Pulmonary vein and cavotricuspid isthmus ablation in situs inversus totalis.
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ABSTRACT:
SUBMITTER: Benjamin MM
PROVIDER: S-EPMC4736523 | biostudies-other | 2016 Feb
REPOSITORIES: biostudies-other
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Project description:BackgroundSitus inversus totalis (SIT) is a rare condition, where all the organs in the body are mirrored. Atrial fibrillation occurs in patients with SIT. We describe the case of pulmonary vein isolation (PVI) in SIT.Case summaryA patient with atrial fibrillation was referred to our hospital due to palpitations. Diagnosis of atrial fibrillation was made by electrocardiogram. The patient reported to have a SIT that was confirmed. Meticulous preparation was done including a three-dimensional model and radiofrequency PVI was performed successfully. No recurrence of atrial fibrillation was detected until last follow-up 2 years after PVI.DiscussionPulmonary vein isolation in SIT can be performed successfully and with excellent long-term result.
| S-EPMC10858676 | biostudies-literature
Project description:Introduction and significanceSitus inversus totalis is characterized by the complete transposition of thoracic and abdominal viscera. Individuals can live asymptomatically with this condition; however, it may be associated with certain abnormalities of the organs involved.Case presentationHerein, we present a case of a situs inversus totalis woman presented with choledocholithiasis. Elective laparoscopic cholecystectomy was performed on the patient with intraoperative modifications. The patient was discharged in a healthy condition.Clinical discussionSeveral case reports have documented the typical presentation of cholelithiasis, which is characterized by pain in the left upper quadrant and epigastric region. Our patient exhibited similar symptoms and was diagnosed with choledocholithiasis.ConclusionsDiagnostic and therapeutic management of morbidities in SIT individuals can be performed with recommended modifications that can lead to favorable outcomes.
| S-EPMC11332169 | biostudies-literature
Project description:We investigated the cause of situs inversus totalis (SIT) in two siblings from a consanguineous family. Genotyping and whole-exome analysis revealed a homozygous change in NME7, resulting in deletion of an exon causing an in-frame deletion of 34 amino acids located in the second NDK domain of the protein and segregated with the defective lateralization in the family. NME7 is an important developmental gene, and NME7 protein is a component of the γ-tubulin ring complex. This mutation is predicted to affect the interaction of NME7 protein with this complex as it deletes the amino acids crucial for the binding. SIT associated with homozygous deletion in our patients is in line with Nme7(-/-) mutant mice phenotypes consisting of congenital hydrocephalus and SIT, indicating a novel human laterality patterning role for NME7. Further cases are required to elaborate the full human phenotype associated with NME7 mutations.
| S-EPMC6066188 | biostudies-literature
Project description:Situs inversus totalis is a rare condition where the visceral organs are organized as a mirror image of default organ position. In this study we picture the co-development between brain and visceral organs in a case of situs inversus totalis from a fetal stage to adolescence and compare our findings to an age-, gender-, and education-matched control with normal position of thoracic and abdominal organs. We show that in this case of situs inversus, functional and structural brain lateralization do not coincide with visceral organ situs. Furthermore, cognitive development in situs inversus is normal. To our knowledge, this is the first report of antenatal cerebral origins of structural and functional brain asymmetry in a case of situs inversus totalis.
| S-EPMC6192509 | biostudies-literature
Project description:BackgroundSitus inversus totalis (SIT) is an uncommon disorder characterized by mirror-image anatomy, which can present unique challenges and potential vascular anomalies in surgical interventions, particularly in gastric cancer patients.AimsWe aim to delineate a rare case of gastric adenocarcinoma in a SIT patient and conduct a thorough review of the existing literature concerning surgical strategies, vascular anomalies, and outcomes observed across varied geographic locales and technological approaches.MethodsA thorough examination of a case involving a 39-year-old male SIT patient who underwent a successful distal gastrectomy with D2 lymph node dissection is presented alongside an expansive literature review. The review encompasses 47 articles, collating data on surgical approaches and vascular anomalies across 49 patients diagnosed with SIT and gastric cancer.ResultsThe patient underwent curative distal gastrectomy and Billroth II with Braun anastomosis within 95 minutes, incurring minimal intraoperative blood loss (100ml). Postoperative pathology confirmed moderately to poorly differentiated gastric adenocarcinoma (pT3N0M0), with no signs of recurrence or metastasis after 6 months of S-1 adjuvant chemotherapy. The literature review revealed vascular anomalies in approximately 20% of reported cases, accentuating its surgical significance. Noteworthy variations in surgical strategies, operative times, blood loss, and complications across different surgical modalities were observed, providing a comprehensive view into the practical management of such cases.ConclusionDespite the inherent challenges associated with SIT, various surgical techniques can be successfully applied with meticulous preoperative planning and understanding vascular anomalies. This compilation of diverse surgical experiences across numerous documented cases seeks to provide a consolidated resource for refining surgical strategies and enhancing postoperative outcomes for gastric cancer patients with SIT, underscoring the imperativeness of further research in this niche domain.
