Elsevier

Cardiovascular Surgery

Volume 7, Issue 4, June 1999, Pages 436-442
Cardiovascular Surgery

Atrial isomerism: a surgical experience

https://doi.org/10.1016/S0967-2109(98)00151-3Get rights and content

Abstract

Most publications on atrial isomerism are autopsy or case reports. The authors review 41 consecutive children operated on from 1980 through to 1996 with emphasis on associated cardiac anomalies, surgical procedures and outcome. Left atrial isomerism was present in 23 patients. Interruption of the inferior vena cava (56%), atrio-ventricular septal defect (47%), common atrium (38%) and cor triatriatum sinistrum (30%) were the most common diagnoses. Biventricular repair was achieved in 17 children and total cavo-pulmonary connection in two. Three underwent staged palliation: modified Blalock–Taussig shunt for two and bidirectional Glenn anastomosis for one. The remainder received a cardiac pacemaker. One patient died early after repair. Two underwent reoperation to correct a regurgitant left atrio-ventricular valve: one of these, in another hospital, had peroperative death. Three died later. Actuarial survival rate after repair and total cavo-pulmonary connection that was stabilized after 2 years was 84%. In the 18 children with right atrial isomerism, pulmonary atresia or stenosis predominated (89%) with discordant ventriculo-arterial connection (72%), atrio-ventricular septal defect (72%), 'single' ventricle (55%) and extracardiac total anomalous pulmonary venous drainage (50%). Biventricular repair was achieved in two patients and complete Fontan circulation in eight. The other eight underwent various staged palliative procedures and correction of extracardiac total anomalous pulmonary venous drainage. Five patients died postoperatively: two in our unit after modified Blalock–Taussig shunt and total cavo-pulmonary connection, three in other hospitals after repair (n=1) and Fontan (n=2). Five died later. One was lost for review. Survival after repair and Fontan stabilized after 6 months at 49%. In conclusion, the cardiovascular malformations associated with left atrial isomerism can often be successfully corrected. Those accompanying right atrial isomerism usually preclude a biventricular repair, require staged palliation and carry a poor prognosis.

Section snippets

Methods

Surgery reports and protocols from January 1980 to December 1996 were reviewed for records of atrial isomerism. Diagnosis was based on the morphology of the atrial appendages for patients undergoing open-heart surgery, and on chest radiography and identification of lung and bronchial anatomy at thoracotomy for those undergoing extracardiac palliative procedures. The cases were then studied for associated vascular and cardiac anomalies, type of operation and postoperative results. The current

Patients and results

Forty-one children among 2696 open-heart surgery and 1358 thoracotomy cases (41/4054=1.0%) were described as having left atrial isomerism in 23 and right atrial isomerism in 18.

Discussion

In the large autopsy study from Children's Hospital of Pittsburgh [3], atrial isomerism was found in 3.1% (58/1842) of cases. Right atrial isomerism was more frequently seen (1.9%: 36/1842) than the left isomerism (1.2%: 22/1842). Atrial isomerism incidence was 1.5% (2/132) at transoesophageal echocardiography in patients prospectively evaluated for congenital cardiac lesions by Stümper et al. [8]. The lower incidence of atrial isomerism and the relative higher incidence of left atrial

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