Skip to main content
Log in

A Case of Cor Triatriatum With an Abnormal P Wave: The Pacemaker Action From the Specialized Tissue in the Abnormal Septum

  • Case Report
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

A boy presented with an abnormal P wave shown on an electrocardiogram (ECG) checkup at school. An echocardiogram and contrast-enhanced computed tomography (CT) showed cor triatriatum with a slit-like opening between the accessory chamber and the left atrium located along the interatrial septum. The boy underwent open heart surgery for excision of the anomalous membrane, and a postoperative ECG showed normal P waves. The excised tissue was examined immnunohistopathologically using antihyperpolarization-activated cyclic nucleotide-gated potassium channel 4 (HCN4) antibody and other staining. The authors confirmed the existence of cells positive to HCN4, indicating that they were sinoatrial node cells or at least cells with electrical automaticity.

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
Fig. 4

References

  1. Biel M, Schneider A, Wahl C (2002) Cardiac HCN channels: structure, function, and modulation. Trends Cardiovasc Med 12:206–212

    Article  PubMed  CAS  Google Scholar 

  2. Chen SA, Hsieh MH, Tai CT et al (1999) Initiation of atrial fibrillation by ectopic beats originating from the pulmonary veins: electrophysiological characteristics, pharmacological responses, and effects of radiofrequency ablation. Circulation 18:1879–1886

    Google Scholar 

  3. Geva T, Van Praagh S (2008) Anomalies of the pulmonary veins. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF (eds) Moss and Adams’ heart disease in infants Children and adolescents. Wolters Kluwer/Lippincott Williams & Wilkins, Philadelphia, pp 761–792

    Google Scholar 

  4. Gharagozloo F, Bulkley BH, Hutchins GM (1977) A proposed pathogenesis of cor triatriatum. Am Heart J 94:618–626

    Article  PubMed  CAS  Google Scholar 

  5. Gilljam T, Freedom RM, Yoo SJ (2004) The syndrome of isomeric left atrial appendages, often associated with polysplenia. In: Freedom RM, Yoo SJ, Mikailian HJ, Williams WG (eds) The natural and modified history of congenital heart disease. Blackwell, Oxford, pp 430–434

    Google Scholar 

  6. Haissaguerre M, Jais P, Shah DC et al (1998) Spontaneous initiation of atrial fibrillation by ectopic beats originating in the pulmonary veins. N Engl J Med 10:659–666

    Article  Google Scholar 

  7. Momma K, Takao A, Shibata T (1990) Characteristics and natural history of abnormal atrial rhythms in left isomerism. Am J Cardiol 65:231–236

    Article  PubMed  CAS  Google Scholar 

  8. Moosmang S, Stieber J, Zong X et al (2001) Cellular expression and functional characterization of four hyperpolarization-activated channels in cardiac and neuronal tissues. Eur J Biochem 268:1646–1652

    Article  PubMed  CAS  Google Scholar 

  9. Niwayama G (1960) Cor triatriatum. Am Heart J 59:291–317

    Article  PubMed  CAS  Google Scholar 

  10. Shi W, Wymore R, Yu H et al (2002) Distribution and prevalence of hyperpolarization-activated cation channels (HCN) mRNA expression in cardiac tissues. Circ Res 85:e1–e6

    Google Scholar 

  11. Stieber J, Herrmann S, Feil S et al (2003) The hyperpolarization-activated channel HCN4 is required for the generation of pacemaker action potentials in the embryonic heart. Proc Natl Acad Sci USA 215:15235–15240

    Article  Google Scholar 

  12. Uemura M, Nishibatake N, Nakazawa M et al (1988) Clinical aspect of cor triatriatum. Acta Paediatr Japonica 92:1294–1300

    Google Scholar 

Download references

Acknowledgment

The authors acknowledge Drs. Shigehiro Morishima, M.D. and Takashi Ono, M.D. for the operation and Dr Hideo Sakuma, M.D. for the pathologic analysis. This work was supported by an intra-institutional fund.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Yoshimichi Kudo.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Movie clip 1 2DE two-dimensional echocardiogram showing the abnormal membrane. (MOV 516 kb)

Movie clip 2 Color Doppler echocardiogram showing the flow from the accessory chamber to the left atrium (LA). (MOV 512 kb)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kudo, Y., Kaneko, M., Nakazawa, M. et al. A Case of Cor Triatriatum With an Abnormal P Wave: The Pacemaker Action From the Specialized Tissue in the Abnormal Septum. Pediatr Cardiol 32, 1244–1248 (2011). https://doi.org/10.1007/s00246-011-0078-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-011-0078-6

Keywords

Navigation