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Background Percutaneous closure is nowadays still deemed challenging in patent forame ovale (PFO) associated to multi-fenestrated atrial septal aneurysm (ASA). This anatomic arrangement is still considered a significant risk factor for recurrence of paradoxical embolism. Theoretically, transcatheter approach could be theoretically even more complex in the case of dextrocardia and visceral situs inversus. Case summary A 59-year-old man with history of migraine with aura and multiple cryptogenic strokes was referred for percutaneous closure of a PFO with associated ASA. He had been previously submitted to repeat attempt of percutaneous closure with not self-centering and self-centering devices that failed due to unfavorable anatomic characteristics (dextrocardia with situs viscerum inversus, huge ASA, multiple fenestrations, large PFO). Based on this "complex" anatomy, a sequential 2-step interventional approach aiming to reduce size and mobility of the atrial septal aneurysm with a suture-based approach (Noblestich™ EL, HeartStitch, Fountain Valley, CA, USA) and to close any eventual accessory fenestrations with a not self-centering occluding device was planned. At the end of the procedure, the ASA completely disappeared and no residual shunt was imaged at TEE bubble test. Discussion We describe a very rare case of symptomatic ASA-PFO in dextrocardia with situs viscerum inversus as well as an innovative approach to treat such complex anatomic setting by using the suture-based closure of a PFO to reduce size and mobility of an ASA in order to deploy dedicated not-self-centering occluding devices. [ABSTRACT FROM AUTHOR]

Abstract Introduction Acute appendicitis is one of the most frequent intra-abdominal diseases requiring emergency surgical consult and treatment. The diagnosis of this condition is based on clinical features and radiologic findings. One-third of patients with acute appendicitis present unusual symptoms. There are several circumstances that may cause misdiagnosis and unclear prognostic prediction. Among these, situs viscerum inversus totalis and midgut malrotation can be challenging scenarios, leading to a delay in treatment, especially when these conditions are unknown. We decided to carry on a systematic review of published cases of acute appendicitis in the context of anatomical anomalies. Methods We used the MESH terms “appendicitis” AND “situs inversus” AND/OR “gut malrotation” to search for titles and abstracts. Inclusion criteria were patients with clinical and/or radiological diagnosis of acute appendicitis, with conservative or surgical management and with preoperative/intraoperative findings of situs viscerum inversus or gut malrotation. Additionally, previous reviews were examined. Exclusion criteria of the studies were insufficient patient clinical and demographic data. Results We included in this review 70 articles concerning 73 cases of acute appendicitis with anatomical anomaly. Patients were aged from 8 to 86 years (median: 27.0 years). 50 were male and 23 were female. 46 patients (63%) had situs viscerum inversus, 24 (33%) had midgut malrotation, 2 (2.7%) had Kartagener’s syndrome, one of them (1.4%) had an undetermined anomaly In 61 patients the anatomical anomaly was unknown previously (83.6%), while 16,4% already were aware of their condition. Conclusion Acute appendicitis can occur in association of rare anatomical anomalies and in these cases diagnosis can be challenging. Situs viscerum inversus and midgut malrotation should always be considered in the differential diagnosis of a patient with left lower quadrant pain, especially in younger population. Besides clinical features, it is fundamental to implement the diagnostic progress with radiological examination. Laparoscopic approach is useful to identify and treat acute surgical emergency and it is also a diagnostic tool and can be tailored in order to offer the best exposition of the operatory field for each single case.

Introduction: Acute appendicitis is one of the most frequent intra-abdominal diseases requiring emergency surgical consult and treatment. The diagnosis of this condition is based on clinical features and radiologic findings. One-third of patients with acute appendicitis present unusual symptoms. There are several circumstances that may cause misdiagnosis and unclear prognostic prediction. Among these, situs viscerum inversus totalis and midgut malrotation can be challenging scenarios, leading to a delay in treatment, especially when these conditions are unknown. We decided to carry on a systematic review of published cases of acute appendicitis in the context of anatomical anomalies. Methods: We used the MESH terms "appendicitis" AND "situs inversus" AND/OR "gut malrotation" to search for titles and abstracts. Inclusion criteria were patients with clinical and/or radiological diagnosis of acute appendicitis, with conservative or surgical management and with preoperative/intraoperative findings of situs viscerum inversus or gut malrotation. Additionally, previous reviews were examined. Exclusion criteria of the studies were insufficient patient clinical and demographic data. Results: We included in this review 70 articles concerning 73 cases of acute appendicitis with anatomical anomaly. Patients were aged from 8 to 86 years (median: 27.0 years). 50 were male and 23 were female. 46 patients (63%) had situs viscerum inversus, 24 (33%) had midgut malrotation, 2 (2.7%) had Kartagener's syndrome, one of them (1.4%) had an undetermined anomaly In 61 patients the anatomical anomaly was unknown previously (83.6%), while 16,4% already were aware of their condition. Conclusion: Acute appendicitis can occur in association of rare anatomical anomalies and in these cases diagnosis can be challenging. Situs viscerum inversus and midgut malrotation should always be considered in the differential diagnosis of a patient with left lower quadrant pain, especially in younger population. Besides clinical features, it is fundamental to implement the diagnostic progress with radiological examination. Laparoscopic approach is useful to identify and treat acute surgical emergency and it is also a diagnostic tool and can be tailored in order to offer the best exposition of the operatory field for each single case. [ABSTRACT FROM AUTHOR]

