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 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.

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 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.

scholarly journals The impact of left atrial strain parameters on systolic and diastolic improvement following TAVI

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
B Vattay ◽  
A I Nagy ◽  
A Apor ◽  
M Kolossvary ◽  
A Manouras ◽  
...  
Keyword(s):  
Left Atrial ◽  
Prognostic Value ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Term Survival ◽  
Lv Mass ◽  
Reverse Remodelling ◽  
The Impact

Abstract Introduction Transcatheter aortic valve implantation (TAVI) can improve left ventricular (LV) mechanics and has been shown to improve long term survival. Data on the prognostic value of left atrial (LA) strain following TAVI are scarce. LA strain – a surrogate of LV filling pressure - can aid the early detection of diastolic dysfunction and correlates with the extent of fibrosis in atrial remodelling. Purpose In this multimodality study, we aimed to evaluate the prognostic value of LA function measured before hospital discharge following TAVI and to further elucidate its association with LV and LA reverse remodelling. Methods In this prospective single center study, we investigated 90 patients (mean age 78.5 years, 46.7% female) with severe, symptomatic aortic stenosis (AS) who underwent transthoracic echocardiography immediately after TAVI and 6 months later. LA and LV global longitudinal strain parameters were obtained by speckle tracking echocardiography. CT angiography (CTA) was performed for pre-TAVI planning and repeated at 6 months follow-up. LV mass values were derived from the serial CTA images. We defined LV reverse remodelling as reduction of myocardial mass quantified on CTA and as an improvement of LV global longitudinal strain (GLS). LA reverse remodelling was assessed based on the peak reservoir strain values (LAGS). The association of LA and LV global strain parameters, LA stiffness, systolic and diastolic functional parameters and LV mass based reverse remodelling were analysed using Pearson correlation coefficient and linear regression models. Results The mean LAGS and LVGLS values were 17.7% and 15.3% at discharge and 20.2% and 16.6% at follow-up, respectively (p=0.024, p&lt;0.001). LA and LV strain values improved in 60.6% and 74.5% of all patients. Reduced LAGS (&lt;20%) was found in 66.7% of all patients at baseline. LA strain at discharge correlated significantly with diastolic parameters (E wave, E/e', LAVI, all p&lt;0.05). Atrial reverse remodelling based on LAGS change correlated with LVGLS change (p&lt;0.01, standardized β=0.53) and LAGS at discharge (p=0.012, standardized β=−0.30). LAGS correlated with the extent of morphological LV remodelling based on LV mass reduction (p=0.002, coeff: 0.36). Elevated LA stiffness at discharge (upper tercile) leads to substantially lower LAGS at 6 months versus patients with lower LA stiffness value (1. and 2. tercile): 16.4±10.0 vs 21.9±9.8, p=0.042. Conclusion Patients with reduced LAGS immediately after TAVI showed a larger extent of LV reverse remodelling during follow up. On the other hand, increased LA stiffness at discharge was consistent with irreversible LA damage as demonstrated by a lack of improvement in LA function. FUNDunding Acknowledgement Type of funding sources: None.

Abstract 14107: Early Right Ventricular Reverse Remodeling Predicts Long-term Survival After Isolated Tricuspid Valve Surgery

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Sri Harsha Patlolla ◽  
Hartzell V Schaff ◽  
Joseph A Dearani ◽  
Kevin L Greason ◽  
Alberto Pochettino ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tricuspid Valve ◽  
Cox Regression ◽  
Multivariable Analysis ◽  
Right Atrial ◽  
Reverse Remodeling ◽  
Term Survival ◽  
Cox Regression Analysis ◽  
The Impact ◽  
Rv Function

