E Common Pitfalls And Side
The biggest concern with pitfalls in PH management is whether a proper diagnosis has been established. Missing a case of CTEPH is depriving that patient from a potential safe and curative intervention. Treating a patient with left heart related PH with PAH targeted therapy risks an adverse outcome from medication side effects and delay in addressing the primary problem. In terms of side effects for PAH patients receiving approved therapy, that depends on numerous factors, including the type or class of medical therapy.
Approved therapies in the U.S. for PAH fall under three pathways. Prostacyclin analogue replacement can be given continuous-intravenously , subcutaneous , or inhaled . The complexity of the parenteral formulas result in these treatments often reserved for higher volume or experienced PH centers. Endothelin receptor antagonists are pills taken orally: bosentan and ambrisentan . Lastly, phosphodiesterase-5 inhibitors are also given orally and are approved for PAH: sildenafil and tadalafil . All these therapies are only approved for WHO group I, PAH. The use in other forms of PH is discouraged and should be reserved only for clinical trials.
Table I.
Standard Pulmonary Hypertension Treatments
Our team includes a nurse practitioner who specializes in caring for people with pulmonary hypertension. You can contact this specialist between appointments if you have any questions about your care plan.
Your personalized care plan may include:
- Healthy living recommendations: We give you helpful tips for getting the most out of treatment and lowering your risk of complications. These tips often include a low-sodium diet and mild exercise.
- Medications: Most people with pulmonary arterial hypertension take medications to help control their symptoms. If standard treatments are not effective, we may recommend new options available through clinical trials.
- Pulmonary rehabilitation: This supervised exercise program is tailored to meet the unique needs of people with lung disease. Pulmonary rehabilitation can help you regain your strength and make a safe return to a more active lifestyle. Learn more about our pulmonary rehabilitation program.
- Monitoring: We develop a lasting relationship with you, including regular follow-up appointments. During these appointments, we determine whether treatments are working as they should. We may make adjustments to your care plan to help you feel your best.
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Pulmonary Hypertension: Diagnosis And Treatment
BETH DUNLAP, MD, Northwestern University, Chicago, Illinois
GEORGE WEYER, MD, University of Chicago, Chicago, Illinois
Am Fam Physician. 2016 Sep 15 94:463-469.
Pulmonary hypertension is a heterogeneous group of disorders characterized by a mean pulmonary arterial pressure of 25 mm Hg or greater at rest during right heart catheterization.14 Patients with pulmonary hypertension may initially present to family physicians, but symptoms such as dyspnea on exertion and fatigue are nonspecific and may be attributed to comorbid conditions.5 Pulmonary hypertension is categorized on the basis of pathophysiology, hemodynamics, and therapeutic options. In 2013, the classification scheme was updated to recognize five groups of pulmonary hypertension6 .
SORT: KEY RECOMMENDATIONS FOR PRACTICE
Echocardiography is the recommended initial test in the evaluation of patients with suspected pulmonary hypertension.
A = consistent, good-quality patient-oriented evidence B = inconsistent or limited-quality patient-oriented evidence C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to .
SORT: KEY RECOMMENDATIONS FOR PRACTICE
Echocardiography is the recommended initial test in the evaluation of patients with suspected pulmonary hypertension.
Clinical Classification of Pulmonary Hypertension
Group 1*: Pulmonary arterial hypertension
*Also includes 1 and 1 .
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Medical History And Physical Exam
Your doctor may ask you about any signs and symptoms you have been experiencing and any risk factors such as other medical conditions you have.
Your doctor will also perform a physical exam to look for signs that may help diagnose your condition. As part of this exam, your doctor may do the following:
- Check whether the oxygen levels in your blood are low. This may be done by pulse oximetry, in which a probe is placed on your finger to check your oxygen levels.
- Feel your liver to see if it is larger than normal.
- Listen to your heart to see if there are changes in how it sounds, and also to find out if your heartbeat is faster than normal or irregular or if you have a new heart murmur.
- Listen to your lungs for sounds that could be caused by heart failure or interstitial lung disease.
