Ideal chest compression site for cardiopulmonary resuscitation in fontan circulation patients with dextrocardia.

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.

Three-dimensional high-definition live tissue virtual dissection of mirror-image dextrocardia with thoracic-abdominal discordance in a fetus.

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.

Radiofrequency ablation of persistent atrial fibrillation in a patient with situs inversus totalis and interrupted inferior vena cava.

INTRODUCTION: Catheter ablation of atrial fibrillation (AF) has emerged as the most effective therapy. However, rare anatomical abnormalities such as situs inversus totalis, dextrocardia, or interrupted inferior vena cava can make ablation challenging. METHODS AND RESULTS: We report a case of a 55-year-old woman with situs inversus totalis, dextrocardia, surgical atrial septal defect repair, left-sided dual chamber pacemaker in place, and symptomatic recurrent persistent AF who underwent successful pulmonary vein and posterior wall isolation by the superior access from the left internal jugular vein. CONCLUSIONS: It is a feasible and safe approach with support of transesophageal echocardiography and multiple emerging technologies.

Robotic magnetic navigation-guided pulmonary vein isolation in an atrial fibrillation patient with dextrocardia situs inversus: techniques and potential advantages.

BACKGROUND: Dextrocardia with situs inversus (DSI) is a very rare congenital anomaly. Catheter manipulation and ablation of atrial fibrillation (AF) in patients with this anatomical variant is challenging for the operators. This case report presents a safe and effective AF ablation guided by the robotic magnetic navigation (RMN) system in combination with intracardiac echocardiograhy (ICE) in a patient with DSI. CASE PRESENTATION: A 64-year-old male with DSI was referred for catheter ablation of symptomatic, drug-refractory paroxysmal AF. One transseptal access was achieved via the left femoral vein under the guidance of ICE. The three-dimensional reconstruction of the left atrium and the pulmonary veins (PVs) were performed by the magnetic catheter using the CARTO and the RMN system. Then, the electroanatomic map and pre-acquired CT images were merged. Finally, bilateral circumferential ablation lines were delivered around the ipsilateral PV ostia to achieve complete PV isolation (PVI). CONCLUSIONS: This case demonstrates that AF catheter ablation under the guidance of the RMN system using ICE is feasible and safe in a patient with DSI. Moreover, the combination of these technologies broadly facilitates treatment of patients with complex anatomy, while reducing the risk of complications.

3D TEE imaging in a patient with severe tricuspid regurgitation with dextrocardia and tricuspid commissural prolapse.

Although dextrocardia is a rare congenital anomaly, it may be accompanied by numerous pathologies. Tricuspid valve prolapse is an extremely uncommon cause of primary tricuspid valve disease, as is its association with dextrocardia. In this instance, we wished to share our knowledge of tricuspid commissural prolapse together with dextrocardia, which we augmented with 3D images.

Result and technique consideration of radiofrequency catheter ablation of tachycardia in patients with dextrocardia.

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.

Successful management of ectopic kidney stones in a patient with situs inversus totalis: a rare case report.

BACKGROUND: Situs inversus totalis is a very rare congenital anatomical variation, in which all thoracic and abdominal organs are right-left inverted. This condition is associated with an increased risk of organ malformations including ectopic kidney, which is a very rare combination. CASE PRESENTATION: A 56-year-old male presented with colicky left iliac pain associated with nausea, vomiting, and irritative lower urinary symptoms. The patient has a medical history of recurrent lower urinary infections and a family history of situs inversus totalis. Radiological images demonstrated dextrocardia, situs inversus totalis of all the abdominal organs, and an ectopic pelvic kidney on the left side, with 4 stones inside it. Left nephrectomy was performed due to extensive renal damage. At discharge and during follow-up, the patient's condition was satisfactory and stable. CONCLUSIONS: The ectopic kidney may present diagnostic and therapeutic challenges when associated with situs inversus.

Severe COVID-19 acute respiratory distress syndrome in an adult with single-ventricle physiology: a case report.

BACKGROUND: COVID-19 can induce acute respiratory distress syndrome (ARDS). In patients with congenital heart disease, established treatment strategies are often limited due to their unique cardiovascular anatomy and passive pulmonary perfusion. CASE PRESENTATION: We report the first case of an adult with single-ventricle physiology and bidirectional cavopulmonary shunt who suffered from severe COVID-19 ARDS. Treatment strategies were successfully adopted, and pulmonary vascular resistance was reduced, both medically and through prone positioning, leading to a favorable outcome. CONCLUSION: ARDS treatment strategies including ventilatory settings, prone positioning therapy and cannulation techniques for extracorporeal oxygenation must be adopted carefully considering the passive venous return in patients with single-ventricle physiology.

