Left Isomerism Research Articles

Comparable long-term results of the extracardiac conduit total cavopulmonary connection in heterotaxy to non-heterotaxy patients.

Fontan operation in heterotaxy patients has been associated with high mortality. We studied whether adoption of the extracardiac conduit (EC) total cavopulmonary connection (TCPC)in heterotaxy demonstrated comparable results to non-heterotaxy population. A retrospective medical record review of 35 consecutive patients with heterotaxy and 70 consecutive patients without heterotaxy syndrome who underwent EC TCPC between 2000 and 2018 was performed. In the 35 heterotaxy patients, 30 were right and 5 were left atrial isomerism. Anomalies of venous return included bilateral superior vena cava in 20 (57.1%), separated hepatic vein in 8 (22.9%), interrupted inferior vena cava in 3 (8.6%), total anomalous pulmonary venous return in 7 (20%), and partial in 2 patients (5.7%). All patients underwent EC TCPC under beating-heart cardiopulmonary bypass except in four patients (11.4%) cardioplegic arrest was needed for cardiac repair. The surgical mortality rate was lower in heterotaxy patients (0% vs. 5.7%; p = 0.299) but statistically not significant. The follow-up ranged from 2 months to 17.8 years (mean 9.4 ± 5.6 years). At 15 years, there was no significant difference between the heterotaxy and non-heterotaxy patients regarding the long-term survival (70% vs. 78.6%; p = 0.443), freedom from reoperation (81.9% vs. 96.5%; p = 0.057), and postoperative arrhythmia (17.1% vs. 7.1%; p = 0.174). EC TCPC can be performed in heterotaxy patients with comparable early and late results to the non-heterotaxy population. However, the late morbidities regarding the Fontan circulation needs careful follow-up.

Pulmonary Versus Systemic Outflow Obstruction in Functionally Univentricular Hearts with Isomerism: An Observation.

Our objective was to review our experience with isomerism associated with univentricular hearts and evaluate the prevalence of pulmonary versus systemic outflow obstruction. We identified those prenatally or postnatally diagnosed, between September 2004 and October 2021, with right and left isomerism and a functionally univentricular heart. We identified 62, 51 prenatally and 11 postnatally. Of the 62, 61 had prenatal care for an 84% (51/61) prenatal detection rate. Of the 62, 36 (58%) had right isomerism. Of the 51 prenatally diagnosed, 36 were live-born, 13 had fetal demise, and two underwent elective termination. Of the total 62, 43 had pulmonary outflow obstruction, 14 had systemic outflow obstruction, three had no outflow obstruction, and two had a common arterial trunk. However, between September 2004 and December 2019, 41 of 52 (79%) had pulmonary outflow obstruction, and between January 2000 and October 2021, 2 of 10 (20%) had pulmonary outflow obstruction (p = 001). We noted a statistically significant temporal change in the prevalence of pulmonary versus systemic outflow obstruction in those with isomeric situs and a functionally univentricular heart. Further, prenatal diagnosis exceeded 80% in the general population of Nevada.

Difficulty in the Diagnosis of Biliary Atresia Splenic Malformation Syndrome In Utero.

The fetus of a 30-year-old pregnant Japanese woman was diagnosed with absence of inferior vena cava (IVC) and azygos continuation of interrupted IVC without cardiac anomalies at 34 weeks of gestation, and a healthy male neonate weighing 2,910 g was delivered at 37 weeks of gestation. On day 42 after birth, direct bilirubin predominant hyperbilirubinemia and high serum gamma-GTP levels were detected. Computed tomography revealed the presence of a lobulated and accessory spleen, and laparotomy demonstrated type III biliary atresia (BA), confirming the final diagnosis of BA splenic malformation (BASM) syndrome. In retrospect, non-visualization of the gallbladder was missed in utero. The combination of the absence of IVC and BA without cardiac anomalies is far less likely to occur in left isomerism. Although BA remains difficult to detect in utero, special attention should be paid to cases of BA associated with findings of left isomerism, including the absence of IVC, to enable early diagnosis and treatment of BASM.