- Look at the veins in your neck to see if they are larger than normal.
- Look for swelling in your abdomen and legs that may be caused by fluid buildup.
- Measure your blood pressure.
Lfts: Liver Function Tests Used To Monitor Safety Of Certain Pulmonary Hypertension Treatments
Liver function tests are used to screen for, detect, evaluate, and monitor for liver inflammation and damage. The panel includes ALT , ALP , AST , bilirubin, albumin, and total protein. It is important to measure liver function for multiple reasons. The increased pressure in the pulmonary arteries leads to the right side of the heart not pumping efficiently, which leads to blood backing up in the body and hepatic congestion, which can cause damage to the organ. Some endothelin receptor antagonists have been shown to have a low risk of liver damage as well so it is important to check monthly liver function tests. The LFT panel offers more complete information on the liver and how well it is functioning than a CMP.
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Cmp: Complete Metabolic Panel A Useful Test For Pulmonary Hypertension Patients
The CMP is a BMP plus albumin, total protein, ALP , ALT AST , and bilirubin. This test is used when the physician wants to measure basic electrolytes, renal function, and liver function. This test is useful in pulmonary hypertension for the same reason a BMP is, plus it has the added benefit of measuring liver function.
It is important to measure liver function for multiple reasons. The increased pressure in the pulmonary arteries leads to the right side of the heart not pumping efficiently, which leads to blood backing up in the body and hepatic congestion, which can cause damage to the organ. Some endothelin receptor antagonists have been shown to have a low risk of liver damage as well so it is important to check monthly liver function tests.
Ph Associated With Hypoxemia
Oxygen opens the lung passages. When the body is deprived of oxygen it can cause constriction of the lung vessels resulting in pulmonary hypertension. Diseases that affect lung breathing passages and decrease oxygen include asthma, emphysema, chronic bronchitis, interstitial lung disease and cystic fibrosis.
Another form of lung disease related to low oxygen and periods of not breathing during the sleep cycle is called sleep apnea. Obstructive Sleep Apnea is the most common form found in overweight middle aged adults. Treatment with breathing machines during sleep helps improve oxygen levels.
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Key Facts About Pulmonary Hypertension
Pulmonary hypertension is a rare and varied disease that has many different causes and can present itself in many different ways. In fact, there are five different groups of pulmonary hypertension, and within each of these groups there are multiple diseases and conditions that can cause a particular type of pulmonary hypertension.
It’s estimated that only 15-50 people out of every million adults develops the disease, but the incidence is much higher for people in at-risk groups .
Idiopathic pulmonary hypertension makes up more than 40% of total cases of pulmonary arterial hypertension. It is also, for unknown reasons, anywhere between 2 and 5 times more common in women than men, and is usually diagnosed around the age of 45 years.
In healthy people, the blood pressure in the lungs is significantly lower than the blood pressure throughout the whole-body, at about 8-20 mm/Hg. Unlike regular blood pressure measurement, testing the blood pressure in the pulmonary arteries requires special, more invasive techniques.
To test pulmonary artery pressure, doctors have to perform a procedure called right heart catheterization. It requires going under anesthesia and having a flexible tube, or catheter, inserted into a major blood vessel in your neck or groin.
Group 3 Pulmonary Hypertension
Lung diseases, such as COPD and interstitial lung disease, can cause group 3 PH. Certain sleep disorders, such as sleep apnea, also can cause group 3 PH.
If you have this type of PH, you may need oxygen therapy. This treatment raises the level of oxygen in your blood. You’ll likely get the oxygen through soft, plastic prongs that fit into your nose. Oxygen therapy can be done at home or in a hospital.
Your doctor also may recommend other treatments if you have an underlying lung disease.
