Surgical Management of Heterotaxy Syndrome: Current Challenges and Opportunities

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.

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Heterotaxy syndrome: This is the left, right?

South African Journal of Radiology ◽

10.4102/sajr.v19i2.902 ◽

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

Cardiology in the Young ◽

10.1017/s1047951121003620 ◽

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.

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The right atrial diameter as a risk factor predicting recurrence of atrial fibrillation after catheter ablation at mid-term follow-up

10.21203/rs.3.rs-23323/v1 ◽

2020 ◽

Author(s):

Wang Xiaofei ◽

Wang Wenli ◽

Zou Cao

Keyword(s):

Risk Factor ◽

Independent Risk Factor ◽

Cox Regression ◽

Survival Curve ◽

Right Atrial ◽

Cutoff Value ◽

Kaplan Meier ◽

Significant Difference ◽

Meier Survival Curve

Abstract Background Left atrial diameter (LAD) has been confirmed to predict recurrence of atrial fibrillation (AF) after catheter ablation (CA). The influence of right atrium (RA) size on the prognosis after CA was relatively unclear and lack of research. The objective of the present study was to investigate the relationship between right atrial diameter (RAD) and the mid-term outcome of AF after CA. Methods This study retrospectively examined 121 patients who underwent initial CA for symptomatic AF. Cox regression model was used to find risk factors of recurrence. Receiver operating characteristic (ROC) curve was used to evaluate predictive power and determine clinic cutoff value. Kaplan-Meier survival curve and log-rank test were used to analyze success rate. Results There were 94 (77.7%) patients of freedom from AF after 24.2 ± 4.5 months’ follow-up. Multivariate Cox regression analysis showed both hypertension and RAD were independent risk factors of arrhythmia recurrence after ablation regardless of AF type (HR: 4.915; 95% CI: 1.370-17.635; P = 0.015 and HR: 1.059; 95% CI: 1.001–1.120; P = 0.045, respectively). However, in patients with paroxysmal AF (par-AF), Multivariate analysis showed RAD become the only independent risk factor (HR: 1.031; 95% CI: 1.016–1.340; P = 0.029). ROC curve demonstrated the cutoff value of RAD was 35.5 mm with an area under the curve (AUC) of 0.715 (95% CI: 0.586–0.843, P = 0.009), sensitivity of 81.3% and specificity of 54.2%. Kaplan-Meier survival curve showed significant difference of freedom from par-AF (67.5 vs. 91.4%, log-rank, P = 0.015) between patients with RAD ≥ 35.5 mm and < 35.5 mm in this subgroup. Nevertheless, in patients with persistent AF (per-AF), no risk factor of arrhythmia recurrence was found. In addition, Kaplan-Meier survival curve showed no significant difference of freedom from per-AF (69.7 vs. 87.5%, log-rank, P = 0.31) between patients with RAD ≥ 35.5 mm and < 35.5 mm. Conclusions RAD was the independent risk factor predicting recurrence of AF after CA only in patients with par-AF. In patients with RAD < 35.5 mm, there was a significantly higher freedom from par-AF recurrence compared with RAD ≥ 35.5 mm after a mid-term follow-up.

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

10.1101/2020.05.24.20112078 ◽

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.

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Improving Outcomes of the Surgical Management of Right Atrial Isomerism

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2011.05.110 ◽

2012 ◽

Vol 93 (3) ◽

pp. 832-839 ◽

Cited By ~ 32

Author(s):

Noritaka Ota ◽

Yoshifumi Fujimoto ◽

Masaya Murata ◽

Yuko Tosaka ◽

Yujiro Ide ◽

...

Keyword(s):

Surgical Management ◽

Right Atrial ◽

Atrial Isomerism

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

Cardiology ◽

10.1159/000440947 ◽

2015 ◽

Vol 133 (3) ◽

pp. 141-146 ◽

Cited By ~ 7

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.

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Apex rotation as a risk factor for total anomalous pulmonary connection repair in single ventricle

10.22541/au.162127822.20134393/v1 ◽

2021 ◽

Author(s):

Hidetsugu Asai ◽

Yasushige Shingu ◽

Jin Ikarashi ◽

Yuchen Cao ◽

Daisuke Takeyoshi ◽

...

