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Pulmonary Hypertension

PH

Pulmonary hypertension (PH) is a rise of blood pressure and resistance in the pulmonary vasculature.

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Presentation

The diagnosis of the disorder is often delayed due to nonspecific symptoms and signs, frequently reflecting the underlying etiology. Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain [2] [3] [4]. Uncommon symptoms include cough, hemoptysis and hoarseness.

Dyspnea
  • The diagnosis of pulmonary hypertension should be suspected in patients with progressive exertional dyspnea, angina or syncope on exertion and a coexisting possible cause of the condition.[symptoma.com]
  • Dyspnea and/or fatigue may be present at rest, and discomfort is increased by any physical activity.[aafp.org]
  • Symptoms and Signs Progressive exertional dyspnea and easy fatigability occur in almost all patients. Atypical chest discomfort and exertional light-headedness or presyncope may accompany dyspnea and indicate more severe disease.[merckmanuals.com]
  • Initial symptoms include dyspnea, fatigue, syncope, chest pain, palpitations and pedal edema.[orpha.net]
Cough
  • Other symptoms include fatigue lethargy, loss of appetite, chest pain, cough, blood in cough, hoarseness of voice. Diagnosis X ray-chest and laboratory studies are the first investigations followed by echocardiography.[symptoma.com]
  • The Ohio State Multidisciplinary Cough Clinic helps people with chronic coughs lasting at least six weeks.[lungcenter.osu.edu]
  • As the disease gets worse, symptoms can include the following: Shortness of breath , with or without activity Cough Fatigue Dizziness Chest pain or pressure Rapid heartbeat Swelling of the ankles, legs and abdomen Symptoms of pulmonary hypertension usually[familydoctor.org]
  • Pulmonary hypertension can lead to bleeding into the lungs and coughing up blood (hemoptysis). This is another potentially fatal complication. Dec. 28, 2017[mayoclinic.org]
Hemoptysis
  • Hemoptysis has also been reported. Etiology IPAH is caused by vascular remodeling of small pulmonary arteries due to unknown causes.[orpha.net]
  • Hemoptysis has also been reported. HPAH patients have a severe clinical with less response to acute vasodilator challenge, lower cardiac index, and higher pulmonary vascular resistance.[orpha.net]
  • Hemoptysis has also been reported.[orpha.net]
  • Uncommon symptoms include cough, hemoptysis and hoarseness. The diagnosis of pulmonary hypertension should be suspected in patients with progressive exertional dyspnea, angina or syncope on exertion and a coexisting possible cause of the condition.[symptoma.com]
  • Pulmonary hypertension can lead to bleeding into the lungs and coughing up blood (hemoptysis). This is another potentially fatal complication. Dec. 28, 2017[mayoclinic.org]
Exertional Dyspnea
  • The diagnosis of pulmonary hypertension should be suspected in patients with progressive exertional dyspnea, angina or syncope on exertion and a coexisting possible cause of the condition.[symptoma.com]
  • Symptoms are fatigue, exertional dyspnea, and, occasionally, chest discomfort and syncope. Diagnosis is made by finding elevated pulmonary artery pressure (estimated by echocardiography and confirmed by right heart catheterization).[merckmanuals.com]
  • PAH should be considered in the differential diagnosis of unexplained fatigue, exertional dyspnea, syncope, angina, and progressive limitation of exercise capacity, particularly in patients without common cardiovascular or respiratory disorders.[imaging.onlinejacc.org]
  • The most common symptoms—exertional dyspnea, fatigue and syncope—reflect an inability to increase cardiac output during activity. Typical angina may occur despite normal coronary arteries.[aafp.org]
  • As the disease progresses and right ventricular after-load increases, the heart’s ability to increase cardiac output with activity declines, resulting in exertional dyspnea, chest pain, or syncope.[aetna.com]
Hoarseness
  • Other symptoms include fatigue lethargy, loss of appetite, chest pain, cough, blood in cough, hoarseness of voice. Diagnosis X ray-chest and laboratory studies are the first investigations followed by echocardiography.[symptoma.com]
  • Cough, hemoptysis, and hoarseness are less common symptoms. Women are more likely to be symptomatic than men. Physical Examination Physical findings in persons with PAH can be quite variable.[emedicine.medscape.com]
  • Other symptoms include the following: Dyspnea upon exertion Fatigue Lethargy Syncope with exertion Chest pain Anorexia Right upper quadrant pain Less common symptoms include the following: Cough Hemoptysis Hoarseness (due to compression of the recurrent[emedicine.medscape.com]
  • Hoarseness due to recurrent laryngeal nerve compression by an enlarged pulmonary artery (ie, Ortner syndrome) also occurs rarely.[merckmanuals.com]
Fatigue
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • Dyspnea and/or fatigue may be present at rest, and discomfort is increased by any physical activity.[aafp.org]
  • Initial symptoms include dyspnea, fatigue, syncope, chest pain, palpitations and pedal edema.[orpha.net]
  • Other symptoms are fatigue, dizziness, swelling in the ankles or legs (edema), bluish lips and skin (cyanosis), chest pain, racing pulse and palpitations.[who.int]
Weakness
  • Symptoms Difficulty in breathing is commonest symptom followed by weakness and fainting. Other symptoms include fatigue lethargy, loss of appetite, chest pain, cough, blood in cough, hoarseness of voice.[symptoma.com]