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Pulmonary Arterial Hypertension Associated With Human Immunodeficiency Virus Infection
The use of highly active antiretroviral therapy and aggressive management of opportunistic infections has contributed to increased life expectancy in HIV-infected patients., Consequently, the spectrum of complications has shifted towards other long-term conditions, including PAH. It is likely that modern HIV management with HAART has also resulted in improved survival and decreased incidence of HIV-associated PAH. Taken together, these effects on survival and incidence have resulted in a stable PAH prevalence in HIV-infected patients over recent decades. A population study indicated that the minimal prevalence of HIV-related PAH was 0.46%, very similar to the prevalence before the HAART era. The pathogenesis of HIV-related PAH remains unclear. The absence of viral particles in the complex plexiform lesions found from these patients suggests that an indirect action of viral infection on inflammation and growth factors may act as a trigger in a predisposed patient.
7.5.1 Diagnosis
7.5.2 Therapy
The recommendations for PAH associated with HIV infection are reported in the Table .
Treatments For Other Conditions
Your doctor may recommend medicines or procedures to treat the condition that is causing your pulmonary hypertension.
- Blood pressure medicines such as angiotensin-converting enzymes inhibitors, beta blockers, or calcium channel blockers when left heart disease is the cause
- Blood transfusions or hydroxyurea to treat sickle cell disease
- Heart valve repair
- Ironsupplements to increase blood iron levels and improve anemia
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How Do You Risk Stratify And Provide A Risk Assessment
Regular risk assessment of patients with pulmonary hypertension is strongly recommended. A comprehensive risk assessment is required since there is no single determinant that provides enough prognostic information. The most important question to be addressed at every clinic visit, is the patient compatible with a good long-term prognosis ? To answer this question, a structured approach is needed, and all pulmonary hypertension specialists should have a checklist to help provide a comprehensive risk assessment:
Achieving low clinical risk also functions as the primary treatment goal, thus treatment should be tailored to achieve and maintain a low-risk status.
How To Assess For Pulmonary Hypertension With Echocardiography
Like I mentioned earlier, echo plays a critical role in not only helping diagnose pulmonary hypertension, but also in helping learn what might be causing the increased pulmonary pressures.
For example, a patient who has significant mitral stenosis can also present with pulmonary hypertension. As a matter of fact, PHTN is something you should always interrogate in these cases.
Ultimately, we want to know what the Pulmonary Artery Systolic Pressure is . This is the measurement that were most interested in. But how do we measure pulmonary artery systolic pressure with echocardiography?
Heres what were going to do:
PASP = RVSP + RAP
And it all starts with tricuspid regurgitation.
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Advancing Research For Improved Health
In support of our mission, we are committed to advancing pulmonary hypertension research in part through the following ways.
- We perform research. Our Division of Intramural Research, which includes investigators from the Sickle Cell Branch, is actively engaged in the study of pulmonary hypertension.
- We fund research. The research we fund today will help improve our future health. Our Division of Lung Diseases and its Lung Biology and Disease Branch oversee much of the research on pulmonary hypertension that we fund. Search the NIH RePORTER to learn about research the NHLBI funds on pulmonary hypertension.
- We stimulate high-impact research. Our Trans-Omics for Precision Medicine Program now includes participants who have pulmonary hypertension. This work may help us understand how genes contribute to differences in disease severity and to how patients respond to treatment, particularly in patients who have sickle cell disease. The NHLBI Strategic Vision highlights ways we may support research over the next decade, including research on pulmonary hypertension.
Learn about exciting research areas the NHLBI is exploring about pulmonary hypertension.
Pulmonary Arterial Hypertension Associated With Connective Tissue Disease
PAH is a well-known complication of CTDs such as SSc, systemic lupus erythematosus, mixed CTD and, to a lesser extent, rheumatoid arthritis, dermatomyositis and Sjögren’s syndrome. PAH associated with CTD is the second most prevalent type of PAH after IPAH in Western countries. SSc, particularly in its limited variant, represents the main CTD associated with PAH in Europe and the USA ., The prevalence of haemodynamically proven pre-capillary PH in large cohorts of SSc patients ranges from 5 to 12%.,,, In these patients, PH may occur in association with interstitial lung disease or as a result of an isolated pulmonary vascular disease, which may affect pre-capillary arterioles and post-capillary venules ., In addition, group 2 pulmonary venous hypertension from LHD may be present.,, It is thus imperative to determine which mechanism is operative since this dictates treatment in the context of a multifaceted disease.
