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3.
Tex Heart Inst J ; 51(1)2024 Apr 16.
Article in English | MEDLINE | ID: mdl-38623731

ABSTRACT

Dextrocardia with situs inversus totalis is a rare hereditary condition characterized by reversed orientation of the major thoracic and abdominal organs. Though dextrocardia itself is not believed to increase the risk of coronary artery disease, the workup and surgical management of patients with this condition may be technically challenging to heart team clinicians. This report describes the case management of a high-risk 56-year-old man with dextrocardia who presented with multivessel coronary artery disease.


Subject(s)
Coronary Artery Disease , Dextrocardia , Situs Inversus , Male , Humans , Middle Aged , Coronary Artery Disease/complications , Coronary Artery Disease/diagnosis , Coronary Artery Disease/surgery , Situs Inversus/complications , Situs Inversus/diagnosis , Situs Inversus/surgery , Dextrocardia/complications , Dextrocardia/surgery , Coronary Artery Bypass , Rare Diseases
4.
Medicine (Baltimore) ; 103(13): e37625, 2024 Mar 29.
Article in English | MEDLINE | ID: mdl-38552073

ABSTRACT

RATIONALE: Situs inversus totalis is a rare malposition of organs that typically involves lesions in the respiratory, circulatory, or urinary systems. Cases of congenital hemivertebrae combined with situs inversus totalis are extremely rare and have limited reports. PATIENT CONCERNS: We report a 2.5 years old girl with 2 congenital hemipyramids and complete visceral inversion who ultimately underwent hemilaminectomy. DIAGNOSIS: Congenital hemivertebrae combined with situs inversus totalis. INTERVENTION: The patient underwent hemilaminectomy. OUTCOMES: The spinal deformity was corrected. LESSONS: For patient with spinal deformities combined with situs inversus totalis, surgery can be an effective treatment method. But we also need to be vigilant about the dysfunction of various systems.


Subject(s)
Dextrocardia , Musculoskeletal Abnormalities , Situs Inversus , Female , Humans , Child, Preschool , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Musculoskeletal Abnormalities/surgery , Laminectomy , Treatment Outcome
5.
J Med Case Rep ; 17(1): 486, 2023 Nov 22.
Article in English | MEDLINE | ID: mdl-37990278

ABSTRACT

BACKGROUND: Mirror dextrocardia (MDC) is a condition in which the heart is located in a mirror-image position on the right side of the chest compared to the normal position in individuals with physiological variations. Patients with MDC and chronic total occlusion (CTO) of the left circumflex branch (LCX) are extremely rare in clinical practice. The treatment of MDC-CTO-LCX differs significantly from patients without mirror dextrocardia and the same condition in terms of instrument selection and procedural techniques. In this article, we report a successful case of interventional treatment in a patient with MDC-CTO-LCX. We summarize the anatomical and electrocardiographic variations in patients with MDC-CTO-LCX, and discuss the selection of interventional instruments and techniques that can be useful for interventionists as well as the diagnostic and therapeutic considerations that can be helpful for clinical physicians. CASE PRESENTATION: A male Han Chinese patient, 51, was admitted, presenting recurrent chest pain for a year and recent onset of exertional fatigue over the past week.He reported episodes of chest pain following physical activities over the past year, lasting between 5 and 20 min.Despite these symptoms, the patient did not seek immediate medical attention, and the occurrence of his chest pain has progressively lessened within the year.A week prior, the patient developed exertional dyspnea after brief walks, though without any episodes of nocturnal paroxysmal dyspnea.Upon arrival at our hospital for evaluation, he was initially diagnosed with chronic coronary syndrome, previous inferior myocardial infarction, atrial arrhythmia, and classified under the New York Heart Association functional class III.Following his admission, a chest X-ray and coronary angiography were conducted.The results indicated mirror dextrocardia and total occlusion of the left circumflex branch. Percutaneous coronary intervention (PCI) was performed on the left circumflex branch. Subsequent angiography demonstrated optimal stent positioning without evidence of hematoma or dissection.Following the procedure, the patient's symptoms of chest pain and exertional dyspnea were resolved, which led to his subsequent discharge.A follow-up electrocardiogram, 10 months post-procedure, displayed a slow and regular atrial rhythm. CONCLUSIONS: The incidence of dextrocardia is very low, and it may appear normal on an electrocardiogram; however, careful diagnosis is required when there is an abnormal direction of the P wave in limb leads. During the operation for chronic occlusive lesions of the right-sided coronary artery, the anomalous anatomical structure necessitates specific requirements for instrument selection and operative techniques. After revascularization of chronic occlusive vessels in dextrocardia, routine electrocardiographic examination may show false normalization, requiring caution in interpretation.


