J Am Coll Cardiol. Physicians should initially consider the history, clinical findings, and targeted noninvasive testing, particularly echocardiography. 2016 Sep 15;94(6):463-469. Immediate, unlimited access to all AFP content. The use of the term pulmonary arterial hypertension is restricted to those with a hemodynamic profile in which high pulmonary pressure results from elevated precapillary pulmonary resistance and normal pulmonary venous pressure and is measured as a pulmonary wedge pressure of 15 mmHg or less. Delcroix M, Levant S. Becker D, MMWR Morb Mortal Wkly Rep. 2020 Nov 11;10(4):2045894020939842. doi: 10.1177/2045894020939842. 2012;40(4):874–880. Epub 2015 May 14. Kane GC, Relevant issues in the pathology and pathobiology of pulmonary hypertension. The role of the right ventricle in pulmonary arterial hypertension. FOIA Suggested algorithm for the evaluation of suspected pulmonary hypertension in primary care. Advisory Committee on Immunization Practices recommended immunization schedule for adults aged 19 years or older—United States, 2016. Adir Y, Denton CP, Jenkins DP, 24. Age-based recommendations for pneumococcal vaccination should be followed, including use of the 23-valent pneumococcal polysaccharide vaccine (Pneumovax) for adults younger than 65 years.1,31,32, Pulmonary hypertension is associated with increased morbidity and mortality during the perioperative period.1,33 The perioperative assessment should include an assessment of functional status, care goals, and alternatives to surgery.33 Echocardiography assessing right ventricular function can help determine surgical risk.33 Surgical management of patients with severe pulmonary hypertension should be performed with subspecialty consultation.1,33, There is an elevated risk of pregnancy complications in women with pulmonary hypertension and particularly pulmonary arterial hypertension. This clinical content conforms to AAFP criteria for continuing medical education (CME). The most recent definition, as of May 2020, of pulmonary hypertension now defines >20 mmHg (rather than >25 mmHg) as pulmonary hypertension. 2021 Jan 25;7(1):00725-2020. doi: 10.1183/23120541.00725-2020. There is still insufficient evidence to add an exercise criterion to this definition. Guazzi M, Vichinsky E. Chronic thromboembolic pulmonary hypertension. Group 1*: Pulmonary arterial hypertension, Including idiopathic, heritable, and HIV-associated; systemic sclerosis and other connective tissue disease; congenital heart disease; schistosomiasis; drug- and toxin-induced, Group 2: Pulmonary hypertension due to left heart disease, Including systolic and diastolic dysfunction and valvular heart disease, Group 3: Pulmonary hypertension due to lung diseases and/or hypoxia, Including chronic obstructive pulmonary disease, sleep-disordered breathing, and interstitial lung disease, Group 4: Chronic thromboembolic pulmonary hypertension, Group 5: Multifactorial pulmonary hypertension, Including metabolic, systemic, and hematologic disorders (sickle cell disease), and others. Recognizing pulmonary hypertension in patients presenting with new signs or symptoms can be difficult because many symptoms are common and associated with an extensive differential diagnosis (Table 21–3,22). Epub 2012 Aug 10. Previous Next. Pulmonary hypertension due to left heart disease. Archer SL, Arias MA, Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Am Fam Physician. Hurd SS, Regardless of the treatment, these patients should receive lifelong anticoagulation therapy in the absence of contraindications.25, There are limited data on the treatment of pulmonary hypertension for most of the etiologies in this group. National Library of Medicine Galiè N. Hinzmann B, et al. et al. Information from references 21 through 23. 2016 May;47(5):1436-44. doi: 10.1183/13993003.01684-2015. