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Systemic-to-Pulmonary Collateral Flow Correlates with Clinical Condition Late After the Fontan Procedure

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Abstract

In the Fontan circulation, there is a substantial degree of systemic-to-pulmonary collateral flow (SPCF), which can be measured by cardiac magnetic resonance (CMR). However, the correlation between the degree of SPCF and long-term outcomes is not fully understood. We retrospectively studied 321 patients who underwent the Fontan procedure and CMR at a single center. Using CMR, we calculated SPCF as pulmonary blood flow − systemic blood flow. %SPCF was defined as SPCF ÷ pulmonary blood flow. The mean age of patients at CMR was 14.3 ± 7.5 years. The average %SPCF was 13.0% ± 11.0%. With a multivariate analysis, %SPCF was significantly correlated with time (i.e., the longer the time period since the Fontan procedure, the lower the %SPCF) (p = 0.006), previous total anomalous pulmonary vein drainage (p = 0.007), a low pulmonary artery index (Nakata index) before the Fontan procedure (p = 0.04), and older age at the time of the Fontan procedure (p = 0.002). Regarding the findings after the Fontan procedure, %SPCF was significantly correlated with ventricular end-diastolic volume (p < 0.001), ventricular end-systolic volume (p < 0.001), central venous pressure (p < 0.001), plasma brain natriuretic peptide concentration (p < 0.001), hemoptysis (p = 0.009), and poor New York Heart Association functional class (p = 0.007). SPCF was correlated with clinical condition after the Fontan procedure. The importance of sufficient growth of the pulmonary vascular bed should be emphasized because the development of SPCF is believed to result from the poor condition of the pulmonary circulation.

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References

  1. Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248

    Article  CAS  Google Scholar 

  2. Marcelletti C, Corno A, Giannico S, Marino B (1990) Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg 100:228–232

    Article  CAS  Google Scholar 

  3. Grosse-Wortmann L, Al-Otay A, Yoo SJ (2009) Aortopulmonary collaterals after bidirectional cavopulmonary connection or Fontan completion quantification with MRI. Circ Cardiovasc Imaging 2:219–225

    Article  Google Scholar 

  4. Whitehead KK, Sundareswaran KS, Parks WJ, Harris MA, Yoganathan AP, Fogel MA (2009) Blood flow distribution in a large series of patients having the Fontan operation: a cardiac magnetic resonance velocity mapping study. J Thorac Cardiovasc Surg 138:96–102

    Article  Google Scholar 

  5. Ait Ali L, Cadoni A, Rossi G, Keilberg P, Passino C, Festa P (2017) Effective cardiac index and systemic-pulmonary collaterals evaluated by cardiac magnetic resonance late after fontan palliation. Am J Cardiol 119:2069–2072

    Article  Google Scholar 

  6. Latus H, Gummel K, Diederichs T, Bauer A, Rupp S, Kerst G, Jux C, Akintuerk H, Schranz D, Apitz C (2013) Aortopulmonary collateral flow is related to pulmonary artery size and affects ventricular dimensions in patients after the Fontan procedure. PLoS ONE 8:e81684

    Article  Google Scholar 

  7. Whitehead KK, Harris MA, Glatz AC, Gillespie MJ, DiMaria MV, Harrison NE, Dori Y, Keller MS, Rome JJ, Fogel MA (2015) Status of systemic to pulmonary arterial collateral flow after the fontan procedure. Am J Cardiol 115:1739–1745

    Article  Google Scholar 

  8. Prakash A, Rathod RH, Powell AJ, McElhinney DB, Banka P, Geva T (2012) Relation of systemic-to-pulmonary artery collateral flow in single ventricle physiology to palliative stage and clinical status. Am J Cardiol 109:1038–1045

    Article  Google Scholar 

  9. Glatz AC, Rome JJ, Small AJ, Gillespie MJ, Dori Y, Harris MA, Keller MS, Fogel MA, Whitehead KK (2012) Systemic-to-pulmonary collateral flow, as measured by cardiac magnetic resonance imaging, is associated with acute post-Fontan clinical outcomes. Circ Cardiovasc Imaging 5:218–225

    Article  Google Scholar 

  10. Odenwald T, Quail MA, Giardini A, Khambadkone S, Hughes M, Tann O, Hsia TY, Muthurangu V, Taylor AM (2012) Systemic to pulmonary collateral blood flow influences early outcomes following the total cavopulmonary connection. Heart 98:934–940

