ArticleHeart (British Cardiac Society)1996
Management and outcome of infants and children with right atrial isomerism.
Article in Heart (British Cardiac Society), 1996. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed.
- Improved Surgical Management of Complex Neonates With Heterotaxy Syndrome.World journal for pediatric & congenital heart surgery · 2026Article
- Incidental Finding of Heterotaxy Syndrome in a Patient With Pulmonary Embolism: A Case Report and Concise Review.Cureus · 2022Article
- Heterotaxy syndrome with complex congenital heart disease, facial palsy, and asplenia: A rare newborn finding.Clinical case reports · 2021Article
- The heterotaxy syndrome: associated congenital heart defects and management.Indian journal of thoracic and cardiovascular surgery · 2021Review
- Complex congenital cardiac anomalies in the setting of right isomerism in a 31-month-old infant: a case report.Journal of medical case reports · 2018Article
- Cardiothoracic Area Ratio Predicts Lethal Pulmonary Venous Obstruction in Patients with Single Ventricle and Total Anomalous Pulmonary Venous Connection.AJP reports · 2018Article
- Right atrial isomerism in children older than 3 years.SpringerPlus · 2016Article
- The outcome of patients with right atrial isomerism is poor.Pediatric cardiology · 2013Article
- Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome.Pediatric cardiology · 2008Article
- Total Anomalous Pulmonary Vein Connection: Diagnosis, Management, and Outcome.Current treatment options in cardiovascular medicine · 2004Article
- Outcome of infants with right atrial isomerism: is prognosis better with normal pulmonary venous drainage?Heart (British Cardiac Society) · 2002Article
- Pulmonary venous obstruction requiring lobectomy for hemoptysis after a Glenn operation.The Japanese journal of thoracic and cardiovascular surgery : official publication of the Japanese Association for Thoracic Surgery = Nihon Kyobu Geka Gakkai zasshi · 2001Article
- Modified Fontan operation in patients with anomalies of systemic and pulmonary venous connection.Pediatric cardiologyArticle
- Right atrial isomerism in four siblings.Pediatric cardiologyArticle
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Authors and funding
7 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease.
settingTertiary referral centre.
methods20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures.
resultsPatients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6.8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) had pulmonary atresia. There were no early deaths. One patient died after a second palliative procedure. There was one late sudden death. Four patients had a Fontan operation with no deaths. Two of the remaining three patients meet the Fontan criteria. The long-term survival rate in this group was 78% (seven of nine).
conclusionsSurgical management of patients with right atrial isomerism who have complex congenital heart disease carries a high mortality and remains palliative. The overall survival rate was 45% (nine of 20); 18% in patients requiring surgery in the first month of life (group A) and 78% in patients requiring surgery after the first month of life (group B) (P < 0.001). Of the total of 20 patients, nine were potential candidates for a Fontan operation. Seven of these have undergone a Fontan procedure with five survivors.
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