Assessment of the state of the cardiorespiratory system in pulmonary hypertension associated with congenital heart disease
Authors:
Company:
1 Bakoulev National Medical Research Center for Cardiovascular Surgery, Moscow, Russian Federation
2 Russian Medical Academy of Continuing Professional Education, Moscow, Russian Federation
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Type: Reviews
DOI:
For citation: Glushko L.A., Shmalts A.A. Assessment of the state of the cardiorespiratory system in pulmonary hypertension associated with congenital heart disease. Creative Cardiology. 2021; 15 (2): 167–79 (in Russ.). DOI: 10.24022/1997-3187-2021-15-2-167-179
Received / Accepted: 23.04.2021 / 23.06.2021
Keywords: pulmonary hypertension congenital heart disease cardiopulmonary exercise testing 6-minute walk test lung function tests lung diffusion capacity test end-expiratory partial pressure of carbon dioxide
Abstract
Pulmonary hypertension (PH) is characterized by an increase in mean pulmonary artery (PA) pressure above 25 mm Hg. PH is common in adults with congenital heart disease (CHD) and significantly affects morbidity and mortality. Careful screening of all CHD patients is critical to identify patients who develop PH and who may benefit from further investigations, including cardiac catheterization and initiation of PAH-specific therapy. The diagnosis of PH is made in the catheterization laboratory by a complete invasive hemodynamic assessment using a vasodilator. Screening for PH is currently done with routine echocardiography. However, with echocardiography, data errors are possible due to the fact that systolic pressure in the right ventricle is estimated by the speed of the tricuspid regurgitation stream, and the diastolic pressure of the pulmonary artery – by the speed of the regurgitation stream of the pulmonary artery valve, additional stenosis of the pulmonary artery is possible. The state of the cardiorespiratory system in patients with CHD complicated by PH can be assessed using a 6-minute walk test and a cardiopulmonary exercise test. Lung function tests and lung diffusion testing may provide additional information.References
- Diller G.P., Kempny A., Inuzuka R., Radke R., Wort S.J., Baumgartner H. et al. Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart. 2014; 100 (17): 1366–72. DOI: 10.1136/heartjnl-2014-305690
- Dimopoulos K., Wort S.J., Gatzoulis M.A. Pulmonary hypertension related to congenital heart disease: a call for action. Eur. Heart J. 2014; 35: 691–700. DOI: 10.1093/eurheartj/eht437
- Diller G.P., Dimopoulos K., Okonko D., Li W., Babu-Narayan S.V., Broberg C.S. et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005; 112 (6): 828–35. DOI: 10.1161/CIRCULATIONAHA.104.529800
- Dimopoulos K., Okonko D.O., Diller G.P., Broberg C.S., Salukhe T.V., Babu-Narayan S.V. et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation. 2006; 113: 2796–802. DOI: 10.1161/CIRCULATIONAHA.105.594218
- Kempny A., Dimopoulos K., Uebing A., Moceri P., Swan L., Gatzoulis M.A. et al. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life–single centre experience and review of published data. Eur. Heart J. 2012; 33: 1386–96. DOI: 10.1093/eurheartj/ehr461
- Daliento L., Somerville J., Presbitero P., Menti L., Brach-Prever S., Rizzoli G. et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur. Heart J. 1998; 19: 1845–55. DOI: 10.1053/euhj.1998.1046 7. Diller G.P., Dimopoulos K., Broberg C.S., Kaya M.G., Naghotra U.S., Uebing A. et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur. Heart J. 2006; 27: 1737–42. DOI: 10.1093/eurheartj/ehl116
- Diller G.P., Alonso-Gonzalez R., Dimopoulos K., Alvarez-Barredo M., Koo C., Kempny A. et al. Disease targeting therapies in patients with Eisenmenger syndrome: response to treatment and long-term efficiency. Int. J. Cardiol. 2013; 167: 840–7. DOI: 10.1016/j.ijcard.2012.02.007
- Dimopoulos K., Inuzuka R., Goletto S., Giannakoulas G., Swan L., Wort S.J. et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010; 121: 20–5. DOI: 10.1161/CIRCULATIONAHA.109.883876
- Galiè N., Beghetti M., Gatzoulis M.A., Granton J., Berger R.M.F., Lauer A. et al., Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006; 114: 48–54. DOI: 10.1161/CIRCULATIONAHA.106.630715
- ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am. J. Respir. Crit. Care Med. 2002; 166 (1): 111–7. DOI: 10.1164/ajrccm.166.1.at1102
- Chazova I.E., Martynyuk T.V., Valieva Z.S., Azizov V.A., Barbarash O.L., Veselova T.N. et al. Eurasian clinical guidelines on diagnosis and treatment of pulmonary hypertension. Eurasian Heart Journal. 2020; 1 (30): 78–122 (in Russ.). DOI: 10.38109/2225-1685-2020-1-78-122
- Pulmonary hypertension, including chronic thromboembolic pulmonary hypertension. Russian clinical guidelines. Moscow; 2020 (in Russ.). Available at:http://cr.rosminzdrav.ru/#!/recomend/137 (accessed 10.03.2021).