Introduction: There are limited data on the impact of isolated tricuspid valve (TV) surgery on recovery of right ventricular (RV) function and RV reverse remodeling. Methods: Among 223 patients who had isolated TV procedures between 2001 and 2017, 60 (27%) underwent TV repair and 163 (73%) received TV replacement. RV reverse remodeling was assessed by echocardiography at a median of 11.3 months (IQR 5.9-13.5) post dismissal. Kaplan-Meier analysis and the log-rank test were used to estimate and create survival curves. Cox proportional hazards analysis was performed to study the association of clinical and echocardiographic parameters with mortality. Results: Mean age was 67.3 ± 13.7 years, and 57% were female. Indication for surgery was functional TR in 64% (n=143) patients. Overall 30-day mortality was 2.7%. After a median follow-up period of 9.5 years (IQR 3.6-12.9) there were a total of 122 (54.7%) deaths. Adjusted Cox regression analysis revealed comparable survival for TV repair and replacement and identified older age, and presence of RV dysfunction (HR 1.84, 95% CI 1.14-2.98; P=0.01) as independent predictors of poor survival. Impact of early RV reverse remodeling on survival after TV surgery was evaluated in 90 patients; In 39 RV function and/or RV size recovered, but in 51 RV dysfunction and/or RV enlargement persisted or worsened. Patients who exhibited RV reverse remodeling had significantly improved survival compared to those who did not (Log-Rank P=0.005) and reverse remodeling was independently associated with improved survival (HR 0.42, 95% CI 0.24-0.74; P=0.003). In a multivariable analysis lower preoperative right atrial pressure (OR 0.83, 95% CI 0.73-0.94; P=0.004) was an independent predictor of early right heart reverse remodeling. Conclusions: Isolated TV surgery can be performed with acceptable outcomes, and overall survival is best in patients who receive the operation before developing severe RV systolic dysfunction. Adjusted survival was similar for patients undergoing TV repair or replacement. Reverse remodeling of RV during the first 18 months after surgery appears to provide survival benefit. Preoperative RA pressure is an independent determinant of postoperative RV remodeling.

scholarly journals Left and right atrial appendage functional features as predictors for voltage-defined left atrial remodelling in patients with long-standing persistent atrial fibrillation

2021 ◽  
Author(s):  
Radoslaw Marek Kiedrowicz ◽  
Maciej Wielusinski ◽  
Andrzej Wojtarowicz ◽  
Jaroslaw Kazmierczak
Keyword(s):  
Atrial Fibrillation ◽  
Left Atrial ◽  
Low Voltage ◽  
Regional Distribution ◽  
Persistent Atrial Fibrillation ◽  
Atrial Appendage ◽  
Right Atrial ◽  
Functional Features ◽  
The Impact ◽  
Flow Velocities

AbstractIt was hypothesised that left atrial (LA) fibrosis identified by the presence of low-voltage areas (LVA) may influence the mechanical and electrical function of the left (LAA) and right (RAA) atrial appendage among the long-standing persistent atrial fibrillation (LSPAF) population. 140 consecutive patients underwent voltage mapping of LA with a multielectrode catheter following pulmonary vein isolation and restoration of sinus rhythm with cardioversion. Echocardiography determined LAA peak outflow and inflow velocities and intracardiac catheter-based mean LAA and RAA AF cycle length (AFCL) were obtained during AF before ablation. The impact of flow velocities and AFCL on the prevalence and location of LVA was further evaluated. LVA were detected in 54% of the patients. 14% of the patients presented severe global LVA burden > 20% of the total LA surface area. 29% of the patients presented a disseminated pattern of remodelling as 3 out of 5 LA segments were affected. LAA AFCL, RAA AFCL, LAA flow velocities did not predict the absolute presence of LVA. However LAA AFCL > 155 ms predicted disseminated LVA pattern and LAA AFCL > 165 ms severe LVA incidence. LAA AFCL > 155 ms was predictive for existence of LVA within antero-septal LA segments whilst LAA emptying velocity ≤ 0.2 m/s within lateral wall. Moreover RAA AFCL > 165 ms was strongly related to the presence of LAA AFCL > 15 ms and > 165 ms. LAA and RAA functional assessment was predictive of the presence of advanced stages of voltage-defined LA fibrosis and its regional distribution among LSPAF population

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