scholarly journals Heterotaxy syndrome: This is the left, right?

2015 ◽  
Vol 19 (2) ◽  
Author(s):  
Lili Huang ◽  
Belinda J. Mitchell ◽  
Savvas Andronikou ◽  
Zarina I. Lockhat ◽  
Farhana Suleman
Keyword(s):  
Morphological Characteristics ◽  
Imaging Modalities ◽  
Right Atrial ◽  
Abdominal Organ ◽  
Heterotaxy Syndrome ◽  
Diagnostic Challenge ◽  
Complex Disorder ◽  
Left And Right ◽  
Atrial Isomerism ◽  
Heterotaxy Syndromes

Heterotaxy syndrome is a rare and complex disorder of the chest and abdominal organ arrangements, and presents a diagnostic challenge to the radiologist. This article describes the morphological characteristics of heterotaxy and situs abnormalities, in particular left and right atrial isomerism, and suggests an approach in evaluating the spectrum of abnormalities associated with heterotaxy syndromes, using appropriate imaging modalities.

Prenatal ultrasound diagnosis of pulmonary atresia in combination with left atrial isomerism

2017 ◽  
Author(s):  
M Medvedev, M.V. Kubrina, O.S. Zarubina et all
Keyword(s):  
Left Atrial ◽  
Reverse Flow ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Prenatal Ultrasound ◽  
Ultrasound Diagnosis ◽  
Right Atrial ◽  
Second Trimester ◽  
Left And Right ◽  
Atrial Isomerism

Two cases of prenatal ultrasound diagnosis of left atrial isomerism in the second trimester of gestation is presented. These two cases were in combination with pulmonary atresia and right aortic arch. Left atrial isomerism was identify by the digit-like shape of the left and right atrial appendages. The pulmonary atresia was identified on the basis of reverse flow in small pulmonary artery. A right aortic was identified by “U”-shaped confluence of aorta and ductus arteriosus in view of three vessels and trachea. The trachea was located between the vessels. The pregnancies were terminated and prenatal diagnosis was conformed at autopsy

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

2021 ◽  
pp. 1-8
Author(s):  
Anastasia Fotaki ◽  
Victoria L. Doughty ◽  
Winston Banya ◽  
Stefano Giuliani ◽  
Sarah Bradley ◽  
...  
Keyword(s):  
Abdominal Surgery ◽  
Left Atrial ◽  
Right Atrial ◽  
Medium Term ◽  
Heterotaxy Syndrome ◽  
Term Survival ◽  
Ladd Procedure ◽  
Atrial Isomerism ◽  
The Impact ◽  
Gastrointestinal Abnormalities

Abstract Background and aim: 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. Methods: 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. Results: 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). Conclusions: 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.

scholarly journals Prenatal Findings in Left Atrial Isomerism and an Overview of Heterotaxy Syndrome

2017 ◽  
Vol 34 (2) ◽  
pp. 148-154
Author(s):  
Claudine Thomas ◽  
Samantha N. Sawyer
Keyword(s):  
Case Report ◽  
Left Atrial ◽  
In Utero ◽  
Heterotaxy Syndrome ◽  
Cardiac Defects ◽  
Complex Disorder ◽  
Atrial Isomerism ◽  
Cardiac Manifestations

Heterotaxy syndrome is a rare, complex disorder involving structural cardiac defects in combination with symmetrical development of abdominal viscera that are normally asymmetrical organs. The vast assortment of cardiac manifestations with accompanying abdominal visceral variations determines the prognosis of heterotaxy syndrome. This case study provides an example of the management of this disease, which is unique to the patient. This is a case report of heterotaxy syndrome diagnosed in utero at 29 weeks and 4 days.

scholarly journals Impact of gastrointestinal comorbidities in patients with right & left atrial isomerism

2020 ◽  
Author(s):  
Anastasia Fotaki ◽  
Victoria L Doughty ◽  
Winston Banya ◽  
Stefano Giuliani ◽  
Sarah Bradley ◽  
...  
Keyword(s):  
Abdominal Surgery ◽  
Survival Data ◽  
Left Atrial ◽  
Right Atrial ◽  
List Type ◽  
Heterotaxy Syndrome ◽  
Term Survival ◽  
Ladd Procedure ◽  
Atrial Isomerism ◽  
The Impact

