Skip to main content
Log in

Pulmonary vascular resistance and compliance in pulmonary blood flow alterations in children with congenital heart disease

  • Original Article
  • Published:
Heart and Vessels Aims and scope Submit manuscript

Abstract

There is a unique hyperbolic relationship between pulmonary vascular resistance (Rp) and compliance (Cp); however, the characteristics of this coupling curve in pulmonary blood flow alterations remains unknown in children with congenital heart disease. We aimed to explore the Rp–Cp coupling and resistant-compliance (RC) time among them. We retrospectively reviewed catheterization data and calculated Rp and Cp in 217 subjects with ventricular septal defect. Median age and weight at catheterization were 2.8 (1.7–4.4) months and 4.3 (3.7–5.3) kg, respectively. Pulmonary hemodynamic parameters were as follows: mean pulmonary arterial pressure: 36 (28–43) mmHg; the amount of pulmonary blood flow (Qp): 14.2 (11.6–17.6) L/min/m2; Rp: 1.95 (1.38–2.59) Wood unit m2; Cp: 2.98 (2.42–3.88) mmHg/mL/m2; and RC time: 0.35 (0.30–0.40) s. RC time remained unchanged according to alterations in Qp (P = 0.206); however, the relationship between logarithm transformations of Rp and Cp showed more steeper according to an increase in Qp. The pulmonary circulation depends upon Cp rather than Rp according to the degree of Qp despite the constancy in RC time. We should take both Rp and Cp into consideration when evaluating the pulmonary circulation among children with congenital heart disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M, ESC Scientific Document Group (2016) 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 37:67–119

    Article  Google Scholar 

  2. Assad TR, Brittain EL, Wells QS, Farber-Eger EH, Halliday SJ, Doss LN, Xu M, Wang L, Harrell FE, Yu C, Robbins IM, Newman JH, Hemnes AR (2016) Hemodynamic evidence of vascular remodeling in combined post- and precapillary pulmonary hypertension. Pulm Circ 6:313–321

    Article  Google Scholar 

  3. Saouti N, Westerhof N, Postmus PE, Vonk-Noordegraaf A (2010) The arterial load in pulmonary hypertension. Eur Respir Rev 19:197–203

    Article  CAS  Google Scholar 

  4. Tedford RJ (2014) Determinants of right ventricular afterload (2013 Grover Conference series). Pulm Circ 4:211–219

    Article  Google Scholar 

  5. Bonderman D, Martischnig AM, Vonbank K, Nikfardjam M, Meyer B, Heinz G, Klepetko W, Naeije R, Lang IM (2011) Right ventricular load at exercise is a cause of persistent exercise limitation in patients with normal resting pulmonary vascular resistance after pulmonary endarterectomy. Chest 139:122–127

    Article  Google Scholar 

  6. de Perrot M, McRae K, Shargall Y, Thenganatt J, Moric J, Mak S, Granton JT (2011) Early postoperative pulmonary vascular compliance predicts outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. Chest 140:34–41

    Article  Google Scholar 

  7. Lankhaar JW, Westerhof N, Faes TJ, Gan CT, Marques KM, Boonstra A, van den Berg FG, Postmus PE, Vonk-Noordegraaf A (2008) Pulmonary Vascular resistance and compliance stay inversely related during treatment of pulmonary hypertension. Eur Heart J 29:1688–1695

    Article  Google Scholar 

  8. Mahapatra S, Nishimura RA, Sorajja P, Cha S, McGoon MD (2006) Relationship of pulmonary arterial capacitance and mortality in idiopathic pulmonary arterial hypertension. J Am Coll Cardiol 47:799–803

    Article  Google Scholar 

  9. Takatsuki S, Nakayama T, Ikehara S, Matsuura H, Ivy DD, Saji T (2017) Pulmonary arterial capacitance index is a strong predictor for adverse outcome in children with idiopathic and heritable pulmonary arterial hypertension. J Pediatr 180:75-79.e2

    Article  Google Scholar 

  10. Dupont M, Mullens W, Skouri HN, Abrahams Z, Wu Y, Taylor DO, Starling RC, Tang WH (2012) Prognostic role of pulmonary arterial capacitance in advanced heart failure. Circ Heart Fail 5:778–785

