Pulmonary Hypertension

PH

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

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Pulmonary hypertensive arteriopathy (4348170715)

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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.

Respiratoric

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]
Pulmonary Hypertension Heart Breathlessness Chest pain Dizziness Fainting Loss of energy Swelling of the arms, legs, ankles or abdomen Dry cough Raynaud’s phenomenon Heart and lung disorders Family history HIV infection Connective tissue disorders Congenital[nm.org]
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]

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]

Hemoptysis

Here, we present the case of a 47-year-old man who presented with a 10-year history of progressive hemoptysis and a 2-year history of shortness of breath, in whom a diagnosis of FM was made.[ncbi.nlm.nih.gov]
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]

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]
The most common symptoms of pulmonary hypertension include: Shortness of breath, particularly with exertion (dyspnea) Chest pain or angina Fatigue Dizziness Fainting spells (syncope) Racing pulse or irregular heart beat Swelling in the ankles or legs[utswmed.org]
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]

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]

Entire body system

Fatigue

Dyspnoea and / or fatigue may even be present at rest. Discomfort is increased by any physical activity.[pulmonaryhypertension.ie]
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]
Signs and symptoms of PH include shortness of breath, fatigue, chest pain, swelling of legs, and fainting, which could also be seen in many other diseases thus making diagnosis of PH challenging.[lluh.org]
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]
This in turn can cause symptoms such as shortness of breath, fatigue, dizziness, fainting, heart palpitations, and chest pain. Among those diagnosed with PAH, heart failure is the leading cause of death.[uabmedicine.org]

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]
High blood pressure in your lung vessels causes your right ventricle and right atrium (heart chambers) to become enlarged and weak and to not pump as well.[wexnermedical.osu.edu]
Over time, the heart can grow weak and proper circulation can diminish throughout the body.[healthline.com]
Heart failure occurs when the heart becomes too weak to pump enough blood to the lungs.[heart.org]

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]
’s disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, and splenectomy[pulmonaryhypertension.ie]
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]

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]

Cardiovascular

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]
Ordinary physical activity causes undue dyspnoea or fatigue, chest pain, or near syncope. Class III : Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest.[pulmonaryhypertension.ie]
Signs and symptoms of PH include shortness of breath, fatigue, chest pain, swelling of legs, and fainting, which could also be seen in many other diseases thus making diagnosis of PH challenging.[lluh.org]
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]

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]

Neurologic

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].

Cardiac Catheterization

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]

Axis

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]

Blocks

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]

P Wave

P Pulmonale

[…] respiratory disease) Examination prominent ‘a’ wave palpable P2 right heart failure – increased JVP, (large V waves), parasternal heave, TR murmur, hepatomegaly, peripherial oedema other signs of CHF hypoxaemia INVESTIGATIONS Bedside ECG – RVH, RAD, p-pulmonale[lifeinthefastlane.com]

Jugular Venous Pressure

Prominent A-Wave

‘a’ wave palpable P2 right heart failure – increased JVP, (large V waves), parasternal heave, TR murmur, hepatomegaly, peripherial oedema other signs of CHF hypoxaemia INVESTIGATIONS Bedside ECG – RVH, RAD, p-pulmonale, tall R waves in V1, right ventricular[lifeinthefastlane.com]
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

We performed a systematic review to assess the efficacy and safety of BPA, especially compared to medical treatment or PEA.[ncbi.nlm.nih.gov]
Before treatment, there were no significant differences in LVEF, LVFS, NTproBNP, hsCRP, 6-MWT, LVD, LAD and LVMI between the treatment group and control group.[ncbi.nlm.nih.gov]
UC San Diego Health is a leader in the diagnosis and treatment of pulmonary arterial hypertension (PAH).[health.ucsd.edu]
Offering the latest in pediatric pulmonary hypertension treatments The best treatment for pulmonary hypertension differs for each child.[chw.org]

Prognosis

PH in SSc can be characterized into distinct clusters that differ in prognosis.[ncbi.nlm.nih.gov]
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]
[…] severity, prognosis, and treatment response in PH.[ncbi.nlm.nih.gov]
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]