- Galiè N., Humbert M., Vachiery J.L., Gibbs S., Lang I., Torbicki A. 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; 37 (1): 67–119. DOI: 10.1093/eurheartj/ehv317
- Pulmonary hypertension in children. Russian clinical guidelines. Moscow; 2017 (in Russ.). Available at: http://cr.rosminzdrav.ru/#!/recomend/901 (accessed 10.03.2021).
- Hypertensive pulmonary vascular disease associated with congenital heart diseases in children. Russian clinical guidelines. Moscow; 2018 (in Russ.). Available at:http://cr.rosminzdrav.ru/#!/schema/356 (accessed 10.03.2021).
- Hansmann G., Koestenberger M., Alastalo T.P., Apitz C., Austin E.D., Bonnet D. et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J. Heart Lung. Transplant. 2019; 38 (9): 879–901. DOI: 10.1016/j.healun.2019.06.022
- Kozlik-Feldmann R., Hansmann G., Bonnet D., Schranz D., Apitz C., Michel-Behnke I. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016; 102 (Suppl. 2): ii42–8. DOI: 10.1136/heartjnl2015-308378
- Gorbachevsky S.V., Shmalts A.A., Plotnikova L.R. Pulmonary hypertension in children with congenital heart disease. Moscow; 2018 (in Russ.).
- Enright P.L., Sherrill D.L. Reference equations for the six-minute walk in healthy adults. Am. J. Respir. Crit. Care Med. 1998; 158 (5, Pt 1): 1384–7. DOI: 10.1164/ajrccm.158.5.9710086
- Wasserman K., Hansen J.E., Sue D.Y., Stringer W.W., Whipp B.J. Principles of exercise testing and interpretation: including pathophysiology and clinical applications. Fifth edition. Philadelphia: Lippincott Williams & Wilkins; 2012.
- Pressler A., Niebauer J. Textbook of Sports and Exercise Cardiology. Springer Nature Switzerland AG; 2020. 23. Klinger J.R., Frantz R.P. (Eds.) Diagnosis and management of pulmonary hypertension. New York, NY: Humana Press; 2015. DOI: 10.1007/978-1-4939-2636-7
- Boсkeria L.A., Boсkeria O.L., Glushko L.A., Mironenko M.Yu., Donakanyan S.A., Biniashvili M.B. et al. Long-term results of state assessment of cardiorespiratory system in patients with chronic ischemic heart disease after incomplete myocardial revascularization. The Bulletin of Bakoulev Center. Cardiovascular Diseases. 2019; 20 (7–8): 602–9 (in Russ.). DOI: 10.24022/1810- 0694-2019-20-7-8-602-609
- American College of Sports Medicine (ACSM) Walking Equation ACSM’s Guidelines for Exercise Testing and Prescription. 2006. (дата обращения 10.03.2021)
- American Thoracic Society, American College of Chest Physicians. ATS/ACCP Statement on cardiopulmonary exercise testing. Am. J. Respir. Crit. Care Med. 2003; 167 (2): 211–77. DOI: 10.1164/rccm.167.2.211
- Guazzi M., Adams V., Conraads V., Halle M., Mezzani A., Vanhees L. et al. European Association for Cardiovascular Prevention and Rehabilitation; American Heart Association. EACPR/AHA Scientific Statement. Clinical recommendations for cardiopulmonary exercise testing data assessment in specific patient populations. Circulation. 2012; 126 (18): 2261–74. DOI: 10.1161/CIR.0b013e31826fb946
- Mantegazza V., Apostolo A., Hager A. Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease. Ann. Am. Thorac. Soc. 2017; 14 (Suppl. 1): S93–S101. DOI: 10.1513/AnnalsATS. 201611-876FR
