Abstract
Objective. To study the parametres of hemodynamics and heart failure in children with a single ventricle to assess the long-term results of Fontan surgery.
Material and methods. Long-term results of Fontan surgery performed from 2009 to 2020 in Federal Center for Cardiovascular Surgery of the Ministry of Health of Russia (Penza) were evaluated. Thirty-seven children aged from 3.1 to 8.7 years (mean age 5.5 ± 1.23 years) were examined. All patients underwent perioperative examination with ECG, D-echoCG, cardiac catheterization (with evaluation of pulmonary artery pressure), angiopulmonography and heart MSCT, transcutaneous saturation (SatpO2) was determined. When studying hemodynamic parameters in the long-term period there were assessed data of D-echoCG, daily ECG monitoring by Holter, 6 minute walking test and blood tests for pro-BNP. New York classification (NYHA) was used to determine the functional class of chronic heart failure (CHF).
Results. In the period of 8.5 years (Me 3.2; 25–75% CI 1.7–4.5) 37 children were examined. There was no long-term postoperative mortality. Additional interventions – closure of collateral vessels – were performed in 3 (7.9%) cases. The freedom from additional interventions was 89.5%. There was a statistically significant decrease in indexed CVD by 14.6 ml/m2 (95% CI 5.3 to 23.8; t = 3.254; p = 0.003), indexed CSR by 4.7 ml/m2 (95% CI 0.7 to 8.7; t =2.433; p = 0.023). None of the study subjects had severe circulatory insufficiency: 66.7% of the children were in NYHA functional class I and 33.3% were in NYHA functional class II. Statistically significant differences in the level of pro-BNP biomarker were obtained for the I and II classes according to NYHA. Single-factor logistic regression demonstrated an association of elevated pro-BNP levels (above the threshold value of 125 pg/ml) for the diagnosis of CHF class with an odds ratio of 22.00 (95% CI 1.857–260.648; p = 0.014), with a sensitivity of 85.7% and specificity of 78.6%.
Conclusions. The current strategy for managing patients after Fontan surgery requires a focus on determinants of long-term outcomes, full comprehensive monitoring of hemodynamic parameters, and determination of biochemical marker levels.
References
- Podzolkov V.P., Chiaureli M.R., Zelenikin M.M., Jurlov I.A., Kovalev D.V., Putjato N.A., et al. Fontan operation: correction or palliative? Russian Journal of Thoracic and Cardiovascular Surgery. 2013; 5: 19–23 (in Russ.).
- Atz A.M., Zak V., Mahony L., Uzark K., Shrader P., Gallagher D. et al. Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort. Congenit. Heart Dis. 2015; 10 (1): E30–42. DOI: 10.1111/chd.12193. Epub 2014 Jun 17. PMID: 24934522; PMCID: PMC4414014.
- Van der Ven J.P.G., van den Bosch E., Bogers A.J.C.C., Helbing W.A. State of the art of the Fontan strategy for treatment of univentricular heart disease. F1000Res. 2018; 7: F1000 Faculty Rev-935. DOI: 10.12688/f1000research. 13792.1.eCollection 2018.
- Bockeria L.A., Gudkova R.G. Cardiovascular Surgery – 2015. Diseases and congenital anomalies of the circulatory system. Moscow; 2016 (in Russ.).
- Poh C.L., d'Udekem Y. Life after surviving Fontan surgery: a meta-analysis of the incidence and predictors of late death. Heart Lung Circ. 2018; 27 (5): 552–9. DOI: 10.1016/j.hlc.2017.11.007
- Gewillig M., Goldberg D.J. Failure of the fontan circulation. Heart Fail. Clin. 2014; 10 (1): 105–16. DOI: 10.1016/j.hfc.2013.09.010
- Ohuchi H. Where is the "optimal" Fontan hemodynamics? Korean. Circ. J. 2017; 47 (6): 842–57. DOI: 10.4070/kcj.2017.0105
- Anderson P.A., Sleeper L.A., Mahony L. Colan S.D., Atz A.M., Breitbart R.E. еt al. Pediatric Heart Network Investigators. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J. Am. Coll. Cardiol. 2008; 52 (2): 85–98. DOI: 10.1016/j.jacc.2008.01.074. PMID: 18598886; PMCID: PMC4385517.