  • Heart failure occurs when the heart becomes too weak to pump enough blood to the lungs.[heart.org]
  • They include: Progressive shortness of breath (especially with activity) Hyperventilation (breathing harder and faster) Fatigue (tiring easily) Progressive weakness Fainting spells Lightheadedness or dizziness Coughing up blood Progressive cyanosis (blue[cincinnatichildrens.org]
  • Your heart has to work harder to pump blood through those arteries, and after a while the heart muscle gets weak. Eventually, it can lead to heart failure . Causes Sometimes doctors can't find a reason for high blood pressure in the lungs.[webmd.com]
  • Presentation [ 2 ] Most commonly presents with progressive breathlessness, weakness and tiredness. Exertional dizziness and syncope may also develop. Oedema and ascites tend to occur late in the disease.[patient.info]
Gaucher Disease
  • disease, thyroid disorders Others: Tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental pulmonary hypertension Definition Pulmonary hypertension is a rise of blood pressure and resistance in the lung vessels.[symptoma.com]
  • Metabolic disorders: glycogen storage disease, Gaucher's disease, thyroid disorders. Others: tumour obstruction, fibrosing mediastinitis, chronic kidney disease, segmental pulmonary hypertension. Epidemiology [ 2 ] Idiopathic PAH is rare.[patient.info]
  • disease Thyroid disorders Other disorders: Fibrosing mediastinitis Tumor, causing obstruction Chronic kidney disease Segmental pulmonary hypertension Adapted from the Fifth World Symposium on PAH, Nice, 2013; Simonneau G, Gatzoulis MA, AdatiaI, et al[merckmanuals.com]
  • disease, and thyroid disorders Subgroup 4 - Miscellaneous conditions, including tumor obstruction, mediastinal fibrosis, and chronic renal failure on dialysis On the basis of information adapted from the executive summary of the world symposium on Primary[emedicine.medscape.com]
  • disease, thyroid disorders 5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH Within this classification, IPAH represents a subset of pulmonary vascular disease called pulmonary arterial hypertension (Group[emedicine.medscape.com]
Anorexia
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • The most common symptoms and their frequency, reported in a national prospective study, are as follows: Dyspnea (60% of patients) Weakness (19%) Recurrent syncope (13%) Additional symptoms include fatigue, lethargy, anorexia, chest pain, and right upper[emedicine.medscape.com]
  • Other symptoms include the following: Dyspnea upon exertion Fatigue Lethargy Syncope with exertion Chest pain Anorexia Right upper quadrant pain Less common symptoms include the following: Cough Hemoptysis Hoarseness (due to compression of the recurrent[emedicine.medscape.com]
Right Upper Quadrant Pain
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • Other symptoms include the following: Dyspnea upon exertion Fatigue Lethargy Syncope with exertion Chest pain Anorexia Right upper quadrant pain Less common symptoms include the following: Cough Hemoptysis Hoarseness (due to compression of the recurrent[emedicine.medscape.com]
Pulsatile Liver
  • The signs of right ventricular failure include a high-pitched systolic murmur of tricuspid regurgitation, hepatomegaly, a pulsatile liver, ascites, and peripheral edema. In this scenario, a right ventricular third heart (S 3 ) sound is also heard.[emedicine.medscape.com]
Chest Pain
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • Symptoms of PH include Shortness of breath during routine activity, such as climbing two flights of stairs Tiredness Chest pain A racing heartbeat Pain on the upper right side of the abdomen Decreased appetite As PH worsens, you may find it hard to do[nlm.nih.gov]
  • Initial symptoms include dyspnea, fatigue, syncope, chest pain, palpitations and pedal edema.[orpha.net]
  • Other symptoms are fatigue, dizziness, swelling in the ankles or legs (edema), bluish lips and skin (cyanosis), chest pain, racing pulse and palpitations.[who.int]
Exertional Syncope
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • Exertional syncope . Sudden cardiac death .[patient.info]
Graham Steell Murmur
  • Pulmonic regurgitation (Graham Steell murmur) may be apparent. A murmur of tricuspid regurgitation can be present, and a right ventricular lift (heave) may be noted.[emedicine.medscape.com]
  • Steell murmur) may be apparent A murmur of tricuspid regurgitation can be present, and a right ventricular lift (heave) may be noted Jugular venous pulsations may be elevated in the presence of volume overload, right ventricular failure, or both; large[emedicine.medscape.com]
Lethargy
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • Other less common symptoms may include: cough , fatigue , lethargy, and dizziness .[emedicinehealth.com]
  • The most common symptoms and their frequency, reported in a national prospective study, are as follows: Dyspnea (60% of patients) Weakness (19%) Recurrent syncope (13%) Additional symptoms include fatigue, lethargy, anorexia, chest pain, and right upper[emedicine.medscape.com]
  • Other symptoms include the following: Dyspnea upon exertion Fatigue Lethargy Syncope with exertion Chest pain Anorexia Right upper quadrant pain Less common symptoms include the following: Cough Hemoptysis Hoarseness (due to compression of the recurrent[emedicine.medscape.com]
  • Pulmonary hypertension symptoms may include: Shortness of breath that worsens with activity Other common complaints are cough , fatigue , dizziness , and lethargy.[medicinenet.com]

