Pediatric Cardiology

, Volume 39, Issue 4, pp 749–756 | Cite as

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

  • Eiji Morihana
  • Kenichiro Yamamura
  • Yuichiro Sugitani
  • Hideaki Kado
  • Yasushi Takahata
  • Toshihide Nakano
  • Yasutaka Nakashima
  • Naoki Fusazaki
  • Shouichi Ohga
Original Article

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.

Keywords

Congenital heart disease Hypoplastic left heart syndrome Fatal arrhythmia Prolonged PR interval 

Notes

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.

Compliance with Ethical Standards

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.

References

  1. 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 37:419–425CrossRefPubMedGoogle Scholar
  2. 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 64:1795–1802CrossRefPubMedGoogle Scholar
  3. 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 107:1121–1127PubMedGoogle Scholar
  4. 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 102:136–141CrossRefGoogle Scholar
  5. 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 144:896–906CrossRefPubMedPubMedCentralGoogle Scholar
  6. 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 139:359–365CrossRefPubMedGoogle Scholar
  7. 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 22:82–89CrossRefPubMedGoogle Scholar
  8. 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–e49CrossRefPubMedGoogle Scholar
  9. 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 e91–93Google Scholar
  10. 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 34:1181–1189CrossRefPubMedGoogle Scholar
  11. 11.
    Mahle WT, Spray TL, Gaynor JW, Clark BJ 3rd (2001) Unexpected death after reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg 71:61–65CrossRefPubMedGoogle Scholar
  12. 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 34:554–560CrossRefPubMedGoogle Scholar
  13. 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 147:897–901CrossRefPubMedGoogle Scholar
  14. 14.
    Simsic JM, Bradley SM, Stroud MR, Atz AM (2005) Risk factors for interstage death after the Norwood procedure. Pediatr Cardiol 26:400–403CrossRefPubMedGoogle Scholar
  15. 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 109:2326–2330CrossRefPubMedGoogle Scholar
  16. 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 161:138–144CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Monaco MA, Liberman L, Starc TJ, Silver ES (2015) Defining the electrocardiogram in the neonate with hypoplastic left heart syndrome. Pediatr Cardiol 36:1014–1018CrossRefPubMedGoogle Scholar
  18. 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 100:591–598CrossRefPubMedGoogle Scholar
  19. 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 20:350–358CrossRefPubMedGoogle Scholar
  20. 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 129:2013–2020CrossRefPubMedPubMedCentralGoogle Scholar
  21. 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 152:189–194CrossRefPubMedGoogle Scholar
  22. 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 115:103–110CrossRefPubMedGoogle Scholar
  23. 23.
    Siffel C, Riehle-Colarusso T, Oster ME, Correa A (2015) Survival of children with hypoplastic left heart syndrome. Pediatrics 136:e864–e870CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Greenleaf CE, Urencio JM, Salazar JD, Dodge-Khatami A (2016) Hypoplastic left heart syndrome: current perspectives. Transl Pediatr 5:142–147CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Eiji Morihana
    • 1
    • 2
  • Kenichiro Yamamura
    • 1
  • Yuichiro Sugitani
    • 1
    • 2
  • Hideaki Kado
    • 3
  • Yasushi Takahata
    • 4
  • Toshihide Nakano
    • 3
  • Yasutaka Nakashima
    • 1
  • Naoki Fusazaki
    • 2
  • Shouichi Ohga
    • 1
  1. 1.Department of Pediatrics, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  2. 2.Division of Neonatal CardiologyFukuoka Children’s HospitalFukuokaJapan
  3. 3.Division of Cardiac SurgeryFukuoka Children’s HospitalFukuokaJapan
  4. 4.Division of NeonatologyFukuoka Children’s HospitalFukuokaJapan

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