ABSTRACTObjectiveHeterotaxy syndrome (HS), being right atrial isomerism (RAI) or left atrial isomerism (LAI) often presents with congenital heart disease (CHD). Intestinal abnormalities including malrotation are common. We aim to assess the impact of gut abnormalities on outcome in HS.MethodsWe reviewed cardiology records of HS patients regarding presence of CHD, time for cardiac intervention, presence of gastrointestinal (GI) abnormalities and type/time of surgery. A questionnaire about GI status was sent to patients <18 years old. Kaplan-Meier curves were derived for survival data.ResultsData were available for 195 patients (49 RAI, 146 LAI) of 247 identified. Questionnaires were sent to 77 families, 47 replied. CHD was present in all RAI and 63.7% of LAI cases. Thirty-eight patients had abdominal surgery (19.5%), similar rate in RAI and LAI (20.4% vs 19.1%, p=0.92). Ladd procedure was performed in 17 (44.7%), non-Ladd in 12 (31.5%) and both procedures in nine patients (23.7%). Ten-year freedom from Ladd procedure for all was 86% (RAI=87%; LAI=85%, p=0.82). Freedom from any GI surgery at one year was 86% (RAI=86%; LAI=86%, p=0.98) and at ten years was 80% (RAI=77%; LAI=81%, p=0.65). Ten-year freedom from cardiac surgery was 34% (RAI=7%; LAI=42%, p<0.0001).ConclusionsIn our cohort, one in five patients required abdominal surgery, mostly in their first year, similar in RAI and LAI. Between one and ten years of follow up the impact of GI abnormalities on outcome was minimal. Medium term survival was related to CHD.ARTICLE SUMMARYSTRENGTHS AND LIMITATIONS OF THIS STUDYThis study is the largest cohort study investigating the impact of gastrointestinal abnormalities in cardiology patients with heterotaxy syndrome.It is the first clinical study to show that HS patients suffer from a wider spectrum of abdominal abnormalities, other than typical malrotation, varying in severity from asymptomatic malrotation to complete non-rotation, namely atresia at multiple intestinal levels. This is relevant in prenatal family counseling but also raises questions regarding the indication of elective Ladd procedure in all heterotaxy syndrome patients, as a different procedure might be indicated.Patient morbidity was investigated from both the cardiology and gastrointestinal point 12 of view.Its main limitations in design is that is not a prospective study. Cardiology records have been reviewed with regards to GI symptomatology, screening investigations and procedures.It involved both reviewing the medical records and directly contacting the family for the subcohort that was below 18 years old and lived in the UK, in an attempt to minimise any data errors. Our records were consistent with the information provided by parents/guardians in all cases but one.

Early- and Middle-Term Surgical Outcomes in Patients with Heterotaxy Syndrome

Cardiology ◽  
2015 ◽  
Vol 133 (3) ◽  
pp. 141-146 ◽  
Author(s):  
Weidan Chen ◽  
Li Ma ◽  
Hujun Cui ◽  
Shengchun Yang ◽  
Yuansheng Xia ◽  
...  
Keyword(s):  
Fontan Procedure ◽  
Univariate Analysis ◽  
Survival Rates ◽  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Heterotaxy Syndrome ◽  
Middle Term ◽  
Palliative Procedure ◽  
Atrial Isomerism

Objectives: Heterotaxy syndrome is a recognized risk factor for surgical cardiac interventions. We evaluated the early- and middle-term results of a surgical intervention for patients with heterotaxy syndrome. Methods: A total of 42 patients with heterotaxy syndrome were enrolled (September 2008 to March 2015). Left and right atrial isomerism were identified in 26% (11 out of 42) and 74% of patients (31 out of 42), respectively. The median age of the patients at the time of surgery was 6.8 months (range: 5 days to 22.3 years). Biventricular repair was completed in 3 patients with left atrial isomerism. Seventeen out of 39 patients who were scheduled for single ventricular repair completed a modified Fontan procedure. Results: The hospital mortality rate was 4.7% (2 out of 42). Another 5 deaths occurred in the remaining survivors following hospital discharge with a follow-up duration of 45.8 ± 23.6 months (range: 13-111 months). The 1-year and 5-year survival rates were 88.1% (37/42) and 83.3% (35/42), respectively. Univariate analysis and multivariate analysis identified pulmonary venous obstruction and atrioventricular valve replacement as additional risk factors for mortality. Conclusions: Right ventricular bypass surgery remains the preferred palliative procedure for patients with heterotaxy syndrome. Based on the current results, the early- and middle-term outcomes are satisfactory.

