data on the optimal medical treatment of TR are limited, because there are no class 1 recommendations in the guidelines (12, 13). Diuretics should be used in patients with significant TR and clinical signs of right heart failure (class 2a recommendation) (13). Initially, treatment with loop diuretics (starting dose: torasemide 5–10 mg daily) should be ordered with early and regular monitoring of efficacy (14). Right heart failure leads to reduction of renal function and perfusion, neurohormonal activation, and reduced intestinal resorption. This may lead to diuretic resistance, necessitating a further increase in dosage or combination with thiazide diuretics (14).
Betablockers (15) and SGLT-2 inhibitors (16) can be considered in patients with right heart failure. In the presence of underlying left heart failure, optimal medical treatment according to the guidelines is recommended (17). Due to ventricular interdependence, heightened left ventricular contractility results immediately in an increase in right ventricular stroke volume. Reduction in right ventricular afterload leads indirectly to improvement in right ventricular function.
In patients with significant prepapillary pulmonary hypertension and elevated pulmonary vascular resistance, a trial of treatment with pulmonary dilators (e.G., sildenafil) should be considered.
Heart rhythm monitoring
Rhythm-control therapy may lead to improvement of right ventricular function and reduced TR in patients with atrial fibrillation. Such treatment should therefore be considered at an early stage for containment of right ventricular failure (18).
Extended diagnostic work-up at the heart valve center
Diagnosis of clinically significant TR should prompt more detailed exploration at a heart valve center. The tricuspid valve pathology has to be evaluated, the underlying causes of TR have to be identified, and the cardiac and extracardiac implications have to be delineated. The multimodal diagnostic investigations conducted to this end form an essential basis for the subsequent treatment recommendations (Figure 2). Since TR occurs in complex clinical scenarios and may show swift progression (19), timely co-management at the heart valve center is advisable.
Figure 2
Multimodal assessment of tricuspid regurgitation (TR) carried out at the heart valve center for evaluation of the TR and the potential treatment options
Detailed echocardiography
Detailed transthoracic and transesophageal echocardiography is required for thorough evaluation of TR. Persons with TR often show pronounced remodeling of the ventricles and/or the atria, and this is quantified by transthoracic echocardiography. The same is true for impaired biventricular function. Three-dimensional determination of right ventricular function is superior to the conventional parameters for estimating the longitudinal function (e.G., tricuspid annular plane systolic excursion, TAPSE) (20).
The severity of TR is assessed by means of multiparametric measurements (qualitative, quantitative, and semiquantitative). The anatomy of the tricuspid valve and the pathomechanism are investigated by transesophageal echocardiography. Approximately 40% of patients with TR show variable segmentation of the leaflets with more than three functional components (21). Echocardiography also serves to assess the interaction of transvalvular pacemaker or ICD electrodes (ICD, implantable cardioverter–defibrillator) with the valvular apparatus and to detect primary valvular pathologies.
Cardiac catheterization
To aid understanding of the causes and consequences of TR, the hemodynamic interaction of the left heart, the pulmonary arteries, and the right ventricle and atrium should be explored by means of right heart catheterization. Moreover, stenotic coronary artery disease must be ruled out. Echocardiographic determination of pulmonary artery pressure is not reliable in severe TR (22). Rather, invasive measurement is necessary to distinguish the different forms of pulmonary hypertension: precapillary, isolated postcapillary, and combined precapillary and postcapillary. Precapillary pulmonary hypertension with severe TR is associated with an unfavorable prognosis (23), and therefore further diagnostic examinations must be performed to distinguish it from pulmonary, pulmonary vascular, and systemic conditions.
Cardiac computed tomography and magnetic resonance imaging
With the expansion of interventional treatment options, contrast-enhanced ECG-synchronized cardiac computed tomography (CT) has gained in importance (24). Both direct interventional annuloplasty and transcatheter tricuspid valve replacement require CT measurement of the tricuspid annulus. In heterotopic implantation of valves into the superior and inferior vena cava, the size of prosthesis selected depends on CT determination of vascular diameter.
Cardiac magnetic resonance imaging is not yet used for routine evaluation of TR, but it will probably gain importance in the near future (25).