- Chernogrivov A.E., Chernogrivov I.E., Sulejmanov Sh.F., Rybakova T.V., Vostokova E.V., Gorelova A.O. et al. Comparative results of bidirectional cavopulmonary anastomosis operation. Russian Bulletin of Perinatology and Pediatrics. 2016; 61 (3): 169 (in Russ.).
- Gessler P., Knirsch W., Schmitt B., Rousson V., von Eckardstein A. Prognostic value of plasma N-terminal probrain natriuretic peptide in children with congenital heart defects and open-heart surgery. J. Pediatr. 2006; 148 (3): 372–6. DOI: 10.1016/j.jpeds.2005.10.039
- Gupta R.K., Zheng H., Cui Y., Qu J., Li L., Liang H. et al. Change in N-terminal pro B-type natriuretic peptide levels and clinical outcomes in children undergoing congenital heart surgery. Int. J. Cardiol. 2019; 283: 96–100. DOI: 10.1016/j.ijcard.2019.02.025. Epub 2019 Feb 14. PMID: 30833104.
- Tupikina A.A., Plotnikova I.V., Kovalev I.A., Svincova L.I., Janulevich O.S., Krivoshhekov E.V. Heart failure in children with a functional single ventricle. Level of biochemical markers after Fontan operation with extracardiac conduit. Siberian Medical Journal. 2016; 31 (2): 66–71 (in Russ.) DOI: 10.29001/2073-8552-2016-31-2-66-71
- Bockeria L.A., Kim A.I., Movsesyan R.R., Boltabaev I.I., Rogova T.V., Zaharchenko A.G. et al. Results of bidirectional cavopulmonary anastomosis operation without cardiopulmonary bypass in the treatment of complex congenital heart defects in children of the first year of life. Bulletin of the Russian Academy of Medical Sciences. 2009; 12: 36–9 (in Russ.).
- Khairy P., Fernandes S.M., Mayer J.E. Jr, Triedman J.K., Walsh E.P., Lock J.E. et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008; 117 (1): 85–92. DOI: 10.1161/CIRCULATIONAHA.107.738559. Epub 2007 Dec 10. PMID: 18071068.
- d'Udekem Y., Iyengar A.J., Galati J.C., Forsdick V., Weintraub R.G., Wheaton G.R. et al. Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation. 2014; 130 (11 Suppl 1): S32–8. DOI: 10.1161/CIRCULATIONAHA.113.007764. PMID: 25200053.
- Atz A.M., Zak V., Mahony L., Uzark K., D'agincourt N., Goldberg D.J. et al. Pediatric Heart Network Investigators. Longitudinal outcomes of patients with single ventricle after the Fontan procedure. J. Am. Coll. Cardiol. 2017; 69 (22): 2735–44. DOI: 10.1016/j.jacc.2017.03.582. PMID: 28571639; PMCID: PMC5604334.
- Rathod R.H., Prakash A., Kim Y.Y., Germanakis I.E., Powell A.J., Gauvreau K. et al. Cardiac magnetic resonance parameters predict transplantation-free survival in patients with Fontan circulation. Circ. Cardiovasc. Imaging. 2014; 7 (3): 502–9. DOI: 10.1161/CIRCIMAGING.113.001473. Epub 2014 Mar
- Erratum in: Circ. Cardiovasc. Imaging. 2018; 11 (11): e000021. Abstract corrected. PMID: 24619103; PMCID: PMC4262249.
