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Human laterality disorders comprise a group of diseases characterized by abnormal location (situs) and orientation of thoraco-abdominal organs and vessels across the left-right axis. Situs inversus totalis is mirror image reversal of thoraco-abdominal organs/great vessels. Situs ambiguus, better known as heterotaxy, is abnormal arrangement of thoraco-abdominal organs across the left-right axis excluding situs inversus totalis. Heterotaxy, also referred to as atrial or atrial appendage isomerism, is characterized by abnormal location of left-sided or right-sided organs with loss of asymmetry of normally paired asymmetric organs. It is associated with a variety of anomalies involving the heart, great vessels, lungs and intra-abdominal organs. Right and left atrial isomerism are associated with multiple complex congenital cardiac and vascular anomalies, many of which are lethal when untreated. Isomerism may also affect the lungs, spleen, liver, gall bladder, and intestines. Innovative surgical therapy of heterotaxy/isomerism has reduced early mortality and markedly improved long-term prognosis., Competing Interests: Declaration of Competing Interest None of the authors report conflicts of interest or competing interests., (Copyright © 2021 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.)

This report presents the case of an 82-year-old man with known dextrocardia and situs inversus totalis who presented with increasing dyspnea on exertion and was diagnosed with severe aortic stenosis. Transcatheter aortic valve replacement was performed and required deviation from standard techniques for patients with normal anatomy and left-sided aortic arch. We describe two technical differences required for patients with dextrocardia and right-sided aortic arch that facilitate transcatheter aortic valve replacement in this patient group., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 4-year-old cocker spaniel, male, of 12kg body weight was presented because of the onset of polyuria or polydipsia. From the first months of its life, the dog had exhibited constant serous to mucopurulent nasal discharge, productive cough, sneezing, reverse sneezing, otitis, and recurrent episodes of fever. The respiratory signs had been treated several times with antibiotics, without ever achieving a complete resolution. Clinical examination revealed normal rectal temperature (38.3°C), increased respiratory rate (40breaths/min), a copious mucous nasal discharge and right deviation of the heart apex beat (ictus cordis). Increased respiratory sounds with moist rales and crackles were found on chest auscultation. An increase in serum creatinine, urea and phosphorus, hypoalbuminemia and proteinuria were found. Lateral and ventrodorsal radiographs of the thorax and of the abdomen showed the transposition of the heart, with the cardiac apex pointing toward the right (dextrocardia), bronchointerstitial lung pattern, areas of consolidation, lesions consistent with bronchiectasis caves and a mirror-image of abdominal organs, confirming the diagnosis of complete situs inversus (CSI). Respiratory signs, combined with CSI, suggested the diagnosis of Kartagener syndrome (KS). Abdominal ultrasound showed an increase in the echogenicity of the renal parenchyma, a loss of definition of the corticomedullary line, slight bilateral pyelectasis, and decreased cortical perfusion. The dog died 2 months later because of a further worsening of the clinical condition. Necroscopy demonstrated the existence of CSI, rhinosinusitis, bronchitis, and bronchiectasis, so confirming the diagnosis of KS, and renal amyloidosis. This is the first case reported in veterinary medicine of the presence of renal amyloidosis together with KS in a dog., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: The right ventricle has a proclivity to wrap around the left ventricle outflow tract (LVOT) in congenitally corrected transposition (CCT) patients with apicocaval ipsilaterality, which may influence the outcome of the double switch operation (DSO). The goal of this study was to determine if the LVOT is compressed by the right ventricle in this setting., Methods: A total of 103 patients with CCT were divided into four groups according to ventricular looping and apical position, including Group A (D-loop and levocardia), Group B (L-loop and dextrocardia), Group C (D-loop and dextrocardia), and Group D (L-loop and levocardia). Computed tomography was used to define left-right laterality and ventro-dorsal relationship of the LVOT., Results: Apicocaval ipsilaterality was found in 57 patients (Group A, n=25; Group B, n=32), in whom the right ventricle was found to wrap around the LVOT. Among them, 49 (86%) had LVOT obstruction. In 46 patients without apicocaval ipsilaterality (Group C, n=10; Group D, n=36), 31 had LVOT obstruction (67.4%). LVOT obstruction was more prone to occur in patients with apicocaval ipsilaterality compared with those without (p=0.025), and was more significant in the situs solitus (p=0.058) than in situs inversus (p=0.547)., Conclusions: LVOT obstruction was prone to occur in CCT patients with situs solitus and apicocaval ipsilaterality (Group B). The ventricular outflow patency was influenced by apical position, which should be considered to avoid a posterior ventricular outflow tract from compression after DSO., (Copyright © 2016 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Complete situs inversus is a rare congenital condition that is characterized by the development of the thoracic and abdominal viscera in a mirror image to their normal orientation. This study describes this condition in 2 dogs: an 8-year-old male dalmatian that was originally evaluated for cystitis and a 3-year-old male crossbreed Pekinese that had a routine echographic study. In dogs, most of the reported cases were associated with the Kartagener syndrome, but our patients had no evidences of this ciliary disorder., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The double discrepancy (DD) is an exceptional cardiopathy, manifested by atrioventricular and ventriculo-arterial discordances. DD is rarely diagnosed in the antenatal period and is often found in adults when cardiac complications occur. We describe six cases of DD, the different forms, their ultrasound semiology, and the assessment of prognosis, correlated with the existence of associated cardiac anomalies., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Objective: Klippel-Feil syndrome is characterized by faulty segmentation of two or more cervical vertebrae and, in its most severe form, consists of massive cervical vertebral fusion, short neck, low posterior hairline, and limitation of head movement. Several cases associating Klippel-Feil syndrome with situs inversus totalis have been reported. In the present study, we describe the clinical features of a novel case of Klippel-Feil syndrome associated with situs inversus totalis and searched for mutations in GDF1, GDF3 and GDF6 genes, which were recently implicated in the development of skeletal and visceral anomalies., Methods: A case of Klippel-Feil syndrome associated with situs inversus totalis underwent a full clinical examination including X-ray of cervical spine and thorax, abdominal ultrasound, and computerized tomography scanning of thorax and abdomen. PCR amplification and automated nucleotide sequencing of coding exons and intron-exon junctions of GDF1, GDF3, and GDF6 genes were performed in genomic DNA., Results: No molecular alterations were found in GDF1, GDF3 and GDF6 genes in this patient., Conclusion: An additional patient associating Klippel-Feil syndrome and situs inversus totalis is reported. Mutations in GDF1, GDF3, and GDF6 genes were excluded as the cause of this unusual clinical association., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