Subject(s)
Atrial Fibrillation , Coronary Occlusion , Dextrocardia , Percutaneous Coronary Intervention , Humans , Male , Chest Pain/etiology , Chronic Disease , Coronary Angiography , Coronary Occlusion/diagnostic imaging , Coronary Occlusion/surgery , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery , Dyspnea , Percutaneous Coronary Intervention/methods , Treatment Outcome , Middle Aged
6.
Heart Lung Circ ; 32(11): 1394-1397, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37833100

ABSTRACT

BACKGROUND: Patients with dextrocardia and d-transposition of the great arteries (d-TGA) present a technical challenge when performing the arterial switch operation (ASO). We sought to determine the long-term outcomes of this rare presentation. METHODS: All patients who underwent the ASO with d-TGA and dextrocardia were identified from the hospital database. RESULTS: There were four patients with dextrocardia (4 of 844, 0.5%) patients; two patients had situs solitus and two patients had situs inversus. Three patients had a ventricular septal defect. Left ventricular outflow tract obstruction was present in one patient and one patient had a hypoplastic arch with severe coarctation of aorta. Anomalous coronary anatomy occurred in two patients. There was one early mortality and the cause of death was sepsis. One patient required late reoperation for severe neo-pulmonary valve regurgitation at 23 years after the ASO. At last follow-up, all three surviving patients were in New York Heart Association Class I. CONCLUSIONS: Patients with d-TGA and dextrocardia who undergo the ASO are extremely rare and require challenging operative management.


Subject(s)
Arterial Switch Operation , Dextrocardia , Heart Septal Defects, Ventricular , Transposition of Great Vessels , Humans , Infant , Transposition of Great Vessels/surgery , Aorta , Heart Septal Defects, Ventricular/surgery , Reoperation , Dextrocardia/complications , Dextrocardia/surgery , Treatment Outcome , Follow-Up Studies
7.
J Int Med Res ; 51(8): 3000605231189129, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37548409

ABSTRACT

Dextrocardia is a rare cardiac malposition that was first described in 1606. Mirror-image dextrocardia is characterized by a mirror-image change of the normal position of the heart. Most cases are accompanied by situs inversus viscerum, whereas only 3% to 10% of cases are associated with intracardiac anomalies. Valve surgery for acquired valvular lesions in patients with mirror-image dextrocardia with situs inversus is rare. Diagnosing situs anomalies in adults is important to prevent errors during surgical operations, emergency procedures, or interventional operations. In this report, we present two cases of mitral regurgitation in patients with mirror-image dextrocardia. One patient had mirror-image dextrocardia with subacute infective endocarditis and mitral regurgitation, and the other patient had mirror-image dextrocardia with mitral Carpentier type I regurgitation. In both patients, mitral valve repair was successfully performed using a transseptal approach.


Subject(s)
Cardiac Surgical Procedures , Dextrocardia , Mitral Valve Insufficiency , Situs Inversus , Adult , Humans , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/surgery , Mitral Valve/diagnostic imaging , Mitral Valve/surgery , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Situs Inversus/surgery
8.
World J Pediatr Congenit Heart Surg ; 14(4): 524-526, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37282584

ABSTRACT

The Konno-Rastan operation is performed for relief of complex left ventricular outflow obstruction with a small aortic annulus. When associated with situs inversus and dextrocardia, important aspects should be kept in mind due to the mirror-image anatomy. In this report, we present a case of a 10-year-old child with the diagnosis of recurrent diffuse subaortic stenosis and situs inversus and dextrocardia who underwent the Konno-Rastan operation successfully and was asymptomatic with normal physical activity after a follow-up period of one year.