    Article  Google Scholar 

  11. Reuben SR (1971) Compliance of the human pulmonary arterial system in disease. Circ Res 29:40–50

    Article  CAS  Google Scholar 

  12. Segers P, Verdonck P, Deryck Y, Brimioulle S, Naeije R, Carlier S, Stergiopulos N (1999) Pulse pressure method and the area method for the estimation of total arterial compliance in dogs: Sensitivity to wave reflection intensity. Ann Biomed Eng 27:480–485

    Article  CAS  Google Scholar 

  13. Chemla D, Lau EM, Papelier Y, Attal P, Hervé P (2015) Pulmonary vascular resistance and compliance relationship in pulmonary hypertension. Eur Respir J 46:1178–1189

    Article  CAS  Google Scholar 

  14. MacKenzie Ross RV, Toshner MR, Soon E, Naeije R, Pepke-Zaba J (2013) Decreased time constant of the pulmonary circulation in chronic thromboembolic pulmonary hypertension. Am J Physiol Heart Circ Physiol 305:H259–H264

    Article  Google Scholar 

  15. Tedford RJ, Hassoun PM, Mathai SC, Girgis RE, Russell SD, Thiemann DR, Cingolani OH, Borlaug BA, Redfield MM, Lederer DJ, Kass DA (2012) Pulmonary capillary wedge pressure augments right ventricular pulsatile loading. Circulation 125:289–297

    Article  Google Scholar 

  16. Muneuchi J, Nagatomo Y, Watanabe M, Joo K, Onzuka T, Ochiai Y, Joo K (2016) Relationship between pulmonary arterial resistance and compliance among patients with pulmonary arterial hypertension and congenital heart disease. J Thorac Cardiovasc Surg 152:507–513

    Article  Google Scholar 

  17. Ghio S, Schirinzi S, Pica S (2015) Pulmonary arterial compliance: How and why should we measure it? Glob Cardiol Sci Pract 2015:58

    Article  Google Scholar 

  18. Gan CT, Lankhaar JW, Westerhof N, Marcus JT, Becker A, Twisk JW, Boonstra A, Postmus PE, Vonk-Noordegraaf A (2007) Noninvasively assessed pulmonary artery stiffness predicts mortality in pulmonary arterial hypertension. Chest 132:1906–1912

    Article  Google Scholar 

  19. Sanz J, Kariisa M, Dellegrottaglie S, Prat-González S, Garcia MJ, Fuster V, Rajagopalan S (2009) Evaluation of pulmonary artery stiffness in pulmonary hypertension with cardiac magnetic resonance. JACC Cardiovasc Imaging 2:286–295

    Article  Google Scholar 

  20. Saouti N, Westerhof N, Helderman F, Marcus JT, Stergiopulos N, Westerhof BE, Boonstra A, Postmus PE, Vonk-Noordegraaf A (2009) RC time constant of single lung equals that of both lungs together: a study in chronic thromboembolic pulmonary hypertension. Am J Physiol Heart Circ Physiol 297:H2154–H2160

    Article  CAS  Google Scholar 

  21. Presson RG Jr, Baumgartner WA Jr, Peterson AJ, Glenny RW, Wagner WW Jr (2002) Pulmonary capillaries are recruited during pulsatile flow. J Appl Physiol (1985) 92:1183–1190

    Article  Google Scholar 

  22. Lankhaar JW, Westerhof N, Faes TJ, Marques KM, Marcus JT, Postmus PE, Vonk-Noordegraaf A (2006) Quantification of right ventricular afterload in patients with and without pulmonary hypertension. Am J Physiol Heart Circ Physiol 291:H1731–H1737

    Article  CAS  Google Scholar 

  23. Hadinnapola C, Pepke-Zaba J (2015) Developments in pulmonary arterial hypertension-targeted therapy for chronic thromboembolic pulmonary hypertension. Expert Rev Respir Med 9:559–569

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Jun Muneuchi.

Ethics declarations

Conflict of interest

All authors have no conflict of interest to declare. This research received no grant from any funding agency in the public, commercial or not-for-profit sectors.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Iwaya, Y., Muneuchi, J., Sugitani, Y. et al. Pulmonary vascular resistance and compliance in pulmonary blood flow alterations in children with congenital heart disease. Heart Vessels 37, 1283–1289 (2022). https://doi.org/10.1007/s00380-021-02009-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00380-021-02009-4

Keywords

Navigation