- Ghelani S.J., Harrild D.M., Gauvreau K., Geva T., Rathod R.H. Comparison between echocardiography and cardiac magnetic resonance imaging in predicting transplant-free survival after the Fontan operation. Am. J. Cardiol. 2015; 116 (7): 1132–8. DOI: 10.1016/j.amjcard.2015.07.011
- Margossian R., Schwartz M.L., Prakash A., Wruck L., Colan S.D., Atz A.M. et al. Pediatric Heart Network Investigators. Comparison of echocardiographic and cardiac magnetic resonance imaging measurements of functional single ventricular volumes, mass, and ejection fraction (from the Pediatric Heart Network Fontan Cross-Sectional Study). Am. J. Cardiol. 2009; 104 (3): 419–28. DOI: 10.1016/j.amjcard.2009.03.058. Epub 2009 Jun 6. PMID: 19616678; PMCID: PMC2741330.
- Lytrivi I.D., Sfyridis P.G., Papagiannis J., Kirvassilis G., Zavaropoulos P., Sarris G.E. Impact of age at Fontan compleChildren’s Heart and Vascular Diseases. 2022; 19 (2) DOI: 10.24022/1810-0686-2022-19-2-108-116 Original articles 116 tion on functional status at mid-term follow up. Hellenic. J. Cardiol. 2011; 52 (2): 118–22. PMID: 21478121.
- Robert J. Dabal, James K. Kirklin, Manisha Kukreja, Robert N. Brown, David C. Cleveland, Michael C. Eddins et al. The modern Fontan operation shows no increase in mortality out to 20 years. J. Thorac. Cardiovasc. Surg. 2014; 148 (6): 2517–24. DOI: 10.1016/j.jtcvs.2014.07.075
- Koch A., Zink S., Singer H. B-type natriuretic peptide in paediatric patients with congenital heart disease. Eur. Heart J. 2006; 27 (7): 861–6. DOI: 10.1093/eurheartj/ehi773
- Саперова Е.В, Вахлова И.В. Клиническое значение натрийуретических пептидов. Патология кровообращения и кардиохирургия. 2017; 21 (1): 117–27. DOI: 10.21688/1681-3472-2017-1-117-127 Saperova E.V., Vakhlova I.V. A clinical importance of natriuretic peptides in pediatric practice. Patologiya Krovoobrashcheniya i Kardiokhirurgiya. 2017; 21 (1): 117–127 (in Russ.). DOI: 10.21688/1681-3472-2017-1-117-127.
- Lowenthal A., Camacho B.V., Lowenthal S., Natal-Hernandez L., Liszewski W., Hills N.K. еt al. Usefulness of B-type natriuretic peptide and N-terminal pro-B-type natriuretic peptide as biomarkers for heart failure in young children with single ventricle congenital heart disease. Am. J. Cardiol. 2012; 109 (6): 866–72. DOI: 10.1016/j.amjcard.2011.10.049. Epub 2011 Dec 22. PMID: 22196786; PMCID: PMC3294194.
- Alsaied T., Bokma J.P., Engel M.E., Kuijpers J.M., Hanke S.P., Zuhlke L. et al. Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies. Congenit. Heart Dis. 2017; 12 (4): 393–8. DOI: 10.1111/chd.12468. Epub 2017 May 8. PMID: 28480627.
About the authors
- Vladlen V. Bazylev, Dr. Med. Sci., Professor, Chief Physician; ORCID
- Aleksey A. Shikhranov, Cand. Med. Sci., Head of the Department; ORCID
- Lyudmila N. Lashmanova, Pediatric Cardiologist; ORCID
- Igor E. Chernogrivov, Dr. Med. Sci., Cardiovascular Surgeon; ORCID
- Tatiana V. Rybakova, Pediatric Cardiologist; ORCID
- Anastasia O. Ryabova, Pediatric Cardiologist; ORCID
- Irina S. Nazarova, Pediatric Cardiologist; ORCID
- Ekaterina V. Vostokova, Pediatric Cardiologist; ORCID
- Ivan S. Makaganchuk, Cardiovascular Surgeon; ORCID
