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Congenital Heart Disease (CHD) is one of the most common developmental anomalies seen in children and there is an increased risk of developing oral disease and its systemic effects in children with CHD. The present case report highlights the dental management of a 10-year-old boy with CHD and dextrocardia. The diagnosis for the patient included dextrocardia (where the position of the heart is developmentally changed from the left to the right-side), complete atrioventricular septal defect and double outlet right ventricle with pulmonary stenosis. After consultation, diagnosis and treatment planning and upon obtaining consent from the parents and modifying the child's behaviour, the dental needs of the child were addressed on a visit-by-visit basis. A quadrant approach was adopted to complete the extractions of all retained carious primary teeth, followed by restorative treatment. Oral prophylaxis was performed, followed by topical fluoride application and the patient was then referred to the Department of Orthodontics for treatment of malalignment. This case report focuses on the guidelines followed for antibiotic prophylaxis against Infective Endocarditis (IE) and highlights the importance of maintaining good oral health for this group of patients. The dental management of children with CHD can be complex, as oral health is often neglected by both parents and patients. Cardiovascular problems can significantly impact both the child and the parent, affecting management and financial implications. The dental considerations require early diagnosis of dental problems and prompt treatment to prevent complications and difficulties in implementing treatment due to systemic repercussions. The present case report aimed to improve dental care for children with severe systemic alterations, as there is a lack of scientific literature regarding the dental management of paediatric patients with CHD. [ABSTRACT FROM AUTHOR]

Dextrocardia is a rare congenital malformation in which the heart is located in the right hemithorax, with an incidence of around 1 in 12,000. Dextrocardia usually remains asymptomatic until accidentally diagnosed through echocardiography and chest radiography, which are mostly performed for other reasons. The complex heart anatomy in dextrocardia can pose great challenges, particularly in patients requiring surgical interventions. In this case presentation, we aimed to emphasize that a multidisciplinary approach is recommended in managing patients with dextrocardia. A 54-year-old female patient arrived with nausea, vomiting, fatigue, and tremor complaints. Medical evaluations revealed bradycardia, which developed after the use of propranolol for a panic attack. The patient underwent an angiography due to the existence of non-ST elevation myocardial infarction and Wenckebach's atrioventricular block, which showed the deviation of the left coronary artery to the right and of the right coronary artery to the left, raising suspicion of dextrocardia. Chest radiography confirmed the presence of dextrocardia. The patient received the necessary treatment, since dextrocardia poses minimal risk, the patient was discharged with follow-up recommendations. [ABSTRACT FROM AUTHOR]

Background: A heart positioned on the right side of the thorax can be more a complex situation than it seems, also the potentially associated congenital cardiopathies are variable. In this regard, patients with dextrocardia presenting with complete atrioventricular block require a thorough anatomical investigation to map the veno-arterial system and potential cardiovascular defects before proceeding with a permanent pacing procedure. Case presentation: A 73-year-old Caucasian woman with no significant medical history presented with syncope. Physical examination showed regular heart sounds with an average heart rate of 35–40 beats per minute and no other relevant physical findings. Chest X-rays revealed a heart positioned in the right hemithorax. A standard electrocardiogram revealed complete atrioventricular block with junctional escape rhythm at nearly 35–40 beats per minute. A transthoracic echocardiogram showed a mirror image dextrocardia without any other remarkable abnormalities. Venogram performed using the left peripheral cephalic vein showed normal venous return into the right atrium positioned on the left side. Surgical intervention consisted of permanent dual chamber pacemaker implantation using the left subclavian vein. Discussion is provided based on the relevant medical literature outlining different potential scenarios with associated cardiac and major vessels abnormalities. Conclusion: This case highlights an atypical presentation of atrioventricular block in a patient with dextrocardia and situs inversus, for whom a successful dual-pacemaker implantation procedure was performed. [ABSTRACT FROM AUTHOR]

