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.

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

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.

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 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 Mid-term results of modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection

2020 ◽  
Vol 58 (6) ◽  
pp. 1261-1268
Author(s):  
Zicong Feng ◽  
Yang Yang ◽  
Fengpu He ◽  
Kunjing Pang ◽  
Kai Ma ◽  
...  
Keyword(s):  
Propensity Score ◽  
Multivariable Analysis ◽  
Pulmonary Venous Obstruction ◽  
Kaplan Meier ◽  
Incision Technique ◽  
Atrial Isomerism ◽  
Anomalous Pulmonary Venous Connection ◽  
Mid Term Results ◽  
Acceptable Outcome

Abstract OBJECTIVES Surgical outcomes of supracardiac total anomalous pulmonary venous connection (TAPVC) repair by the posterior technique (PT) remain unsatisfactory. This study aimed to compare the outcomes of the modified L-shaped incision technique with the PT for supracardiac TAPVC repair. METHODS From January 2009 to December 2019, 121 consecutive patients with supracardiac TAPVC undergoing surgical repair in our institution were included (L-group, n = 53; PT group, n = 68). A propensity score-matched analysis was performed. Patients with single-ventricle physiology or atrial isomerism were excluded. All clinical data were retrospectively analysed. RESULTS In the unmatched cohort, the median follow-up duration was 33 months (interquartile range 26–65 months). There were 5 operative mortalities (4.1%) and 12 late mortalities (9.9%). Postoperative pulmonary venous obstruction (PVO) was documented in 21 patients. After matching (52 pairs), the overall survival rate in the L-group was 88.2% at both 3 and 5 years. For the propensity score-matched patients with preoperative PVO (n = 20), statistically significant differences (P = 0.002) were found by Kaplan–Meier curves with freedom from death and postoperative PVO at 1 and 3 years of 100% and 85.7% [standard deviation (SD): 13.2%] in the L-group and 90% (SD: 9.5%) and 22.9% (SD: 14.1%) in the PT group, respectively. Multivariable analysis revealed that the use of the PT was an independent risk factor for death and postoperative PVO (hazard ratio 4.12, 95% confidence interval 1.12–15.16; P = 0.03). CONCLUSIONS The modified L-shaped incision technique provided an acceptable outcome for supracardiac TAPVC repair. Compared with PT, the modified L-shaped incision technique was significantly associated with decreased death and postoperative PVO in patients with obstructed supracardiac TAPVC.

scholarly journals Impact of intrapulmonary-artery septation to pulmonary vein obstruction for two-lung Fontan

2020 ◽  
Vol 58 (1) ◽  
pp. 177-185
Author(s):  
Motonori Ishidou ◽  
Keisuke Ota ◽  
Kentaro Watanebe ◽  
Hiroshi Koshiyama ◽  
Kazuyoshi Kanno ◽  
...  
Keyword(s):  
Pulmonary Circulation ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Pulmonary Venous Obstruction ◽  
Operative Outcomes ◽  
Group 12 ◽  
Lung Area ◽  
Pulmonary Vein Obstruction ◽  
Significant Factors

Abstract OBJECTIVES Patients with unbalanced pulmonary artery (PA) growth and decreased unilateral pulmonary circulation are considered unsuitable candidates for the Fontan procedure. Following our previous study on the utility of intrapulmonary-artery septation for patients with PA hypoplasia, we investigated its use in patients with pulmonary venous obstruction (PVO). METHODS We recruited 42 patients who underwent intrapulmonary-artery septation for unilateral PA hypoplasia and/or PVO between 1998 and 2018 and classified them into no PVO or PVO group. We analysed overall survival, success of the Fontan procedure and data from catheterization and echocardiography. In PVO, we evaluated the functional lung area before the Fontan procedure and the relevance of this parameter to operative outcomes. RESULTS The PVO and no-PVO group included 24 and 18 patients, respectively. One patient in the no-PVO and 6 patients in the PVO group died during follow-up; this difference was statistically significant (log-rank P = 0.040). In the no-PVO group, 15 (83%) patients achieved two-lung Fontan circulation. In the PVO group, 12 (50%) patients achieved two-lung Fontan circulation. Multivariate analysis revealed that functional lung area and shunt size [significantly larger in patients with functional lung area ≥50% in affected lung (P = 0.040)] were significant factors for successful two-lung Fontan procedure (P = 0.030). CONCLUSIONS Intrapulmonary-artery septation may contribute to increase functional lung area after PVO release to establish two-lung Fontan circulation in patients with PVO who have unbalanced PA growth and/or decreased unilateral pulmonary circulation.

scholarly journals P03.02 Risk of leptomeningeal dissemination in patients treated with postoperative stereotactic radiotherapy of brain metastases

