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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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Pulmonary hypertensive arteriopathy (4348170715)
Pulmonary hypertensive arteriopathy (4348170715)
Pulmonary hypertensive arteriopathy (4348170715), CC BY-SA 2.0

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
  • He presented with progressive severe dyspnea on exertion and lower extremity edema.[ncbi.nlm.nih.gov]
  • The greatest risk is a diagnostic delay due to focus on discarding a pulmonary etiology of dyspnea. Physical examination and early echocardiographic assessment are the keys to avoid overlooking this uncommon pathology.[ncbi.nlm.nih.gov]
  • A 58-year-old woman was referred to our hospital with a chief complaint of exertional dyspnea.[ncbi.nlm.nih.gov]
  • A 63-year-old woman with a history of neurofibromatosis type-1 (NF-1) and pulmonary arterial hypertension (PAH) thought to be secondary to the NF-1 presented with a few weeks of worsening dyspnea on exertion.[ncbi.nlm.nih.gov]
  • This patient visited our hospital at 37 weeks of gestation after experiencing dyspnea and chest pain. Clinical evaluation revealed severe fixed pulmonary hypertension.[ncbi.nlm.nih.gov]
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]
  • Other symptoms may include: Chest pain (angina pectoris) Dizziness Fainting (syncope) Loss of energy Swelling of the arms, legs, ankles, or abdomen (edema) Dry cough Raynaud’s phenomenon (chalky white or dusky blue fingers that may be painful and can[pfizer.com]
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
  • A 58-year-old woman was referred to our hospital with a chief complaint of exertional dyspnea.[ncbi.nlm.nih.gov]
  • 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]
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]
  • These may 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 laryngeal[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]
  • However, the dyspnea and fatigue subsequently got worsened. Tracing back his family history, together with the electromyography, nerve conduction studies, and the result of muscle biopsy, mitochondrial disease was confirmed.[ncbi.nlm.nih.gov]
  • Dyspnoea and / or fatigue may even be present at rest. Discomfort is increased by any physical activity.[pulmonaryhypertension.ie]
  • Initial symptoms include dyspnea, fatigue, syncope, chest pain, palpitations and pedal edema.[orpha.net]
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]
Raynaud Phenomenon
  • More rarely, clubbing of digits and Raynaud phenomenon may be observed. Hemoptysis has also been reported. Etiology IPAH is caused by vascular remodeling of small pulmonary arteries due to unknown causes.[orpha.net]
  • phenomenon (mostly in females) have been observed.[orpha.net]
  • More rarely, abdominal distension, clubbing of digits and Raynaud phenomenon (predominantly in females) are observed. Hemoptysis has also been reported.[orpha.net]
  • 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]
  • Other symptoms may include: Chest pain (angina pectoris) Dizziness Fainting (syncope) Loss of energy Swelling of the arms, legs, ankles, or abdomen (edema) Dry cough Raynaud’s phenomenon (chalky white or dusky blue fingers that may be painful and can[pfizer.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]
  • These may 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 laryngeal[emedicine.medscape.com]
Chest Pain
  • This patient visited our hospital at 37 weeks of gestation after experiencing dyspnea and chest pain. Clinical evaluation revealed severe fixed pulmonary hypertension.[ncbi.nlm.nih.gov]
  • 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]
  • A 26-year-old man, previously diagnosed as a case of primary pulmonary hypertension, presented to the emergency department (ED) with chest pain and breathlessness on exertion. Cardiac biomarkers were within their normal ranges.[ncbi.nlm.nih.gov]
  • Initial symptoms include dyspnea, fatigue, syncope, chest pain, palpitations and pedal edema.[orpha.net]
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]
Dizziness
  • Patients experience symptoms such as shortness of breath, dizziness, and fatigue, and the severity of symptoms usually correlates with the progression of the disease.[pfizer.com]
  • Fatigue, dizziness, and fainting spells also can be symptoms. Swelling in the ankles, abdomen or legs, bluish lips and skin, and chest pain may occur as strain on the heart increases.[my.clevelandclinic.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]
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]
  • These may 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 laryngeal[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
  • Surface electrocardiogram showed right atrial and ventricular overload with right axis deviation. Chest imaging noted enlarged central pulmonary vascularity with bilateral plethoric lung fields.[ncbi.nlm.nih.gov]
  • 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]
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 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]
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
  • Before treatment, there were no significant differences in LVEF, LVFS, NT–proBNP, hsCRP, 6-MWT, LVD, LAD and LVMI between the treatment group and control group.[ncbi.nlm.nih.gov]
  • Comprehensive evaluations are needed to determine the severity of PH and associated diseases and to initiate treatment focused on FC.[ncbi.nlm.nih.gov]
  • We achieved a rapid improvement in clinical conditions and pulmonary pressure normalized after 42 h of treatment.[ncbi.nlm.nih.gov]
  • Both mechanism and treatment for pulmonary hypertension complicated with mitochondrial disease are unclear. Further study is necessary.[ncbi.nlm.nih.gov]
  • Herein, we describe a case of a 66-year-old woman who exhibited deterioration of mean pulmonary artery pressure and exercise tolerance after a 4-month treatment with riociguat.[ncbi.nlm.nih.gov]

Prognosis
  • PH in SSc can be characterized into distinct clusters that differ in prognosis.[ncbi.nlm.nih.gov]
  • The diagnosis of PH is associated with poor prognosis and significant morbidity and mortality.[ncbi.nlm.nih.gov]
  • PVOD is difficult to diagnose antemortem and has a poor prognosis. Lung transplantation is the only curative treatment for PVOD.[ncbi.nlm.nih.gov]
  • Development of sarcoidosis-associated pulmonary hypertension (SAPH) significantly worsens prognosis in sarcoidosis patients. Unfortunately, there is no treatment of proven benefit for this condition.[ncbi.nlm.nih.gov]
  • They may address risk assessment, disease progression, response to medical and surgical therapy, risk of right heart failure, and prognosis.[ncbi.nlm.nih.gov]

Etiology
  • It has a very low prevalence and its etiology is heterogeneous. Complex congenital cardiac malformations are also rare.[ncbi.nlm.nih.gov]
  • 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]
  • The greatest risk is a diagnostic delay due to focus on discarding a pulmonary etiology of dyspnea. Physical examination and early echocardiographic assessment are the keys to avoid overlooking this uncommon pathology.[ncbi.nlm.nih.gov]
  • The primary etiology of PH is unclear, although it can be from the heart, lung, or systemic disease.[ncbi.nlm.nih.gov]

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]
  • 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]
  • 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]
  • Epidemiology and genetics of pulmonary hypertension 4.1 Epidemiology and risk factors 4.2 Genetics 5.[escardio.org]
Sex distribution
Age distribution

Pathophysiology
  • The activation of molecular pathways is the pathophysiological underpinning of the biomarkers assessed in peripheral venous blood.[ncbi.nlm.nih.gov]
  • While the pathophysiology underlying the development of pulmonary hypertension (PH) in patients with HHT is not fully understood, it is believed to occur by one of two mechanisms: increases in pulmonary vascular resistance or cardiac output.[ncbi.nlm.nih.gov]
  • 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]
  • Pathophysiology The pathophysiology of IPAH is poorly understood.[emedicine.medscape.com]
  • 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]

Prevention
  • 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]
  • Given the need for improved diagnostic and therapeutic options for PH, the development or repurposing of metabolic tracers and medications could provide an effective avenue for preventing or even reversing disease progression.[ncbi.nlm.nih.gov]
  • ., 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]
  • 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)

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.

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Last updated: 2018-06-22 01:09