Third Atrial Chamber

A 10-MONTH-OLD BOY (weight: 5.8 kg; height: 66 cm; body mass index: 13) was taken to the cardiac catheterization laboratory before the creation of a bidirectional Glenn shunt. At 4 days of age, transthoracic echocardiography done for the evaluation of persistent cyanosis revealed heterotaxy (left atrial isomerism), situs ambiguous with levocardia, bilateral superior vena cava, atrial septal defect (9 mm), partial anomalous right pulmonary venous return to the morphologic right atrium, discordant atrioventricular connection and concordant ventriculoarterial connection, with a hypoplastic right ventricle. The child underwent stenting of the patent ductus arteriosus and balloon atrial septostomy on the 11th day of life. Pulmonary artery banding was done at the age of 1 month. During the current admission, a computerized tomography scan revealed the complex connections of the great vessels (Fig 1). A 4-chamber transesophageal echocardiography view displayed the morphologic left atrium, which was on the right side, and multiple coronary artery fistulae opening into an enlarged chamber located superiorly (Fig 2; Video 1). The atrial septal defect could be visualized in the bicaval view (Fig 3; Video 2). A modified midesophageal 4-chamber view displayed a large superior chamber, as well as the atrial septal defect (Fig 4). Agitated saline was injected into a right upper extremity vein. The agitated saline appeared first in the right internal jugular vein and then appeared in the upper atrial chamber, right side atrium (morphologic left atrium), followed by an appearance in the right ventricle (Fig 5, A-D; Video 3). Slight retraction of the probe from the 4-chamber view displayed a right superior vena cava on the right side that was moving into close juxtaposition with the morphologic left atrium (Fig 6; Video 4). Correlation with computerized tomography findings revealed the identity of the structure seen by transesophageal echocardiography into which the right superior vena cava and the coronary artery fistulae were draining. What is the diagnosis? Fig. 2(A) Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with (B) color Doppler displaying an enlarged chamber (white query sign) with multiple coronary artery fistulae opening into the chamber. mLA, morphologic left atrium; RV, right ventricle; TV, tricuspid valve View Large Image Figure Viewer Download Hi-res image Fig. 3Bicaval midesophageal transesophageal echocardiography view displaying the atrial septal defect. View Large Image Figure Viewer Download Hi-res image Fig. 4Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with color-flow Doppler displaying the atrial septal defect and a large upper chamber marked by white query sign. View Large Image Figure Viewer Download Hi-res image Fig. 5Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying (A) the agitated saline echo contrast injected into an upper limb vein entering the right superior vena cava, (B) the coronary sinus and the morphologic right atrium on the right side, (C) the morphologic left atrium on the right side, and (D) the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 6Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying the right superior vena cava and morphologic right atrium in close proximity. ASD, atrial septal defect; LAA, morphologic left atrial appendage; mLeft atrium, morphologic left atrium; mR, morphologic right atrium; RSVC, right superior vena cava; RV, right ventricle; TV, tricuspid valve. View Large Image Figure Viewer Download Hi-res image

Total anomalous systemic venous connection with left isomerism in an adult - A rare cause of cyanosis

Total anomalous systemic venous connection with left isomerism in an adult - A rare cause of cyanosis

Anomalous subaortic course of brachiocephalic vein: Evaluation on multidetector computed tomography angiography