Subject(s)
Aortic Stenosis, Subvalvular , Cardiomyopathy, Hypertrophic , Dextrocardia , Situs Inversus , Ventricular Outflow Obstruction, Left , Child , Humans , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Situs Inversus/surgery , Cardiomyopathy, Hypertrophic/complications , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery
10.
J Card Surg ; 37(8): 2446-2449, 2022 Aug.
Article in English | MEDLINE | ID: mdl-35637597

ABSTRACT

INTRODUCTION: Situs inversus totalis, dextrocardia with interrupted inferior vena cava, and azygos vein continuation concomitant with symptomatic atrial fibrillation requiring ablation. This case was deemed not suitable for percutaneous ablation due to anatomic variations and the lack of case reports in the literature. METHODS AND RESULTS: We performed bilateral thoracoscopic epicardial ablation and epicardial left atrial appendage exclusion. The direct vision allowed for a complete box lesion set with bipolar radiofrequency device. Patient remained in sinus rhythm at the 12-months follow-up. CONCLUSION: Surgical thoracoscopic epicardial ablation is safe and effective also in congenital defects. Multidisciplinary expertise can offer minimally invasive ablation treatments.


Subject(s)
Atrial Fibrillation , Catheter Ablation , Dextrocardia , Heart Defects, Congenital , Situs Inversus , Atrial Fibrillation/complications , Atrial Fibrillation/surgery , Azygos Vein/abnormalities , Azygos Vein/surgery , Dextrocardia/complications , Dextrocardia/surgery , Heart Defects, Congenital/surgery , Humans , Situs Inversus/complications , Situs Inversus/surgery , Vena Cava, Inferior/abnormalities , Vena Cava, Inferior/surgery
11.
Kyobu Geka ; 75(2): 88-91, 2022 Feb.
Article in Japanese | MEDLINE | ID: mdl-35249082

ABSTRACT

Situs inversus totalis is a congenital anomaly characterized by a mirror image transposition of the normal visceral organs, which makes it difficult to perform aortic surgery accurately. Stanford type A aortic dissection in patients with this condition is very rare and difficult to assess and manage. We report a case of Stanford type A aortic dissection with situs inversus totalis. The patient presented with severe tricuspid regurgitation with annulus enlargement due to chronic atrial fibrillation, requiring ascending aortic replacement and tricuspid annuloplasty. These procedures were performed after the operator swapped the left and right positions during the operation. Postoperative course was uneventful. By carefully checking the preoperative computed tomography images and changing the operator's position during the operation, it is possible to safely perform Stanford type A aortic dissection surgery in patients with situs inversus totalis.


Subject(s)
Aortic Dissection , Dextrocardia , Situs Inversus , Tricuspid Valve Insufficiency , Aortic Dissection/complications , Aortic Dissection/diagnostic imaging , Aortic Dissection/surgery , Dextrocardia/surgery , Humans , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Situs Inversus/surgery , Tricuspid Valve/diagnostic imaging , Tricuspid Valve/surgery , Tricuspid Valve Insufficiency/complications , Tricuspid Valve Insufficiency/diagnostic imaging , Tricuspid Valve Insufficiency/surgery
12.
J Cardiothorac Surg ; 17(1): 56, 2022 Mar 27.
Article in English | MEDLINE | ID: mdl-35346291

ABSTRACT

BACKGROUND: Coronary artery bypass grafting in situs inversus totalis patients has been seldom reported in the literature. CASE PRESENTATION: A 76-year-old woman visited our hospital for chest pain and dyspnea that had started about 5 years earlier. Coronary angiography revealed triple-vessel disease, and computed tomography showed situs inversus totalis. Coronary artery bypass grafting was performed. In this case, the main operating surgeon stood on the right side of the patient until cardiopulmonary bypass was established and then switched positions to the left side of the patient for anastomosis. CONCLUSION: CABG was successfully completed in a patient with situs inversus totalis. The position shift helped improve the safety and ease of the surgery.


