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1.
Kyobu Geka ; 77(6): 446-449, 2024 Jun.
Article in Japanese | MEDLINE | ID: mdl-39009539

ABSTRACT

Dextrocardia associated with situs inversus totalis is a rare congenital anomaly. We herein report a patient with this condition and ischemic coronay artery disease who underwent emergency surgical myocardial revascularization. A 76-year-old man was admitted to our hospital with a diagnosis of unstable angina pectoris. He had incessant ventricular fibrillation attack on the second day of hospitalization. Emergent coronary angiography revealed total obstruction of the anatomically left anterior descending and right coronary arteries, and severe stenosis in the anatomically left circumflex artery. Emergent off-pump coronary artery bypass using saphenous vein grafts was successfully performed. Intraoperatively, the main surgeon continuouly stood on the patient's right side except during anastosiso of a vein graft to the left circumflex artery. Postoperative course was almost uneventful aside from transient renal replacement and prolonged ventilation.


Subject(s)
Coronary Artery Bypass, Off-Pump , Situs Inversus , Humans , Male , Aged , Situs Inversus/complications , Situs Inversus/surgery , Situs Inversus/diagnostic imaging , Coronary Angiography , Dextrocardia/complications , Dextrocardia/surgery , Dextrocardia/diagnostic imaging
2.
Sci Rep ; 14(1): 16266, 2024 07 15.
Article in English | MEDLINE | ID: mdl-39009665

ABSTRACT

To evaluate the genetic etiology of fetal dextrocardia, associated ultrasound anomalies, and perinatal outcomes, we investigated the utility of whole exome sequencing (WES) for prenatal diagnosis of dextrocardia. Fetuses with dextrocardia were prospectively collected between January 2016 and December 2022. Trio-WES was performed on fetuses with dextrocardia, following normal karyotyping and/or chromosomal microarray analysis (CMA) results. A total of 29 fetuses with dextrocardia were collected, including 27 (93.1%) diagnosed with situs inversus totalis and 2 (6.9%) with situs inversus partialis. Cardiac malformations were present in nine cases, extra-cardiac anomalies were found in seven cases, and both cardiac and extra-cardiac malformations were identified in one case. The fetal karyotypes and CMA results of 29 cases were normal. Of the 29 cases with dextrocardia, 15 underwent WES, and the other 14 cases refused. Of the 15 cases that underwent WES, clinically relevant variants were identified in 5/15 (33.3%) cases, including the diagnostic variants DNAH5, DNAH11, LRRC56, PEX10, and ZIC3, which were verified by Sanger sequencing. Of the 10 cases with non-diagnostic results via WES, eight (80%) chose to continue the pregnancies. Of the 29 fetuses with dextrocardia, 10 were terminated during pregnancy, and 19 were live born. Fetal dextrocardia is often accompanied by cardiac and extra-cardiac anomalies, and fetal dextrocardia accompanied by situs inversus is associated with a high risk of primary ciliary dyskinesia. Trio-WES is recommended following normal karyotyping and CMA results because it can improve the diagnostic utility of genetic variants of fetal dextrocardia, accurately predict fetal prognosis, and guide perinatal management and the reproductive decisions of affected families.


Subject(s)
Dextrocardia , Exome Sequencing , Prenatal Diagnosis , Humans , Dextrocardia/genetics , Dextrocardia/diagnosis , Dextrocardia/diagnostic imaging , Female , Pregnancy , Prenatal Diagnosis/methods , Adult , Tertiary Care Centers , Fetus/abnormalities , Genetic Testing/methods , Ultrasonography, Prenatal , Karyotyping
3.
Int Heart J ; 65(4): 775-777, 2024 Jul 31.
Article in English | MEDLINE | ID: mdl-39010225

ABSTRACT

Dextrocardia is a very rare congenital malposition, and most cardiologists are not familiar with the radiographic angiograms of this condition. Here, we first report a case of dextrocardia with a chronic total occlusion (CTO) lesion undergoing retrograde percutaneous coronary intervention (PCI). Significant difficulties in lesion interpretation and device manipulation were encountered with the original angiograms. These challenges were not significantly improved until we adopted the double-inversion technique. The procedure was finally accomplished by using the kissing wire technique with a poor angle of attack. Retrograde CTO PCI for patients with dextrocardia is feasible with adequate techniques.