Workup

The diagnosis of pulmonary hypertension should be suspected in patients with progressive exertional dyspnea, angina or syncope on exertion and a coexisting possible cause of the condition. Electrocardiography (ECG), laboratory studies (complete blood count (CBC), liver function tests, HIV serology, prothrombin time (PT), and activated partial thromboplastin time (aPTT)), arterial blood gas measurements and X-rays of the chest should be obtained. Echocardiography with Doppler flow studies is the most useful noninvasive method and can be employed to estimate the pulmonary artery pressure and ventricular function [5] [6]. Depending on the etiology, further evaluation may include pulmonary function testing, high-resolution computed tomography of the chest, polysomnography, ventilation-perfusion lung scanning and right cardiac catheterization. The diagnosis of most forms of pulmonary hypertension requires right heart catheterization and is confirmed when the mean pulmonary artery pressure is greater than 25 mmHg at rest [1].

Pulmonary Arterial Pressure Increased
  • The current Nice Classification system of PH now lists the following genetic defects that are known to be associated with PAH [8] : BMPR2 ALK1 ENG SMAD9 CAV1 KCNK3 Early in IPAH (and probably in APAH), as the pulmonary artery pressure increases because[emedicine.medscape.com]
Right Axis Deviation
  • In pulmonary hypertension, the electrocardiogram (ECG) may demonstrate signs of right ventricular hypertrophy, such as tall right precordial R waves, right axis deviation and right ventricular strain ( Figure 1 ) .[aafp.org]
  • These signs include the following: Right-axis deviation R/S ratio greater than 1 in lead V1 Right atrial enlargement as indicated by an increased P-wave amplitude in lead II Right bundle branch block PPH is diagnosed if no underlying etiology is found[emedicine.medscape.com]
  • Common ECG findings include right axis deviation, R S in V 1 , S 1 Q 3 T 3 (suggesting right ventricular hypertrophy), and peaked P waves (suggesting right atrial dilation).[merckmanuals.com]
  • axis deviation, right ventricular hypertrophy, and characteristic ST depression and T-wave inversions in the anterior leads.[emedicine.medscape.com]
  • The electrocardiogram might show right ventricular hypertrophy, right-axis deviation, or right atrial enlargement.[clevelandclinicmeded.com]
Incomplete Right Bundle Branch Block
  • Right axis deviation (short arrow), increased P-wave amplitude in lead II (black arrowhead), and incomplete right bundle branch block (white arrowhead) are highly specific but lack sensitivity for the detection of right ventricular hypertrophy. 12 View[aafp.org]
Prominent A-Wave
  • A prominent A wave may be observed in the jugular venous pulse. A right-sided fourth heart sound (S 4 ) with a left parasternal heave may be auscultated. Right ventricular failure leads to systemic venous hypertension and cor pulmonale.[emedicine.medscape.com]