To evaluate the morphology and associated cardiovascular abnormalities in patients with an anomalous subaortic course of brachiocephalic vein on multidetector computed tomography (CT) angiography. A retrospective study was performed at a tertiary referral institute to identify patients with subaortic brachiocephalic vein on multidetector CT (MDCT) angiography using dual source CT scanner between January 2014 and July 2021. The morphology of the subaortic brachiocephalic vein along with the cardiovascular anatomy and associated anomalies were evaluated. Out of 4349 patients who had undergoneMDCT angiography for evaluation of congenital heart diseases, we identified 126 (2.9%) patients with subaortic brachiocephalic vein. The subaortic brachiocephalic vein was left-sided in 125 patients while a right-sided subaortic brachiocephalic vein was identified in a patient with left isomerism. Common cardiovascular associations included tetralogy of Fallot (109/126; 88.1%), double outlet right ventricle (8/126; 6.3%) and common arterial trunk (5/126; 3.9%). The presence of a right aortic arch was seen in 78/126 (62%) patients. Some degree of right ventricular outflow obstruction was present in 119/126 (94.4%) patients; pulmonary stenosis was seen in 78 (62%) patients while pulmonary atresia was seen in 41 (32.5%) patients. A subaortic brachiocephalic vein can coexist with various complex congenital heart diseases, most commonly tetralogy of Fallot and commonly associated with right aortic arch and pulmonary stenosis/atresia. It is important to identify this anomalous course of brachiocephalic vein before performing surgical procedures or venous catheterization to avoid potential complications.

Analysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery.

Laterality anomalies are almost always associated with severe cardiac anomalies. Demographic properties, type of the procedures, associated anomalies, and early and mid-term prognosis of four types of laterality anomalies were analysed. A total of 64 consecutive patients with laterality anomalies were enrolled between July 2014 and July 2020. We grouped the patients as situs solitus dextrocardia (SSD) (n = 12; 18.7%); situs inversus (SI) (n = 16; 25%); right atrial isomerism (RAI) (n = 29; 45.3%); and left atrial isomerism (LAI) (n = 7; 10.9%). TAPVC was only present in the RAI group (31%). Incidence of mitral or tricuspid atresia was higher in the SSD group (25%). All the patients were followed up with a mean of 19.06 ± 17.6 (0.1-72) months. Early postoperative mortality was 17 patients, among 107 procedures (15.8%). Twelve patients were in the neonatal period. All ten patients survived after isolated ductal stenting. Fourteen of the deaths were in the RAI group (48.3%). The 3-year survival rates were 85% in LAI, 78.7% in SI, 55.8% in SSD, and 38% in RAI groups. According to the multivariable Cox regression model, mechanical ventilation, kidney injury, RAI, and complex surgery in the neonatal period were independent risk factors for early mortality. Laterality anomalies are one of the most challenging patients who commonly had univentricular physiology. The most prevalent anomaly was RAI, and RAI had the worst outcome and survival. Ductal stent is an acceptable first intervention during the neonatal period in suitable patients. Complex procedures may carry a high risk of death in the neonatal period.

OC18.09: Splenic vessels: runway lights to spot the fetal pancreas

To assess the utility of splenic vessels in delineation of fetal pancreas and its implications in clinical practice. 200 normal singleton fetuses between 17–22 weeks of gestation with normal pancreas and splenic vessels were included. 4 fetuses with variant anatomy were also included. The fetal pancreas was imaged using a transabdominal approach in an oblique section caudal to the plane of abdominal circumference using both grey scale and power Doppler with PRF setting between 0.6-0.9 kHz. The pancreas and splenic vessels were imaged with the fetal spine position between 3 to 9 o'clock position. The normal pancreas was seen as an echogenic structure with the splenic vessels coursing along its posterior border in all controls (figure 1a-d). Splenic artery is seen in figure 1b and splenic vein in figure 1d. One case each of enlarged pancreas (figure 1e) in a fetus with suspected Beckwith Wiedemann syndrome confirmed postnatally, normal right sided pancreas (figure 1f) in situs inversus, truncated pancreas (figure 1g) in left atrial isomerism and normal left sided pancreas with absent splenic vessels (figure 1h) likely due to asplenia in right atrial isomerism were included. Fetal pancreas is challenging to spot on grey scale ultrasound. Power Doppler lights up the splenic vessels which course along the posterior border of the pancreas serving as a useful landmark to delineate the pancreas. The knowledge of normal pancreatic and splenic vessel anatomy can help in detecting anomalies which are a part of several syndromes and situs abnormalities. This serves as a useful tool in our armamentarium to elucidate the fetal phenotype. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

VP22.24: Idiopathic DA constriction observed during left isomerism

VP22.24: Idiopathic DA constriction observed during left isomerism

Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China.

