Resting respiratory lung volumes are “healthier” than exercise respiratory volumes in different types of palliated or corrected congenital heart disease
Corresponding Author
Marianna Fabi MD, PhD
Department of Cardiac, Thoracic and Vascular Sciences, Paediatric Cardiology and Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Correspondence Marianna Fabi, Department of Cardiac, Thoracic and Vascular Sciences, Paediatric Cardiology and Adult Congenital Unit, University of Bologna, S. Orsola-Malpighi Hospital, Via Massarenti 9, 40138 Bologna, Italy.
Email: [email protected]
Search for more papers by this authorAnna Balducci MD, PhD
Department of Cardiac, Thoracic and Vascular Sciences, Paediatric Cardiology and Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorSalvatore Cazzato MD, PhD
Department of Mother and Child Health, Azienda Ospedaliero Universitaria Ospedali Riuniti di Ancona Umberto I G M Lancisi G Salesi, Ancona, Marche, Italy
Search for more papers by this authorArianna Aceti MD, PhD
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorMarcella Gallucci MD
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorEmanuela Di Palmo MD
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorGaetano Gargiulo MD
Department of Pediatric and Adult Congenital Heart Cardiac Surgery, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorAndrea Donti MD
Department of Cardiac, Thoracic and Vascular Sciences, Paediatric Cardiology and Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorMarcello Lanari MD, PhD
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorCorresponding Author
Marianna Fabi MD, PhD
Department of Cardiac, Thoracic and Vascular Sciences, Paediatric Cardiology and Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Correspondence Marianna Fabi, Department of Cardiac, Thoracic and Vascular Sciences, Paediatric Cardiology and Adult Congenital Unit, University of Bologna, S. Orsola-Malpighi Hospital, Via Massarenti 9, 40138 Bologna, Italy.
Email: [email protected]
Search for more papers by this authorAnna Balducci MD, PhD
Department of Cardiac, Thoracic and Vascular Sciences, Paediatric Cardiology and Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorSalvatore Cazzato MD, PhD
Department of Mother and Child Health, Azienda Ospedaliero Universitaria Ospedali Riuniti di Ancona Umberto I G M Lancisi G Salesi, Ancona, Marche, Italy
Search for more papers by this authorArianna Aceti MD, PhD
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorMarcella Gallucci MD
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorEmanuela Di Palmo MD
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorGaetano Gargiulo MD
Department of Pediatric and Adult Congenital Heart Cardiac Surgery, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorAndrea Donti MD
Department of Cardiac, Thoracic and Vascular Sciences, Paediatric Cardiology and Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorMarcello Lanari MD, PhD
Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Search for more papers by this authorAbstract
Aims
Cardiac surgery has improved life expectancy of patients with congenital heart diseases (CHDs). Exercise capacity is an important determinant of survival in patients with CHDs. There is a lack of studies focusing on the role of resting respiratory performance in reducing exercise tolerance in these patients.
Objectives
To determine the prevalence and severity of respiratory functional impairment in different types of corrected/palliated CHDs, and its impact on an exercise test.
Materials and Methods
Retrospective single-center study involving 168 corrected/palliated patients with CHD and 52 controls. Patients CHD were divided into subgroups according to the presence of native pulmonary blood flow or total cavopulmonary connection (TCPC). All subjects performed complete pulmonary function tests and gas diffusion; patients with CHD also performed cardiopulmonary exercise test (CPX).
Results
Mean values of lung volumes were within the normal range in all CHD groups. Comparing to controls, patients with the reduced pulmonary flow and with TCPC had the highest reduction in lung volumes. CPX was reduced in all groups, most severely in TCPC, and it was correlated to decreased dynamic volumes in all CHD groups except in TCPC. Younger age at intervention and number of surgical operations negatively affected lung volumes.
Conclusions
Respiratory function is within the normal range in our patients with different CHDs at rest but altered in all CHDs during exercise when cardiorespiratory balance is likely to be inadequate. Comparing the different groups, patients with reduced pulmonary flow and TCPC are the most impaired.
CONFLICT OF INTERESTS
The authors declare that there are no conflict of interests.
REFERENCES
- 1Baumgartner H, Bonhoeffer P, De Groot NMS, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010; 31(23): 2915-2957.
- 2Nieminen HP, Jokinen EV, Sairanen HI. Causes of late deaths after pediatric cardiac surgery: a population-based study. J Am Coll Cardiol. 2007; 25(13): 1263-1271.
- 3Rabinovitch M, Keane JF, Norwood WI, Castaneda AR, Reid L. Vascular structure in lung tissue obtained at biopsy correlated with pulmonary hemodynamic findings after repair of congenital heart defects. Circulation. 1984; 69(4): 655-667.