Treatment

  • UC San Diego Health is a leader in the diagnosis and treatment of pulmonary arterial hypertension (PAH).[health.ucsd.edu]
  • In fact, as an academic medical center, we are one of a select number of treatment centers nationwide to offer the highly complex and leading edge treatments we do for conditions such as pulmonary embolism and pulmonary vascular disease.[froedtert.com]
  • Pulmonary Hypertension and Treatment Options While there is no cure for pulmonary hypertension, thanks to advances in the treatment over the past decade, more effective options are available to patients than ever before.[umcvc.org]
  • Proper treatment has been found to extend life and improve quality of life. However, early intervention remains the key to maximizing treatment efficacy and patient outcomes.[ochsner.org]
  • Through this program, patients have access to the latest diagnostic techniques, treatments and therapies to manage PH.[vcuhealth.org]

Prognosis

  • PAH associated with systemic sclerosis carries a worse prognosis than idiopathic PAH. However, PAH associated with congenital heart disease has a better prognosis than idiopathic PAH.[patient.info]
  • Prognosis Prognosis varies between different forms, but is generally poor; the mean survival rate after diagnosis is The documents contained in this web site are presented for information purposes only.[orpha.net]
  • Prognosis Prognosis of Pulmonary Hypertension. Early diagnosis and treatment substantially improves the prognosis for pulmonary hypertension associated with systemic sclerosis (scleroderma). ISN.[sclero.org]
  • The prognosis and life expectancy for a person with pulmonary hypertension is improving as newer treatment options become available; however, prognosis may depend on the underlying disease or condition that is causing pulmonary hypertension.[medicinenet.com]
  • HPAH due to ACVRL1 or TBX4 gene mutations occurs more commonly in children with rapid progression and poor prognosis. Etiology HPAH has been linked to mutations in BMPR2 ((2q33) in majority of cases.[orpha.net]

Etiology

  • The diagnosis of the disorder is often delayed due to nonspecific symptoms and signs, frequently reflecting the underlying etiology.[symptoma.com]
  • Clinical description Clinical manifestations of IPAH are similar to other forms of PAH regardless of its etiology. IPAH develop in adults and in rare cases in children; women are twice as likely as men to be affected.[orpha.net]
  • On the basis of the results, a more focused approach to establish the etiology of the pulmonary hypertension may then involve tests 6-9.[emedicine.medscape.com]
  • Etiology HPAH has been linked to mutations in BMPR2 ((2q33) in majority of cases. However, other genes implicated in HPAH have been described in few cases, and include ACVRL1 ((12q13)), KCNK3 (2p23), CAV1 (7q31), TBX4 (17q21) and SMAD9 (13q12) .[orpha.net]
  • Etiology PAH is caused by vascular remodeling of pulmonary arteries leading to widespread obliteration of the smallest arteries; sufficient occlusion of these vessels increases the resistance to pulmonary blood flow and a compensating increased pressure[orpha.net]

Epidemiology

  • "Obesity Hypoventilation Syndrome Epidemiology and Diagnosis" . Sleep medicine clinics . 9 (3): 341–347. doi : 10.1016/j.jsmc.2014.05.007 . ISSN 1556-407X . PMC 4210766 . PMID 25360072 .[en.wikipedia.org]
  • Epidemiology and genetics of pulmonary hypertension 4.1 Epidemiology and risk factors 4.2 Genetics 5.[escardio.org]
  • Summary Epidemiology Prevalence of PAH, in all of its forms, is estimated at around 1/67,000. IPAH is one of the most commonly diagnosed forms.[orpha.net]
  • Summary Epidemiology HPAH represent less than 4% of PAH. Prevalence is estimated at 1/million people. Clinical description HPAH develop usually in adults, and rarely in children; women are twice as likely as men to be affected.[orpha.net]
  • Summary Epidemiology Prevalence of PAH, in all of its forms, is estimated around 5/1,000,000. Clinical description Most forms of PAH develop in adults, and in rare cases in children; women are twice as likely as men to be affected.[orpha.net]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology The pathophysiology of IPAH is poorly understood.[emedicine.medscape.com]
  • Although previously thought to be uncommon, canine pulmonary hypertension is now diagnosed with relative frequency. 1 Understanding the pathophysiology of pulmonary hypertension is paramount to successful treatment.[veterinarymedicine.dvm360.com]
  • March 19, 2011 "Primary Pulmonary Hypertension: Practice Essentials, Background, Pathophysiology" . Archived from the original on 2015-12-24. York, Michael; Farber, Harrison W. (November 2011).[en.wikipedia.org]
  • Pathophysiology Blood returning to the lungs is pumped through the lungs by the right ventricle of the heart. Blood pressure in the lungs is ordinarily rather low, for example 20/10, in contrast to body blood pressure which is usually around 110/70.[med.umich.edu]
  • Definitions [ 1 ] PH is a haemodynamic and pathophysiological condition defined as an increase in mean PAP 25 mm Hg at rest as assessed by right heart catheterisation.[patient.info]

