Skip to main content
Log in

Prolonged PR Interval at Birth Predicting the High Occurrence of Fatal Atrioventricular Block in Hypoplastic Left Heart Syndrome

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

Abstract

Infants with hypoplastic left heart syndrome (HLHS) are at high mortality especially when they are associated with bradyarrhythmias. However, the risk factor of developing high-grade atrioventricular block (HAVB) is still unclear. Seventy-three patients with HLHS in our institutions from 2002 to 2011 were enrolled. The survival rate was assessed by the anatomical types, treatments, occurrence of HAVB, severe tricuspid regurgitation (TR), and restrictive atrial septal defect (ASD) along with electrocardiogram findings at birth. There were 23 (32%) cardiogenic and 7 (10%) non-cardiogenic deaths. The occurrence rate of HAVB but not severe TR or restrictive ASD was higher in 30 deceased patients than in 43 survived patients [7 (23%) vs. 1 (2.3%), p = 0.0038]. The overall mortality rate was higher in patients with HAVB than in those without it (p = 0.0002). Of 7 deceased patients with HAVB, 6 HAVB occurred within 10 days post-surgery, and 3 HAVB led to the early death. The mortality rate of patients with prolonged PR (≥ 0.15 s) but not wide QRS (> 0.08 s) or prolonged QTc (> 0.43 s) at birth was higher than each without it (p = 0.0106). Multivariate analysis indicated that prolonged PR but no other variables was independently associated with the mortality (hazard ratio: 2.948, p = 0.0104). Prolonged PR at birth in HLHS infants predicts the development of fatal HAVB.

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. Kane JM, Canar J, Kalinowski V, Johnson TJ, Hoehn KS (2016) Management options and outcomes for neonatal hypoplastic left heart syndrome in the early twenty-first century. Pediatr Cardiol 37:419–425

    Article  PubMed  Google Scholar 

  2. Bartram U, Grunenfelder J, Van Praagh R (1997) Causes of death after the modified Norwood procedure: a study of 122 postmortem cases. Ann Thorac Surg 64:1795–1802

    Article  CAS  PubMed  Google Scholar 

  3. Jonas RA, Hansen DD, Cook N, Wessel D (1994) Anatomic subtype and survival after reconstructive operation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 107:1121–1127

    CAS  PubMed  Google Scholar 

  4. Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark BJ 3rd (2000) Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 102:136–141

    Article  Google Scholar 

  5. Ghanayem NS, Allen KR, Tabbutt S, Atz AM, Clabby ML, Cooper DS, Eghtesady P, Frommelt PC, Gruber PJ, Hill KD, Kaltman JR, Laussen PC, Lewis AB, Lurito KJ, Minich LL, Ohye RG, Schonbeck JV, Schwartz SM, Singh RK, Goldberg CS, Pediatric Heart Network Investigators (2012) Interstage mortality after the Norwood procedure: results of the multicenter Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg 144:896–906

    Article  PubMed  PubMed Central  Google Scholar 

  6. Furck AK, Uebing A, Hansen JH, Scheewe J, Jung O, Fischer G, Rickers C, Holland-Letz T, Kramer HH (2010) Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: a 12-year single-center survey. J Thorac Cardiovasc Surg 139:359–365

    Article  PubMed  Google Scholar 

  7. Gaynor JW, Mahle WT, Cohen MI, Ittenbach RF, DeCampli WM, Steven JM, Nicolson SC, Spray TL (2002) Risk factors for mortality after the Norwood procedure. Eur J Cardiothorac Surg 22:82–89

    Article  PubMed  Google Scholar 

  8. Hansen JH, Uebing A, Furck AK, Scheewe J, Jung O, Fischer G, Kramer HH (2011) Risk factors for adverse outcome after superior cavopulmonary anastomosis for hypoplastic left heart syndrome. Eur J Cardiothorac Surg 40:e43–e49

    Article  PubMed  Google Scholar 

  9. Hehir DA, Dominguez TE, Ballweg JA, Ravishankar C, Marino BS, Bird GL, Nicolson SC, Spray TL, Gaynor JW, Tabbutt S (2008) Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants. J Thorac Cardiovasc Surg 136:94–99, 99 e91–93

  10. Hoque T, Richmond M, Vincent JA, Bacha E, Torres A (2013) Current outcomes of hypoplastic left heart syndrome with restrictive atrial septum: a single-center experience. Pediatr Cardiol 34:1181–1189

    Article  PubMed  Google Scholar 

  11. Mahle WT, Spray TL, Gaynor JW, Clark BJ 3rd (2001) Unexpected death after reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg 71:61–65