Specific treatment at the heart valve center
If significant and symptomatic TR persists despite optimal treatment of underlying conditions and medical therapy, one should weigh up whether specific tricuspid valve treatment is indicated. The best treatment in each individual case is decided by a multidisciplinary heart team (Figure 3). Taking the patient's wishes into consideration, the treatment options are discussed in terms of the individual treatment risk, technical/anatomical practicability, and anticipated treatment effect. Efficient reduction of the TR (26, 27) and treatment at a stage of the disease where right ventricular function is still preserved (20) and there is no secondary organ damage (28) are predictors of a favorable postprocedural outcome. To date, the only evidence from randomized controlled trials is for interventional treatments. These include edge-to-edge repair by means of clipping (27) and interventional tricuspid valve replacement with the Evoque system (Edwards Lifesciences; Table 2). An overview of the concluded and ongoing randomized controlled trials on tricuspid valve treatment can be found in Table 2.
Figure 3
Treatment algorithm for use in persons with isolated severe tricuspid regurgitation
Table 2
Concluded and ongoing randomized controlled trials on tricuspid valve treatment
Surgical treatment options
The currently prevailing guidelines recommend tricuspid valve surgery, with simultaneous operative treatment of the left heart in the presence of severe TR or dilatation of the tricuspid annulus in patients with low surgical risk (12, 13). This strategy has been shown to be beneficial in patients with moderate to severe TR, but no benefit has yet been demonstrated for those with low-grade TR (29).
Patients with isolated TR are relatively rarely treated surgically (0.3% of the cardiac surgery interventions in Germany). The reasons are the high—taking the whole risk spectrum into account—in-hospital mortality of around 10%, the frequent occurrence of severe complications (over 30%: death, shock, pericardial tamponade, acute kidney injury requiring dialysis, prolonged ventilation for more than 72 hours) (30), and the lack of evidence from randomized trials. The surgical risk can be estimated by use of the TRI-SCORE on the basis of eight parameters (Figure 4) (31). Only in patients with few comorbidities and no signs of right heart failure is the risk acceptably low (31). Isolated surgical interventions on the tricuspid valve should nowadays be carried out in the form of minimally invasive annuloplasty (lateral minithoracotomy) without arresting the patient's heartbeat (32). A recent retrospective multicenter analysis showed that the 2-year survival rate was higher after surgery than after interventional and medical treatment (33). In view of the inherent selection bias of this study, together with the rapid pace of progress and accumulating experience in surgical and interventional therapy, a prospective randomized trial comparing different options would be very desirable for future treatment recommendations.
Figure 4
Risk score for estimation of in-hospital mortality after isolated tricuspid valve surgery
Interventional treatment options
Numerous interventional treatment procedures have been developed on the basis of surgical techniques in recent years. These procedures are divided into interventions to restore leaflet coaptation (essentially edge-to-edge repair by means of clipping), annuloplasties, and direct (orthotopic) or indirect (heterotopic) transcatheter tricuspid valve replacement. The most widely performed procedure is CE-certified edge-to-edge repair using the TriClip system (Abbott Vascular) or the PASCAL system (Edwards Lifesciences). The leaflets of the valve are grasped into the clip and anchored. The practicability of the technique depends on the morphological complexity (34), the size of the coaptation defect, and how well the valve can be imaged by transesophageal echocardiography, which is essential to guide the procedure. The intervention is characterized by a high degree of safety. A 2.7% rate of serious adverse events in the first 30 days was reported for experienced heart valve centers (36). A randomized controlled trial (TRILUMINATE Pivotal) confirmed the safety of the procedure and demonstrated for the first time the superiority of tricuspid interventions over medical treatment. One year after interventional therapy there was an effect on the composite endpoint of mortality, hospitalization due to heart failure, and quality of life (win ratio 1.48 [1.06; 2.13]). The endpoint was affected by the improvement in quality of life (win ratio 3.06 for improvement of the score on the Kansas City Cardiomyopathy Questionnaire [KCCQ] by at least 15 points, p < 0.001), while the overall low rates of hospitalization (win ratio 0.68, p = 0.41) and mortality (win ratio 1.09, p = 0.75) were comparable across the treatment groups (27). Bearing in mind the promising data from retrospective registries (5), it seems likely that the treatment effect might be particularly pronounced in specific patient populations or with efficient TR reduction. The impact of edge-to-edge repair on harder clinical endpoints is currently being investigated in randomized controlled trials, among others the multicenter TRIC-I-HF (tricuspid intervention in heart failure) study in Germany.