Subject(s)
Coronary Artery Disease , Dextrocardia , Situs Inversus , Aged , Coronary Angiography , Coronary Artery Bypass/methods , Coronary Artery Disease/surgery , Dextrocardia/surgery , Female , Humans , Situs Inversus/complications , Situs Inversus/surgery
13.
J Vis Exp ; (180)2022 02 11.
Article in English | MEDLINE | ID: mdl-35225283

ABSTRACT

Atrial fibrillation (AF) is the most common cardiac arrhythmia. The use of ablation technologies made the Cox-Maze IV procedure (CMP-IV) technically easier, faster, becoming the gold standard for the surgical treatment of AF. However, the efficacy and safety of CMP-IV in situs inversus dextrocardia are largely unknown. This paper summarizes the CMP-IV procedure performed concomitantly with valvular surgery in patients with situs inversus dextrocardia at this institution. From February 2016 to September 2020, three dextrocardia patients with persistent AF and valvular diseases were referred to this institution for valvular and CMP-IV surgery. CMP-IV was performed using either cryoablation with a nitrous oxide (N2O)-based cryoprobe or a bipolar radiofrequency clamp and bipolar radiofrequency pen. Mechanical valve replacement or mitral vavuloplasty was performed in another patient in addition to tricuspid annuloplasty. Transmurality of the ablated atrial tissues was evaluated by electron microscopy. Heart function was assessed by transthoracic echocardiography. Cardiac rhythm was monitored by 24 h Holter at 3, 6, 12, 18, 24, and 48 months follow-up. All the AF was successfully eliminated in the ablation procedure without recurrence or other complications during hospitalization. The mean bypass and crossclamp times were similar in all the patients. The postoperative ventilator support time, the duration of stay in the ICU, and postoperative residence time were also not significantly different among the patients. Transmural atrial necrosis was detected in the ablated atrial tissues. Sinus rhythm maintenance was achieved at 3, 6, 12, 18, 24, and 48 months follow-up in all the patients. All valve protheses switched freely; no tricuspid regurgitation was observed. The results of the present study demonstrate that the CMP-IV is safe and effective in eliminating AF in dextrocardia patients concomitant with valvular surgery.


Subject(s)
Atrial Fibrillation , Catheter Ablation , Dextrocardia , Situs Inversus , Atrial Fibrillation/surgery , Dextrocardia/complications , Dextrocardia/surgery , Humans , Maze Procedure , Situs Inversus/complications , Situs Inversus/surgery , Treatment Outcome
14.
Pacing Clin Electrophysiol ; 45(3): 340-347, 2022 Mar.
Article in English | MEDLINE | ID: mdl-35044698

ABSTRACT

BACKGROUND: Patients with tachycardia, in the context of pre-existing dextrocardia, could benefit from catheter ablation. However, anatomical complexities hinder effective conduct of this procedure. We aimed to retrospectively summarize the clinical characteristics and the safety and efficiency, and recommended the technique considerations. METHODS: Twenty-one cases from 19 patients with tachycardia and dextrocardia, who underwent catheter ablation between 2009 and 2021, were enrolled. All patients underwent echocardiography and computed tomography (CT) to confirm the anatomical malformations. Transseptal puncture was guided by fluoroscopy or intracardiac echocardiography when left atrial access was necessary and the ablation process was guided by three-dimensional (3D) mapping. RESULTS: Six cases exhibited situs solitus while nine cases exhibited situs inversus. Fourteen cases had atrial fibrillation, seven had atrial flutter, and two had atrioventricular reentrant tachycardia (AVRT); two cases had combined atrial fibrillation and atrial flutter. Acute success was achieved in 18 cases (85.7%, 18/21). The 3D mapping system was not employed in the three cases which failed. During long-term follow-up (20.71 ± 21.86 months), eight cases (72.7%, 8/11) of atrial fibrillation with dextrocardia successfully attained sinus rhythm. None of AVRT cases had recurrence. Half of the atrial flutter cases with dextrocardia, especially those with a history of surgical correction for cardiac malformations, underwent recurrence. One case had cardiac tamponade. CONCLUSIONS: Catheter ablation for tachycardia patients with dextrocardia is safe, efficient, and feasible. It is imperative to integrate echocardiography, cardiac computer tomography, and 3D mapping, and apply 3D reconstruction to facilitate the success of catheter ablation.