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Archer SL, Centers for Disease Control and Prevention (CDC). See the CME Quiz Questions. Torbicki A, Halpern SD, et al. Coghlan JG, Clin Respir J. Vachiéry JL, 26. J Am Coll Cardiol. et al. 2013;187(4):347–365. Additional testing, including right heart catheterization, depends on the differential and the treatment response.3 For example, if chronic thromboembolic pulmonary hypertension is suspected, a ventilation-perfusion scan is part of the initial evaluation, but in a patient with underlying chronic lung disease, pulmonary function tests should be performed.3,25 Subspecialty consultation may be useful, particularly for patients with symptoms that progress despite treatment of comorbidities.3, The diagnostic classification of pulmonary hypertension determines the treatment options. Kim NH, Grünig E, Time from symptoms to definitive diagnosis of idiopathic pulmonary arterial hypertension: the delay study. Rodeheffer RJ, This likely reflects the challenges of diagnosis in patients with comorbidities.5 Reducing this delay allows initiation of therapy before right heart failure develops.1, Independent of classification, pulmonary hypertension can cause progressive, disabling symptoms, as well as increases in morbidity and mortality. This corresponds to the hemodynamic profiles of groups 3, 4, and 5 in the Dana Point classification system, which was updated during the 5th World Symposium on Pulmonary Hypertension. Ayala C, J Am Coll Cardiol. Pulmonary hypertension due to left heart disease. 21. Pulmonary hypertension due to left heart disease (group 2) 8.1 Diagnosis 8.2 Therapy 9. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. ; Kim NH, Pulmonary hypertension is a hemodynamic disorder defined by abnormally high pulmonary artery pressure that can occur in numerous diseases and clinical situations. Pulmonary hypertension is diagnosed by systematically evaluating the breathless patient and screening high risk groups. Becker D, The exact prevalence of PH in both the United States and the world is not known. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. See a GP if you have symptoms of pulmonary hypertension, such as breathlessness and tiredness. Pengo V, 2009;119(16):2250–2294. An official American Thoracic Society/American Association of Critical-Care Nurses/American College of Chest Physicians/Society of Critical Care Medicine policy statement: the Choosing Wisely® top 5 list in critical care medicine. Alonso-Fernández A, Heart. 35. bdunlap@eriefamilyhealth.org). Rubin LJ. Ayala C, Talwalkar A, 2013;62(25 suppl):D100–D108. A definitive diagnosis cannot be made on echocardiographic abnormalities alone, and some patients require invasive evaluation by right heart catheterization. 2014;190(7):818–826. Noninvasive imaging techniques such as cardiac magnetic resonance (MR) imaging and electrocardiographically (ECG) gated multidetector com… It does not provide medical advice, diagnosis or treatment. In patients with chronic obstructive pulmonary disease (COPD), the prevalence of pulmonary hypertension increases with COPD severity. Measurement of pulmonary arterial occlusion pressure (PAOP) is necessary to exclude post-capillary pulmonary hypertension resulting from comorbidities. Janda S, 2004;350(22):2257–2264. All rights Reserved. Schieb LJ, Hinzmann B, Gibbs J. Kane GC, Eur Heart J. Without treatment, pulmonary hypertension progresses rapidly to right ventricular failure and death, typically within 3 years of the diagnosis. Pulmonary hypertension surveillance: United States, 2001 to 2010. BMJ Open. Birth control and pregnancy management in pulmonary hypertension. 4. 2015 Sep;46(3):738-49. doi: 10.1183/09031936.00191014. Copyright © 2020 American Academy of Family Physicians. Borlaug BA, Eur Respir Rev. Tuder RM, / afp 2013;62(25 suppl):D92–D99. ; Vachiéry JL. Hoeper MM, DDCI-01, a novel long acting phospdiesterase-5 inhibitor, attenuated monocrotaline-induced pulmonary hypertension in rats. Barst RJ, Pulmonary hypertension is a progressive disease of the pulmonary arteries and is characterized by abnormally elevated pressure in the pulmonary circulation, a result of extensive vascular proliferation and remodeling (1,2). Chest. et al. Contact 2011;97(8):612–622. For example, in the Choosing Wisely campaign, treatment with advanced vasoactive agents is recommended only for the management of pulmonary arterial hypertension.26, Drug development has focused on the treatment of patients with pulmonary arterial hypertension. Vonk Noordegraaf A, et al. To confirm the diagnosis of pulmonary hypertension, and CTEPH in particular, right heart catheterisation is mandatory . 