Leadless pacemaker implantation (LPI) has fewer device complications and reduced chance of infection compared to conventional pacemakers. Dextrocardia with situs viscerum inversus (DC+SVI) is a rare condition, which seldom leads to cardiac complications. However, its presence poses a challenge to operators in cardiac procedures. LPI reports in DC patients are scarce. We report a case of LPI in a DC+SVI patient, followed by a brief but comprehensive literature review. [ABSTRACT FROM AUTHOR]

We describe the case of a 32-year-old man who developed a liver neoplasm due to previous Fontan surgery (FS) for a single ventricle anomaly and situs viscerum inversus. He was admitted to our hospital for suspected hepatocellular carcinoma during an Ultrasound (US) follow up. Computed tomography (CT) showed features of chronic liver disease and 7 cm hepatic nodule with arterial enhancement. Laboratory analyses documented preserved liver function and increased levels of alpha-fetoprotein. Trans-arterial-chemoembolization (TACE) was performed obtaining complete necrosis at 4 weeks of follow up and significant reduction of alpha-fetoprotein. The patient is currently in follow-up, being evaluated for further treatments and/or combined liver-heart transplantation. TACE is a therapeutic option for the treatment of patients with unresectable hepatocellular carcinoma (HCC) and with severe heart disease, like those submitted to FS and with also other vascular abnormalities like those correlated to situs viscerum inversus.

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Randomization of left–right body asymmetry, situs viscerum inversus (heterotaxy), is commonly associated with primary ciliary dyskinesia (PCD) resulting from an abnormal ciliary structure, with approximately 50% of PCD patients exhibiting organ laterality defects. I herein report an intrauterine fetal death case, in which an autopsy revealed two lobes of the bilateral lungs as well as heterotaxy of abdominal organs (right-sided spleen and inversion of the alimentary and biliary organs). Whole-exome sequencing (WES) identified a heterozygous single-nucleotide change (c.12775T>C) in exon 68 of the DNAH9 gene, which is a rare single-nucleotide polymorphism (SNP) of rs746081639 and results in the amino acid change of p.C4259R. WES also identified a rare SNP of rs763089682 (c.121G>A) in the RSPH1 gene that causes a heterozygous amino acid alteration of p.G41R. The frequencies of both SNPs, C in rs746081639 and A in rs763089682, are 0.00000824, and a polyphen-2 analysis predicted these amino acid changes to be probably damaging, with a score of 1.000. The combination of extremely rare SNPs in DNAH9 and RSPH1 genes might have been the possible mechanism underlying the development of the laterality defect in the present case. [ABSTRACT FROM AUTHOR]

Kartagener syndrome (KS) is a rare autosomal recessive disease. The disease is characterized by three typical symptoms: chronic sinusitis, situs viscerum inversus (SVI), and bronchiectasis. The laparoscopic cholecystectomy (LC) is the standard procedure in most cases of cholelithiasis, but in SVI patients it can be difficult, especially for right-handed surgeons. We report the case of a 24-year-old female affected by KS, presenting with a history of symptomatic cholelithiasis. Ultrasound and magnetic resonance cholangiopancreatography confirmed SVI totalis and cholelithiasis. The patient underwent a laparoscopic cholecystectomy by a right-handed surgeon performed with a mixed approach without complications. Laparoscopic cholecystectomy in SVI patients can be a safe and reliable technique especially for a left-handed surgeon. The described technique is also easy for a right-handed surgeon. However, it is considered a technically challenging procedure and often requires technical modification. [ABSTRACT FROM AUTHOR]

