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.

• 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]

  Dyspnoea and / or fatigue may even be present at rest. Discomfort is increased by any physical activity. [pulmonaryhypertension.ie]

  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]

  Initial symptoms include dyspnea, fatigue, syncope, chest pain, palpitations and pedal edema. [orpha.net]

• Weakness

  Also, because your heart is working harder than normal, your right ventricle becomes strained and weak. Your heart may become so weak that it can't pump enough blood to your lungs. This causes heart failure. [web.archive.org]

  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]

  Other symptoms include: Ankle and leg swelling Bluish color of the lips or skin ( cyanosis ) Chest pain or pressure, usually in the front of the chest Dizziness or fainting spells Fatigue Increased abdomen size Weakness People with pulmonary hypertension [nlm.nih.gov]

  Symptoms Mild pulmonary hypertension usually causes no symptoms, but as the pressures in the pulmonary artery increase, shortness of breath, weakness, lethargy, and fatigue become common. [verywellhealth.com]

• Raynaud Phenomenon

  […] doi: 10.1097/BOR.0b013e32834ba6a7 Raynaud phenomenon, scleroderma, overlap syndromes and other fi brosing syndromes: Edited by John Varga Abstract Author Information Authors Article Metrics Metrics The review provides an update of the epidemiology, pathogenesis [journals.lww.com]

  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]

  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]

• 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]

  Many of the medications are associated with side effects of anorexia or nausea, so careful attention to nutritional status is needed to maintain overall health. Infusion pump management. [ncbi.nlm.nih.gov]

• Impaired Exercise Tolerance

  DOI: INTRODUCTION Dyspnoea on exertion, impaired exercise tolerance, and fatigue are most often due to common cardiac and pulmonary diseases such as heart failure or COPD, but may be also due to pulmonary hypertension, a pathophysiological state of diverse [bjgp.org]

• Dyspnea

  He presented with progressive severe dyspnea on exertion and lower extremity edema. [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]

• 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]

  Other symptoms, such as coughing (rarely, coughing up blood) and wheezing, are usually caused by the underlying lung disorder. [merckmanuals.com]

  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]

  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]

  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]

  Rarely, people with pulmonary hypertension become hoarse. [merckmanuals.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]

  Affected individuals may also have a cough, sometimes with blood (hemoptysis), an enlarged heart and liver, low blood pressure (hypotension), and hoarseness due to compression of a nerve in the chest by an enlarged pulmonary artery. [rarediseases.org]

• Heart Failure

  It affects approximately 50% to 60% of patients with heart failure with preserved ejection fraction as well as 60% of those with heart failure with reduced ejection fraction and contributes significantly to disease progression and unfavorable outcomes [ncbi.nlm.nih.gov]

  […] to diastolic heart failure ( table 1 ) [ 4 ]. [doi.org]

• Chest Pain

  We describe a rare case of a right coronary artery fistula draining to the right atrium, manifesting in chest pain and pulmonary arterial hypertension (PAH). The fistula was detected on transesophageal echocardiography during the workup for PAH. [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]

  Signs and symptoms of pulmonary hypertension (PH) may 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 [web.archive.org]

  Other symptoms include: Ankle and leg swelling Bluish color of the lips or skin ( cyanosis ) Chest pain or pressure, usually in the front of the chest Dizziness or fainting spells Fatigue Increased abdomen size Weakness People with pulmonary hypertension [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.com]

• Jugular Venous Distention

  As the disease progresses, additional signs of right heart failure from PAH can be detected with jugular venous distention, right-sided S3, hepatomegaly, ascites, lower extremity edema, and peripheral cyanosis. [thecardiologyadvisor.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]

• 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]

  Symptoms Mild pulmonary hypertension usually causes no symptoms, but as the pressures in the pulmonary artery increase, shortness of breath, weakness, lethargy, and fatigue become common. [verywellhealth.com]

  The day of admission he had decreased PO intake, lethargy, increased WOB. Patient had no primary care but had his 2 month vaccines from the health department. He was found to have profound respiratory acidosis and hypoxemia. Post navigation [ultrasoundoftheweek.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]

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]

  INVESTIGATIONS The ECG may show right axis deviation and incomplete RBBB. A normal ECG, however, does not exclude pulmonary hypertension. The chest X-ray may show dilated pulmonary arteries (Figure 1) and an enlarged right ventricle. [bjgp.org]

  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.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]

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

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.

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.