Etiology

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]
The diagnosis of the disorder is often delayed due to nonspecific symptoms and signs, frequently reflecting the underlying etiology.[symptoma.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]
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 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

"Pulmonary hypertension in COPD: epidemiology, significance, and management: pulmonary vascular disease: the global perspective". Chest . 137 (6 Suppl): 39S–51S. doi : 10.1378/chest.10-0087 . PMID 20522579 .[en.wikipedia.org]
Epidemiology and genetics of pulmonary hypertension 4.1 Epidemiology and risk factors 4.2 Genetics 5.[escardio.org]
The present review is focused on hypertension, pulmonary hypertension, ischemic heart disease, and CHF to newly summarize the studies available to date from the viewpoints of epidemiology, pathogenesis, and treatment.[ncbi.nlm.nih.gov]
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]

Sex distribution

Age distribution

Pathophysiology

Pathophysiology of pulmonary hypertension in acute lung injury. American journal of physiology. Lung cellular and molecular physiology. 302(9):L803-15. 2012. [ pubmed ] [ free full text ] Stamm JA, Risbano MG, Mathier MA.[lifeinthefastlane.com]
Pathophysiology The pathophysiology of IPAH is poorly understood.[emedicine.medscape.com]
March 19, 2011 "Primary Pulmonary Hypertension: Practice Essentials, Background, Pathophysiology" . Archived from the original on 2015-12-24. York M, Farber HW (November 2011). "Pulmonary hypertension: screening and evaluation in scleroderma" .[en.wikipedia.org]
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]
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]

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]
Sleep-disordered breathing (SDB) is a risk factor for cardiovascular disease including acute coronary syndrome and acute myocardial infarction, and treating SDB prevents their development and recurrence and improves a patient's prognosis.[ncbi.nlm.nih.gov]
Prevention Can pulmonary hypertension be prevented or avoided? Not all pulmonary hypertension can be prevented. But you can make an effort to prevent other conditions that can cause the disease.[familydoctor.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)

Idiopathic PAH
Heritable (BMPR2/ALK1, endoglin, SMAD9, CAV1, KCNK3/Unknown)
Drug- and toxin-induced
Associated with Connective tissue diseases/HIV infection/Portal hypertension/Congenital heart diseases/Schistosomiasis
Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)
Persistent pulmonary hypertension of the newborn (PPHN)

Pulmonary hypertension owing to left heart disease

Left ventricular systolic dysfunction
Left ventricular diastolic dysfunction
Valvular heart disease
Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies

Pulmonary hypertension owing to lung diseases and/or hypoxia

Chronic obstructive pulmonary disease
Interstitial lung disease
Other pulmonary diseases with mixed restrictive and obstructive pattern
Sleep-disordered breathing
Alveolar hypoventilation disorders
Chronic exposure to high altitude
Developmental abnormalities

Chronic thromboembolic pulmonary hypertension (CTEPH)

Pulmonary hypertension with unclear multifactorial mechanisms

Hematologic disorders: Chronic hemolytic anemia, myeloproliferative disorders, splenectomy
Systemic disorders: Sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis
Metabolic disorders: Glycogen storage disease, Gaucher disease, thyroid disorders
Others: Tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental pulmonary hypertension

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

Simonneau G, GalièN, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(12 Suppl S):5S.
Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet 2003; 361:1533.
Peacock AJ. Primary pulmonary hypertension. Thorax 1999; 54:1107.
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.
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.
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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Cardiovascular manifestations in systemic lupus erythematosus. Prospective study of 100 patients - E Badui, D Garcia-Rubi, E Robles, J Jimenez, L Juan… - Angiology, 1985 - ang.sagepub.com [ang.sagepub.com]
Cardiovocal (Ortner's) syndrome left recurrent laryngeal nerve palsy associated with cardiovascular disease. - P Chan, CP Lee, JT Ko, JS Hung - The European journal of medicine, 1992 - ukpmc.ac.uk [ukpmc.ac.uk]
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Pulmonary Hypertension

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