Prevention

  • ., dizziness, chest pain, ankle swelling, feeling your heart race or pound [palpitations]). 2,3 Prevention and Treatment While not all pulmonary hypertension can be prevented, efforts to prevent high blood pressure, coronary heart disease, chronic liver[cdc.gov]
  • Using pulmonary rehabilitation, medications, surgery, and the latest research, we aim to prevent your condition from getting worse and help you maintain as normal a lifestyle as possible.[dukemedicine.org]
  • Pulmonary Hypertension Prevention Prevention of pulmonary hypertension may focus on reducing risk factors that may lead to the underlying cause. This is not always possible, especially in situations where the risk factors are not controllable.[emedicinehealth.com]
  • A Centers for Disease Control and Prevention (CDC) review of a decade's worth of data shows that deaths from PAH are increasing among men and women of all ages. Hospitalization for PAH has almost doubled for people over 85.[everydayhealth.com]
  • Preventing Endocarditis People with PH and certain underlying congenital heart defects may need antibiotics before certain dental procedures to prevent endocarditis.[heart.org]

Summary

Pulmonary hypertension is characterized by a mean pulmonary arterial pressure exceeding 25 mm Hg at rest or 30 mm Hg during exercise [1]. The World Health Organization (WHO) classified pulmonary hypertension into five main groups based upon etiology and disease mechanisms:

Pulmonary arterial hypertension (PAH)

Pulmonary hypertension owing to left heart disease

Pulmonary hypertension owing to lung diseases and/or hypoxia

Chronic thromboembolic pulmonary hypertension (CTEPH)

Pulmonary hypertension with unclear multifactorial mechanisms

Patient Information

Definition

Pulmonary hypertension is a rise of blood pressure and resistance in the lung vessels.

Cause

Pulmonary hypertension may be without any cause or with some associated factors like cirrhosis of the liver, appetite suppressants, HIV infection, alpha adrenergic stimulants like amphetamine and cocaine. It may also be due to some underlying diseases like left side heart diseases such as valvular abnormalities, myocardial diseases, and compression of pulmonary vein or respiratory conditions including COPD, interstitial diseases, sleep apnea and high altitude sickness for a long time. It may occur after thromboembolism episode or recurrent thromboembolism.

Symptoms

Difficulty in breathing is commonest symptom followed by weakness and fainting. Other symptoms include fatigue lethargy, loss of appetite, chest pain, cough, blood in cough, hoarseness of voice.

Diagnosis

X ray-chest and laboratory studies are the first investigations followed by echocardiography. Further investigations depend on the underlying cause. To confirm the diagnosis, right-sided cardiac catheterization may be done.

Treatment

Cases are treated with oxygen, pharmacological therapy and if required surgical intervention. In secondary cases management of primary condition is important. Pharmacological agents include calcium channel blockers, endothelin-receptor antagonists, Phosphodiesterase type 5 inhibitors, soluble guanylate cyclase (sGC) stimulators, prostacyclins, digoxin, and anticoagulants.

References

Article

  1. Simonneau G, GalièN, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(12 Suppl S):5S.
  2. Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet 2003; 361:1533.
  3. Peacock AJ. Primary pulmonary hypertension. Thorax 1999; 54:1107.
  4. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016 Jan 1;37(1):67-119.
  5. Jacobs W, Konings TC, Heymans MW, et al. Noninvasive identification of left-sided heart failure in a population suspected of pulmonary arterial hypertension. Eur Respir J 2015; 46:422.
  6. Berger M, Haimowitz A, Van Tosh A, et al. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol 1985; 6:359.

Symptoms

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