Mirror-image dextrocardia: why is the apex impulse not on the right?

This article presents the case of an 11-year-old girl with a history of Fortan surgery who presented to the authors' department with shortness of breath, orthopnea, and cyanosis. Electrocardiography (ECG) was indicative of mirror-image dextrocardia despite location of the apex impulse on the left. Echocardiography suggested mirror-image dextrocardia accompanied by levoversion, a large atrial septal defect and left ventricular atresia (functional single atrium and single ventricle), and right ventricular hypertrophy. ECG with corrected leads placement showed a sinus rhythm, biatrail enlargement, and right ventricular hypertrophy. Based on echocardiography and medical history, the case was rediagnosed as mirror-image dextrocardia with levoversion.

Aortic valve replacement for dextrocardia with situs inversus totalis.

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.

Pulmonary root translocation in corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis with dextrocardia.

We present 13 years old with history of cyanosis and diagnosis of congenitally corrected transposition of great arteries with ventricle septal defect and pulmonary stenosis with dextrocardia. He underwent modified Senning procedure with pulmonary root translocation. Postoperative recovery was uneventful and was extubated on postoperative day 1. Patient remained in sinus rhythm. Postoperative echocardiography showed good biventricular function with no baffle obstruction and mild pulmonary regurgitation with no significant left and right outflow tract obstruction. Patient was discharged on postoperative day 6 and remained asymptomatic on follow up at 3 months.

Pulmonary Vein Isolation and Pacemaker Implantation in a Patient with Dextrocardia Situs Inversus.

A 70-year-old female with dextrocardia with situs inversus (DSI) totalis and inferior vena cava occlusion underwent radiofrequency catheter ablation because she had symptomatic paroxysmal atrial fibrillation (AF). Careful preoperative examination made successful pulmonary vein isolation through the left jugular vein approach. One-year later, however, AF recurred, and symptomatic sinus bradycardia or junctional bradycardia often occurred. Then, the pacemaker was implanted. We here reported a rare case of congenital abnormality, DSI with inferior vena cava occlusion who had undergone successful pulmonary vein isolation and pacemaker implantation without any complications.

Dextrocardia, situs solitus, inlet ventricular septal defect (VSD), and iatrogenic tricuspid regurgitation (TR)-A surgical perspective.

Dextrocardia with situs solitus is a rare congenital anomaly, often associated with intra- or extracardiac malformations. A clear understanding of the spatial orientation of the chambers of the heart for planning surgical approach, is imperative. Access to the right side of the heart, especially for repair of the tricuspid valve can be challenging. We describe a case of dextrocardia, situs solitus, inlet ventricular septal defect (VSD) with iatrogenic tricuspid regurgitation (TR) in an adult patient, highlighting the surgical aspects essential for a successful outcome.

Transseptal Approach for Mitral Valve Replacement in Dextrocardia with Situs Inversus Totalis: A Case Report and Review of the Literature.

We consider mitral valve disease requiring surgery in a patient with dextrocardia and situs inversus totalis to be an exceptional finding. The transseptal approach for mitral valve surgery in dextrocardia represents a technical challenge owing to its anatomic particulars. We present the case of a 56-year-old female patient who had been diagnosed with situs inversus totalis in childhood and with chronic atrial fibrillation in adulthood and was under oral anticoagulant treatment. She was referred to our hospital for increasing dyspnea and palpitation. Transthoracic echocardiography detected severe mitral regurgitation associated with moderate tricuspid regurgitation, with normal left and right ventricular function. Contrast chest computed tomography (CT) and preoperative abdominal CT showed both dextrocardia and situs inversus totalis, with normal continuity of the inferior vena cava. Biatrial cannulation was performed with the surgeon standing on the right side of the patient, and mitral valve replacement using a transseptal approach was performed with the surgeon standing on the left side of the patient. In this case report, we emphasize the rarity of mitral valve disease in a patient with dextrocardia and the inherent potential difficulty that can appear in this particular anatomic condition.

Stress cardiomyopathy in dextrocardia with situs inversus and anomalous coronary arteries.

We present a 53-year-old male with the rare constellation of stress cardiomyopathy, dextrocardia with situs inversus and anomalous coronary anatomy. This case highlights the difficulties faced when managing patients with uncommon disorders and demonstrates a rare overlap of acquired and CHD.