Subject(s)
Atrial Fibrillation , Atrial Flutter , Catheter Ablation , Dextrocardia , Tachycardia, Atrioventricular Nodal Reentry , Tachycardia, Supraventricular , Atrial Fibrillation/surgery , Atrial Flutter/surgery , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery , Humans , Retrospective Studies , Tachycardia/surgery , Treatment Outcome
15.
J Int Med Res ; 50(1): 3000605211072785, 2022 Jan.
Article in English | MEDLINE | ID: mdl-35023389

ABSTRACT

Cases of patients complicated with dextrocardia who suffer from acute cerebral infarction with large vessel occlusion and receive emergency thrombectomy are particularly rare and have not been widely reported. This article aimed to increase the awareness and knowledge of these cases. We report the case of a patient with mirror-image dextrocardia who suffered from cerebral infarction with large vessel occlusion and received emergency thrombectomy. A male patient in his early 60s with dextrocardia had acute cerebral infarction with posterior circulation large vessel occlusion and underwent emergency thrombectomy. During the operation, the rapid confirmation of dextrocardia and use of flexible interventional instruments helped establish a pathway for blood flow. We used an intracranial thrombectomy stent and intracranial balloon dilation catheter to restore the cerebral blood supply. The Modified Rankin Scale score was 0 at 3 months after thrombectomy, indicating a good prognosis of the patient. Acute cerebral infarction with large vessel occlusion in patients with dextrocardia is extremely rare. Emergency thrombectomy is feasible to recanalize cerebral blood flow and give patients a chance to recover.


Subject(s)
Cerebrovascular Disorders , Dextrocardia , Stroke , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery , Humans , Male , Retrospective Studies , Thrombectomy , Treatment Outcome
16.
World J Pediatr Congenit Heart Surg ; 13(2): 257-259, 2022 03.
Article in English | MEDLINE | ID: mdl-34661458

ABSTRACT

We report a case of a 2-year-old female presenting with cyanosis since birth and having rare cardiac anatomy of visceral situs inversus, dextrocardia, discordant atrioventricular connections with double outlet right ventricle, atrial communication, subaortic interventricular communication, anteriorly leftward aorta with right aortic arch, single coronary artery with a branch crossing right ventricle outflow tract, pulmonary stenosis, and bilateral superior vena cava. Corrective repair with atrial switch (Senning technique), intracardiac baffle, and right ventricle outflow reconstruction was successfully performed with challenges from altered anatomical location and orientation of the heart and abnormal coronary artery.


Subject(s)
Dextrocardia , Double Outlet Right Ventricle , Heart Septal Defects, Ventricular , Heterotaxy Syndrome , Pulmonary Valve Stenosis , Situs Inversus , Child, Preschool , Congenitally Corrected Transposition of the Great Arteries , Coronary Vessels , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery , Double Outlet Right Ventricle/complications , Double Outlet Right Ventricle/diagnostic imaging , Double Outlet Right Ventricle/surgery , Female , Heart Atria/surgery , Heart Septal Defects, Ventricular/surgery , Humans , Pulmonary Valve Stenosis/complications , Pulmonary Valve Stenosis/diagnostic imaging , Pulmonary Valve Stenosis/surgery , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Situs Inversus/surgery , Vena Cava, Superior
17.
Turk Kardiyol Dern Ars ; 49(7): 588-592, 2021 10.
Article in English | MEDLINE | ID: mdl-34623303

ABSTRACT

The procedure of stenting the patent ductus arteriosus (PDA) is a palliative procedure applied as an alternative to surgery in newborns with ductus-dependent pulmonary circulation. However, it is still a very challenging method in patients with aortic arch anomalies. We describe our experience with a newborn with right atrial isomerism and dextrocardia, complete atrioventricular septal defect, aortic outlet right ventricle with pulmonary atresia, right aortic arch, and a PDA from the left innominate artery. Because the PDA was long and tortuous, we preferred placing three short stents instead of a single long stent. The procedure applied the femoral artery approach with a Glidesheath Slender to decrease arterial injuries. PDA stenting in challenging morphologies can be performed successfully using multiple short stents and via Glidesheath Slenders.


