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Ebstein's anomaly. Clinical profile in 174 patients
F Attie1, J M Casanova, C Zabal
1Instituto Nacional de Cardiología Ignacio Chávez, INCICH, México, D.F.
Insights
Medical treatment offers good survival for early-stage Ebstein's anomaly. However, advanced clinical deterioration significantly increases mortality, necessitating timely surgical intervention before progression to severe stages.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiac Surgery
Background:
- Ebstein's anomaly is a congenital heart defect affecting the tricuspid valve.
- Long-term outcomes for non-operated patients with Ebstein's anomaly vary significantly based on clinical status.
Purpose of the Study:
- To evaluate the long-term survival rates and predictors of mortality in patients with Ebstein's anomaly who did not undergo surgical treatment.
- To establish optimal timing for surgical intervention based on clinical deterioration and risk stratification.
Main Methods:
- Retrospective analysis of 148 non-operated patients and 26 surgically treated patients with Ebstein's anomaly.
- Patients were categorized into three groups based on functional class and cardiothoracic index (CTR).
- Kaplan-Meier survival analysis and multivariate regression were used to identify mortality predictors.
Main Results:
- Overall survival rate was 81% in non-operated patients.
- Significant mortality differences were observed between clinical deterioration groups (p < 0.001 for I vs. III, p < 0.02 for II vs. III).
- Predictors of death included advanced functional class (III/IV), high CTR (>65%), cyanosis, and arrhythmias. Patients in deterioration group III had a 38.2% survival rate.
Conclusions:
- Medical management provides good long-term survival for patients in stable clinical condition (Group II).
- High mortality rates in advanced clinical deterioration (Group III) underscore the need for early surgical intervention before disease progression.
- Surgical indications should weigh institutional surgical risk against survival rates without surgery, prioritizing intervention before severe deterioration.
Abstract:
The study population consisted of 148 patients who did not undergo surgical treatment and 26 who were operated, most of them diagnosed after the age of 2, with a follow-up from 6 months to 25.3 years. Patients were divided in three groups of clinical deterioration according to their functional class and cardiothoracic index (CTR) long-term follow-up in 148 nonoperated patients showed significant differences for mortality between groups I and III (p < 0.001), and between groups II and III (p < 0.02). Predictors of death included the association among functional class III or IV CTR > or = 65% with either cyanosis or arrhythmias (p < 0.05). The multivariate analysis showed that clinical deterioration (p < 0.0001), CTR (p < 0.0002) and functional class (p < 0.001), were significant for mortality. Kaplan-Meier analysis showed a survival rate of 81% in the overall patients free from surgical treatment. According to Kaplan-Meier analysis, the rate of survival was lower in patients with CTR > or = 65% (63.5%), in patients who had functional class IV (52.5%) and in patients included in group III of clinical deterioration (38.2%). Despite the fact that the association of functional class III or IV plus CTR > or = 65% with either cyanosis or arrhythmias is a good predictor for death, the mortality in patients who had only one of these variables was lower. Patients included in group II of clinical deterioration in stable condition presented long survival with medical treatment. Due to the high mortality rate found in group III, surgical treatment of Ebstein's anomaly must be done before deteriorating into group III. Surgical indication must be done considering the surgical risk of each group according to the experience of the Institution and comparing the rate of surgical mortality with the rate of survival without surgery.