• Abdominal pain due to acute appendicitis in one of the most causes of access to Emergency Room requiring surgical consult and treatment. • The occurrence of anatomical anomalies should be considered especially when clinical and imaging features are misleading. In these cases laparoscopic surgery can be a safe tool in order to confirm uncertain diagnosis. • We report a case of acute appendicitis in a 23-year-old Caucasian men with situs viscerum inversus detected on radiological investigation. • Laparoscopic approach was used to confirm the diagnosis and to perform appendectomy. Trocars placement was tailored for this peculiar case. • Situs viscerum inversus and midgut malrotation should be taken into consideration in patients with findings of the physical examination suspicious for left-sided acute appendicitis. Abdominal pain due to acute appendicitis in one of the most causes of access to Emergency Room requiring surgical consult and treatment. The occurrence of anatomical anomalies should be considered especially when clinical and imaging features are misleading. In these cases laparoscopic surgery can be a safe tool in order to confirm uncertain diagnosis. We report a case of acute appendicitis in a 23-year-old Caucasian men with situs viscerum inversus detected on radiological investigation. Laparoscopic approach was used to confirm the diagnosis and to perform appendectomy. Trocars placement was tailored for this peculiar case. One third of patient with acute appendicitis complains abdominal pain in an unexpected location due to various anatomical position of appendix. Left-sided acute appendicitis is a cause of misdiagnosis and it can occur in association with anatomical anomalies such as situs viscerum inversus and midgut malrotation. Laparoscopic surgery may represent a valuable approach in terms of differential diagnosis and treatment in these patients. Left-sided acute appendicitis should always be considered in young male patients with left lower quadrant pain. Laparoscopic approach is useful and safe procedure both for diagnosis and treatment of these unclear clinical pictures. [ABSTRACT FROM AUTHOR]

Primary ciliary dyskinesia (PCD) is a rare genetic diseases with diverse clinical symptoms. Clinicians should think of PCD in the differential diagnostic process in both children and adults who suffer from recurrent or chronic upper and lower respiratory tract symptoms frequently accompanied by hearing impairment. The first manifestations of PCD often occur very early in life and might present as an acute respiratory distress syndrome of a newborn or neonatal rhinitis. During the whole life the patients suffer from chronic wet cough and recurrent airway and lung inflammations, which can result in bronchiectasis. Situs viscerum inversus, other laterality defects and fertility disorders are other common symptoms in PCD. Early diagnosis and complex therapy play a key role in the prognosis of PCD patients, as deterioration of lung functions and lung tissue destruction may occur at an early age in some phenotypes. [ABSTRACT FROM AUTHOR]

Situs viscerum inversus (SVI) is a very rare condition in that abdominal and thoracic organs are located reversed. Abdominal aortic aneurysm (AAA) is a life-threatening pathology due to progressive aortic enlargement until the rupture. The association between SVI and AAA is very infrequent. The aim of this study is to identify the surgical procedures available to treat AAA in SVI. We performed a literature review of all studies about AAA in SVI patients, analyzing PubMed/MEDLINE, EMBASE, Web of Science (WOS), Google Scholar databases. The survey includes all publications until June 2023. The outcomes include demographic findings, type of surgical procedure, intraoperative and postoperative complications, follow-up. A total of 12 studies, including 12 patients, were considered eligible for the review. AAA mean size was 70.5 mm (range: 55-90 mm); the most common localization was in the infrarenal aortic portion. 6 studies reported data on elective surgery, and 6 on emergency procedures. In one case endovascular treatment was performed. No intraoperative complications are reported; 3 postoperative complications are registered. Medium follow-up period was 13.5 months (range: 3-60). According to the available literature, the treatment of AAA in SVI is feasible and does not show an incremented morbidity compared to patients with a normal visceral configuration. This treatment seems to be effective also in case of endovascular treatment. AAA treatment in SVI should be performed (especially in elective settings) in high volume centers where it is possible to bring on collaboration across different surgical specialists., Competing Interests: None.The authors have no conflicts of interest to disclose., (2024, International Research and Cooperation Association for Bio & Socio - Sciences Advancement.)

