Skip to main content

Advertisement

SpringerLink
Log in

Decreased Pulmonary Arterial Compliance is a Predictor for Poor Outcomes in Infants with Isolated Atrial Septal Defect and Pulmonary Hypertension

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

This study aimed to investigate risk factors for poor outcomes in infants with isolated atrial septal defect (ASD) and pulmonary hypertension who had unexpectedly fatal course. We retrospectively reviewed 22 infants with isolated ASD and pulmonary hypertension, and analyzed the relationship between clinical outcomes and pulmonary hemodynamic parameters including pulmonary arterial resistance (Rp) and compliance (Cp) based on cardiac catheterization among them. Age and weight at cardiac catheterization were 5 (1–11) months and 4.9 (3.1–9.2) kg, respectively. There were 17 individuals with Down syndrome. Pulmonary hemodynamic parameters were shown as follows: the ratio of pulmonary to systemic blood flow: 2.0 (0.6–3.8), mean pulmonary arterial pressure: 41 (20–60) mmHg, the ratio of pulmonary to systemic mean pressure (Pp/Ps): 0.67 (0.46–1.13), Rp: 4.11 (0.68–15.80) Wood units m2, and Cp: 1.80 (0.63–6.16) mL/mmHg m2. There were 4 deaths during the follow-up period of 40 (7–241) months. Univariable logistic regression analysis revealed that Pp/Ps (odds ratio [OR]: 18,500, 95% confidential interval [CI] 1.48–230,659,690, P = 0.041) and Cp (OR: 0.03, 95% CI 0.001–0.73, P = 0.031) were significantly related to death. A Pp/Ps cutoff value more than 0.94 and a Cp cutoff value less than 0.97 mL/mmHg m2 yielded as the predictors of death with sensitivity of 98% and 94%, specificity of 75% and 75%, respectively. Decreased Cp is a previously unrecognized predictor for poor outcome in infants with isolated ASD and pulmonary hypertension.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Availability of Data and Materials

All data will be offered if applicable.

Code Availability

Statistical analysis was performed using the Analysis TollPak In the Microsoft Office Excel™ add-in software.

References

  1. Geggel RL (2017) Clinical detection of hemodynamically significant isolated secundum atrial septal defect. J Pediatr 190:261–264.e1. https://doi.org/10.1016/j.jpeds.2017.07.037

    Article  PubMed  Google Scholar 

  2. Lammers A, Hager A, Eicken A, Lange R, Hauser M, Hess J (2005) Need for closure of secundum atrial septal defect in infancy. J Thoracic Cardiovasc Surg 129(6):1353–1357. https://doi.org/10.1016/j.jtcvs.2004.10.007

    Article  Google Scholar 

  3. Tanghöj G, Liuba P, Sjöberg G, Rydberg A, Naumburg E (2019) Adverse events within 1 year after surgical and percutaneous closure of atrial septal defects in preterm children. Cardiol Young 29(5):626–636. https://doi.org/10.1017/S1047951119000350

    Article  PubMed  Google Scholar 

  4. Charisopoulou D, Bini RM, Riley G, Janagarajan K, Moledina S, Marek J (2020) Repair of isolated atrial septal defect in infants less than 12 months improves symptoms of chronic lung disease or shunt-related pulmonary hypertension. Cardiol Young. https://doi.org/10.1017/S1047951120000463

    Article  PubMed  Google Scholar 

  5. Lee JS, Cha SG, Kim GB, Lee SY, Song MK, Kwon HW, Bae EJ, Kwak JG, Kim WH, Lee JR (2019) Reversibility of pulmonary hypertension following surgical atrial septal defect closure in children with down syndrome. J Cardiovasc Imag 27(4):247–253. https://doi.org/10.4250/jcvi.2019.27.e33

    Article  Google Scholar 

  6. Saji T (2014) Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome. Pediatr Int 56(3):297–303. https://doi.org/10.1111/ped.12349

    Article  CAS  PubMed  Google Scholar 

  7. 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. https://doi.org/10.1016/j.jpeds.2016.10.003

    Article  PubMed  Google Scholar 

  8. 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 Thoracic Cardiovasc Surg 152(2):507–513. https://doi.org/10.1016/j.jtcvs.2016.03.080

    Article  Google Scholar 

  9. Okada S, Muneuchi J, Nagatomo Y, Watanabe M, Iida C, Shirouzu H, Matsuoka R, Joo K (2017) Pulmonary arterial resistance and compliance in preterm infants. Int J Cardiol 244:265–270. https://doi.org/10.1016/j.ijcard.2017.06.056

    Article  PubMed  Google Scholar 

  10. Cheng XL, Liu ZH, Gu Q, Ni XH, Luo Q, Zhao ZH, He JG, Xiong CM (2017) Prognostic value of pulmonary artery compliance in patients with pulmonary arterial hypertension associated with adult congenital heart disease. Int Heart J 58(5):731–738. https://doi.org/10.1536/ihj.16-449