Subject(s)
Coronary Angiography , Coronary Occlusion , Dextrocardia , Percutaneous Coronary Intervention , Humans , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Percutaneous Coronary Intervention/methods , Coronary Occlusion/surgery , Coronary Occlusion/diagnosis , Coronary Occlusion/complications , Male , Chronic Disease , Aged , Middle Aged
6.
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
8.
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
10.
Am J Case Rep ; 25: e942006, 2024 Mar 07.
Article in English | MEDLINE | ID: mdl-38451882

ABSTRACT

BACKGROUND Duplication of the gastrointestinal tract is a rare congenital malformation that can develop in any part of the digestive tract. These duplications may be asymptomatic into adult age. Situs inversus totalis is a rare congenital anomaly characterized by a mirror transposition of thoracic and abdominal organs. We present a case of a pancreatic pseudocyst in a patient with a combination of situs inversus totalis and doubling of the esophagus, stomach, and first part of the duodenum. CASE REPORT A 64-year-old woman presented with epigastric pain. Abdominal computed tomography revealed a pancreatic pseudocyst and a previously identified duplication of the esophagus, stomach, and duodenum with situs inversus totalis. The patient underwent esophagogastroduodenoscopy (EGD) with endoscopic ultrasonography for pseudocyst drainage. During EGD, a bifurcation of the esophagus was found. Duplication of the esophagus, stomach, and first part of the duodenum was evident on further advancement. A week later, there was repeated filling of the pseudocyst with a liquid component, and the patient underwent cystogastrostomy with stenting. Five months after discharge, the stent was removed without complications. CONCLUSIONS Duplication of the gastrointestinal tract and situs inversus totalis are very rare congenital malformations that require early diagnosis. While situs inversus totalis does not represent any medical disadvantage, physicians should be aware of abnormal anatomy before procedures to prepare specialists for this in case of the need for special techniques. Endoscopic treatment of pancreatic pseudocysts is safe and effective even in such rare cases. The use of endoscopic methods also minimizes intervention and decreases the length of the patients' stays in the hospital.


Subject(s)
Dextrocardia , Pancreatic Pseudocyst , Situs Inversus , Female , Humans , Middle Aged , Abdomen , Dextrocardia/complications , Pancreatic Pseudocyst/diagnostic imaging , Pancreatic Pseudocyst/surgery , Pancreatic Pseudocyst/complications , Situs Inversus/complications , Situs Inversus/diagnosis , Tomography, X-Ray Computed
13.
Catheter Cardiovasc Interv ; 103(4): 565-569, 2024 03.
Article in English | MEDLINE | ID: mdl-38344889

ABSTRACT

Coronary angioplasty in patients with a right-sided heart may be difficult due to challenges in engaging the coronary arteries, interpreting angiogram, and further delivering intracoronary therapies. We present our experience of percutaneous coronary intervention in two cases and propose a practical algorithm to approach cardiac catheterization in these patients.


Subject(s)
Angioplasty, Balloon, Coronary , Dextrocardia , Percutaneous Coronary Intervention , Humans , Treatment Outcome , Percutaneous Coronary Intervention/adverse effects , Coronary Angiography
14.
Am J Case Rep ; 25: e942323, 2024 Feb 27.
Article in English | MEDLINE | ID: mdl-38409778

ABSTRACT

BACKGROUND Situs inversus totalis (SIT) is a rare congenital abnormality that includes mirror-image transposition of both the abdominal and the thoracic organs. It may remain undetected into adulthood until an acute medical emergency results in diagnostic imaging. This report presents a challenging case of left-sided acute appendicitis in a 45-year-old man with SIT. CASE REPORT A 45-year-old man with a medical history of gastroesophageal reflux disease, class 2 obesity, prediabetes, and elevated low-density lipoprotein cholesterol presented with severe acute abdominal pain localized in the left lower quadrant with localized tenderness, nausea, and 2 episodes of non-bloody and non-bilious emesis that started a day before the clinical encounter. Computed tomography of the abdomen and pelvis revealed previously undiagnosed congenital SIT. In addition, physical, laboratory, and radiological findings suggested early acute appendicitis with no evidence of complications. Hence, the patient was managed with an emergent laparoscopic appendectomy. Acute appendicitis was confirmed in the post-surgery histopathological examination. The post-surgery recovery was uneventful, and the patient was discharged home on the second postoperative day. CONCLUSIONS This report highlights that SIT may remain undiagnosed into adulthood and poses a challenge in the diagnosis of left-side appendicitis due to atypical symptom presentation, supporting the findings of previous case reports. Therefore, the inclusion of left-sided acute appendicitis in the differential diagnosis of abdominal pain in the left lower quadrant is warranted.


Subject(s)
Appendicitis , Dextrocardia , Laparoscopy , Situs Inversus , Male , Humans , Middle Aged , Appendectomy/adverse effects , Appendicitis/complications , Appendicitis/diagnosis , Appendicitis/surgery , Dextrocardia/complications , Situs Inversus/diagnosis , Abdominal Pain/etiology , Abdominal Pain/surgery , Acute Disease , Laparoscopy/methods
15.
Echocardiography ; 41(2): e15788, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38400626

ABSTRACT

Three-dimensional virtual dissection using high-definition live tissue rendering ultrasound tool of a 23-week gestation fetus with situs solitus, mirror image dextrocardia, ventricular septal defect, aortic override, and pulmonary atresia.