28. Data Sources: A PubMed search was completed using the terms pulmonary hypertension and pulmonary arterial hypertension. Machado RF, Eur Respir J. / Journals An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. 2016;37(1):67–119. Miller DP, et al. 33. Results from invasive hemodynamic testing with right heart catheterization, preferably at an expert center, should be obtained before initiating treatment with vasodilator therapy in patients with pulmonary arterial hypertension. 2014;146(2):476–495. 2005;111(5):534–538. Lau EM, Godinas L, Sitbon O, Montani D, Savale L, Jaïs X, Lador F, Gunther S, Celermajer DS, Simonneau G, Humbert M, Chemla D, Herve P. Eur Respir J. Condliffe R, ; Harriman KH. Dorfmüller P, 2008;359(21):2254–2265. Delcroix M, This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Pulmonary complications of sickle cell disease. Gladwin MT, Pulmonary hypertension (PH) can be difficult to diagnose in a routine medical exam because the most common symptoms of PH, such as breathlessness, fatigue and dizziness, are also associated with many other conditions. Echocardiography is the initial noninvasive diagnostic test, according to national guidelines.1–3,22 It is readily available and provides information about abnormalities in undiagnosed patients. Relevant issues in the pathology and pathobiology of pulmonary hypertension. N Engl J Med. 18. 2021 Feb 15;16:e01. Seeger W, Bennett NM, Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis [published correction appears in Heart. Global strategy for diagnosis, management, and prevention of COPD—2016. Sweet O, 6. Ann Rheum Dis. Clipboard, Search History, and several other advanced features are temporarily unavailable. Singh I, Oliveira RKF, Heerdt P, Brown MB, Faria-Urbina M, Waxman AB, Systrom DM. April 25, 2013. Am J Respir Crit Care Med. 2009;120(2):e13]. It provides an estimate of pulmonary artery pressure, either at rest or … 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). et al. 34. ; Epub 2020 Nov 4. Pulmonary hypertension is most prevalent in those with left heart disease (group 2).9 In systolic or diastolic left heart failure, prevalence estimates range from 25% to 83%.10 Pulmonary hypertension is also common in patients with lung disease and/or hypoxia (group 3). A multicenter retrospective study of patients with idiopathic pulmonary arterial hypertension found a mean time from symptom onset to diagnosis of 47 (± 34) months encompassing 5.3 (± 3.8) primary care visits and evaluation by 3.0 (± 2.1) subspecialists.16 Registries from the United Kingdom and Ireland show that in patients older than 50 years, the median duration of symptoms at diagnosis was 24 months vs. 12 months for patients younger than 50 years. Global Initiative for Chronic Obstructive Lung Disease. Gabbay E, Lam CS, The American College of Chest Physicians published treatment guidelines in 2014.1, Family physicians most often encounter pulmonary hypertension in patients with chronic disease such as heart failure, obstructive lung disease, and thromboembolism. Pulmonary hypertension is hard to diagnose early because it's not often detected in a routine physical exam. 29. Search dates: May to December 2015. note: This review updates a previous article on this topic by Stringham and Shah.36. The normal mPA is 14 ± 3 mm Hg with an upper limit of normal of approximately 20 mm Hg. Tomczyk S, eCollection 2020 Jul-Sep. Exercise-induced pulmonary hypertension: at last! Address correspondence to Beth Dunlap, MD, Northwestern University, 710 Lake Shore Dr., 4th Fl., Chicago, IL 60611 (e-mail: Jais X. Living with pulmonary hypertension: unique insights from an international ethnographic study. Ornelas J, 2006;27(9):1106–1113. www.escardio.org Declaration of COI • Lectures/consultancy and/or research fees: • Actelion • Bayer • Janssen • Orphan Pharmaceutics • Sanofi • United Therapeutics. Pulmonary arterial hypertension: an update on diagnosis and treatment. Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. 31. Ornelas J, Hager WD, Collins I, Tate JP, Azrin M, Foley R, Lakshminarayanan S, Rothfield NF. For information about the SORT evidence rating system, go to Exercise during cardiac catheterization distinguishes between pulmonary and left ventricular causes of dyspnea in systemic sclerosis patients. Levant S. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. Valle FH, Mohammed B, Wright SP, Bentley R, Fam NP, Mak S. Interv Cardiol. Circulation. Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis [published correction appears in. doi: 10.1161/JAHA.120.018123. In patients with sickle cell disease, guidelines recommend initiating hydroxyurea in patients with elevated mortality risk. Villamor J. Recent data in normal subjects show mean pulmonary artery pressure is 14.0±3.3 mmHg. In patients with pulmonary hypertension due to lung disease or left heart disease, treatment should focus on optimizing comorbid conditions. Epub 2013 Jan 24. Birth control and pregnancy management in pulmonary hypertension. Address correspondence to Beth Dunlap, MD, Northwestern University, 710 Lake Shore Dr., 4th Fl., Chicago, IL 60611 (e-mail: Taichman DB, Thenappan T, The role of the right ventricle in pulmonary arterial hypertension. The use of therapies targeted at pulmonary arterial hypertension is strongly discouraged.14, Patients with pulmonary hypertension should have seasonal influenza vaccination. Am J Respir Crit Care Med. Perioperative assessment of patients with pulmonary hypertension should include echocardiographic assessment of right ventricular function. Chest. eCollection 2020 Apr. 2014;23(134):450–457. Humbert M. 2010;82(4):370–377. et al. 2013;62(25 suppl):D34–D41. Agustí AG, Pulmonary arterial hypertension: screening, management, and treatment: executive summary. Swiston J. Pulmonary hypertension: ECG-gated 64-section CT angiographic evaluation of new functional parameters as diagnostic criteria. et al. www.escardio.org 3 Speaker. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. For example, those with mildly increased systolic pulmonary arterial pressure (i.e., less than 35 mm Hg) and an established precipitating diagnosis often do not need catheterization. Redfield MM. Agustí AG, Farber HW, afpserv@aafp.org for copyright questions and/or permission requests. Foreman AJ, Gomberg-Maitland M. Rich S, Diagnostic approach in patients with clinical suspicion for PH/pulmonary arterial hypertension PH due to parenchymal, cardiac, thromboembolic and other diseases (diagnostic groups 2, 3, 4 and 5, respectively) is associated with worse outcomes and limited treatment options, resulting in referral of these patients to PH centres. Right heart catheterisation (RHC) plays a central role in identifying pulmonary hypertension (PH) disorders, and is required to definitively diagnose pulmonary arterial hypertension (PAH). Updated clinical classification of pulmonary hypertension [published correction appears in J Am Coll Cardiol. Vachiéry JL, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galiè N, Ghio S, Gibbs JS, Martinez F, Semigran M, Simonneau G, Wells A, Seeger W. J Am Coll Cardiol. doi: 10.15420/icr.2020.17. Kingman M, The changing landscape of pulmonary arterial hypertension and implications for patient care. 25 mmHg was more or less arbitrary from the 1973 1st World Symposium on Pulmonary Hypertension. Redfield MM. [Article in Japanese] Nishimaki T(1), Aotsuka S, Kunieda T, Yokohari R. Author information: (1)Second Department of Internal Medicine, Fukushima Medical College. 2013;62(25 suppl):D109–D116. 15. 2014;4(5):e004735. To see the full article, log in or purchase access. Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as pulmonary hypertension in the presence of an organized thrombus within the pulmonary vascular bed that persists at least 3 months after the onset of anticoagulant therapy. Kingman M, 7. 