    Article  PubMed  Google Scholar 

  11. Tonelli AR, Alnuaimat H, Mubarak K (2010) Pulmonary vasodilator testing and use of calcium channel blockers in pulmonary arterial hypertension. Respir Med 104(4):481–496. https://doi.org/10.1016/j.rmed.2009.11.015

    Article  PubMed  Google Scholar 

  12. Goetschmann S, Dibernardo S, Steinmann H, Pavlovic M, Sekarski N, Pfammatter JP (2008) Frequency of severe pulmonary hypertension complicating “isolated” atrial septal defect in infancy. Am J Cardiol 102(3):340–342. https://doi.org/10.1016/j.amjcard.2008.03.061

    Article  PubMed  Google Scholar 

  13. Pellegrini P, Rossi A, Pasotti M, Raineri C, Cicoira M, Bonapace S, Dini FL, Temporelli PL, Vassanelli C, Vanderpool R, Naeije R, Ghio S (2014) Prognostic relevance of pulmonary arterial compliance in patients with chronic heart failure. Chest 145(5):1064–1070. https://doi.org/10.1378/chest.13-1510

    Article  PubMed  Google Scholar 

  14. 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(1):34–41. https://doi.org/10.1378/chest.10-1263

    Article  CAS  PubMed  Google Scholar 

  15. Iwaya Y, Muneuchi J, Inoue Y, Watanabe M, Okada S, Ochiai Y (2019) Relationship Between Pulmonary Arterial Resistance and Compliance in Patients with Down Syndrome. Pediatr Cardiol 40(4):841–847. https://doi.org/10.1007/s00246-019-02080-9

    Article  PubMed  Google Scholar 

  16. Schwarz MA, Caldwell L, Cafasso D, Zheng H (2009) Emerging pulmonary vasculature lacks fate specification. Am J Physiol Lung Cell Mol Physiol 296(1):L71–L81. https://doi.org/10.1152/ajplung.90452.2008

    Article  CAS  PubMed  Google Scholar 

  17. Parera MC, van Dooren M, van Kempen M, de Krijger R, Grosveld F, Tibboel D, Rottier R (2005) Distal angiogenesis: a new concept for lung vascular morphogenesis. Am J Physiol Lung Cell Mol Physiol 288(1):L141–L149. https://doi.org/10.1152/ajplung.00148.2004

    Article  CAS  PubMed  Google Scholar 

  18. Armulik A, Abramsson A, Betsholtz C (2005) Endothelial/pericyte interactions. Circul Res 97(6):512–523. https://doi.org/10.1161/01.RES.0000182903.16652.d7

    Article  CAS  Google Scholar 

  19. Peng T, Tian Y, Boogerd CJ, Lu MM, Kadzik RS, Stewart KM, Evans SM, Morrisey EE (2013) Coordination of heart and lung co-development by a multipotent cardiopulmonary progenitor. Nature 500(7464):589–592. https://doi.org/10.1038/nature12358

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  20. Lévy M, Maurey C, Dinh-Xuan AT, Vouhé P, Israël-Biet D (2005) Developmental expression of vasoactive and growth factors in human lung. Role in pulmonary vascular resistance adaptation at birth. Pediatr Res 57(5 Pt 2):21R–25R. https://doi.org/10.1203/01.PDR.0000159575.58834.8D

    Article  PubMed  Google Scholar 

  21. Muneuchi J, Ochiai Y, Masaki N, Okada S, Iida C, Sugitani Y, Ando Y, Watanabe M (2019) Pulmonary arterial compliance is a useful predictor of pulmonary vascular disease in congenital heart disease. Heart Vessels 34(3):470–476. https://doi.org/10.1007/s00380-018-1263-9

    Article  PubMed  Google Scholar 

Download references

Funding

This work received no specific grant from any funding agency.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, 1-8-1, Kishinoura, Yahatanishi-ku, Kitakyushu, Fukuoka, 806-8501, Japan

    Yuki Iwaya, Jun Muneuchi, Mamie Watanabe & Yuichiro Sugitani

  2. Department of Cardiovascular Surgery, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan

    Yoshie Ochiai

Authors
  1. Yuki Iwaya
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jun Muneuchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mamie Watanabe
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Yuichiro Sugitani
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Yoshie Ochiai
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

The authors’ contributions are as the follows; YI: writing original draft, investigation, J M: conceptualization, methodology, editing, MW: conceptualization, data curation, YS; data curation, YO; critical review.

Corresponding author

Correspondence to Jun Muneuchi.

Ethics declarations

Conflict of interest

All authors have no conflict of interest to declare.

Ethical Standards

This work does not involve any human/animal experimentation.

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., Watanabe, M. et al. Decreased Pulmonary Arterial Compliance is a Predictor for Poor Outcomes in Infants with Isolated Atrial Septal Defect and Pulmonary Hypertension. Pediatr Cardiol 41, 1408–1413 (2020). https://doi.org/10.1007/s00246-020-02400-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-020-02400-4

Keywords

Search

Navigation