• All symptomatic paraesophageal hiatal hernias should be repaired, particularly those with acute obstructive symptoms or which have undergone volvulus. • Laparoscopic hiatal hernia repair is as effective as open transabdominal repair, with a reduced rate of perioperative morbidity and with shorter hospital stays. It is the preferred approach for the majority of hiatal hernias. • Robotic Assisted Giant-Paraesophageal Hernia repair remain technically challenging predominantly in the dissecation of the hernia sac from the posterior mediastinum. • The robotic platform have the same benefits of the laparoscopic approach in terms of complication rate, total surgical time, and hospital length of stay and in particular case is superior. Giant hiatus hernia is defined as migration of >30% of the stomach with or without other intra-abdominal organs into the chest. Situs Viscerum Inversus is a rare congenital condition in which the major visceral organs are reversed from their normal arrangement; they are translated (completely or partially) on the opposite side of the body. Diagnosis is often incidental. We report a Robot-assisted Toupet fundoplication for a giant hiatal hernia with gastro oesophageal reflux disease and cholelithiasis, in a 63-years-old woman with situs viscerum inversus. A 63-year-old woman with Situs Viscerum Inversus was diagnosed with giant sliding hiatus hernia. We performed a Robot-assisted procedure of reduction of hiatal hernia in abdomen and Toupet fundoplication with Bio A mesh placement and gastropexy procedure associated to cholecystectomy. The operation time was of 190 min. The patient was discharged on third postoperative day after X-ray check and he tolerated a solid food. Minimally invasive surgery represents, nowadays, the standard approach for hiatal hernia and cholelithiasis. In challenging cases as the giant hernias ad rare anomaly as situs viscerum inversus, the surgical treatment can be facilitated by the use of robotic technology. [ABSTRACT FROM AUTHOR]

Key Clinical Message: After transvenous lead extraction, leadless pacemaker might be a valid alternative to the traditional two‐step strategy including an active fixation leads temporary PM and subsequent contralateral permanent implantation in patients who are pacemaker‐dependent. Moreover, leadless PM might be of great importance in patients presenting with congenital vascular or cardiac abnormality. [ABSTRACT FROM AUTHOR]

Abstract: A situs inversus with dextrocardia (DC) is a rare condition in adults. Usually, patients have structurally normal hearts and normal life expectancy. The incidence of coronary artery disease in this setting is similar to that in the general population. Coronary revascularization may present potential difficulties related to the unusual anatomy. Although the radial artery is a safe and effective site of access for coronary interventions, some anatomical variations may make this procedure more complicated. We describe two cases of patients with situs viscerum inversus and acute myocardial infarction who underwent successful transradial percutaneous coronary intervention (PCI). We will show that coronary angioplasty with stent application via the radial approach in patients with DC is feasible and effective also in emergency and urgent care. [Copyright &y& Elsevier]

Abstract: Primary cilia are assembled and maintained by evolutionarily conserved intraflagellar transport (IFT) proteins that are involved in the coordinated movement of macromolecular cargo from the basal body to the cilium tip and back. The IFT machinery is organized in two structural complexes named complex A and complex B. Recently, inactivation in the mouse germline of Ift genes belonging to complex B revealed a requirement of ciliogenesis, or proteins involved in ciliogenesis, for Sonic Hedgehog (Shh) signaling in mammals. Here we report on a complex A mutant mouse, defective for the Ift122 gene. Ift122-null embryos show multiple developmental defects (exencephaly, situs viscerum inversus, delay in turning, hemorrhage and defects in limb development) that result in lethality. In the node, primary cilia were absent or malformed in homozygous mutant and heterozygous embryos, respectively. Impairment of the Shh pathway was apparent in both neural tube patterning (expansion of motoneurons and rostro-caudal level-dependent contraction or expansion of the dorso-lateral interneurons), and limb patterning (ectrosyndactyly). These phenotypes are distinct from both complex B IFT mutant embryos and embryos defective for the ciliary protein hennin/Arl13b, and suggest reduced levels of both Gli2/Gli3 activator and Gli3 repressor functions. We conclude that complex A and complex B factors play similar but distinct roles in ciliogenesis and Shh/Gli3 signaling. [Copyright &y& Elsevier]

: We report the case of a 66-year-old caucasian woman affected by Kartagener’s syndrome (KS), a genetically transmitted disorder characterised by situs viscerum inversus, bronchiectasis and sinusitis, who also developed rheumatoid arthritis (RA). The impaired mucociliary function typical of KS caused recurrent paranasal sinus and lung infections, as shown by CT scans of the sinuses and chest. The coexistence of KS and RA in our patient was probably accidental. Given the small number of patients in whom an association of the two disorders has been described, it is impossible to establish whether KS might play a role in the pathogenesis of RA. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 65-year-old man who was suffering from haemoptysis, and showed the symptoms of type 1 situs viscerum inversus without Kartagener syndrome.