Objectives: The management of atrial isomerism with complex congenital heart disease remains challenging. Experience has been largely obtained in advanced countries. The clinical diversity is greater in China. We evaluated the early- and medium-term outcomes of surgical treatment of these patients.Methods: We reviewed 86 patients of atrial isomerism with complex congenital heart disease undergoing varied surgeries in our center in 2008–2020. Cox regression models were used to analyze the risk factors for mortality.Results: There were 75 cases of right and 11 of left atrial isomerism. Eighty-three (96.5%) patients underwent single-ventricle staged palliation approach, with 10 early and 7 late deaths. The overall 1-, 5-, and 10-year survival rates were 84.7, 79.3, and 79.3%, respectively. Thirty-six (43.4%) patients completed the Fontan procedure with median age of 48 months and freedom from death or Fontan failure at 1-, 5-, and 8-years were 94.4, 87.4, and 80.7%, respectively. Concomitant total anomalous pulmonary venous connection [hazard ratio (HR): 5.15 (1.95–12.94), p = 0.008], more than moderate atrioventricular valve regurgitation [HR: 4.82 (2.42–6.79), p = 0.003], and the need for first-stage palliative surgery [HR: 4.58 (1.64–10.76), p = 0.015] were independent risk factors for mortality.Conclusions: Despite even greater clinical diversity, the surgical outcomes of atrial isomerism with complex congenital heart disease are improving in China. The early and intermediate outcomes are comparable to many previous reports. Concomitant total anomalous pulmonary venous connection, moderate or severe atrioventricular valve regurgitation, and the need for a first-stage palliative surgery are still independent risk factors for mortality.

Novel MYO1D Missense Variant Identified Through Whole Exome Sequencing and Computational Biology Analysis Expands the Spectrum of Causal Genes of Laterality Defects.

Laterality defects (LDs) or asymmetrically positioned organs are a group of rare developmental disorders caused by environmental and/or genetic factors. However, the exact molecular pathophysiology of LD is not yet fully characterised. In this context, studying Arab population presents an ideal opportunity to discover the novel molecular basis of diseases owing to the high rate of consanguinity and genetic disorders. Therefore, in the present study, we studied the molecular basis of LD in Arab patients, using next-generation sequencing method. We discovered an extremely rare novel missense variant in MYO1D gene (Pro765Ser) presenting with visceral heterotaxy and left isomerism with polysplenia syndrome. The proband in this index family has inherited this homozygous variant from her heterozygous parents following the autosomal recessive pattern. This is the first report to show MYO1D genetic variant causing left–right axis defects in humans, besides previous known evidence from zebrafish, frog and Drosophila models. Moreover, our multilevel bioinformatics-based structural (protein variant structural modelling, divergence, and stability) analysis has suggested that Ser765 causes minor structural drifts and stability changes, potentially affecting the biophysical and functional properties of MYO1D protein like calmodulin binding and microfilament motor activities. Functional bioinformatics analysis has shown that MYO1D is ubiquitously expressed across several human tissues and is reported to induce severe phenotypes in knockout mouse models. In conclusion, our findings show the expanded genetic spectrum of LD, which could potentially pave way for the novel drug target identification and development of personalised medicine for high-risk families.

Fetal Ultrasonic Evaluation of Bronchial Morphology in Fetuses with Isomerism

Objective: The aim of the study was to evaluate whether fetal ultrasound could determine bronchial isomerism and distinguish left isomerism from right isomerism. Methods: We identified 110 healthy fetuses and 28 fetuses with isomerism. The outer angle between the tracheal midline and the inner margin of the bronchus is measured. The bronchial angles and the ratio of left/right bronchial angle were used to differentiate bronchial morphology and confirm the presence of bronchial isomerism in pathological cases. Results: The normal angles of the left and right bronchi were 146.98° (95% CI, 145.15–147.81°) and 167.37° (95% CI, 166.30–168.44°), separately. The cutoff bronchial angle of 156.5° was used to distinguish left bronchus from right bronchus. The bronchial isomerism could be identified in all pathological cases by autopsy and bronchial-atrial concordance occurred in 27 pathological cases (96.4%). In 21 pathological cases, the bilateral bronchial angle was <156.5 versus >156.5 differentiated left from right isomerism, respectively. The ratio of the left/right bronchial angle of >0.935 identified 92.9% (26/28) of all pathological cases, with a sensitivity of 89.7%. Conclusions: Fetal ultrasound can detect the bronchial morphology and the presence of bronchial isomerism in fetuses with isomerism according to bronchial angles and the ratio of left/right bronchial angle.