Subject(s)
Dextrocardia , Heart Defects, Congenital , Heart Septal Defects, Ventricular , Humans , Dextrocardia/complications , Dextrocardia/diagnostic imaging , Aorta/abnormalities , Fetus
17.
BMC Cardiovasc Disord ; 24(1): 22, 2024 01 03.
Article in English | MEDLINE | ID: mdl-38172727

ABSTRACT

BACKGROUND: We aimed to identify the ideal chest compression site for cardiopulmonary resuscitation (CPR) in patients with a single ventricle with dextrocardia corrected by Fontan surgery. METHODS: The most recent stored chest computed tomography images of all patients with a single ventricle who underwent Fontan surgery were retrospectively analysed. We reported that the ideal chest compression site is the largest part of the compressed single ventricle. To identify the ideal chest compression site, we measured the distance from the midline of the sternum to the point of the maximum sagittal area of the single ventricle as a deviation and calculated the area fraction of the compressed structures. RESULTS: 58 patients (67.2% male) were analysed. The mean right deviation from the midline of the sternum to the ideal compression site was similar to the mean sternum width (32.85 ± 15.61 vs. 31.05 ± 6.75 mm). When chest compression was performed at the ideal site, the area fraction of the single ventricle significantly increased by 7%, which was greater than that of conventional compression (0.15 ± 0.10 vs. 0.22 ± 0.11, P < 0.05). CONCLUSIONS: When performing CPR on a patient with Fontan circulation with dextrocardia, right-sided chest compression may be better than the conventional location.


Subject(s)
Cardiopulmonary Resuscitation , Dextrocardia , Fontan Procedure , Humans , Male , Female , Cardiopulmonary Resuscitation/adverse effects , Fontan Procedure/adverse effects , Retrospective Studies , Sternum , Dextrocardia/diagnostic imaging
18.
J Cardiovasc Electrophysiol ; 35(3): 418-421, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38213071

ABSTRACT

INTRODUCTION: Patients with congenital heart disease are at increased risk for requiring cardiac pacing during their lifetime. METHODS: We present the first described case of using two leadless pacing systems manufactured by separate companies implanted within the same patient to provide atrial and ventricular pacing due to complex congenital anatomy. RESULTS: A 27-year-old male with dextrocardia with double outlet right ventricle, subaortic ventricular septal defect, and pulmonary stenosis status-post pulmonary valve replacement complicated by ventricular pacing dependence and subsequent atrial pacing dependence after atriotomy-based atypical flutter ablation developed recurrent mediastinitis and pocket infection with erosion despite prolonged antibiotic treatment. Due to atrial and ventricular pacing dependence, a comprehensive congenital care team concluded the need for lead extraction and replacement of pacemaker via leadless peacemaking device. Laser-lead extraction and temporary atrial pacemaker placement was performed. Afterward, a transesophageal echocardiogram guided implantation of both a Micra AV 2 (Medtronic) leadless pacemaker in the interventricular septum within the right ventricle and an Aveir (Abbott) leadless pacemaker in the superior base of the right atrial appendage was performed with successful pacing. Although there is no communication between these devices, atrial-mechanical ventricular pacing was reliable with good implant thresholds, impedances and sensing from both devices. CONCLUSION: Our case demonstrates the feasibility of using dual leadless pacing modalities to simultaneously pace someone at complex, prohibitive risk for temporary permanent or permanent pacemaker devices.


Subject(s)
Dextrocardia , Double Outlet Right Ventricle , Pacemaker, Artificial , Male , Humans , Adult , Cardiac Pacing, Artificial/adverse effects , Heart Ventricles , Double Outlet Right Ventricle/etiology , Treatment Outcome , Pacemaker, Artificial/adverse effects , Equipment Design
19.
Am J Cardiol ; 211: 137-140, 2024 Jan 15.
Article in English | MEDLINE | ID: mdl-37918473

ABSTRACT

We present the case of a patient with known situs inversus referred for cardiac catheterization, which revealed a chronic total occlusion of the right coronary artery. Situs inversus, a rare congenital abnormality, is a term used to describe the inverted position of the chest and abdominal organs. Cardiac catheterization is rare in patients with this particular abnormality. It is important to customize techniques to engage coronary arteries and optimize guide support if percutaneous coronary intervention is required in these particular cases.


Subject(s)
Dextrocardia , Situs Inversus , Vascular Diseases , Humans , Coronary Angiography , Dextrocardia/complications , Situs Inversus/complications , Angioplasty
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