Catheter ablation of supraventricular tachycardia in patients with dextrocardia and situs inversus.

BACKGROUND: Dextrocardia with situs inversus is a rare cardiac positional anomaly. Catheter ablation procedures performed in this set of patients have not been sufficiently reported. METHODS: A total of 10 patients with dextrocardia and situs inversus who received catheter ablation for supraventricular tachycardia (SVT) were included from a cohort of over 20 000 cases of catheter ablation for SVT in three centers from 2005 to 2016. All patients underwent electrophysiologic study and catheter ablation of SVT. Ablation targets were selected based on different tachycardia mechanisms with the primary endpoint of noninduction of tachycardia. RESULTS: The average age was 32.4 ± 5.6 years. Congenitally corrected transposition of great arteries (TGA) with situs inversus and D-looping of the ventricles and aorta (congenitally corrected TGA {I,D,D}) was found in four patients, while the other six patients exhibited mirror-image dextrocardia {I,L,L}. The mechanisms of SVT were atrioventricular nodal reentrant tachycardia in four patients, atrioventricular reentrant tachycardia in three, typical atrial flutter in one, intra-atrial reentrant tachycardia in one, and focal atrial tachycardia in one. Immediate procedural success was achieved in 9 out of 10 patients with no procedural complications. During a follow-up period of 6.3 ± 3.5 years on average, all patients remained free from recurrent tachycardia. CONCLUSIONS: For patients with dextrocardia and situs inversus, catheter ablation of SVT is safe and feasible. Differences in catheter maneuver and fluroscopy projection, along with difficulties in distorted anatomy are major obstacles for successful ablation.

Congenital bronchopulmonary foregut malformation: systematic review of the literature.

BACKGROUND: Congenital bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory system. Early diagnosis is difficult, and delayed recognition may result in considerable complications. The aim of the study was to identify and analyze the clinical characteristics and radiological features of CBPFMs. METHODS: A systematic review was conducted in accordance with PRISMA guidelines. PubMed, Ovid database, EMBASE were searched for relevant publications to identify all published case-reports of CBPFM since 1992. Data about the demography, clinical presentation, pathology, imaging features, treatment and prognosis were collected. RESULTS: Sixty-one cases were included in our study. Cases were aged from 1 day to 59 years with the majority aged 3 years or younger. The most common type was group III (37.7%), followed by group II (29.5%)group I (27.9%) and group IV (4.9%). The presentations included respiratory distress (32.8%), cough/choking following food intake and other presentations associated respiratory infection. Thirty-eight cases (62.3%) were diagnosed by upper gastrointestinal series (UGI). Misdiagnosis was common. Eight cases (13.1%) of the included cases died. CONCLUSIONS: Early recognition and extensive delineation of the anatomy of CBPFM are important to correct these anomalies successfully. UGI is the first choice to confirm the abnormal bronchus communicating with the esophagus. Resection of abnormal pulmonary tissue, lobe or even unilateral lung is preferred. Reconstruction procedures are feasible in selected patients.

Scimitar syndrome: A rare case of recurrent pneumonia.

Scimitar syndrome is a rare, congenital malformation. It is characterized by partial anomalous pulmonary venous connection to inferior vena cava, hypoplastic right lung along with dextroposition of the heart. Depending on the severity of the defect it can present as early as in neonatal period or incidentally later in life. Its incidence is 1-3 per 100,000 patients. We present a case of a two-year-old girl who presented with recurrent fever, cough and was later diagnosed as scimitar syndrome.

STEMI in a patient with mirror-image dextrocardia.

ST-segment elevation myocardial infarction (STEMI) is a potentially life-threatening diagnosis that cannot be missed on ECG interpretation. However, ECG interpretation may not be straightforward in patients with dextrocardia. This case report discusses the presentation of a patient with situs inversus with dextrocardia who was found to have an acute inferior MI, and how this anatomical change results in an atypical ECG and clinical presentation.

Narrow QRS tachycardia with extreme rightward axis. What is the mechanism?

A 25 year old lady presented with palpitation to the emergency department. Her pulse rate was 210 beats per minute. She was hemodynamically stable with a blood pressure of 100/60 mm Hg. 12 lead surface electrocardiogram (ECG) was recorded during the presentation (Fig. 1) and rhythm strip (Fig. 2A) during administration of IV adenosine. What is the likely diagnosis?