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii24-iii25
Author(s):  
A Mousli ◽  
B Bihin ◽  
T Gustin ◽  
G Koerts ◽  
M Mouchamps ◽  
...  
Keyword(s):  
Brain Metastases ◽  
Stereotactic Radiotherapy ◽  
Univariate Analysis ◽  
Survival Rates ◽  
Safety Margin ◽  
Incidence Rates ◽  
Leptomeningeal Dissemination ◽  
En Bloc ◽  
Tumor Dissemination

Abstract Background There is a body of evidence that the risk of leptomeningeal dissemination (LMD) is increased in the postoperative stereotactic radiotherapy (SRT) of brain metastases (BM) compared to adjuvant whole brain radiotherapy (WBRT). The proposed mechanism is an iatrogenic tumor dissemination into the cerebrospinal fluid at time of surgery. Including a wider volume of meningeal wall and the entire surgical track in the definition of the postoperative SRT clinical target volume (CTV) to decrease LMD is still controversial. The aim of this study was to retrospectively analyze the outcome of adjuvant SRT targeted at resection cavities of BM without previous WBRT. MATERIAL / METHODS We reviewed 70 patients treated with postoperative SRT for BM. Stereotactic planning computed tomography and planning MRI were imported into iPlan RT image software for image registration and TV delineation. The CTV consisted of any residual enhancement and all resected cavity including a safety margin of 1 to 2 mm. Only in cases of superficial initial tumor with meningeal contact was the CTV enlarged to the adjacent meningeal wall, but never included edema or the entire surgical track. Patients underwent regular follow-up MRI. The cumulative incidence rates of LMD was retrospectively calculated as well as patterns of failure. RESULTS The most common histological type was non small cell lung cancer in 61.4%. There were 38.6% infratentorial locations and 37.2 % superficial lesions. En bloc resection was achieved in 60% and compete resection in 75.7%. After a median imaging follow up time of 16.7 months, 54.3% of patients experienced distant brain failure. LMD occurred in 9 of 70 patients (12.9 %) at a median time of 10.7 months. Survival without LMD was 88% at 1 year (IC 95% 79%-97%) and 82% at 2years (IC 95% 72%-94%). In three quarter of cases, LMD interested superficial lesions. In univariate analysis, survival rates without LMD at 1 year for superficial and deep lesions were 88 % and 94 %, respectively (p=0.49). We report only one recurrence in the surgical track (1.42%). CONCLUSION The risk of LMD was comparable to the literature (11–17%). Superficial lesions were slightly more likely to relapse in the meninges, but it was non-significant. The risk of recurrence in the surgical track is negligible. Our results do not support the current guidelines recommending the systematic inclusion of the surgical track and the related meninges in the CTV.

Sarcoligo: Impact of local ablative treatment of oligometastatic sarcomas on overall survival.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10042-10042
Author(s):  
Juliette Thariat ◽  
Laurence Moureau-Zabotto ◽  
Nicolas Penel ◽  
Antoine Italiano ◽  
Jacques-Olivier Bay ◽  
...  
Keyword(s):  
Overall Survival ◽  
Lung Metastases ◽  
Univariate Analysis ◽  
Survival Rates ◽  
Locoregional Treatment ◽  
Metastatic Sites ◽  
The Impact

10042 Background: 40-50% of sarcomas become metastatic. Median survival of metastatic patients has improved over time. The probably multifactorial reasons for such improvement are not fully clear. Noteworthy, for patients with a controlled primary and a limited number of lung metastases, complete resection of their metastases yields survival rates of up to 40% at three years. Advances in surgery, radiotherapy and radiofrequency have fostered the use of local treatments for various metastatic sites (lung, liver, spine...). Methods: A multicentric retrospective study of the Groupe Sarcome Francais (GSF-GETO); approved by the nationally-review board and ethical committee, was conducted to assess the impact of local ablative treatment on overall survival. Patients who had had oligometastases (any site, 1-5 synchronous metastases) at diagnostic or during the course of disease between 2000 and 2010 were included. Results: Median age of the 243 oligometastatic sarcoma patients was 53 years-old (11-86). Patients had grade I, II and III in 7.5%, 29.6% and 63.3% of cases, respectively with various histologies. 69% of patients underwent local ablative treatment of metastases. Median follow-up was 59 months (4-212) for living patients. Median overall survival was 51 months (1-348). On univariate analysis, grade, histology, absence of chemotherapy, local ablative treatment (surgery, irradiation, radiofrequency or chemoembolisation) correlated with survival but not age or site of oligometastasis. On multivariate analyses, grade (hazard ratio HR 0.12 [CI95 0.3-0.6]) and local ablative treatment (HR 3.8 [CI95 2.1-7.1]) remained significant. Conclusions: Local ablative treatment of metastases is associated with better survival in sarcoma patients with oligometastatic disease. The role of the locoregional treatment of metastases and its impact on quality of life should be assessed prospectively.

scholarly journals Adult Granulosa Cell Tumors of the Ovary: A Retrospective Study of 36 FIGO Stage I Cases with Emphasis on Prognostic Pathohistological Features

