Atrial Fibrillation/surgery* ; Catheter Ablation ; Heterotaxy Syndrome/surgery* ; Humans

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.
Arrhythmias, Cardiac ; Arteriovenous Fistula ; Biliary Atresia ; Follow-Up Studies ; Heart ; Heart Diseases ; Heterotaxy Syndrome ; Humans ; Isomerism* ; Medical Records ; Mortality ; Prognosis ; Survival Rate

Heterotaxy syndrome (HS) is a congenital disorder resulting from an abnormal arrangement of visceral organs across the normal left-right axis in the embryonic period. HS is usually associated with multiple anomalies, including defects of the major cardiovascular system and the extracardiovascular system such as intestinal malrotation, abnormal lung lobulation, bronchus anomalies, and pancreatic dysplasia. Although pancreatic dysplasia is occasionally accompanied with HS, the occurrence of diabetes mellitus (DM) due to pancreatic dysplasia in HS is rarely reported. We here report a case involving 13-year-old girl with DM caused by agenesis of the dorsal pancreas and HS diagnosed on the basis of the presence of a double-outlet right ventricle with bilateral pulmonary stenosis and intestinal malrotation with duodenal cyst. Timely diagnosis and treatment with insulin improved glycemic control.
Adolescent ; Bronchi ; Cardiovascular System ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Diabetes Mellitus* ; Diagnosis ; Double Outlet Right Ventricle ; Female ; Heterotaxy Syndrome* ; Humans ; Insulin ; Lung ; Pancreas* ; Pulmonary Valve Stenosis

OBJECTIVE: To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes. METHODS: A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution. RESULTS: Of 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%. CONCLUSION: Our study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.
Atrioventricular Block ; Bradycardia ; Double Outlet Right Ventricle ; Echocardiography ; Fetus ; Heart Block ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular ; Heterotaxy Syndrome ; Humans ; Infant ; Isomerism* ; Live Birth ; Lost to Follow-Up ; Prenatal Diagnosis* ; Prevalence ; Pulmonary Atresia ; Retrospective Studies ; Survival Rate ; Vena Cava, Inferior ; Vena Cava, Superior

Situs inversus of the abdominal organs in the presence of normally placed heart on the left side of the thorax is known as situs inversus with isolated levocardia. This rare condition is commonly associated with severe congenital defects of the heart. We report a case of situs inversus with levocardia in a 19-year-old asymptomatic male patient with completely normal heart on the left chest. Spiral computed tomography of the thorax and abdomen and echocardiographic studies revealed situs inversus of abdominal organs, normal heart (levocardia), mirrored left lungs, a midline liver, a left-sided inferior vena cava connecting to the right atrium, multiple splenic masses in the abdominal right upper quadrant, and aneurysmal dilatation of a splenic artery.
Abdomen ; Aneurysm ; Congenital Abnormalities ; Dilatation ; Heart ; Heart Atria ; Heterotaxy Syndrome* ; Humans ; Levocardia* ; Liver ; Lung ; Male ; Situs Inversus ; Splenic Artery ; Thorax ; Tomography, Spiral Computed ; Vena Cava, Inferior ; Young Adult

A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.
Acute Disease ; Congenital Abnormalities/*diagnosis/ultrasonography ; Endosonography ; Female ; Heterotaxy Syndrome/*diagnosis/ultrasonography ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Pancreas/abnormalities/ultrasonography ; Pancreatitis/*diagnosis ; Tomography, X-Ray Computed

Situs ambiguous is rare congenital anomaly in adults. In 2 adult patients who admitted for different cardiac problems, situs ambiguous with polysplenia was detected. A 42-year-old male admitted for radio frequent catheter ablation of atrial fibrillation, and he had left-sided inferior vena cava (IVC), hepatic segment of IVC interruption with hemiazygos continuation, multiple spleens and intestinal malrotation. And in a 52-year-old female case who was hospitalized due to infective endocarditis after implanting pacemaker for sick sinus syndrome, multiple spleens, left-sided stomach, bilateral liver with midline gallbladder, and left-sided IVC were found. Those findings were consistent with situs ambiguous with polysplenia, but their features were distinctive.
Adult ; Atrial Fibrillation ; Catheter Ablation ; Endocarditis ; Female ; Gallbladder ; Heterotaxy Syndrome ; Humans ; Liver ; Male ; Middle Aged ; Sick Sinus Syndrome ; Spleen ; Stomach ; Vena Cava, Inferior

Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. This broad term includes patients with a wide variety of very complex cardiac lesions. Patients with heterotaxy can be stratified into the subsets of asplenia syndrome and polysplenia syndrome, or the subsets of heterotaxy with isomerism of the right atrial appendages and heterotaxy with isomerism of the left atrial appendages. Treatment of patients with isomerism is determined by the nature and severity of the associated cardiac and extracardiac lesions. Most cardiac operations for patients with isomerism are palliative in nature, since normal anatomy is rarely achieved and mortality rates remain high for patients with heterotaxy syndrome. Patients with left isomerism in general have less severe cardiac malformations than those with right isomerism and, hence, more chance of biventricular repair. For almost all patients with right isomerism, and for many with left isomerism, biventricular repair will not be feasible, and all palliative protocols are then staging procedures prior to a Fontan-type repair. Recent advances in medical management, and improvements in surgical techniques have resulted in improved survival for these patients, and the surgical outcomes are comparable to those with Fontan circulation irrespective of the presence or absence of heterotaxy.
Atrial Appendage ; Axis, Cervical Vertebra ; Heterotaxy Syndrome ; Humans ; Isomerism