| S-EPMC10635521 | biostudies-literature
Project description:BackgroundMotile cilia dysfunction causes primary ciliary dyskinesia (PCD), situs inversus totalis (SI), and a spectrum of laterality defects, yet the prevalence of laterality defects other than SI in PCD has not been prospectively studied.MethodsIn this prospective study, participants with suspected PCD were referred to our multisite consortium. We measured nasal nitric oxide (nNO) level, examined cilia with electron microscopy, and analyzed PCD-causing gene mutations. Situs was classified as (1) situs solitus (SS), (2) SI, or (3) situs ambiguus (SA), including heterotaxy. Participants with hallmark electron microscopic defects, biallelic gene mutations, or both were considered to have classic PCD.ResultsOf 767 participants (median age, 8.1 years, range, 0.1-58 years), classic PCD was defined in 305, including 143 (46.9%), 125 (41.0%), and 37 (12.1%) with SS, SI, and SA, respectively. A spectrum of laterality defects was identified with classic PCD, including 2.6% and 2.3% with SA plus complex or simple cardiac defects, respectively; 4.6% with SA but no cardiac defect; and 2.6% with an isolated possible laterality defect. Participants with SA and classic PCD had a higher prevalence of PCD-associated respiratory symptoms vs SA control participants (year-round wet cough, P < .001; year-round nasal congestion, P = .015; neonatal respiratory distress, P = .009; digital clubbing, P = .021) and lower nNO levels (median, 12 nL/min vs 252 nL/min; P < .001).ConclusionsAt least 12.1% of patients with classic PCD have SA and laterality defects ranging from classic heterotaxy to subtle laterality defects. Specific clinical features of PCD and low nNO levels help to identify PCD in patients with laterality defects.Trial registryClinicalTrials.gov; No.: NCT00323167; URL: www.clinicaltrials.gov.
| S-EPMC4219335 | biostudies-literature
Project description:Dextrocardia with situs inversus totalis is a rare congenital condition. We report herein a first experience of video-assisted minimally invasive mitral and pulmonary valve replacement through right anterior mini-thoracotomy as reoperation in patient with this complex anomaly. The good clinical and cosmetic results demonstrate that this innovative technique can be safely performed even in difficult anatomical conditions.
| S-EPMC10435878 | biostudies-literature
Project description:Dextrocardia associated with situs inversus totalis is a rare congenital condition. A small number of cases with these conditions have been reported who underwent myocardial revascularization via the on-pump or off-pump techniques. Among them, only 1 patient with dextrocardia and situs inversus totalis was reported to have the procedure performed with minimally invasive coronary surgery via a right anterior small thoracotomy. However, the case was a single-vessel disease and only one graft was achieved. We describe the case of a 65-year old female patient with triple-vessel obstructive coronary diseases who was successfully revascularized with three grafts using a minimally invasive technique. This was achieved via partial sternotomy and employing off-pump coronary artery bypass grafting.
| S-EPMC3686400 | biostudies-literature
Project description:INTRODUCTION:Situs inversus is a rare congenital anomlay in which the thoracic and abdominal organs are sited on the opposite side to that of normal anatomy. Its etiology is still unknown. It is classified into situs inversus partialis and situs inversus totalis. It is discovered incidentally in most cases. Any surgical intervention for such patients may encounter intra-operative technical difficulties due to anatomical differences, particularly if a laparoscopic approach is adopted, as in bariatric surgeries where the obesity itself opposes an added surgical challenge. CASE PRESENTATION:A female patient with a BMI of 52.1?Kg/m2 was being prepared for biliopancreatic diversion. She was diagnosed incidentally as having situs inversus totalis during the preoperative evaluation. She underwent an uneventful laparoscopic biliopancreatic diversion. She was followed up regularly in the bariatric surgery clinic for 9 years. Her last BMI was 29?Kg /m2 with no signs or symptoms of metabolic complications. CONCLUSION:This case report demonstrates that biliopancreatic diversion can be done safely in morbidly obese patients with situs inversus by an experienced bariatric surgeon in a specialized well-equipped center.
| S-EPMC6047057 | biostudies-literature
Project description: Not available
| S-EPMC7426708 | biostudies-literature