A 24-year-old male pedestrian with situs inversus and dextrocardia was struck by a car and sustained multiple injuries, including a pseudoaneurysm of the proximal descending thoracic aorta. A thoracic endograft was deployed to exclude the blunt aortic injury. We are not aware of any report of endovascular repair of blunt aortic injury in a patient with this congenital finding. A brief review of the literature is also included., (Copyright © 2011 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)

Polysplenia syndrome (PS) is rarely encountered in elderly. It is characterized by multiple spleens associated with various cardiac and gastrointestinal abnormalities including partial or complete agenesis of dorsal pancreas. Situs inversus totalis (SIT) is a rare congenital anomaly with mirror image of viscera combined with dextrocardia. Occurrence of SIT and PS in the same patient is exceedingly rare. We present the first case of adult PS with SIT accompanied with annular pancreas., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Heart transplantation represents a valuable therapeutic option for patients with congenital heart disease and end-stage heart failure. We report the case of a 15-year-old patient with situs inversus and additional complex congenital malformations of the heart who underwent several previous palliative operations. Orthotopic cardiac transplantation with several technical modifications was performed successfully. At 18 months post-operatively the patient is free from rejection and doing well in New York Heart Association (NYHA) Class I status.

Background: Despite improved outcome for many single ventricle lesions, staged reconstruction for heterotaxy syndrome with a functional single ventricle continues to have a high mortality. Prenatal identification of heterotaxy syndrome may improve long-term survival., Methods: Our database was reviewed from January 1996 to December 2004 for patients with heterotaxy syndrome. Assessment was made for prenatal diagnosis and echocardiographic characteristics of heterotaxy syndrome. We sought to assess the accuracy of fetal echocardiography in the diagnosis of heterotaxy syndrome and determine whether prenatal diagnosis and other risk factors have an impact on survival in patients with heterotaxy syndrome., Results: Of 81 patients that met criteria, 43 (53%) had prenatal diagnosis. Prenatal diagnosis had high specificity and positive predictive value for all findings but had low sensitivity for anomalous pulmonary veins. Among the 70 patients born alive, survival was 60% with median follow-up of 51.4 months (range, 6.5 to 109.7 months). Prenatal diagnosis did not improve survival (p = 0.09). None of the 11 patients with complete heart block (CHB) survived past 3 months of age. Two patients underwent heart transplantation as their first intervention and have survived. CHB and anomalous pulmonary venous connection were associated with shorter duration of survival., Conclusions: Prenatal diagnosis of heterotaxy syndrome does not improve survival in patients who undergo single ventricle reconstruction. The most potent risk factors for poor outcome (CHB, anomalous pulmonary veins) are likely not impacted by identification in utero. In light of the poor outcome, cardiac transplantation as an initial therapy may be a viable option for some patients.