Subject(s)
Dextrocardia/diagnosis , Ductus Arteriosus, Patent/diagnosis , Heart Septal Defects/diagnosis , Pulmonary Atresia/diagnosis , Stents , Computed Tomography Angiography , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery , Diagnosis, Differential , Ductus Arteriosus, Patent/complications , Ductus Arteriosus, Patent/diagnostic imaging , Ductus Arteriosus, Patent/surgery , Female , Heart Septal Defects/complications , Heart Septal Defects/diagnostic imaging , Heart Septal Defects/surgery , Humans , Infant, Newborn , Pulmonary Atresia/complications , Pulmonary Atresia/diagnostic imaging , Pulmonary Atresia/surgery
18.
Int J Med Robot ; 17(5): e2304, 2021 Oct.
Article in English | MEDLINE | ID: mdl-34197045

ABSTRACT

BACKGROUND: This paper describes a case of a patient with situs inversus totalis (SIT) and dextrocardia in which robotic atrial septal defect (ASD) repair was successfully performed in a beating heart. METHODS AND RESULTS: A 45-year-old female patient who had SIT and dextrocardia was diagnosed with secundum ASD 5 years ago. Because of progressive dyspnoea, fatigue, and obvious cough, she came to our hospital for surgical treatment. Transthoracic echocardiography showed the defect located in the middle and lower segments of the atrial septum with a maximum diameter of 27 mm, with a left-to-right shunt. Transcatheter ASD closure could not be performed because there was not enough tissue surrounding the defect. After communicating with the patient, we performed robotic ASD repair in a beating heart using the da Vinci surgical system. The operation was successful, and the patient recovered quickly. CONCLUSION: As a minimally invasive approach, robotic cardiac surgery has many advantages and is feasible and safe in suitable patients.


Subject(s)
Cardiac Surgical Procedures , Dextrocardia , Heart Septal Defects, Atrial , Robotic Surgical Procedures , Robotics , Dextrocardia/complications , Dextrocardia/surgery , Female , Heart Septal Defects, Atrial/complications , Heart Septal Defects, Atrial/surgery , Humans , Middle Aged
19.
J Card Surg ; 36(9): 3378-3380, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34148254

ABSTRACT

Dextrocardia is a rare congenital cardiac positional anomaly. A 74-year-old woman was referred for aortic regurgitation (AR). Chest X-ray revealed a mildly enlarged heart that directed to the right side. Transthoracic echocardiography showed AR due to cusp prolapse with a dilated left ventricle and reduced wall motion. Computed tomography demonstrated the heart and great vessels in a mirror image of their normal positions. A median sternotomy performed by the surgeon standing on the right side of the patient. After establishing a cardiopulmonary bypass, the surgeon moved to the left side of the patient, and then performed aortic valve replacement.


Subject(s)
Dextrocardia , Situs Inversus , Aged , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Dextrocardia/surgery , Echocardiography , Female , Humans , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Situs Inversus/surgery , Tomography, X-Ray Computed
20.
Am J Med Sci ; 362(3): 233-242, 2021 09.
Article in English | MEDLINE | ID: mdl-34052215

ABSTRACT

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.


Subject(s)
Disease Management , Heterotaxy Syndrome/diagnostic imaging , Heterotaxy Syndrome/surgery , Situs Inversus/diagnostic imaging , Situs Inversus/surgery , Dextrocardia/diagnostic imaging , Dextrocardia/epidemiology , Dextrocardia/surgery , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/surgery , Heterotaxy Syndrome/epidemiology , Humans , Situs Inversus/epidemiology
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