2018 ◽  
Vol 2018 ◽  
pp. 1-11
Author(s):  
Emina Babarović ◽  
Ivan Franin ◽  
Marko Klarić ◽  
Ani Mihaljević Ferrari ◽  
Ružica Karnjuš-Begonja ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Univariate Analysis ◽  
Survival Rates ◽  
Disease Free Survival ◽  
Figo Stage ◽  
Free Survival ◽  
Granulosa Cell Tumors ◽  
Diffuse Growth ◽  
Disease Free

Objective. Adult granulosa cell tumors (AGCTs) represent 2%–5% of all ovarian malignancies. The aim of this study was to analyze clinical and pathohistological parameters and their impact on recurrence, overall, and disease-free survival in FIGO stage I AGCT patients. Methods. The tumor specimens analyzed in this retrospective study were obtained from a total of 36 patients with diagnosis of ovarian AGCT surgically treated at the Department of Gynecology, Rijeka University Hospital Centre, between 1994 and 2012. Clinical, pathological, and follow-up data were collected. Results. The mean age at diagnosis was 54.5 years with a range of 24–84. The majority of the patients, 30 (83%), were in FIGO stage IA, 3 (8%) in stage IC1, 1 (3%) in stage IC2, and 2 (6%) in stage IC3. During follow-up period (median 117.5 months, range 26–276), recurrence occurred in 4 patients (12%) with 2 deaths of the disease recorded. In univariate analysis, the 5-year survival rates were significantly shorter in patients with FIGO substage IC (p=0.019), with positive LVSI (p=0.022), with presence of necrosis (p=0.040), and with hemorrhage (p=0.017). In univariate analysis, the 5-year disease-free survival rates were significantly shorter in patients treated with fertility surgery (p=0.004), with diffuse growth pattern (p=0.012), with moderate and severe nuclear atypia (p=0.032), and with presence of hemorrhage (p=0.022). FIGO substage IC proved to be independent predictor for recurrence (OR = 16.87, p=0.015, and OR = 23.49, p=0.023, resp.) and disease-free survival (p=0.0002; HR 20.84, p=0.02) at the uni- and multivariate analyses. Conclusions. FIGO substage IC is predictive of recurrence and disease-free survival in patients with early-stage AGCTs. LVSI, presence of necrosis and hemorrhage, diffuse growth pattern, and nuclear atypia in AGCTs seem to be associated with overall and disease-free survival, so these pathological features should be taken into consideration when managing patients with AGCT.

Systemic ventricle morphology impact on ten-year survival after Fontan surgery

2018 ◽  
Vol 26 (9) ◽  
pp. 677-684 ◽  
Author(s):  
Mira Fauziah ◽  
Oktavia Lilyasari ◽  
Lies Dina Liastuti ◽  
Budi Rahmat
Keyword(s):  
Survival Rate ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Survival Rates ◽  
Univentricular Heart ◽  
Thromboembolic Events ◽  
Term Survival ◽  
Systemic Ventricle ◽  
The Impact

Background The Fontan procedure has been applied in many patients with single-ventricle physiology, with quite low mortality rates all over the world, and a 8.6%–9.1% rate in Indonesia. Structural and characteristic differences in the morphologically left and right ventricles influence the role of the systemic ventricle in the functionally univentricular heart and impact on postoperative outcomes. Mid- and long-term survival based on systemic ventricle morphology remains controversial. This study aimed to investigate the impact of systemic ventricle morphology on ten-year survival after the Fontan procedure. Methods The 162 patients who underwent a Fontan operation at our institution between 2008 and February 2018 and survived to discharge were reviewed and followed up until March 2018. Data were extracted from the registry and pediatric surgical conferences, medical records, surgery reports, echocardiography and catheterization reports, and follow-up to the end of the study period. Median follow-up was 26.5 months (range 10.75–54 months). Results The patients were divided into 2 groups based on systemic ventricle morphology. Seventy-four patients were included in the left morphology group and 88 in the right morphology group. There was no difference in 10-year survival rates between the two type of systemic ventricle morphology. Postoperative thromboembolic events influenced the 10-year survival rate after the Fontan procedure (hazard ratio 4.84, 95% confidence interval: 1.26–18.55, p = 0.021). Conclusion Systemic ventricle morphology was not associated with the 10-year survival rate after the Fontan procedure. Postoperative thromboembolic events accounted for a 4.84-times higher mortality risk after the Fontan procedure.

Masked gradient of infundibular stenosis in right atrial isomerism with pulmonary venous obstruction

1994 ◽  
Vol 73 (11) ◽  
pp. 829-831 ◽  
Author(s):  
Jou-Kou Wang ◽  
Hung-Chi Lue ◽  
Ing-Sh Chiu ◽  
Mei-Hwan Wu ◽  
Chung-I Chang
Keyword(s):  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Venous Obstruction ◽  
Infundibular Stenosis ◽  
Atrial Isomerism
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