Heterotaxy Syndrome with Polysplenism and Left Atrial Isomerism

2021 ◽  
Author(s):  
Jordan See ◽  
E. Colin Daingerfield ◽  
Michael A. Santos
Keyword(s):  
Left Atrial ◽  
Heterotaxy Syndrome ◽  
Atrial Isomerism

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.

scholarly journals Complicated acute appendicitis in a child with left atrial isomerism: a case report

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Faysal Tabbara ◽  
Karim Wehby Ataya ◽  
Youssef Annous ◽  
Kawsar Alamiddine ◽  
Ahmad Zaghal
Keyword(s):  
Acute Appendicitis ◽  
Left Atrial ◽  
High Index ◽  
Heterotaxy Syndrome ◽  
Lower Quadrant Pain ◽  
Lower Quadrant ◽  
Trocar Placement ◽  
Atypical Presentations ◽  
Atrial Isomerism ◽  
High Index Of Suspicion

Abstract Background Heterotaxy syndrome is a rare clinical entity that is characterized by abnormal visceral organ arrangement in the chest and abdomen. Left atrial isomerism is a subcategory of heterotaxy syndrome characterized by the presence of multiple spleens with or without cardiac anomalies. Patients may remain asymptomatic their whole lives until they are diagnosed incidentally. Given that patients with left atrial isomerism might demonstrate atypical presentations of acute intrabdominal pathologies, it is important to keep a high index of suspicion when encountering such cases. Case presentation In this report, we present a case of a 9-year-old boy with a known history of left atrial isomersim who presented with left lower quadrant pain and positive McBurney, psoas, and obturator signs on the left side. A computed tomography scan of the abdomen confirmed the diagnosis of perforated acute appendicitis on the left, which prompted an emergent laparoscopic appendectomy. Conclusion Our case highlights the importance of keeping a high index of suspicion for a heterotaxy syndrome that is complicated by acute appendicitis in pediatric patients presenting with vague abdominal pain. Planning the location of trocar placement in patients with situs anomalies is of paramount importance to avoid technical difficulties in laparoscopic procedures.

scholarly journals Successful Palliation via Kawashima Procedure of an Infant With Heterotaxy Syndrome and Left-Atrial Isomerism

2018 ◽  
Vol 18 (4) ◽  
pp. 406-412
Author(s):  
Hannah Lively-Endicott ◽  
Diego A. Lara
Keyword(s):  
Left Atrial ◽  
Heterotaxy Syndrome ◽  
Atrial Isomerism

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

‘Sinus node’ dysfunction associated with left atrial isomerism

2008 ◽  
Vol 9 (9) ◽  
pp. 953-956 ◽  
Author(s):  
Paolo Ferrero ◽  
Riccardo Massa ◽  
Claudia Amellone ◽  
Giampaolo Trevi
Keyword(s):  
Left Atrial ◽  
Sinus Node ◽  
Sinus Node Dysfunction ◽  
Atrial Isomerism

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.

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