Background Cardiac interventions may be challenging in patients with congenital cardiac abnormalities. This case reports cardiac resynchronization therapy pacemaker (CRT-P) implantation and single catheter ablation of atrioventricular node (AVN) with remote magnetic navigation (RMN) via peripheral vascular access in a patient with Kartagener's syndrome and permanent atrial fibrillation (AF). Case summary A 74-year-old male with situs inversus presented for treatment of permanent AF and severe heart failure. In echocardiography, left ventricular ejection fraction was 30%, and there was severe dyskinesia due to a left bundle branch block. After successful CRT-P implantation, we performed AVN ablation because biventricular (BiV) pacing was <75% despite maximal rate control medication. The ablation catheter was inserted from the right basilic vein, and no other catheters were used. Despite peripheral vascular access, manipulation of the ablation catheter with RMN was easy, and the ablation was successful. After the ablation, BiV pacing instantly increased to 100%, and left ventricular function and symptomatic status improved gradually. Conclusions Cardiac resynchronization therapy pacemaker implantation and RMN-guided single catheter ablation of the AVN in a patient with dextrocardia via peripheral vascular access was effective and safe. The use of RMN and peripheral vascular access may offer important advantages also in other patient groups. [ABSTRACT FROM AUTHOR]

Introduction: Kartagener Syndrome is a clinical variant of primary ciliary dyskinesia It is a rare autosomal recessive disease, one in every 32,000 births, characterized by a triad of bronchiectasis, chronic sinusitis and situs inversus partial o total. We present the case female with this disease and review the diag-nostic, therapeutic strategies and prognosis. Case description: A 20-year-old female student denies toxic habits with history of repeated respiratory infections since childhood, nasal polypec-tomy; presents persistent cough with purulent expectoration, mild dyspnea, sporadic febrile episodes, and constitutional symptoms. On physical examination: regular general condition, tachypnea, tachycardia hypoxemia sat O2 89% baseline, lungs were auscul-tated crackles at the left base. Tests: hemogram (leukocytosis with neutrophilia), 3 sputum smears (negative), Sudan IV (negative), amylase (negative), chest X-ray showing dextrocardia and bron-chiectasis in the lingula high resolution chest tomography, showed bilateral cystic bronchiectasis predominantly in lingual abdominal ultrasound revealed: situs inversus, electrocardiogram: right axis, Conclusion: Kartagener Syndrome is a rare disease that has a clinical presentation similar to other respiratory tract conditions. It is important to know its pathophysiology and clinical characteristics to establish a proper diagnosis and guarantee specialized mul-tidisciplinary treatment. This clinical entity should be considered when finding partial or total situs inversus to specify a diagnosis.

Background: Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. Methods: We retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. Results: Dextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. Conclusion: Our study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.

Abstract Background A heart positioned on the right side of the thorax can be more a complex situation than it seems, also the potentially associated congenital cardiopathies are variable. In this regard, patients with dextrocardia presenting with complete atrioventricular block require a thorough anatomical investigation to map the veno-arterial system and potential cardiovascular defects before proceeding with a permanent pacing procedure. Case presentation A 73-year-old Caucasian woman with no significant medical history presented with syncope. Physical examination showed regular heart sounds with an average heart rate of 35–40 beats per minute and no other relevant physical findings. Chest X-rays revealed a heart positioned in the right hemithorax. A standard electrocardiogram revealed complete atrioventricular block with junctional escape rhythm at nearly 35–40 beats per minute. A transthoracic echocardiogram showed a mirror image dextrocardia without any other remarkable abnormalities. Venogram performed using the left peripheral cephalic vein showed normal venous return into the right atrium positioned on the left side. Surgical intervention consisted of permanent dual chamber pacemaker implantation using the left subclavian vein. Discussion is provided based on the relevant medical literature outlining different potential scenarios with associated cardiac and major vessels abnormalities. Conclusion This case highlights an atypical presentation of atrioventricular block in a patient with dextrocardia and situs inversus, for whom a successful dual-pacemaker implantation procedure was performed.

Background: Situs inversus (SI) totalis is a rare congenital anomaly characterized by complete inversion of abdominal and thoracic visceral organs. While renal abnormalities have been documented in such cases, the association with nephrotic syndrome (NS) is exceptionally rare. Clinical Description: A 2.5-year-old girl, diagnosed with NS, a year ago, presented with anasarca due to relapse. Routine clinical examination showed normal vital parameters, anthropometry, mild pallor, and pitting edema. While abdominal examination revealed ascites, cardiovascular examination was suggestive of dextrocardia which was confirmed by echocardiogram without structural heart defects. Ultrasound abdomen liver, spleen, and stomach showed lateral inversion. Laboratory investigations were consistent with a relapse of NS. Management and Outcome: The child was managed with corticosteroids along with anti-edema measures, resulting in remission. Conclusion: This case brings out the exceptional rarity of SI totalis coexisting with NS and encourages further research into potential genetic and immunological factors which may explain this coexistence.

