Click on the image to see a larger version. Selected References These references are in PubMed. This may not be the complete list of references from this article. Isomerism of the cardiac atria in the asplenia syndrome.
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Abstract Objective: To compare the outcome of infants and children who have right atrial isomerism and normal pulmonary venous drainage with those who have anomalous drainage, and to determine factors associated with poor outcome. Design and patients: Retrospective review of management and outcome of infants and children determined to have right atrial isomerism between January and December Setting: Tertiary paediatric cardiac centre. Results: The patients presented at a median of one day range 1 day to 3.
The mean SEM survival estimates for those with normal pulmonary venous drainage at 1, 5, 10, and 15 years was 81 5. Independent risk factors for mortality included pulmonary venous obstruction relative risk 3. An analysis of only patients with normal pulmonary venous drainage identified no risk factors for mortality.
Conclusions: The long term outcome of infants and children with right atrial isomerism in association with a normal pulmonary venous drainage remains unfavourable.
Sepsis and sudden death that may potentially be related to cardiac arrhythmia are major causes of late mortality. Keywords: right atrial isomerism, pulmonary venous drainage, outcome The complex congenital cardiac malformations associated with right atrial isomerism are well documented.
In contrast to the uniformly poor prognosis of patients with right isomerism and total anomalous pulmonary venous drainage, the outcome of those with normal, non-obstructed pulmonary venous drainage has not been defined. Previous reports documented the outcome in a relatively small number of patients who underwent palliative surgery, the majority or all of whom had total anomalous pulmonary venous drainage.
We compared the outcome of those with normal pulmonary venous drainage with those with anomalous drainage and determined factors that are associated with a poor outcome. Diagnosis The diagnostic criteria of right atrial isomerism remain controversial. The bronchial pattern and position of the liver on chest and abdominal radiography, respectively, may suggest the diagnosis. The intracardiac anatomy was delineated by two dimensional echocardiography. For this study, a normal pulmonary venous connection refers to connection of all pulmonary veins to the left sided atrium, although it is recognised that the right sided atrium is the pulmonary venous atrium in some cases of dextrocardia.
The diagnosis of pulmonary venous obstruction was based on clinical presentation, chest roentgenographic appearance, two dimensional echocardiographic imaging, and Doppler assessment of pulmonary venous flow velocities. Data collection The following data were retrieved from the clinical records and entered as covariates in a Cox regression model to determine risk factors for mortality: 1 demographic data including age and year at presentation and sex; 2 morphological variables including location of cardiac apex, number of atrioventricular valves, atrioventricular valvar regurgitation, number of ventricles, morphology of the main ventricular chamber, aortic outflow obstruction, pulmonary outflow obstruction, pulmonary arterial anatomy, type of pulmonary venous drainage, and presence of pulmonary venous obstruction; 3 haemodynamic data including oxygen saturation at presentation, and use of intravenous prostaglandin infusion; 4 electrocardiographic variables including P wave axis, P wave morphology, heart block, and history of arrhythmia.
Other data that were retrieved were the type of surgical intervention offered and the outcome; for patients who died, the age at death and cause of death; and for survivors, their further management and follow up duration. Statistical analysis Results are expressed as mean SEM , unless otherwise specified. The survival of patients was analysed by Kaplan-Meier actuarial survival analysis and patient groups were compared by log rank test.
The independent effects of demographic, morphological, haemodynamic, and electrocardiographic variables on survival were analysed by Cox regression model.
All statistical analyses were performed using SPSS version 7. The median age at presentation was 1 day range 1 day to 3. Seventy six per cent of patients presented within their first week of life. Surgery was not planned in 31 patients, all of whom died.
The median follow up duration of the remaining 85 patients who either had or are awaiting surgery was 5. Three patients were lost to follow up. It was supracardiac in 28, cardiac in 18, infradiaphragmatic in 11, and partial in 2 patients; one patient had complete atresia of pulmonary veins. Of these, 26 had cardiac lesions considered too complex for surgery and five had parents who refused surgery; they all died soon after birth. Surgical interventions to be performed at an older age were planned in 11 patients who had pulmonary stenosis with a balanced haemodynamic status at initial presentation.
Of these, three were lost to follow up; one died of viral pneumonia while awaiting intervention; two were subsequently managed conservatively, one because of pulmonary hypertension and one unfavourable pulmonary arterial anatomy; and five were awaiting interventions.
Heterotaxy Syndrome (Isomerism)
Acute symptoms can be due to both cardiac and non-cardiac defects. Cyanosis or blue skin coloration, primarily affecting the lips and fingernails, can indicate a systemic or circulatory issue. Poor feeding, failure to thrive , and rapid shallow breathing may also be observed due to poor circulation. Upon examination, arrhythmia and heart murmur may raise further suspicion of a cardiac abnormality. Non-cardiac symptoms include impairments of the liver and gastrointestinal tract.
Asplenia with cardiovascular anomalies
Contact Us Online What is heterotaxy syndrome isomerism? Heterotaxy syndrome is a rare birth defect that involves the heart and other organs. All usually involve heart defects of varying types and severity. In addition, organs such as the stomach, intestines, liver and lungs may be in abnormal places in the chest and abdomen. The intestines may have malrotation, which is when the loops of bowel are lined up incorrectly.