If edge-to-edge repair is technically infeasible, alternative forms of treatment are evaluated. Interventional implantation of annuloplasty systems is performed only occasionally due to the prolonged nature of the procedure and the potential for damage to the right coronary artery (10%) (37). One promising treatment option is interventional orthotopic transcatheter tricuspid valve replacement. To date, only the Evoque system (Edwards Lifesciences) has been CE-certified. The valve prosthesis is secured by pressure on the annulus together with the anchoring of small hooks at the base of the leaflets. In the case of anatomical compatibility, extremely efficient TR reduction can be achieved with this procedure (38). Long-term improvement of clinical endpoints and reverse remodeling after treatment have been described in prospective observational studies (39). Moreover, recently presented preliminary results from a randomized controlled trial (TRISCEND-II) confirm the safety and efficacy of the procedure and show the beneficial effect on quality of life (see Table 2). The final results, including the precise effect of the treatment on hospitalization and mortality, remain to be published. Another interventional treatment option is heterotopic valve replacement, in which one or more valvular prostheses are implanted into the superior and inferior vena cava. The impact of TR on the venous circulation, and thus the severity of symptoms, was successfully reduced by this means (40).
Conclusion
Severe TR is a serious multifactorial disease which, if left untreated, leads to progressive right heart failure and is associated with a poor prognosis. To prevent irreversible damage to the right heart and the development of secondary organ complications, early outpatient diagnosis with timely referral to an experienced heart valve center is crucial. As part of an expanded diagnostic work-up, each individual case is discussed and evaluated by a specialized multidisciplinary heart team. The options for medical therapy are limited, and the risk involved in surgical treatment is often too high. Against this background new interventional treatment options have been developed, the techniques of which are still being examined and optimized. It is anticipated that this process will lead not only to improvement of patients' quality of life, but also to stable control of right heart failure and reduction of mortality.
Conflict of interest statementTJS has received honoraria for lectures from Edwards Lifesciences. TJS and JH are principal investigators of the DZHK study "TRIC-I-HF." The study was partly financed by funding from Edwards Lifesciences to Ludwig Maximilians University Munich. CB has received institutional honoraria for lectures from Edwards Lifesciences and Abbott. HT has received honoraria for his work as a consultant to TRiCares (transcatheter tricuspid valve implantation).
Manuscript received on 15 January 2024, revised version accepted on 14 May 2024.
Translated from the original German by David Roseveare.
Corresponding authorPD Dr. Thomas J. StockerMedizinische Klinik und Poliklinik ILMU Klinikum, Universität MünchenMarchioninistr. 1581377 München, Germanythomas.Stocker@med.Uni-muenchen.De
Cite this as:Stocker TJ, Besler C, Treede H, Hausleiter J: The diagnosis and treatment of tricuspid regurgitation. Dtsch Arztebl Int 2024; 121: 551–8. DOI: 10.3238/arztebl.M2024.0104
Treating Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is one of five types of pulmonary hypertension. It happens when the blood vessels in your lungs narrow and scar so much that it's hard for blood to move through them. This can trigger high blood pressure in the lungs.
PAH can be hard to diagnose. That's because the symptoms – like shortness of breath – are common to several other medical conditions. There's no cure. But there are FDA-approved treatments. Here's what you need to know.
How Is Pulmonary Arterial Hypertension Treated?What caused your PAH, the symptoms, and how advanced your condition is all impact what treatment might work best for you. It may take time to figure out, but your doctor is there to help.
Options include:
Prescription drugs called vasodilators specifically treat PAH. These may be taken by mouth or given through an IVor subcutaneously, meaning under the skin. Some you inhale, or breathe in, from a nebulizer.
In some cases, you may need more than one type of drug to get your condition under control. You might also need other therapies like extra oxygen along with diet and lifestyle changes to help you feel better.
