Outcome of Left Atrial Isomerism at a Single Institution

Abstract

Left atrial isomerism includes a complex spectrum of cardiac and extracardiac anomalies. The records of all patients with left isomerism born during the period of 1973-2010 and treated at the Children's Hospital, Helsinki were reviewed. The short- and long-term outcomes were studied. The review included 38 patients (50% females). The overall survival with left atrial isomerism was 63% during a median follow-up time of 16 years (range, 4-30 years). Extracardiac anomalies were noted in 14 (37%) of 38 cases. Cardiac defects included dextrocardia in 26%, partially or totally anomalous pulmonary venous return in 29%, common atrium in 50%, atrioventriculoseptal defect in 73%, single ventricle in 40%, ventriculoseptal defect without atrioventricular defect in 11%, transposition in 21%, double outlet of the right ventricle in 26%, pulmonary stenosis or atresia in 61%, and left ventricular outflow obstruction in 24% of the cases. Cardiac arrhythmias were presented in 71% and pacemaker treatment in 29% of the cases. Of the 38 patients, 33 had cardiac surgery. Simple palliative methods were used in 11 cases, single-ventricle palliation in 12 cases, and operation with a biventricular track in 10 cases. In the groups that had surgery, 3 of 11 patients, 3 of 12 patients, and 3 of 10 patients died, respectively. In this review, 14 deaths occurred, associated with extracardiac anomalies in five cases and with cardiac arrhythmia in four cases. Five postoperative deaths occurred. At this writing, all three patients who had heart transplantation are alive. Complicated heart defects associated with severe arrhythmias and extracardiac anomalies contribute to a high mortality rate with left isomerism. Cardiac transplantation was considered a good option for selected patients.

Perinatal and Infant Outcomes of Prenatal Diagnosis of Heterotaxy Syndrome (Asplenia and Polysplenia)

“…In the PSP group, the survival with a biventricular circulation was significantly higher than previously reported. 3,10 Although the survival for both ASP and PSP of continued pregnancies in our study was slightly better than previously reported (44% vs 12% to 21% for ASP 12,15 and 79% vs 39% to 61% in PSP 1,12,13,15 ), the prognosis for patients with HS is still dismal, especially for those with ASP. The improvement in our survival rate can be explained by a most recent era analyzed and in the case of PSP by the inclusion of a high proportion of normal hearts.…”

“…Our results support the previously published data that pulmonary vein stenosis and noncardiac anomalies strongly affect the survival for HS patients. 3,4,8,10,12,13,28 In our cohort, only 5 patients with TAPVC are still alive, all of them <2 years. Therefore, for ASP patients with TAPVC, prenatal counseling should describe the potential for pulmonary vein stenosis.…”

“…Five patients died from noncardiac causes, confirming previous published data showing that noncardiac anomalies contribute to the high mortality rate of HS. 3,10,12 …”

Human Heterotaxy Syndrome

“…Biventricular repair with a 2-patch method is usually performed for the complete type of AVSD with balanced ventricles. 79 AVSD with unbalanced ventricles is sometimes seen in patients with left isomerism, where either of the ventricles and the inflow tract is significantly hypoplastic, for instance in less than 30%. 80 In these cases, biventricular repair is not feasible.…”

Left Atrial Isomerism in Afrocaribbean Adolescents and Adults with no Intervention: Echocardiographic Findings

“…Respiratory disorders can include tracheoesphageal fistula and absence of one lung. Neurologic disorders include myelomeningocoele and en-Williams-Phillips and Hoo Sang (3,4).…”