Cardiac surgery in patients with atrial isomerism: Long-term results and outcomes.

The aim of the study was to identify, determine, and analyze the clinical and surgical outcomes of patients with atrial isomerism (AI) undergoing cardiac surgery. A retrospective study was carried out. Patients with diagnosis of AI undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2010 and March 31, 2020 were included; demographic characteristics and perioperative conditions of the patients were considered. Sixty-five patients were included, with an average age of 6.4 ± 4.9 years, 50.8% males. Thirty-eight (58.5%) had right atrial isomerism (RAI) and 27 (41.5%) had left atrial isomerism (LAI); univentricular physiology (78.5%) predominated. Atrioventricular septal defect (AVSD) in RAI and septal defects in LAI were identified as the main associated defects. The most common surgical procedures performed were modified Blalock-Taussig shunt (MBTS) (27.6%), MBTS with total anomalous pulmonary venous connection (TAPVC) repair (15.3%) and total cavopulmonary connection (TCPC) with an extracardiac conduit fenestrated (10.8%); 100% RAI required a univentricular approach, while in LAI it was 48.1%. Overall survival was 92.3%, with 100% survival in LAI with biventricular physiology and 86.8% in RAI with univentricular physiology. The survival of our institution is similar to that of other referral centers, where patients with LAI had a better evolution than RAI; in addition, the univentricular approach was required in all with RAI. Patients with AI must undergo a rigorous evaluation to determine an adequate repair strategy, considering univentricular RAI with a high possibility of morbidity and mortality.

Impact of gastrointestinal comorbidities in patients with right and left atrial isomerism.

Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome. We reviewed the cardiology records of heterotaxy syndrome patients from two centres, regarding the presence of CHD, time for cardiac intervention, presence of gastrointestinal abnormalities, and type/time of surgery. A questionnaire about gastrointestinal status was sent to patients <18 years old. Kaplan-Meier curves were derived for survival data and freedom from intervention. Data were included for 182 patients (49 RAI and 133 LAI) of 247 identified. Questionnaires were sent to 77 families and 47 replied. CHD was present in all RAI and 61.7% of LAI cases. Thirty-eight patients had abdominal surgery (20.9%), similar for RAI and LAI (20.4% versus 21%, p> 0.99): Ladd procedure in 17 (44.7%), non-Ladd in 12 (31.5%), and both procedures in 9 (23.7%). Ten-year freedom from Ladd procedure for all was 86% for the whole cohort (RAI = 87%; LAI = 85%, p = 0.98). Freedom from any gastrointestinal surgery at 10 years was 79% for the whole cohort (RAI = 77%; LAI = 80%, p = 0.54). Ten-year freedom from cardiac surgery was 31% for the whole cohort (RAI = 6%; LAI = 43%, p < 0.0001). In our cohort, one in five patients required abdominal surgery, mostly in their first year of life, similar for RAI and LAI. Between 1 and 10 years of follow-up, the impact of gastrointestinal abnormalities on outcome was minimal. Medium term survival was related to CHD.

Fetal heterotaxy: Should we still categorize?