OBJECTIVE: To determine the clinical significance of persistent left superior vena cava (PLSVC) in a fetus with and without cardiac and extra-cardiac anomalies. METHODS: This was a retrospective review of all cases of PLSVC detected prenatally at our institution between May 2001 and May 2008. This retrospective study included 85 fetuses with PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Patient charts and recorded images were reviewed in order to identify associated conditions and outcomes. Telephone interviews were conducted to check patients' conditions in cases of isolated PLSVC. RESULTS: Eighty-five cases of PLSVC were detected prenatally during this study period. Of these 85 fetuses, 11 were aborted due to associated, prenatally proven, severe congenital heart anomalies or chromosomal anomalies, and 52 fetuses were delivered. The cases for other 22 fetuses were lost to follow-up. Postnatal echocardiography was performed in the 33, surviving patients, and PLSVC was confirmed in 32 of these patients. The most common associated congenital cardiac anomalies were seen included VSD, AVSD, and DORV (54.1%, 17.6% and 17.6%, respectively) (Table 3). PLSVC was also seen in seven cases (8.2%) of right isomerism and in four cases (4.7%) of left isomerism. In only two cases was the coexistence of PLSVC and extra-cardiac anomalies noted in this study. Fifteen cases were prenatally diagnosed as isolated PLSVC and all of them had live births. The follow-up period in our isolated PLSVC patients ranged from 0.5 to 84 months (Mean 24.5 months). Thirteen of these infants were doing well at the time of preparing this document and one case was diagnosed as TAPVR on postnatal echocardiography and one case was lost to follow-up. CONCLUSION: We strongly suggest that PLSVC is a benign vascular malformation and does not affect to the patient after birth. However, PLSVC is frequently associated with heterotaxy syndromes as well as other cardiac malformations and can be misdiagnosed as TAPVR. So if we find PLSVC in prenatal ultrasonography, meticulous inspection of the fetal anatomy must be performed.
Echocardiography ; Female ; Fetus ; Follow-Up Studies ; Glycosaminoglycans ; Heart ; Heterotaxy Syndrome ; Humans ; Infant ; Interviews as Topic ; Isomerism ; Live Birth ; Lost to Follow-Up ; Parturition ; Pregnancy ; Pregnancy Outcome ; Prenatal Diagnosis ; Pulmonary Artery ; Retrospective Studies ; Scimitar Syndrome ; Ultrasonography, Prenatal ; Vascular Malformations ; Vena Cava, Superior

PURPOSE: Despite extracardiac conduit Fontan had many advantages, long-term results related to longevity of conduit, and anticoagulation were not proven. This study was to evaluate the long-term outcome of hospital survivors with extracardiac Fontan circulation. METHODS: Between 1996 and 2006, 200 patients underwent extracardiac conduit Fontan operation. Median age at the Fontan operation was 3.4 years (range:16 months-35.7 years). All patients (89.5 %) except 21 patients of one stage Fontan operation underwent bi-directional cavopulmonary shunt. Fenestration was required in 85 patients (42.5%). RESULTS: There has been 6 hospital mortalities (3.0%), and 7 late mortalities (3.6%) at a mean follow-up of 52.4+/-32.2 months (range; 18days-120 months). Overall 10-years survival was 92.4 2.1%. Multivariate analysis identified severe infection at early postoperative periods (hazard ratio =12.439, P=0.001), and high pulmonary arterial pressure at preoperative period (hazard ratio=3.445, P=0.038) as risk factors for mortality. Reoperation was performed in 24 patients (12.0%), and freedom from reoperation was 82.4+/-4.1% at 10 years. Arrhythmia occurred in 32 patients (16.0%) after Fontan operation, and freedom from arrhythmia was 85.14.4% at 10 years. Risk factors for arrhythmia were heterotaxy syndrome (P=0.001), Follow up duration (P=0.027) and the age at Fontan operation (P=0.001). Freedom from thromboembolism was 92.91.9% at 10 years. The conduit cross- sectional area decreased by 14%, and the extent of decrease of the conduit cross-sectional remained stable irrespective of the follow-up duration. 95.2% of patients had New York Heart Association class I. CONCLUSIONS: After 10 years of follow-up, the overall survival, and the functional status of survivors of the extracardiac Fontan procedure are satisfactory. Fenestration has a beneficial effect on the results of high-risk Fontan patients. The incidence of late deaths, reoperations, obstructions of the cavopulmonary pathway, arrhythmias and thromboembolisms is low.
Arrhythmias, Cardiac ; Arterial Pressure ; Follow-Up Studies ; Fontan Procedure* ; Freedom ; Heart ; Heart Bypass, Right ; Heterotaxy Syndrome ; Hospital Mortality ; Humans ; Incidence ; Korea* ; Longevity ; Mortality ; Multivariate Analysis ; Postoperative Period ; Preoperative Period ; Reoperation ; Risk Factors ; Survivors ; Thromboembolism