- Buys R., Cornelissen V., Van De Bruaene A., Stevens A., Coeckelberghs E., Onkelinx S. et al. Measures of exercise capacity in adults with congenital heart disease. Int. J. Cardiol. 2011; 153 (1): 26–30. DOI: 10.1016/j.ijcard.2010.08.030
- Chen S.S.M., Dimopoulos K., Sheehan F.H., Gatzoulis M.A., Kilner P.J. Physiologic determinants of exercise capacity in patients with different types of right-sided regurgitant lesions: Ebstein's malformation with tricuspid regurgitation and repaired tetralogy of Fallot with pulmonary regurgitation. Int. J. Cardiol. 2016; 205: 1–5. DOI: 10.1016/j.ijcard.2015.10.175
- Greutmann M., Le T.L., Tobler D., Biaggi P., Oechslin E.N., Silversides C.K. et al. Generalised muscle weakness in young adults with congenital heart disease. Heart. 2011; 97 (14): 1164–8. DOI: 10.1136/hrt.2010.213579
- Alonso-Gonzalez R., Borgia F., Diller G.P., Inuzuka R., Kempny A., Martinez-Naharro A. et al. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013; 127 (8): 882–90. DOI: 10.1161/CIRCULATIONAHA.112.126755
- Ferguson G.T., Enright P.L., Buist A.S., Higgins M.W. Office spirometry for lung health assessment in adults: a consensus statement from the National Lung Health Education Program. Chest. 2000; 117 (4): 1146–61. DOI: 10.1378/chest. 117.4.1146
- Kusunose K., Yamada H. Rest and exercise echocardiography for early detection of pulmonary hypertension. J. Echocardiogr. 2016; 14 (1): 2–12. DOI: 10.1007/s12574-015-0268-y
- Zhao Q.H., Wang L., Pudasaini B., Jiang R., Yuan P., Gong S.G. et al. Cardiopulmonary exercise testing improves diagnostic specificity in patients with echocardiography-suspected pulmonary hypertension. Clin. Cardiol. 2017; 40 (2): 95–101. DOI: 10.1002/clc.22635
- Farina S., Correale M., Bruno N., Paolillo S., Salvioni E., Badagliacca R. et al. “Right and Left Heart Failure Study Group” of the Italian Society of Cardiology. The role of cardiopulmonary exercise tests in pulmonary arterial hypertension. Eur. Respir. Rev. 2018; 27 (148): 170134. DOI: 10.1183/16000617.0134-2017
- Johnson J.T., Yetman A.T. Cardiopulmonary exercise testing in adults with congenital heart disease. Prog. Pediatr. Cardiol. 2012; 34: 47–52. DOI: 10.1016/j.ppedcard.2012.05.011
- Sun X.G., Hansen J.E., Oudiz R.J., Wasserman K. Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation. 2001; 104: 429–35.
- Ferrazza A.M., Martolini D., Valli G., Palange P. Cardiopulmonary exercise testing in the functional and prognostic evaluation of patients with pulmonary diseases. Respiration. 2009; 77 (1): 3–17. DOI: 10.1159/000186694
- asunobu Y., Oudiz R.J., Sun X.G., Hansen J.E., Wasserman K. End-tidal PCO2 abnormality and exercise limitation in patients with primary pulmonary hypertension. Chest. 2005; 127 (5): 1637–46. DOI: 10.1378/chest.127.5.1637
- Bockeria L.A., Glushko L.A. State of the cardiorespiratory system in patients with hypertrophic cardiomyopathy. J. Cardioresp. Res. 2020; 1 (Is. 1): 16–22. DOI: 10.26739/2181-0974- 2020-1-1
- Feltez G., Coronel C.C., Pellanda L.C., Lukrafka J.L. Exercise capacity in children and adolescents with corrected congenital heart disease. Pediatr. Cardiol. 2015; 36 (5): 1075–82. DOI: 10.1007/s00246-015-1129-1