Intravenous (IV) treatments
Epoprostenol is a vasodilator that relaxes your blood vessels so it's easier for blood to move through them. There are two brands: Flolan and Veletri.
Flolan. In 1995, this was the first drug the FDA approved to treat PAH. It's given as a continuous injection through a vein for short-term use. A doctor can also surgically place a special catheter for long-term use. One study found that it helped make exercise easier and boosted survival rates.
Flolan has to be mixed every day. You must also:
Your catheter also needs to be checked often for signs of possible infection.
Side effects may include:
Selexipag IV (Uptravi). It can help slow the progression of PAH and lower your risk of being hospitalized. It's used only in patients who temporarily can't take oral medication.
Treprostinil (Remodulin). This is another IV formula of the same class of drugs as Flolan and Veletri. It's also stable at room temperature and doesn't have to be mixed daily. It can also be given under the skin.
Veletri. It's a newer version of epoprostenol approved by the FDA in 2010. It doesn't have to be mixed daily, and you can keep it at room temperature. Your doctor may suggest Veletri if you have severe PAH and other treatments haven't worked.
Side effects for Veletri and Remodulin are similar to those of Flolan. Catheters used for IVs should be checked regularly to avoid infections.
Nebulizer treatments
Iloprost (Ventavis). You breathe it in through a nebulizer about six to nine times every day. If you have asthma or other conditions that can make it hard to breathe, this may worsen those symptoms.
Treprostinil (Tyvaso). Your doctor may recommend this drug if you have moderate to severe PAH symptoms. You'll likely take it about four times a day. Research suggests it can help you walk longer distances.
Side effects of iloprost and treprostinil are similar to those of IV-based PAH drugs. But you may have less flushing or diarrhea with iloprost. Treprostinil may cause a sore throat and cough when you first start to use it.
Oral treatments
Ambrisentan (Letairis). This is a once-a-day medication, but unlike bosentan, it doesn't affect your liver.
Bosentan (Tracleer). This medication is taken two times a day. The drug could affect your liver function and hemoglobin levels. It's important to get your blood count and liver function tested regularly when you're taking bosentan.
Neither bosentan nor ambrisentan is recommended for people who are pregnant. Experts recommend two separate birth control options. Monthly pregnancy tests are required to continue using these drugs.
Sildenafil (Revatio). Small studies suggest this drug can improve blood flow in your lungs. The recommended dose is a 20-milligram pill taken three times a day, but it can be increased if you have a more severe form of PAH.
Side effects of sildenafil include:
Tadalafil (Adcirca). This is a once-a-day pill prescribed if you have moderate to severe PAH.
Treprostinil (Orenitram) is the oral formulation of Remodulin and can substitute for the IV medications after you have stabilized.
Other Ways to Treat Pulmonary Arterial HypertensionThese include:
Calcium channel blockers. This class of drugs, in select patients, can help lower the blood pressure in your lungs.
Blood thinners. Your risk of forming blood clots is higher if you have PAH. Blood thinners, like warfarin (Coumadin), are given to most people with PAH to cut down on clots and improve overall survival. But warfarin interacts with several foods and drugs. Your doctor can tell you what to avoid.
Diuretic. Sometimes called water pills, diuretics can help your body get rid of extra salt and water. This happens to most people with PAH. Your doctor will usually recommend one pill a day. But if you have a severe form of PAH, they may up the dosage.
Digoxin. This is an older medication sometimes prescribed to people with severe PAH. It's designed to help your heart pump blood better.
Oxygen therapy
When you have moderate to severe PAH, it can make it hard to breathe properly and affect your day-to-day life. The low levels of oxygen can make the blood vessels constrict further, which can make your PAH worse.
Getting a little extra oxygen can help you not only breathe better but also be able to get through daily tasks and routines. It can also boost your mood and sleep. While some may need extra oxygen every now and then, others may need it continuously.
Oxygen therapy comes in three types:
Before you try oxygen therapy, ask your doctor how much you need and how often you may need it. They can also guide you toward the type that works best for your lifestyle.