We present the case of a 52-year-old female patient on optimal medical therapy (OMT) for heart failure with severely reduced ejection fraction. She was diagnosed with dextrocardia and congenital corrected transposition of the great arteries (ccTGA) by echocardiography. Despite 3 months of OMT and a persisting NYHA II functional state, she was referred for primary prevention ICD implantation. At admission echocardiography revealed a hypertrophic systemic ventricle with an ejection fraction of 27%. Cardiac CT confirmed ccTGA, dextrocardia, a normal extracardiac and epicardial venous system. Coronary artery disease was excluded. Our team opted for left-sided VDD-ICD implantation in case of a need for AV-synchronous pacing and to enhance arrhythmia discrimination. An active fixation shock lead was placed in the mid-septal region of the functional right ventricle. The defibrillation threshold test (DFT) was successful at 20 J. No periprocedural complications were observed. At the 12-month ambulatory visit, device interrogation showed good function, no detected arrhythmias, and no antitachycardia therapy was delivered. Preoperative imaging is essential prior to any invasive cardiac procedure in patients with congenital heart disease. Performing a DFT test at adult congenital heart disease patients may be reasonable.

Benign situs inversus totalis of the viscerum is often diagnosed accidentally, rarely in adults, and more frequently in children and neonates, affecting both sexes. In this report, a young female patient accidentally discovered a situs inversus totalis after computed tomography for acute abdominal pain. In this uncommon anatomical abnormality, the major visceral organs are reversed in the opposite direction. This report highlights the importance of being aware of and considering situs inversus in clinical practice, particularly when interpreting imaging findings and planning medical procedures. This is critical for differential diagnosis and comorbidities that may affect those patients. The cause of situs inversus totalis is still unknown; however, this condition is frequently asymptomatic, particularly in infants, and is sometimes associated with other syndromes. The patient arrived at the emergency department with left flank pain, nausea, and fever. In the first ultrasonography, a strange anatomy was suspected; thus, a contrasted computed tomography was performed. The patient had never had a computed tomography scan before. The identification of situs inversus totalis was unexpected and coincidental; the computed tomography images were carefully examined. In patients with chest or abdominal pain, clinicians may consider situs inversus totalis based on computed tomography, particularly if without clinical and imaging history. This knowledge can help in the differential diagnosis, avoiding unneeded interventions. Moreover, comorbidities that affect several systems, particularly cardiovascular and pulmonary systems, affect quite a few patients with situs inversus totalis, who require careful examination and lifelong monitoring.

Background: Phlebectasia is a term used for the dilatation of a vein without tortuosity. Rarely, the condition may affect the internal jugular vein (IJV), causing prominence in the neck, which may be confused with other neck swellings. Clinical Description: A 6-year-old boy presented with bilateral episodic swelling in the neck noticed since 2 years of age and a hoarse voice since 3 years of age. On examination, the neck swelling was soft, cystic, compressible, nontender, and nonpulsatile, increasing in size by all maneuvers increasing intrathoracic pressure. Systemic examination revealed the apex beat was on the right side, and the liver was palpable below the left subcostal margin. Management and Outcome: The workup for tuberculosis was negative, and direct laryngoscopy was normal. X-ray of the chest with abdomen showed dextrocardia and stomach shadow on the right side. Color Doppler revealed nontortuous dilatation of both IJVs. Dynamic computed tomography venography confirmed the diagnosis of bilateral IJV phlebectasia (IJVP). The child was started on speech therapy. The mother was counseled regarding the benign nature of the disease and advised regular follow-up. The child had no complications, and his voice improved with speech therapy at 1-year follow-up. Conclusion: Pediatricians should be aware that IJVP may rarely be the cause of neck swelling associated with hoarseness of voice. Increase in the size of the swelling with Valsalva maneuvers and color Doppler ultrasound helps to confirm the diagnosis.

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.

