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Review article. Congenital complete heart block
Insights
Congenital complete heart block has varied causes, often linked to maternal connective tissue disease. Prognosis is challenging, with neonatal factors like low heart rate and prolonged QT time indicating poor outcomes.
Area of Science:
- Cardiology
- Pediatrics
- Genetics
Background:
- Isolated congenital complete heart block (CCHB) presents a heterogeneous etiology.
- Maternal connective tissue disease is identified in approximately one-third of CCHB cases.
- Mortality rates for CCHB vary significantly by age, being highest in neonates.
Purpose of the Study:
- To investigate the etiology and long-term outcomes of isolated congenital complete heart block.
- To identify prognostic indicators for patients with CCHB.
- To evaluate the need for and indications of pacemaker implantation in CCHB patients.
Main Methods:
- Retrospective analysis of patients with isolated congenital complete heart block.
- Review of maternal and patient medical histories, including signs of connective tissue disease.
- Assessment of mortality, pacemaker implantation rates, and clinical presentation across different age groups.
Main Results:
- Congenital complete heart block etiology is diverse, with maternal connective tissue disease being a significant factor.
- Mortality is highest in the neonatal period, decreasing in childhood and adolescence, then slowly increasing in adulthood.
- Pacemaker implantation rates were approximately 10% for patients under 15 and 25% for those over 15, with neonatal heart failure being a primary indication.
- Stokes-Adams attacks can occur at any age.
- Poor prognostic signs include a fixed or decreasing low neonatal ventricular rate and prolonged QT interval.
Conclusions:
- Congenital complete heart block requires careful prognostic evaluation due to its varied etiology and clinical course.
- Neonatal factors such as low ventricular rate and prolonged QT time are critical indicators of poor prognosis.
- Pacemaker implantation is a common intervention, particularly for heart failure in neonates and for managing arrhythmias in older patients.
Abstract:
Isolated congenital complete heart block has a heterogenous aetiology. In approximately one-third of the patients in the present material the mothers had symptoms or signs of connective tissue disease. The mortality is highest in the neonatal period, much lower during childhood and adolescence and increases slowly later in life. There are elderly patients who are completely asymptomatic but patients are also encountered with signs and symptoms of more or less severe myocardial damage. Stokes-Adams attacks may occur at any age. About 10% of patients below 15 years of age in this material and about 25% of those above 15 years (mean age 30 years) were paced. In the neonatal period the predominant indication for pacing was heart failure. It is difficult to make a prognosis in the individual patient. Apart from already known risk factors, a fixed or decreasing low ventricular rate neonatally and a prolonged QT time seem to be bad prognostic signs.