Keyword(s):

Risk Factor ◽

Vertebral Body ◽

Single Ventricle ◽

Right Atrial ◽

Pulmonary Venous Obstruction ◽

Sutureless Technique ◽

High Incidence ◽

Atrial Isomerism ◽

Anomalous Pulmonary Venous Connection

Background: The high incidence of postoperative pulmonary venous obstruction (PVO) is a major mortality-associated concern in patients with right atrial isomerism and extracardiac total anomalous pulmonary venous connection (TAPVC). We evaluated new anatomical risk factors for reducing the space behind the heart after TAPVC repair. Methods: 18 patients who underwent TAPVC repair between 2014 and 2020 were enrolled. Sutureless technique was used in 12 patients and conventional repair in six patients. The angle between the line perpendicular to the vertebral body and that from the vertebral body to the apex was defined as the “vertebral-apex angle (V-A angle).” The ratio of post- and preoperative angles, indicating the apex’s lateral rotation, was compared between patients with and without PVO. Results: The median (interquartile range) age and body weight at repair were 102 (79-176) days and 3.8 (2.6-4.8) kg, respectively. The 1-year survival rate was 83% (median follow-up, 29 [11-36] months). PVO occurred in seven patients (39%), who showed an obstruction of one or two branches in the apex side. The postoperative V-A angle (46° [45°-50°] vs. 36° [29°-38°], P = 0.001) and the ratio of post- and preoperative V-A angles (1.27 [1.24-1.42] vs. 1.03 [0.98-1.07], P = 0.001) were significantly higher in the PVO group than in the non-PVO group. The cut-off values of the postoperative V-A angle and ratio were 41° and 1.17, respectively. Conclusions: A postoperative rotation of the heart apex into the ipsilateral thorax was a risk factor for branch PVO after TAPVC repair.

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One-year change in plasma volume and mortality in the Japanese general population: An observational cohort study

PLoS ONE ◽

10.1371/journal.pone.0254665 ◽

2021 ◽

Vol 16 (7) ◽

pp. e0254665

Author(s):

Yoichiro Otaki ◽

Tetsu Watanabe ◽

Tsuneo Konta ◽

Masafumi Watanabe ◽

Koichi Asahi ◽

...

Keyword(s):

Risk Factor ◽

General Population ◽

Plasma Volume ◽

Additional Risk Factor ◽

Plasma Volume Expansion ◽

C Statistic ◽

Kaplan Meier ◽

Observational Cohort ◽

Cox Proportional Hazard Regression ◽

One Year

Background Changes in plasma volume, a marker of plasma volume expansion and contraction, are gaining attention in the field of cardiovascular disease because of its role in the prevention and management of heart failure. However, it remains unknown whether a 1-year change in plasma volume is a risk factor for all-cause, cardiovascular, and non-cardiovascular mortality in the general population. Methods and results We used a nationwide database of 134,291 subjects (age 40–75 years) who participated in the annual “Specific Health Check and Guidance in Japan” check-up for 2 consecutive years between 2008 and 2011. A 1-year change in plasm volume was calculated using the Strauss–Davis-Rosenbaum formula. There were 220 cardiovascular deaths, 1,001 non-cardiovascular deaths including 718 cancer deaths, and 1,221 all-cause deaths during the follow-up period of 3.9 years. All subjects were divided into quintiles based on the 1-year change in plasma volume. Kaplan–Meier analysis demonstrated that the highest 5th quintile had the greatest risk among the five groups. Multivariate Cox proportional hazard regression analysis demonstrated that a 1-year change in plasma volume was an independent risk factor for all-cause, cardiovascular, non-cardiovascular, and cancer deaths. The addition of a 1-year change in plasma volume to cardiovascular risk factors significantly improved the C-statistic, net reclassification, and integrated discrimination indexes. Conclusions Here, we have demonstrated for the first time that a 1-year change in plasma volume could be an additional risk factor for all-cause, cardiovascular, and non-cardiovascular (mainly cancer) mortality in the general population.

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