A rare case of double cancer with situs ambiguus with polysplenia is presented. A 58-year-old patient was initially diagnosed with an early gastric cancer. On evaluation, the computed tomography of the abdomen demonstrated situs ambiguus with polysplenia. We performed a subtotal gastrectomy with the stomach being reconstructed in a Billroth-II fashion. Three months after the operation, he again visited our department complaining nausea and dysphagia. Examinations confirmed the other oesophageal malignancy with advanced stage. Because of unfamiliarity to situs anomaly and rarity of double cancer, we missed the other coexistent cancer. This is the first case presentation of a double carcinoma occurring in a patient with situs ambiguus with polysplenia. The literature is reviewed and the importance of preoperative evaluation is discussed.

The differential diagnosis of left lower quadrant abdominal pain in an adult man includes, among others, sigmoid diverticulitis; leaking abdominal aortic aneurysm; renal colic; epididymitis; incarcerated hernia; bowel obstruction; regional enteritis; psoas abscess; and in this rare instance, situs inversus with acute appendicitis. We report a case of situs inversus totalis with left-sided appendicitis and a brief review of the literature. There were several subtle indicators of total situs inversus present that were missed by the physicians and surgeons who initially evaluated the patient prior to surgery. Computed tomography scan with contrast, however, revealed the diagnosis immediately, and treatment was successfully initiated.

We performed a combined atrial (Mustard) and ventricular (Rastelli) repair on a previously palliated patient with situs inversus, atrioventricular discordance, ventricular septal defect, and pulmonary atresia. The suitability and durability of this operative strategy is supported by the satisfactory hemodynamic and functional status of the patient 10 years later.

A case of situs inversus with discordant atrio-ventricular and ventriculo-arterial structures was diagnosed at the ultrasonography examination performed at 34 weeks gestation. This observation underscores the importance of recognizing the position of the viscerae and discordant segments of the heart, atria, ventricles and greater vessels. Overall frequency of situs inversus is estimated at 1/10,000 births. It is often associated with an inborn cardiopathy, especially corrected transposition of the greater vessels resulting from abnormal rotation of the cardiac tube. The anatomic, pathologic, embryologic, etiologic and and sonographic features of situs inversus and related anomalies of the cardiac structures is presented.

Isolated atrial inversion in situs inversus is a rare congenital cardiac malformation. Its physiology resembles transposition of great vessels, and the best option for its surgical treatment is the atrial switch operation. In this article, we present a case of isolated atrial inversion in concordance with visceral situs inversus diagnosed at birth by echocardiography and cardiac catheterization, which was successfully treated at 8 months of age by a Senning procedure.

Ivemark Syndrome is a multiple organ syndrome associated with splenic abnormalities, complex cardiac pathology and an abnormality of the abdominal viscera. The incidence in our department is 1/6000 deliveries and the teratogenic effect seems to occur between the 30th and 40th days of intrauterine life, but the cause is unknown. The principal warning signal is bradycardia. Prenatal diagnosis can be made by ultrasound. The prognosis depends on the degree of malformation of the heart.

The report describes a rare case of a complex congenital heart disease wherein the splenic status and the relationship of the suprarenal abdominal great vessels strongly suggested right isomerism, but atrial morphology demonstrated usual atrial arrangement. It emphasises the increasing recognition that the arrangement of the abdominal organs need not accurately reflect atrial arrangement.

Hypoplasia of the morphological left ventricle associated with severe pulmonic stenosis and an intact ventricular septum in the setting of congenitally corrected transposition of the great arteries is a rare lesion. We report the clinical and echocardiographic findings of this lesion in a patient with atrial situs inversus.

A right-sided heart associated with mirror-image atrial arrangement (situs inversus) is a rare condition. The incidence of coronary arterial disease in this setting is probably similar to that in the general population. Description of coronary angiography in this condition is very rare. We have recently catheterized such a patient. The necessary approach to coronary angiography is emphasized.

Determination of the arrangement of the atrial chambers ("atrial situs") is the first step in sequential chamber analysis of complex congenital heart disease. Although bronchial pattern, as detected by chest X-ray, is an excellent guide for prediction of atrial arrangement, exceptions to this rule based on post-mortem examination have been described. We report a case in which discordance between the atrial arrangement (mirror-image) and bronchial morphology (usual) was diagnosed during life.

A 7-month-old infant with ventricular inversion without transposition of the great arteries in situs solitus, associated with a ventricular septal defect, is reported. When the infant was 13 months of age, successful surgical repair was undertaken by performing an intraatrial transposition of venous return using the Senning procedure and by closing the ventricular septal defect with a patch. The postoperative course was uneventful except for a well-tolerated complete heart block. No systemic valve incompetence appeared during the 53 months of actual follow-up. A complete review of the literature suggests that this is the sixth reported instance of successful repair. Injury of the abnormally located conduction tissue is regarded as the most threatening complication.

We report a patient with situs inversus and ischemic heart disease who had myocardial revascularization with anastomosis of the right mammary artery to the anterior descending coronary artery.