Kefelegn Dejene Tadesse,1 Yidnekachew Asrat Birhan2 1Cardiology Department, Cardiac Center of Ethiopia, Addis Ababa, Ethiopia; 2Internal Medicine Department, Addis Ababa University, Addis Ababa, EthiopiaCorrespondence: Kefelegn Dejene Tadesse, Email kefruit@gmail.comAbstract: Percutaneous transvenous mitral commissurotomy is an established treatment for rheumatic mitral stenosis. Dextrocardic heart can be affected by rheumatic pathology just like the normal heart. However, it is technically demanding to percutaneous interventions especially the septal puncture which should be done in a mirror image of the normal. Here we are reporting a 45 year old woman with severe rheumatic mitral stenosis with dextrocardia with situs inversus totalis. She has undergone a successful percutaneous transvenous mitral commissurotomy.Keywords: dextrocardia, mitral stenosis

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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. [ABSTRACT FROM AUTHOR]

Background. Biliary atresia is the leading cause of liver transplantation in children. It is associated with other congenital anomalies. The presentation of biliary atresia with situs inversus is a rare case. Case presentation. A 2-month-old female presented with jaundice since 15 days of life, acolic stools, and tea-colored urine. On further evaluation, she was found to have dextrocardia and situs inversus. A liver biopsy was performed and showed hepatic fibrosis due to extrahepatic cholestasis. Abdominal ultrasound and MRCP confirmed a liver in the left hypochondrium and suggested biliary atresia. Conclusion. Although rare, every patient with biliary atresia should be screened for congenital situs inversus, which has a poor prognosis. [ABSTRACT FROM AUTHOR]

ABSTRACT Background Dextrocardia with atrial fibrillation (AF) complicates radiofrequency ablation treatment. Methods A case of successful AF ablation in dextrocardia, guided by intracardiac echocardiography (ICE) and the Carto 3 high‐density mapping system, is reported. Results ICE‐guided transseptal puncture and three‐dimensional mapping facilitated successful pulmonary vein isolation (PVI). The patient had a good recovery with no recurrence. Conclusion ICE and Carto 3 system's high‐density mapping aid in ablation for abnormal cardiac anatomy, reducing surgical complications.

Introduction: Situs inversus totalis (SIT), a rare congenital disorder, involves complete reversal of the heart's position and mirrored arrangement of visceral organs. Early diagnosis is crucial for managing complications like transposition of the great vessels and Kartagener syndrome, marked by bronchiectasis and sinusitis due to ciliary dysfunction. Case presentation: A 52-year-old male with diabetes mellitus presented with large amounts of watery diarrhea, vomiting, dizziness, and palpitations. He managed his diabetes with 70 units of insulin daily and had a history of chronic obstructive pulmonary disease. Physical examination revealed hypotension, tachycardia, and a right-sided apex beat. Investigations, including chest X-ray, ultrasound, and ECG, confirmed dextrocardia with situs inversus and hypovolemic shock secondary to acute gastroenteritis. The patient responded well to treatment and was discharged on the second day, continuing diabetic management. Discussion: SIT is a genetically determined anomaly with an incidence ranging from 1:35 000 to 1:1400, averaging 1:10 000, with a higher prevalence in males. Many patients are asymptomatic, with the condition often detected incidentally. Diagnostic imaging techniques, including chest radiography, CT scans, MRI, and ultrasonography, are crucial in confirming SIT. ECG findings, such as right-axis deviation and reversed QRS complexes, are valuable in diagnosing dextrocardia. Patient education is essential to empower individuals with SIT to navigate their healthcare, prevent complications, and reduce anxiety. Conclusion: SIT poses diagnostic challenges due to its incidental discovery. Accurate diagnosis relies on thorough imaging and clinical evaluation. Patient education is vital for informed care and anxiety reduction. Case reviews underscore the importance of healthcare providers' readiness to diagnose SIT effectively despite unrelated symptoms. Resumen: Introducción: Situs inversus totalis, un trastorno congénito raro, implica la inversión completa de la posición del corazón y la disposición en espejo de los órganos viscerales. El diagnóstico temprano es crucial para manejar complicaciones como la transposición de los grandes vasos y el síndrome de Kartagener, caracterizado por bronquiectasias y sinusitis debido a la disfunción ciliar. Presentación del Caso: Un hombre de 52 años con diabetes mellitus presentó grandes cantidades de diarrea acuosa, vómitos, mareos y palpitaciones. Controlaba su diabetes con 70 unidades de insulina diarias y tenía antecedentes de EPOC. El examen físico reveló hipotensión, taquicardia y un latido apical en el lado derecho. Las investigaciones, incluyendo radiografía de tórax, ecografía y ECG, confirmaron dextrocardia con situs inversus y choque hipovolémico secundario a gastroenteritis aguda (AGE). El paciente respondió bien al tratamiento y fue dado de alta al segundo día, continuando con el manejo de la diabetes. Discusión: SIT es una anomalía determinada genéticamente con una incidencia que varía de 1:35,000 a 1:1400, promediando 1:10,000, con una mayor prevalencia en hombres. Muchos pacientes son asintomáticos, con la condición a menudo detectada incidentalmente. Las técnicas de diagnóstico por imagen, incluyendo radiografía de tórax, tomografías computarizadas, resonancia magnética y ultrasonografía, son cruciales para confirmar SIT. Los hallazgos del ECG, como la desviación del eje derecho y los complejos QRS invertidos, son valiosos para diagnosticar la dextrocardia. La educación del paciente es esencial para empoderar a las personas con SIT a navegar su atención médica, prevenir complicaciones y reducir la ansiedad. Conclusión: Situs inversus totalis (SIT) presenta desafíos diagnósticos debido a su descubrimiento incidental. Un diagnóstico preciso depende de una evaluación clínica e imagenológica exhaustiva. La educación del paciente es vital para una atención informada y la reducción de la ansiedad. Las revisiones de casos subrayan la importancia de que los proveedores de atención médica estén preparados para diagnosticar SIT de manera efectiva a pesar de los síntomas no relacionados.

