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

Abstract

Background and ObjectivesWe studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis.Subjects and MethodsWe reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014.ResultsOf 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.ConclusionPatients 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.

Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia

“…[19][20][21] Among the cardiac abnormalities of LI found in the literature, the main ones are dextrocardia/mesocardia (24%-42% and 2% of all cases, respectively), anomalous pulmonary venous return (37%-56%), persistent left superior vena cava (33%-59%), unroofed coronary sinus (26%-42%), absence of the suprahepatic segment of the IVC (86%-100%), hepatic venous drainage into the left atrium (32%-41%), common atrium or interatrial communication (80%), total or partial atrioventricular septal defect (49%-80%), univentricular atrioventricular connection (20%-40%), ventricular septal defect (11%), transposition of the great arteries (5%-21%), double-outlet right ventricle (17%-37%), PVS or pulmonary atresia (28%-61%), and aortic stenosis/ atresia or coarctation of the aorta (7%-45%). 19,[22][23][24][25][26][27][28] The literature on the prevalence of heart disease among patients with BA and LI is scarce. Using the MEDLINE, LILACS, and SciELO databases and associating the main descriptors in different manners, our search did not retrieve more than a few case series.…”

Clinical Implications of Airway Anomalies and Stenosis in Patients with Heterotaxy Syndrome

“…In most Western countries, patients with LAI account for more than half of patients with heterotaxy syndrome [6][7][8][9] . In contrast, more heterotaxy syndrome patients have RAI in oriental reports [10][11][12][13] . However, the pulmonary variations and airway anomalies in patients with heterotaxy remain unknown.…”

Clinical implications of airway anomalies and stenosis in patients with heterotaxy syndrome

“…In most Western countries, patients with LAI account for more than half of patients with heterotaxy syndrome 6–9 . In contrast, more heterotaxy syndrome patients have RAI in oriental reports 10–13 . However, the pulmonary variations and airway anomalies in patients with heterotaxy remain unknown.…”

“…[6][7][8][9] In contrast, more heterotaxy syndrome patients have RAI in oriental reports. [10][11][12][13] However, the pulmonary variations and airway anomalies in patients with heterotaxy remain unknown. Most patients with heterotaxy syndrome need to be palliated by multiple staged interventions to achieve a single ventricle due to the associated complex CHD physiology.…”