    Article  Google Scholar 

  11. Grosse-Wortmann L, Drolet C, Dragulescu A, Kotani Y, Chaturvedi R, Lee KJ, Mertens L, Taylor K, La Rotta G, Van Arsdell G, Redington A, Yoo SJ (2012) Aortopulmonary collateral flow volume affects early postoperative outcome after Fontan completion: a multimodality study. J Thorac Cardiovasc Surg 144:1329–1336

    Article  Google Scholar 

  12. Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG (2009) Research electronic data capture (REDCap)-a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 42:377–381

    Article  Google Scholar 

  13. Nakata S, Imai Y, Takanashi Y, Kurosawa H, Tezuka K, Nakazawa M, Ando M, Takao A (1984) A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 88:610–619

    Article  CAS  Google Scholar 

  14. Grosse-Wortmann L, Hamilton R, Yoo S-J (2007) Massive systemic-to-pulmonary collateral arteries in the setting of a cavopulmonary shunt and pulmonary venous stenosis. Cardiol Young 17:548–550

    Article  Google Scholar 

  15. Whitehead KK, Gillespie MJ, Harris MA, Fogel MA, Rome JJ (2009) Noninvasive quantification of systemic-to-pulmonary collateral flow: a major source of inefficiency in patients with superior cavopulmonary connections. Circ Cardiovasc Imaging 2:405–411

    Article  Google Scholar 

  16. Ishikawa Y, Ishikawa S, Sagawa K, Ushinohama H, Nakamura M, Kado H (2016) Favorable effects of oxygen inhalation in patients after bidirectional glenn procedure as assessed by cardiovascular magnetic resonance flow measurement. Circ J 80:1378–1385

    Article  Google Scholar 

  17. Charan NB, Carvalho P (1997) Angiogenesis in bronchial circulatory system after unilateral pulmonary artery obstruction. J Appl Physiol 82:284–291

    Article  CAS  Google Scholar 

  18. Mitzner W, Lee W, Georgakopolous D, Wagner E (2000) Angiogenesis in the mouse lung. Am J Pathol 157:93–101

    Article  CAS  Google Scholar 

  19. Gewillig M, Brown SC (2016) The Fontan circulation after 45 years: update in physiology. Heart 102:1081–1086

    Article  Google Scholar 

  20. Bédard E, Lopez S, Perron J, Houde C, Couture C, Vaillancourt R, Côté JM, Delisle G, LeBlanc MH, Chetaille P, Lamarre A, Rodés-Cabau J (2008) Life-threatening hemoptysis following the Fontan procedure. Can J Cardiol 24:145–147

    Article  Google Scholar 

  21. Averin K, Byrnes JW, Benscoter DT, Whiteside W, Desena H, Hirsch R, Goldstein BH (2018) Life-threatening airway bleeding after palliation of single ventricle congenital heart disease. Heart 104:254–260

    Article  Google Scholar 

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Acknowledgments

We thank Emily Woodhouse, PhD, from Edanz Group (https://en-author-services.edanzgroup.com/ac) for editing a draft of this manuscript.

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Authors and Affiliations

  1. Department of Pediatric Cardiology, Fukuoka Children’s Hospital, 5-1-1 Kashiiteriha, Fukuoka, Japan

    Yoshihiko Kodama, Yuichi Ishikawa, Ayako Kuraoka, Makoto Nakamura & Koichi Sagawa

  2. Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, Fukuoka, Japan

    Shinichiro Oda, Toshihide Nakano & Hideaki Kado

  3. Department of Cardiovascular Medicine, Kyushu University Hospital, Fukuoka, Japan

    Yoshihiko Kodama, Ichiro Sakamoto, Kisho Ohtani, Tomomi Ide & Hiroyuki Tsutsui

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  1. Yoshihiko Kodama
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  2. Yuichi Ishikawa
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  3. Ayako Kuraoka
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  4. Makoto Nakamura
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Correspondence to Yoshihiko Kodama.

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This retrospective study was approved by the institutional review boards of Fukuoka Children’s Hospital and Kyushu University Hospital.

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Kodama, Y., Ishikawa, Y., Kuraoka, A. et al. Systemic-to-Pulmonary Collateral Flow Correlates with Clinical Condition Late After the Fontan Procedure. Pediatr Cardiol 41, 1800–1806 (2020). https://doi.org/10.1007/s00246-020-02450-8

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  • DOI: https://doi.org/10.1007/s00246-020-02450-8

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