Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block
Background: Isolated congenital complete heart block (CCHB) is rare cardiac condition associated with maternal Anti-Rho antibodies. It is managed with permanent pacemaker insertion. We sought to determine the long-term outcomes of individuals with CCHB who had undergone pacemaker insertion. Methods:...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-09-01
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| Series: | International Journal of Cardiology Congenital Heart Disease |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666668522000209 |
| _version_ | 1797999916251873280 |
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| author | Samuel Seitler Isma Rafiq Jonathan M Behar |
| author_facet | Samuel Seitler Isma Rafiq Jonathan M Behar |
| author_sort | Samuel Seitler |
| collection | DOAJ |
| description | Background: Isolated congenital complete heart block (CCHB) is rare cardiac condition associated with maternal Anti-Rho antibodies. It is managed with permanent pacemaker insertion. We sought to determine the long-term outcomes of individuals with CCHB who had undergone pacemaker insertion. Methods: A database search was performed at a UK tertiary cardiac referral centre. The online medical records of patients with CCHB were analysed for pacing requirements and complications, echocardiographic data, and clinical status. Results: A total of 72 patients (female n = 46) were identified, 20% of whom were autoantibody positive. Mean age of initial implantation was 15.5 years (SD 12.7) with mean follow up of 21 years (SD 8.3). 88% of patients were NYHA class I at censure of data. Major adverse cardiac events (MACE) were observed in 2 patients with 0% mortality throughout the follow up period. 16 patients (22.2%) developed cardiomyopathy, of which 15 had CRT devices inserted.Five patients had device related infections and 7 required system extractions. Mean left ventricular ejection fraction on most recent echocardiogram was 53.7% (SD 8.40) with no significant change compared with their historic scan. Mild tricuspid regurgitation was the most frequently observed valvular pathology, identified in 28% of patients. Only 2 patients (2.7%) had severe valve incompetence but neither required surgical intervention. Conclusion: Long-term outcomes for patients with congenital complete heart block who undergo pacemaker insertion are highly favourable. Despite high pacing requirements over an extended period, the incidence of MACE and pacing related complications is low. Cardiac function and valvular competence are largely preserved but dilated cardiomyopathy remains a late concern in a minority; ongoing clinical surveillance is paramount in this population. |
| first_indexed | 2024-04-11T11:12:11Z |
| format | Article |
| id | doaj.art-c16c626401fa4d35b016a16a47c2dc30 |
| institution | Directory Open Access Journal |
| issn | 2666-6685 |
| language | English |
| last_indexed | 2024-04-11T11:12:11Z |
| publishDate | 2022-09-01 |
| publisher | Elsevier |
| record_format | Article |
| series | International Journal of Cardiology Congenital Heart Disease |
| spelling | doaj.art-c16c626401fa4d35b016a16a47c2dc302022-12-22T04:27:26ZengElsevierInternational Journal of Cardiology Congenital Heart Disease2666-66852022-09-019100337Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart blockSamuel Seitler0Isma Rafiq1Jonathan M Behar2Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; Department of Imaging Sciences and Biomedical Engineering, Kings College London, United KingdomAdult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; Department of Imaging Sciences and Biomedical Engineering, Kings College London, United KingdomCorresponding author. Consultant Cardiologist & Electrophysiologist, Guy's and St Thomas' NHS Foundation Trust, United Kingdom.; Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; Department of Imaging Sciences and Biomedical Engineering, Kings College London, United KingdomBackground: Isolated congenital complete heart block (CCHB) is rare cardiac condition associated with maternal Anti-Rho antibodies. It is managed with permanent pacemaker insertion. We sought to determine the long-term outcomes of individuals with CCHB who had undergone pacemaker insertion. Methods: A database search was performed at a UK tertiary cardiac referral centre. The online medical records of patients with CCHB were analysed for pacing requirements and complications, echocardiographic data, and clinical status. Results: A total of 72 patients (female n = 46) were identified, 20% of whom were autoantibody positive. Mean age of initial implantation was 15.5 years (SD 12.7) with mean follow up of 21 years (SD 8.3). 88% of patients were NYHA class I at censure of data. Major adverse cardiac events (MACE) were observed in 2 patients with 0% mortality throughout the follow up period. 16 patients (22.2%) developed cardiomyopathy, of which 15 had CRT devices inserted.Five patients had device related infections and 7 required system extractions. Mean left ventricular ejection fraction on most recent echocardiogram was 53.7% (SD 8.40) with no significant change compared with their historic scan. Mild tricuspid regurgitation was the most frequently observed valvular pathology, identified in 28% of patients. Only 2 patients (2.7%) had severe valve incompetence but neither required surgical intervention. Conclusion: Long-term outcomes for patients with congenital complete heart block who undergo pacemaker insertion are highly favourable. Despite high pacing requirements over an extended period, the incidence of MACE and pacing related complications is low. Cardiac function and valvular competence are largely preserved but dilated cardiomyopathy remains a late concern in a minority; ongoing clinical surveillance is paramount in this population.http://www.sciencedirect.com/science/article/pii/S2666668522000209PacemakerCardiomyopathyLong-term outcomesCongenital Complete Heart Block |
| spellingShingle | Samuel Seitler Isma Rafiq Jonathan M Behar Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block International Journal of Cardiology Congenital Heart Disease Pacemaker Cardiomyopathy Long-term outcomes Congenital Complete Heart Block |
| title | Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block |
| title_full | Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block |
| title_fullStr | Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block |
| title_full_unstemmed | Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block |
| title_short | Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block |
| title_sort | long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block |
| topic | Pacemaker Cardiomyopathy Long-term outcomes Congenital Complete Heart Block |
| url | http://www.sciencedirect.com/science/article/pii/S2666668522000209 |
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