7.3.1. Diagnosis
7.3.2 Therapy
Treatment of patients with CTD-associated PAH is more complex than that of IPAH. Immunosuppressive therapy combining glucocorticosteroids and cyclophosphamide may result in clinical improvement in patients with PAH associated with systemic lupus erythematosus or mixed CTD.
Long-term favourable response to CCB treatment is reported in < 1% of cases. In SSc, the long-term risk:benefit ratio of oral anticoagulation is less favourable than in IPAH because of an increased risk of bleeding.
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Type : Pulmonary Arterial Hypertension
Your specialist will offer you treatments called pulmonary vasodilators. They aim to lower the blood pressure in your lungs by relaxing and opening up the pulmonary arteries. With more space for the blood to pass through, the right side of your heart is under less strain and should work better. This means that your symptoms should improve. Pulmonary vasodilators include medications called:
The choice of medication is complex and your specialist will discuss this with you.
Other treatments will help with your symptoms, called background therapy.
They may include:
- diuretics or water tablets: These remove excess fluid from your body. They treat symptoms like swollen ankles.
- oxygen therapy: This will increase the level of oxygen in your blood and may make you feel less breathless.
- anticoagulation: With some forms of PAH you have a greater risk of getting blood clots. Anticoagulant medicines like warfarin help prevent blood clots forming.
- pulmonary rehabilitationor PR: PR is a programme of exercise and advice to help cope with breathlessness.
Your specialist will advise you:
- not to become pregnant
- not to travel to high altitude . You may also need oxygen when flying ask your health care professional about a fitness-to-fly test
- to keep up to date with flu and pneumonia vaccinations
How Do You Define And Diagnose Pulmonary Hypertension
Pulmonary hypertension is defined by either a mean pulmonary artery pressure at rest of 20 mm Hg or more as measured by a right heart catheterization or a right ventricular systolic pressure of 30 mm Hg or more as measured by an echocardiogram. An RHC is the diagnostic gold standard and an essential component in the evaluation of pulmonary hypertension. While an echocardiogram can be a useful screening tool for the presence of pulmonary hypertension, it only provides an estimate of RVSP. An RHC is absolutely required to confirm the diagnosis, assess the severity of hemodynamic impairment, provide risk assessment, and guide pulmonary hypertension-specific therapy.
The threshold to perform a left heart catheterization in addition to an RHC should be low in patients with risk factors for heart disease and when the measurement of left ventricular end-diastolic pressure is important to avoid potential misclassification of pulmonary hypertension.
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Group 1 Pulmonary Arterial Hypertension
Group 1 PAH includes:
Group 5 includes PH caused by various other diseases or conditions. Examples include:
- Blood disorders, such as polycythemia vera and essential thrombocythemia .
- Systemic disorders, such as sarcoidosis and vasculitis . Systemic disorders involve many of the body’s organs.
- Metabolic disorders, such as thyroid disease and glycogen storage disease. properly.)
- Other conditions, such as tumors that press on the pulmonary arteries and kidney disease.
A History Part I: Pattern Recognition:
The history of PAH can be traced back to the aminorex epidemic in Europe causing primary pulmonary hypertension back in the 1960s and 70s. In response, the National Institutes of Health conducted a registry in the U.S. of PPH patients. The typical patient at that time was an otherwise healthy young woman in her 30s, with an average life expectancy after diagnosis of approximately two and a half years.
With introduction of modern PH therapies, the outlook has improved significantly. Fast forwarding to the current era, the typical PAH patient tends to be older and with other comorbidities either linking with pulmonary hypertension or challenging its timely diagnosis and treatment. The most common presenting symptom remains shortness of breath on exertion, which is present in just about every patient with PAH. Other common symptoms include fatigue, light-headedness with exertion, cough, chest pain, and palpitation. These symptoms are nonspecific and therefore contribute to delayed diagnosis of PAH.
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