    Article  CAS  PubMed  Google Scholar 

  12. Rychik J, Rome JJ, Collins MH, DeCampli WM, Spray TL (1999) The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol 34:554–560

    Article  CAS  PubMed  Google Scholar 

  13. Shamszad P, Gospin TA, Hong BJ, McKenzie ED, Petit CJ (2014) Impact of preoperative risk factors on outcomes after Norwood palliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 147:897–901

    Article  PubMed  Google Scholar 

  14. Simsic JM, Bradley SM, Stroud MR, Atz AM (2005) Risk factors for interstage death after the Norwood procedure. Pediatr Cardiol 26:400–403

    Article  CAS  PubMed  Google Scholar 

  15. Vlahos AP, Lock JE, McElhinney DB, van der Velde ME (2004) Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy. Circulation 109:2326–2330

    Article  PubMed  Google Scholar 

  16. Trivedi B, Smith PB, Barker PC, Jaggers J, Lodge AJ, Kanter RJ (2011) Arrhythmias in patients with hypoplastic left heart syndrome. Am Heart J 161:138–144

    Article  PubMed  PubMed Central  Google Scholar 

  17. Monaco MA, Liberman L, Starc TJ, Silver ES (2015) Defining the electrocardiogram in the neonate with hypoplastic left heart syndrome. Pediatr Cardiol 36:1014–1018

    Article  PubMed  Google Scholar 

  18. Alsoufi B, Mori M, Gillespie S, Schlosser B, Slesnick T, Kogon B, Kim D, Sachdeva R, Kanter K (2015) Impact of patient characteristics and anatomy on results of Norwood operation for hypoplastic left heart syndrome. Ann Thorac Surg 100:591–598

    Article  PubMed  Google Scholar 

  19. Baffa JM, Chen SL, Guttenberg ME, Norwood WI, Weinberg PM (1992) Coronary artery abnormalities and right ventricular histology in hypoplastic left heart syndrome. J Am Coll Cardiol 20:350–358

    Article  CAS  PubMed  Google Scholar 

  20. Newburger JW, Sleeper LA, Frommelt PC, Pearson GD, Mahle WT, Chen S, Dunbar-Masterson C, Mital S, Williams IA, Ghanayem NS, Goldberg CS, Jacobs JP, Krawczeski CD, Lewis AB, Pasquali SK, Pizarro C, Gruber PJ, Atz AM, Khaikin S, Gaynor JW, Ohye RG, Pediatric Heart Network Investigators (2014) Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial. Circulation 129:2013–2020

    Article  PubMed  PubMed Central  Google Scholar 

  21. Mah DY, Cheng H, Alexander ME, Sleeper L, Newburger JW, Del Nido PJ, Thiagarajan RR, Rajagopal SK (2016) Heart block following stage 1 palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 152:189–194

    Article  PubMed  Google Scholar 

  22. Donnelly JP, Raffel DM, Shulkin BL, Corbett JR, Bove EL, Mosca RS, Kulik TJ (1998) Resting coronary flow and coronary flow reserve in human infants after repair or palliation of congenital heart defects as measured by positron emission tomography. J Thorac Cardiovasc Surg 115:103–110

    Article  CAS  PubMed  Google Scholar 

  23. Siffel C, Riehle-Colarusso T, Oster ME, Correa A (2015) Survival of children with hypoplastic left heart syndrome. Pediatrics 136:e864–e870

    Article  PubMed  PubMed Central  Google Scholar 

  24. Greenleaf CE, Urencio JM, Salazar JD, Dodge-Khatami A (2016) Hypoplastic left heart syndrome: current perspectives. Transl Pediatr 5:142–147

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

We thank Junji Kishimoto (Department of Research and Development of Next Generation Medicine, Faculty of Medical Sciences, Kyushu University) for the contribution of statistical analysis. We show gratitude to Ms. Miho Masunaga and Ms. Ayumi Uchida (Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University) for their helpful support in this study.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kenichiro Yamamura.

Ethics declarations

Conflict of interest

All authors have no conflict of interest to report.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed Consent

Not obtained due to retrospective study design.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Morihana, E., Yamamura, K., Sugitani, Y. et al. Prolonged PR Interval at Birth Predicting the High Occurrence of Fatal Atrioventricular Block in Hypoplastic Left Heart Syndrome. Pediatr Cardiol 39, 749–756 (2018). https://doi.org/10.1007/s00246-018-1815-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-018-1815-x

Keywords

Navigation