- 4Borowski A, Reinhardt H, Schickendantz S, Korb H. Pulmonary artery growth after systemic-to-pulmonary shunt in children with a univentricular heart and a hypoplastic pulmonary artery bed. Implications for Fontan surgery. Jpn Hear J. 1998; 39(5): 671-680.
- 5Larsson ES, Eriksson BO, Sixt R. Decreased lung function and exercise capacity in Fontan patients. A long-term follow-up. Scand Cardiovasc J. 2003; 37(1): 58-63.
- 6Laohachai K, Winlaw D, Selvadurai H, et al. Inspiratory muscle training is associated with improved inspiratory muscle strength, resting cardiac output, and the ventilatory efficiency of exercise in patients with a Fontan circulation. J Am Heart Assoc. 2017; 6(8): 1-11. https://doi.org/10.1161/JAHA.117.005750
- 7Wu FM, Opotowsky AR, Denhoff ER, et al. A pilot study of inspiratory muscle training to improve exercise capacity in patients with Fontan physiology. Semin Thorac Cardiovasc Surg. 2018; 29: 462-469.
- 8Diller G-P, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005; 9(6): 828-835.
- 9Inuzuka R, Diller G-P, Borgia F, et al. Comprehensive use of cardiopulmonary exercise testing identifies adults with congenital heart disease at increased mortality risk in the medium term. Circulation. 2012; 125(2): 250-259.
- 10Callegari A, Neidenbach R, Milanesi O, et al. A restrictive ventilator pattern is common in patients with univentrcular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life. Congenit Heart Dis. 2018; 14: 1-9. https://doi.org/10.1111/chd.12694
- 11Opotowsky AR. Abnormal spirometry in congenital heart disease: where do we go from here? Circulation. 2013; 26(8): 865-867.
- 12Hsia CC. Cardiopulmonary limitations to exercise in restrictive lung disease. Med Sci Sport Exerc. 1999; 31: S28-S32.
- 13Scharf SM, Bianco JA, Tow DE, Brown R. The effect of large negative intrathoracic pressure on left ventricular function in patients with coronary artery disease. Circulation. 1981; 63: 871-875.
- 14Lang RM, Badano LP, Mor-Avi V, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 2015; 16(3): 233-70. https://doi.org/10.1093/ehjci/jev014 Review. Erratum in: Eur Heart J Cardiovasc Imaging. 2016 Apr;17(4):412.Eur Heart J Cardiovasc Imaging. 2016 Sep;17 (9):969.
- 15Brusasco V, Crapo R, Viegi G. Coming together: the ATS/ERS consensus on clinical pulmonary function testing. Eur Respir J. 2005; 26(1): 1-2.
- 16Pellegrino R. Interpretative strategies for lung function tests. Eur Respir J. 2005; 26(5): 948-968.
- 17 American Thoracic Society; American College of Chest Physician. ATS/ACCP Statement on cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2003; 167(2): 211-277.
- 18Wasserman K, Hansen J, Sue D, et al. Principles of Exercise Testing and Interpretation: Including Pathophysiology and Clinical Applications. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 2012.
- 19Sulc J, Samánek M, Zapletal A, Vorísková M, Hucín B, Skovránek J. Lung function in VSD patients after corrective heart surgery. Pediatr Cardiol. 1996; 17(1): 1-6.
- 20Sulc J, Andrle V, Hruda J, Hucín B, Samánek M, Zapletal A. Pulmonary function in children with atrial septal defect before and after heart surgery. Heart. 1998; 80(5): 484-488.
- 21Hruda J, Sulc J, Radvanský J, Hucín B, Samánek M. Good exercise tolerance and impaired lung function after atrial repair of transposition. Eur J Cardiothorac Surg. 1997; 12(2): 184-189.
- 22Matthews IL, Fredriksen PM, Bjørnstad PG, Thaulow E, Gronn M. Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity. Cardiol Young. 2006; 16(3): 261-267.
- 23Ginde MD, Bartz PJ, Hill GD, et al. Restrictive lung disease is an independent predictor of exercise intolerance in the adult with congenital heart disease. Congenit Heart Dis. 2013; 8: 246-254.
- 24Alonso-Gonzalez R, Borgia F, Diller G-P, et al. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013; 127(8): 882-890.
- 25Lubica H. Pathologic lung function in children and adolescents with congenital heart defects. Pediatr Cardiol. 1996; 17(5): 314-315.
- 26Samánek M, Sulc J, Zapletal A. Lung function in simple complete transposition after intracardiac repair. Int J Cardiol. 1989; 24(1): 13-17.
- 27Jonsson H, Ivert T, Jonasson R, Wahlgren H, Holmgren A, Björk VO. Pulmonary function thirteen to twenty-six years after repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 1994; 108(6): 1002-1009.