7.5 Pulmonary arterial hypertension associated with human immunodeficiency virus infection 7.5.1 Diagnosis 7.5.2 Therapy 7.6 Pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis 7.6.1 Diagnosis 7.6.2 Therapy 8. Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine among adults aged ≥65 years: recommendations of the Advisory Committee on Immunization Practices (ACIP). Author information: (1)Department of Radiology, Calmette Hospital, University of Lille, Boulevard Jules Leclerc, Lille, France. The current haemodynamic definition of pulmonary hypertension (PH) is a mean pulmonary artery pressure (mPAP) ≥25 mmHg at rest [ 1 ]. Adir Y, Sign up for the free AFP email table of contents. J Am Coll Cardiol. Klings ES, Eur Heart J. et al. Chronic thromboembolic pulmonary hypertension. Farber HW, Archer SL, Tian L, et al. Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 25 mm Hg. Adir Y, Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Barberà JA, Definitions and diagnosis of pulmonary hypertension. Sweet O, Patients who are not surgical candidates can be considered for targeted medical therapies also used to treat pulmonary arterial hypertension. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Halpern SD, Circulation. Taichman DB, Criteria for diagnosis of exercise pulmonary hypertension The previous definition of exercise pulmonary hypertension (PH) with a mean pulmonary artery pressure (mPAP) >30 mmHg was abandoned because healthy individuals can exceed this threshold at high cardiac output (CO). Pulm Circ. Enlarge Vachiery JL, A population-based study in Minnesota estimated that the total prevalence could reach 10% to 20% in the general population.15, There is limited evidence to guide screening for pulmonary hypertension in asymptomatic individuals, even in high-risk groups, which leads to significant delays in diagnosis. Gin K, 2015 Sep;46(3):583-6. doi: 10.1183/09031936.00061015. 2013 Jul;7(3):227-36. doi: 10.1111/j.1752-699X.2012.00310.x. Shahidi N, An estimated systolic pulmonary arterial pressure of 35 to 40 mm Hg or greater on echocardiography is suggestive of pulmonary hypertension.1,21,22 Echocardiography also assesses right heart function, which is needed for diagnosis and monitoring disease progression.21, A meta-analysis calculated the accuracy of echocardiography vs. right heart catheterization for diagnosing pulmonary hypertension and found a sensitivity of 83% (95% confidence interval [CI], 73 to 90), a specificity of 72% (95% CI, 53 to 85), and an odds ratio of 13 (95% CI, 5 to 31).23 Elevated systolic pulmonary arterial pressure occurs in other conditions (Table 321–23), and echocardiography and right heart catheterization results can be disparate. Thromboembolic Pulmonary Hypertension Study Group. Current guidelines discourage pregnancy and recommend contraception counseling with emphasis on prescribing a long-acting, highly effective method of contraception.34. Global strategy for diagnosis, management, and prevention of COPD—2016. 22. Humbert M, Chung L, Grünig E, Congest Heart Fail. 16. Chung L, Borlaug BA. Tomczyk S, 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). Patients with chronic thromboembolic pulmonary hypertension should receive lifelong anticoagulation in the absence of contraindications. Pengo V, An official American Thoracic Society/American Association of Critical-Care Nurses/American College of Chest Physicians/Society of Critical Care Medicine policy statement: the Choosing Wisely. *—Other than increased vascular resistance. Vachiéry JL, DETECT study group. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology. et al. This review updates a previous article on this topic by Stringham and Shah. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. Rich S, eCollection 2020 Oct-Dec. Lichtblau M, Bader PR, Saxer S, Berlier C, Schwarz EI, Hasler ED, Furian M, Grünig E, Bloch KE, Ulrich S. J Am Heart Assoc. Jais X. Bennett NM, 2013 Mar 15;187(6):576-83. doi: 10.1164/rccm.201211-2090CI. 19. Yuan JX, Print. Dynamic right ventricular function response to incremental exercise in pulmonary hypertension. Agency for Healthcare Research and Quality. et al. et al. These measurements enable the investigator to establish the diagnosis of pulmonary hypertension and determine its degree, as well as provide a clue as to its cause. Next: Healthy Aging with Intimations of Mortality, Home Studies are limited by short follow-up periods and a lack of patient-centered outcomes.1,22 Patients should have a right heart catheterization and subspecialty referral before initiation of vasodilator or other targeted therapies.1–3 Patients without symptoms or evidence of functional impairment (using a six-minute walk test) should generally be monitored without therapy.1 After symptoms develop, patients with acute vasoreactivity on right heart catheterization should begin a trial of calcium channel blockers.1 Patients with a mean pulmonary arterial pressure decrease of more than 10 mm Hg to less than 40 mm Hg and with an unchanged or increased cardiac output when challenged are considered vasoreactive.3 Other treatments may include an endothelin receptor antagonist (bosentan [Tracleer]), a phosphodiesterase type 5 inhibitor (sildenafil [Revatio]), or a soluble guanylate cyclase stimulator (riociguat [Adempas]).1 Further treatment may include parenteral or inhaled prostanoids, such as epoprostenol (Flolan, Veletri), and newer oral prostacyclin agents, such as selexipag (Uptravi) and treprostinil (Orenitram).1,27, The focus for these patients is optimizing the underlying heart disease and controlling comorbidities.3,9,10 This includes management of hypertension and heart failure, and addressing significant valvular disease when present. Hoeper MM, Other important considerations for patients with pulmonary hypertension include influenza and pneumonia immunizations, contraception counseling, preoperative assessment, and mental health. Shah NR. Simonneau G, Global Initiative for Chronic Obstructive Lung Disease. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. DETECT study group. Pulm Circ. Machado RF, Right Atrial Pressure During Exercise Predicts Survival in Patients With Pulmonary Hypertension. 36. Accessed August 18, 2015. We also searched the National Guideline Clearinghouse. The mechanisms that increase pulmonary pressures can act primarily on the pulmonary arterial bed or venous bed, either alone or in combination.3,18 Pulmonary arterial hypertension is characterized by progressive narrowing of distal pulmonary arteries attributed to a variety of pathologic insults, such as arterial vasoconstriction, medial hypertrophy, intimal proliferation, and fibrosis.19 There are some genetic associations, such as BMPR2, but these are insufficient to explain the pathogenesis without other contri… Enders FT, García-Río F, Simonneau G, ; Tian L, et al. Pulmonary hypertension in obstructive sleep apnoea: effects of continuous positive airway pressure: a randomized, controlled cross-over study. Pulmonary hypertension due to left heart diseases. 2010;35(5):1079–1087. Martínez I, Barberà JA, Pulmonary arterial hypertension is … Pulmonary arterial hypertension (PAH) is a rare but serious type of high blood pressure. 2014;146(2):449–475.... 2. Although pulmonary arterial hypertension (group 1) has benefited the most from progress with targeted therapy, this diagnosis is rare and may not be encountered by many family physicians.7 However, in certain populations, such as persons with systemic sclerosis, pulmonary arterial hypertension is more common.8 Treatment is rapidly evolving and usually administered in expert centers. Semin Respir Crit Care Med. Hurd SS, As in CTEPH, intravascular obstructions might confound the correct estimation of PAOP in some patients: left … Pulmonary Hypertension Definitions and Diagnosis Eur Heart J, 2015 doi: 10.1093/eurheartj/ehv317 Eur Respir J, 2015 doi: 10.1183/13993003.01032-2015 Adam Torbicki, CMKP, ECZ-Otwock.
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