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Echocardiographic Evaluation of Atrial Communications before Transcatheter Closure
Published on: February 8, 2022
Right atrial isomerism in children older than 3 years
Sun Yan1, Wang Jianpeng1, Quan Xin1
1Department of Echocardiography, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, No. 167, Bei Li Shi Road, Xicheng District, Beijing, 100037 China.
Insights
Children with right atrial isomerism (RAI) surviving without early surgery have complex heart defects. Survival after total cavopulmonary connection is good, but atrioventricular valve repair is frequent.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- High infant mortality associated with right atrial isomerism (RAI).
- Limited understanding of long-term outcomes for older children with RAI.
- Focus on evaluating outcomes in RAI patients surviving past 3 years without prior surgical intervention.
Purpose of the Study:
- To assess the clinical characteristics and outcomes of children with right atrial isomerism (RAI) who have survived to older childhood without early surgical intervention.
- To analyze the types of cardiac abnormalities present in this cohort.
- To evaluate the results of surgical interventions performed after 3 years of age.
Main Methods:
- Retrospective analysis of 33 consecutive patients with RAI, aged 3-32 years, who did not undergo surgical intervention before age 3.
- Detailed review of cardiac abnormalities, including position, valve anomalies, ventricular structure, outflow tract obstruction, great vessel anomalies, and venous drainage.
- Assessment of surgical interventions performed after age 3, including total cavopulmonary connection and atrioventricular valve repair.
Main Results:
- The cohort of 33 patients (mean age 6 years) presented with significant cardiac anomalies: 100% abnormal aorta origin, 97% pulmonary obstruction, 87% atrioventricular valve anomaly, 66% anomalous pulmonary venous drainage, 55% single ventricle, and 39% altered cardiac position.
- 61% of patients underwent surgical intervention after age 3; none had biventricular repair. 10 patients had total cavopulmonary connection, with 40% requiring concurrent atrioventricular valve repair, particularly those with common atrioventricular valve (CAVV).
- Post-operative mortality occurred in one patient after total cavopulmonary connection. Moderate to severe regurgitation was noted in 69% of CAVV patients and 27% of single atrioventricular valve patients.
Conclusions:
- Children with right atrial isomerism (RAI) surviving without early surgery exhibit complex congenital heart disease.
- Single-stage total cavopulmonary connection demonstrates favorable survival rates in this population.
- Atrioventricular valve repair is a frequent necessity in patients undergoing later surgical management for RAI.
Background:
There is a high mortality in infants with right atrial isomerism (RAI). However, less is known about outcome in older children with RAI. This study sought to evaluate those patients with RAI who survived older than 3 years of age without surgical intervention.
Results:
A total of 33 consecutive patients (20 males) were enrolled in the study, mean age 6 years (range 3-32). None of the patients had surgical intervention for the RAI before age 3. Cardiac abnormalities include altered cardiac position (39 %), atrioventricular valve anomaly (87 %), single or functional single ventricle (55 %), pulmonary/subpulmonary obstruction (97 %), abnormal origin of the aorta (100 %), bilateral superior vena cava (67 %), and anomalous pulmonary venous drainage (66 %). Surgical intervention was performed after 3 years of age in 20 patients (61 %). None of them planned or had biventricular repair performed. 10 patients underwent the total cavopulmonary connection procedure, including four (40 %) who had atrioventricular valve (AVV) repair at the same time [all with common atrioventricular valve (CAVV)]. One patient died the day after the operation. A total of 69 % of patients with a CAVV had moderate or severe regurgitation, while 27 % with a single atrioventricular valve had moderate or severe regurgitation.
Conclusion:
Patients with RAI who have survived to early childhood without surgical intervention have complex cardiac abnormalities. Survival after single stage total cavopulmonary connection is good but AVV repair is common.
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02:52Stereoisomerism
Isomers are different chemical species that have the same chemical formula.
Transition metal complexes often exist as geometric isomers, in which the same atoms are connected through the same types of bonds but with differences in their orientation in space. Coordination complexes with two different ligands in the cis and trans positions from a ligand of interest form isomers. For example, the octahedral [Co(NH3)4Cl2]+ ion has two isomers (Figure 1) In the cis...