- 28Hawkins SMM, Taylor AL, Sillau SH, Mitchell MB, Rausch CM. Restrictive lung function in pediatric patients with structural congenital heart disease. J Thorac Cardiovasc Surg. 2014; 20(1): 207-211.
- 29Gilljam T, Sixt R. Lung function in relation to haemodynamic status after atrial redirection for transposition of the great arteries. Eur Heart J. 1995; 16(12): 1952-1959.
- 30Zapletal A, Samanek M, Hruda J, Hucin B. Lung function in children and adolescents with tetralogy of Fallot after intracardiac repair. Pediatr Pulmonol. 1993; 16(1): 23-30.
- 31Zhang X, Ruan Y, Liu Y, Yu C. Quantitative structural study of pulmonary artery in patients with pulmonary atresia with ventricular septal defect. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Acad Med Sin. 2004; 26(3): 241-246.
- 32Gaultier C, Boule M, Thibert M, Leca F. Resting lung function in children after repair of tetralogy of Fallot. Chest. 1986; 89(4): 561-567.
- 33Zhang X-T, Liu Y-L, Ruan Y-M, Yu C-T. Relationship between the quantitative structural study of lung and the right ventricle outflow tract reconstruction in infants with tetralogy of Fallot. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Acad Med Sin. 2006; 28(3): 402-405.
- 34Zhang X-T, Liu Y-L, Ruan Y-M, Yu C-T, Liu L. Lung pathology of complex congenital heart defect with diminished pulmonary blood flow but without aortopulmonary collateral artery and patent ductus arteriosus in infants and young children. Zhongguo Dang Dai Er Ke Za Zhi. 2008; 10(3): 311-314.
- 35Hislop A, Reid L. Structural changes in the pulmonary arteries and veins in tetralogy of Fallot. Br Heart J. 1973; 35(11): 1178-1183.
- 36Rabinovitch M, Herrera-deLeon V, Castaneda AR, Reid L. Growth and development of the pulmonary vascular bed in patients with tetralogy of Fallot with or without pulmonary atresia. Circulation. 1981; 64(6): 1234-1249.
- 37Johnson RJ, Haworth SG. Pulmonary vascular and alveolar development in tetralogy of Fallot: a recommendation for early correction. Thorax. 1982; 37(12): 893-901.
- 38Ohuchi H, Ohashi H, Takasugi H, Yamada O, Yagihara T, Echigo S. Restrictive ventilatory impairment and arterial oxygenation characterize rest and exercise ventilation in patients after Fontan operation. Pediatr Cardiol. 2004; 25(5): 513-521.
- 39Ovroutski S, Ewert P, Miera O, et al. Long-term cardiopulmonary exercise capacity after modified Fontan operation. Eur J Cardiothorac Surg. 2010; 37(1): 204-209.
- 40Starnes SL, Duncan BW, Kneebone JM, et al. Angiogenic proteins in the lungs of children after cavopulmonary anastomosis. J Thorac Cardiovasc Surg. 2001; 122(3): 518-523.
- 41Xu Y-Q, Liu Y-L, Lü X-D, Ruan Y-M, Yu C-T. Further development of pulmonary artery after Glenn procedure: effect of different antegrade pulmonary blood flows on cyanotic congenital heart defects. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Acad Med Sin. 2008; 30(6): 717-722.
- 42Greutmann M, Le TL, Tobler D, et al. Generalised muscle weakness in young adults with congenital heart disease. Heart. 2011; 97(14): 1164-1168.
- 43Fredriksen PM, Veldtman G, Hechter S, et al. Aerobic capacity in adults with various congenital heart diseases. Am J Cardiol. 2001; 87(3): 310-314.
- 44Fredriksen PM, Therrien J, Veldtman G, et al. Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart. 2001; 85(3): 295-299.
- 45Kempny A, Mouldings K, Uebing 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(11): 1386-1396.
- 46Nanas S, Nanas J, Papazachou O, et al. Resting lung function and hemodynamic parameters as predictors of exercise capacity in patients with chronic heart failure. Chest. 2003; 123(5): 1386-1393.
- 47Diller GP, Giardini A, Dimopoulos K, et al. Predictors of mortality in contemporary Fontan patients: results from a multicentre study including cardiopulmonary testing in 321 patients. Eur Heart J. 2010; 31: 3073-3083. https://doi.org/10.1093/eurheart/ehq356
- 48Claessen G, La Gerche A, Van De Bruaene A, et al. Heart rate reserve in Fontan patients: chronotropic incompetence or hemodynamic limitation? J Am Heart Assoc. 2019; 8:e012008. https://doi.org/10.1161/JAHA.119.012008
Citing Literature