Surgical Management of Heterotaxy Syndrome: Current Challenges and Opportunities

2020 ◽  
Vol 11 (2) ◽  
pp. 166-176
Author(s):  
Ashlyn M. Alongi ◽  
James K. Kirklin ◽  
Luqin Deng ◽  
Luz Padilla ◽  
Jozef Pavnica ◽  
...  
Keyword(s):  
Risk Factor ◽  
Surgical Management ◽  
Major Risk Factor ◽  
Right Atrial ◽  
Heterotaxy Syndrome ◽  
Cardiac Operation ◽  
Early Repair ◽  
Kaplan Meier ◽  
One Year ◽  
Atrial Isomerism

Introduction: Heterotaxy syndrome presents a unique challenge in surgical management, even in the current era. We hypothesized that certain anatomic subsets merit novel strategies. Methods: We analyzed morphologic details, surgeries, comorbidities, subsequent admissions, and survival using Kaplan-Meier methods and multivariable risk models from a single-institution experience of 103 consecutive patients with heterotaxy who underwent cardiac surgery between January 1, 1990, and May 31, 2016. Results: Of the 103 patients (50 males and 53 females), 31 had left atrial isomerism, 64 had right atrial isomerism (RAI), and 8 patients’ isomerism was indeterminate (IND), with first cardiac operation at a mean 1.0 year (standard deviation ±3.0 years) of age. Kaplan-Meier overall survival estimate was 83.1% at six months, 77.8% at one year, 65.9% at five years, and 52.1% at ten years. Survival was particularly low among RAI following repair of total anomalous pulmonary venous connection (TAPVC) at first operation, with one- and five-year survival of 57% and 46%, respectively. By multivariable analysis, the only risk factor for death during the early phase (hazard model) was repair of TAPVC at the first cardiac operation (hazard ratio [HR]: 4.4, P = .01), and risk factors during the longer term constant phase were atrioventricular valve (AVV) regurgitation (HR: 4.2, P < .01), male gender (HR: 3.7, P < .01), and two-ventricle repair (HR: 3.0, P = .02). Patients with heterotaxy undergoing the Fontan procedure had excellent subsequent survival (85% at ten years). Conclusions: This analysis of over 100 patients with heterotaxy identified TAPVC requiring initial repair as the major risk factor for early death and important AVV regurgitation as the major risk factor in the longer term. Survival with RAI and early repair of TAPVC were poor, with one-year mortality exceeding 40%. Patients with single ventricle completing the Fontan operation enjoyed outstanding ten-year survival (85%). Initial management of RAI requiring early repair of TAPVC remains challenging. For this high-risk subset, alternative strategies such as early referral for cardiac transplantation evaluation warrant consideration.

scholarly journals Inducibility, but not stability, of atrial fibrillation is increased by NOX2 overexpression in mice

2021 ◽  
Author(s):  
Alexandra S Mighiu ◽  
Alice Recalde ◽  
Klemen Ziberna ◽  
Ricardo Carnicer ◽  
Jakub Tomek ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Superoxide Production ◽  
Right Atrial ◽  
Burst Stimulation ◽  
Lv Mass ◽  
Surface Ecg ◽  
Tissue Homogenates ◽  
Genotype Difference ◽  
Left And Right

Abstract Aims Gp91-containing NADPH oxidases (NOX2) are a significant source of myocardial superoxide production. An increase in NOX2 activity accompanies atrial fibrillation (AF) induction and electrical remodelling in animal models and predicts incident AF in humans; however, a direct causal role for NOX2 in AF has not been demonstrated. Accordingly, we investigated whether myocardial NOX2 overexpression in mice (NOX2-Tg) is sufficient to generate a favourable substrate for AF and further assessed the effects of atorvastatin, an inhibitor of NOX2, on atrial superoxide production and AF susceptibility. Methods and results NOX2-Tg mice showed a 2- to 2.5-fold higher atrial protein content of NOX2 compared with wild-type (WT) controls, which was associated with a significant (twofold) increase in NADPH-stimulated superoxide production (2-hydroxyethidium by HPLC) in left and right atrial tissue homogenates (P = 0.004 and P = 0.019, respectively). AF susceptibility assessed in vivo by transoesophageal atrial burst stimulation was modestly increased in NOX2-Tg compared with WT (probability of AF induction: 88% vs. 69%, respectively; P = 0.037), in the absence of significant alterations in AF duration, surface ECG parameters, and LV mass or function. Mechanistic studies did not support a role for NOX2 in promoting electrical or structural remodelling, as high-resolution optical mapping of atrial tissues showed no differences in action potential duration and conduction velocity between genotypes. In addition, we did not observe any genotype difference in markers of fibrosis and inflammation, including atrial collagen content and Col1a1, Il-1β, Il-6, and Mcp-1 mRNA. Similarly, NOX2 overexpression did not have consistent effects on RyR2 Ca2+ leak nor did it affect PKA or CaMKII-mediated RyR2 phosphorylation. Finally, treatment with atorvastatin significantly inhibited atrial superoxide production in NOX2-Tg but had no effect on AF induction in either genotype. Conclusion Together, these data indicate that while atrial NOX2 overexpression may contribute to atrial arrhythmogenesis, NOX2-derived superoxide production does not affect the electrical and structural properties of the atrial myocardium.

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