Surgery
If you have severe PAH and medications aren't working, your doctor may recommend a lung transplant. Lung transplant surgery is challenging and can come with serious complications. These include:
Your health care team can help you decide if it's right for you.
Things You Can Do to Manage Pulmonary Arterial HypertensionThere are some lifestyle changes you can try to help ease your symptoms.
You can:
Try to exercise and stay as active as you can. But don't lift heavy weight or do intense workouts. Talk to your doctor first if you're not sure what's safe.
Treatments For Pulmonary Arterial Hypertension
While there's no cure for pulmonary arterial hypertension (PAH), many types of medicines and procedures can ease your symptoms and make your day-to-day life better. Everyone's condition is different, so your doctor will help you figure out the treatments that will work best for you.
The first step of any plan is to treat the cause of your PAH. For example, if chronic obstructive pulmonary disease (COPD) is the reason you have it, treatment for that problem will improve pulmonary hypertension, too. But some people need more help to improve their breathing and lower the blood pressure in their lungs.
To help you choose a treatment plan, your doctor will see how severe your symptoms are and how much your disease keeps you from being active.
These work in a few ways. Some let blood flow more easily through the arteries in your lungs, and others help your heart and lungs work better. You can take them as pills, medicines that you breathe in, drugs you get through an IV, or through the skin.
Calcium channel blockers are drugs that lower your blood pressure by relaxing the muscles that control the arteries. Before your doctor prescribes these pills, they'll see how well they work on your blood vessels during a procedure called a vasodilator test.
Digoxin is a daily pill that helps the heart beat more strongly, which can help if the cause of your PAH is heart failure or an irregular heart rhythm. The challenge of this drug is to get the right dose. If you have too much, you could have side effects like nausea, changes in vision, and irregular heartbeats. Digoxin is not used as much any more because there are often newer and better treatment options.
Warfarin (Coumadin) thins your blood to keep clots from building up, which can make PAH worse. But it can also make you bruise and bleed easily.
Medicines that keep excess fluid out of the body, called diuretics, can make the heart and lungs work better and ease the symptoms of PAH. You usually take these pills once or twice a day. You may need regular blood tests if you take diuretics, in order for your doctor to monitor for dehydration, low blood pressure, chemical imbalances in the blood, and kidney problems.
Other PAH pills lower the blood pressure in the pulmonary arteries by keeping blood vessels open or preventing them from narrowing. These include:
Drugs called vasodilators open up blood vessels, but some have an effect on the body for only a few minutes. To administer the most common one, epoprostenol (Flolan, Veletri), you need a small battery-powered pump so you can get a steady dose. The pump delivers medicine through a thin IV tube, and you wear it in a pack on your belt or shoulder. You can expect to spend a few days in the hospital to get the pump set up and to learn how to use it. There is also a subcutaneous (under the skin) form of these medicines called treprostinil (Remodulin).
Other vasodilators are medicines that you inhale, like iloprost (Ventavis) and treprostinil (Tyvaso). They go straight to your lungs and can quickly relieve shortness of breath. To take them, you'll get a nebulizer, a machine that vaporizes these medications and lets you breathe them in. Iloprost can be given six to nine times a day. Tyvaso can be given four times a day, and there is also a form of treprostinil (Remodulin) that can be used with a pump to be injected continuously under the skin.
Some people with PAH need oxygen therapy to get enough oxygen in their blood. You breathe it through a face mask or prongs that fit in your nose. It's especially helpful for people who also have sleep apnea or who live at high altitudes. Some people with PAH eventually need oxygen therapy around the clock.
If you have severe PAH or medications don't help your symptoms, your doctor may recommend one of two types of operations:
Atrial septostomy: A surgeon makes an opening between the upper chambers of the left and right sides of your heart to lower the pressure on one side. This procedure can have serious side effects, so doctors don't often recommend it.
Lung and heart transplants: Doctors recommend them for people who have tried drugs to treat their disease without success and whose condition is getting worse.
A transplant can improve your life, but it's serious surgery. It can take several months to recover completely. Though you may not need PAH medications, you'll need to take drugs to keep your body from rejecting the new organs for the rest of your life. You'll want to talk to your doctor about the balance of benefits and risks before you go ahead with this treatment.
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