Heterotaxy (HTX) is usually stratified in right (RI) and left isomerism (LI). We analyzed 61 fetal specimens with HTX divided in 5 groups: classic LI [CLI: bronchopulmonary LI, bilaterally absent pectinate muscles (PM), polysplenia, n = 22], non-classic LI (NCLI: one discordant feature, n = 13), classic RI (CRI: bronchopulmonary RI, bilateral extent of PM to the crux, asplenia, n = 12), non-classic RI (NCRI: one discordant feature, n = 6, totally discordant features, n = 8). Non-classic patterns were found in 44% (33% RI, 37% LI). Among those, the status of the spleen was highly variable (NCLI with asplenia 38%, NCRI with single spleen 66%), while the bronchial status was almost always concordant. Intestinal malrotation was present in 83% CRI, 64% CLI, 50% NCLI, 50% NCRI. Extent of PM was highly variable in NCLI (bilaterally left 46%, normal 46%, bilaterally right 8%), and bilaterally right in 83% of NCRI only. Interrupted inferior caval vein was seen in LI only; total extracardiac anomalous pulmonary venous connection (APVC) was seen only in RI, ipsilateral APVC only in LI. A common atrioventricular (AV) junction was constant in RI vs. 66% in LI, with a complete AV canal in 100% RI, 75% LI. Functionally univentricular hearts were found in 67% RI, 46% LI, right in 62.5%. Ventriculo-arterial (VA) connections were abnormal in 100% RI, 59% LI, double outlet right ventricle (DORV) being the most frequent type in all categories. Pulmonary atresia and stenosis were frequent in RI (77%), rare in LI. Aortic arch hypoplasia and coarctation were found almost exclusively in LI (57%). Right-sided aortic arch was found in 67% RI, 6% LI. HTX and isomerism are not synonymous. Extent of PM is not always symmetrical (15%) and cannot therefore be considered the hallmark of HTX. Because HTX includes both abnormal symmetry and random organization of organs, each anatomic feature should be analysed individually rather than trying to categorize.

Main pulmonary artery swing back for univentricular heart

Left pulmonary artery (LPA) surgical arterioplasty done for juxtaductal pulmonary artery coarctation is a challenging technique that has often produced unsatisfactory outcomes because of poor outcome later and incomplete understanding of the evolution of structural changes in these vessels. We report a unique technique for LPA plasty involving main pulmonary artery (MPA) swing back, in a toddler who presented with history of increased work of breathing, cyanosis and failure to thrive since birth, where the MPA was detached and was swung back cranially to be anastomosed to LPA. This technique can be done, especially in univentricular heart (future Fontan candidate), since MPA needs to be interrupted. The patient had heterotaxy syndrome, left atrial isomerism, hypoplastic pulmonary valve with severe sub-valvar stenosis, duct dependent pulmonary circulation, good sized confluent branch pulmonary arteries with LPA narrowing at ductal insertion site, interrupted IVC with hemiazygous continuation and left SVC draining into coronary sinus. Initially underwent right modified Blalock taussig (BT) shunt, atrial septectomy, LPA plasty and PDA ligation at 6 months of age. Upon follow up after a year, diagnostic angiogram confirmed good flow in BT shunt and LPA re-stenosis with malpositioned great arteries. Redo sternotomy and Kawashima procedure was performed. BT shunt and MPA stump were divided. To conclude, the detached MPA segment was swung cranially and anstomosed to LPA (end to end) inferiorly and left SVC-LPA anstomosed superiorly onto this by end to side fashion. The early outcome was satisfactory, however long-term outcome needs to be followed up

Multiple Color Doppler Flow Jets Into The Atria in a Child With Heterotaxy Syndrome

