A Case of Residual Atrial Septal Defect with Left Atrial Isomerism Requiring Reoperation 37 Years after the First Operation / 日本心臓血管外科学会雑誌

A 47-year-old female was admitted to our hospital for management of dyspnea. She had undergone surgery for an atrial septal defect (ASD) at the age of 17. Computed tomography revealed left isomerism, inferior vena cava interruption with azygos continuation and a residual ASD. Intra-operative findings showed that the residual ASD was positioned across the orifice of the hepatic vein. The previous suture line could be identified in the partially-closed atrial septum above the residual defect. Re-closure was performed without difficulties, and the patient's condition was good at discharge. Closure of ASD is a simple and basic procedure in cardiac surgery but care must be taken not to leave a shunt at the lower part of the defect, especially in cases with ASD defects in the lower margin.

Clinical Characteristics and Independent Factors Related to Long-Term Outcomes in Patients with Left Isomerism

BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age. CONCLUSION: Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.

Results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly / 中华胸心血管外科杂志

Objective To evaluate the results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly.Methods From September 2008 to October 2013,34 patients with heterotaxy syndrome underwent 46 cardiac operations at Guangzhou Women and Children Medical Center.22 were male,and 12 female.Mean age at operation was 22 months (4 months to 14 year).There were right atrial isomerism 24 cases,left atrial isomerism 10 cases.Only one had a single VSD,and others were all with complex cardiac anomaly,including complete atrioventricular canal defect 26 cases,tricuspid atresia 4 cases,mitral atresia 1 case,anomalous pulmonary venous connection 18 cases,pulmonary venous stenosis 4 cases,double superior vena cava 17 cases,and interrupted hepatic portion of the inferior vena cava 5 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results There were 4 died during hospitalization.The follow-up duration was 1 month to 5 years.There were 2 died.The early to middle term mortality was 17.6％ (6/34).In the same period the mortality for 103 patients without heterotaxy syndrome underwent univentricular repair was 4.8％ (5/103).There was significant statistical difference (P ＜ 0.05).Conclusion Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.

Atrial Rhythms in Patients with Left Atrial Isomerism

PURPOSE: Histologic studies have revealed ectopic location and hypoplasia of sinus nodal tissue in left atrial isomerism. The purpose of this study is to characterize the atrial rhythms corresponding to these histologic abnormalities in left atrial isomerism. Electrocardiograms in left atrial isomerism are characterized by multiple atrial rhythms with abnormal P-wave axes, but the complex nature of atrial rhythms in left atrial isomerism remains to be clarified. METHODS: Surface electrocardiograms and Holter recordings of 16 patients with left atrial isomerism were retrospectively reviewed. For comparison, atrial rhythms in 8 patients with right atrial isomerism were studied with the same study protocol. RESULTS: In left atrial isomerism, atrial rhythm with abnormal superior P-wave axis was the most frequent in 69% of patients and multiple atrial rhythms were observed in 37% in surface electrocardiograms. In Holter recordings, transient or persistent slow atrial rhythms with or without visible P waves associated with junctional escape were documented in 9 out of 16(56%) patients. In comparison, multiple atrial rhythms were as frequent as in left atrial isomerism, but slow atrial rhythm with junctional escape was not present in 8 patients with right atrial isomerism. CONCLUSION: It is concluded that transient or persistent slow atrial rhythms associated with junctional escape, suggestive of atrial nodal dysfunction, are characteristic and helpful in the diagnosis of left atrial isomerism.