A nine-month-old girl (weight: 7.92 kg; height: 70 cm) was taken to the operating room for the creation of a bilateral bidirectional Glenn shunt and atrial septectomy. Patent ductus arteriosus stenting had been done when the child was four days old. Transthoracic echocardiography showed heterotaxy (left atrial isomerism) with situs inversus, discordant atrioventricular and concordant ventriculoarterial connections (ventricular ‘noninversion’), a large ventricular septal defect, membranous pulmonary atresia with a hypoplastic main pulmonary artery, confluent branch pulmonary arteries, aorta anterior and to the right of the pulmonary artery, and bilateral superior vena cava with no bridging vein. The left superior vena cava was draining into the left-sided atrium (morphologic right atrium), while the right superior vena cava was draining into the same atrium via a massively dilated coronary sinus. The inferior vena cava was interrupted, with hemzygous continuation to the left superior vena cava. A computerized tomography scan revealed a right-sided stomach with a midline liver, and demonstrated the complex vascular communications (Fig 1, A and B). Transesophageal echocardiography confirmed the preoperative diagnosis (Fig. 2, Fig. 3; Videos 1 and 2). Color- flow Doppler mapping in the mid-esophageal view demonstrated multiple jets into both the atria and the right-sided ventricle (Fig 4, A and B; Videos 3 and 4). Pulse-wave Doppler of a dominant jet revealed a biphasic flow (Fig 5; Video 5). The intramyocardial origin of the jets was traced toward the aorta (Fig 6; Video 6). Fig. 2The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map showing demonstrating the anatomic abnormality in the child. View Large Image Figure Viewer Download Hi-res image Fig. 3The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map demonstrating the left superior vena cava. Left SVC, left superior vena cava. View Large Image Figure Viewer Download Hi-res image Fig. 4(A) The midesophageal transesophageal echocardiography views with color-flow Doppler map demonstrating jets (white arrows) into the left atrium. (B) The right atrium and the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 5The midesophageal pulse-wave Doppler signal interrogation of the jet by transesophageal echocardiography. View Large Image Figure Viewer Download Hi-res image Fig. 6The midesophageal transesophageal echocardiography view displaying the color-flow Doppler mapping delineating the origin of the jet (white arrows). View Large Image Figure Viewer Download Hi-res image

Surgical management of lesions encountered in the setting of the retroaortic left brachiocephalic vein.

Although the retroaortic left brachiocephalic vein in isolation is of no clinical importance, its recognition in the setting of associated lesions is important. We sought to address issues concerning the influence of isomerism, the establishment of diagnosis, and its importance in various surgical and interventional procedures. A total of 80 published clinical and necropsy studies in the setting of a retroaortic left brachiocephalic vein described 250 patients. Clinical presentation, radiographic, ultrasonographic findings, contrast echocardiography, computed-tomographic angiocardiography, magnetic resonance imaging, and angiocardiography provided the diagnostic information prior to considering the surgical approach to the associated cardiac anomalies. Among 250 reported cases, three-quarters had associated congenitally malformed hearts. Of these 189 patients, all but seven had usual atrial arrangement. Right isomerism was reported in five patients and two patients having left isomerism. Almost two-thirds had tetralogy of Fallot or its variants, over four-fifths had malformations involving the outflow tract, two-thirds had a right aortic arch with two patients having a cervical aortic arch, and onepatient had double aortic arch. Various innovative individualized surgical procedures were employed with an overall perioperative mortality of 3.4%. Although the retroaortic left brachiocephalic vein is asymptomatic, its recognition during clinical investigation should raise the possibility of an association with other malformations, especially right aortic arch, ventricular septal defect, and anomalies of the outflow tracts. We submit that an increased appreciation of this venous anomaly may facilitate surgical planning, endovascular procedures, placement of central venous lines, and transvenous pacemakers.

Intra-Atrial Rerouting of Anomalous Systemic Venous Connection and Septation of Common Atrium in Intracardiac Repair of Complete Atrioventricular Septal Defect with Left Isomerism and Dextrocardia: A Case Report

Intra-Atrial Rerouting of Anomalous Systemic Venous Connection and Septation of Common Atrium in Intracardiac Repair of Complete Atrioventricular Septal Defect with Left Isomerism and Dextrocardia: A Case Report

Heterotaxy syndrome with left atrial isomerism and polysplenia is a unique entity

A state of a patient with heterotaxy syndrome with polysplenia was indiscriminately diagnosed through visualized estimation (computed tomography and pelviabdominal ultrasound) regardless of the pathological state. This syndrome depends on a diversity of anomalies of design and morphology of thoracoabdominal organ configuration. Imaging studies are the foundation in diagnosis.