Background: Dextrocardia with atrial fibrillation (AF) complicates radiofrequency ablation treatment. Methods: A case of successful AF ablation in dextrocardia, guided by intracardiac echocardiography (ICE) and the Carto 3 high‐density mapping system, is reported. Results: ICE‐guided transseptal puncture and three‐dimensional mapping facilitated successful pulmonary vein isolation (PVI). The patient had a good recovery with no recurrence. Conclusion: ICE and Carto 3 system's high‐density mapping aid in ablation for abnormal cardiac anatomy, reducing surgical complications. [ABSTRACT FROM AUTHOR]

Dextrocardia with situs inversus are rare congenital anomalies which can be asymptomatic and compatible with normal life. They are characterized by mirror images of all intra-thoracic and intra-abdominal viscera. Our aim is to report an incidental finding of dextrocardia with situs inversus in a neonate with neonatal sepsis. A day-old male term neonate presented with features of infection. Physical examination revealed cardiac apex on the 4th right intercostal space, along the mid-clavicular line. Chest radiograph and abdominal ultrasound confirmed the diagnosis of dextrocardia with situs inversus. Bilateral cervical ribs were also seen on chest radiograph. He was managed with antibiotics and discharged. Newborn babies should have a thorough physical examination after delivery before discharge to enable early diagnosis of congenital anomalies for appropriate referral.

The extracardiac Fontan procedure is the final common pathway for palliation of patients with univentricular physiology. The procedure is typically performed on cardiopulmonary bypass with or without fenestration using an adequate-sized polytetrafluoroethylene graft as the extracardiac conduit. On a few occasions, some patients may develop narrowing of their conduit with or without calcifications, which is often managed by various transcatheter techniques. Rarely, some of these conduits must be replaced surgically. In the current report, we present an alternative to standard surgical replacement of the calcified Fontan conduit in a patient with dextrocardia.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital anomaly defined by double discordance – atrioventricular and ventricular-arterial. ccTGA frequently has associated lesions, which influence long-term survival.

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.

A five-month-old male child was brought by his parents to the Paediatric Outpatient Department, MGACH and RC with complaints of a persistent cough for a week. The cough was non productive and associated with mild fever. There was no significant past history of Inpatient Department admissions or any surgical procedures. The infant was born to a primiparous mother at full term via normal delivery. There was no significant family history of diabetes mellitus, hypertension, or known cases of any serious illness in close relatives.

Respiratory distress in the newborn is associated with numerous etiologies, some common and some rare. When respiratory distress is accompanied by laterality defects, namely, situs inversus (SI), the index of suspicion for comorbid primary ciliary dyskinesia (PCD) should be raised. Primary ciliary dyskinesia is characterized by ciliary dysmotility and the accumulation of thick secretions in the airways that obstruct air and gas exchange. Neonatal clinicians should know that while PCD is definitively diagnosed in infancy or early childhood, findings suspicious for PCD should be communicated to primary care providers at discharge from the hospital to facilitate timely subspecialty involvement, diagnosis, and treatment. This article will present a case report of a term newborn with SI totalis who was later diagnosed with PCD. We will discuss epidemiology, pathophysiology, clinical manifestations, and diagnostics, followed by management strategies. Additionally, we discuss the outpatient needs and lifespan implications. [ABSTRACT FROM AUTHOR]

Situs inversus totalis (SIT) is a rare medical condition characterized by a complete mirror-image reversal of the normal positioning of the internal organs. Aristotle initially described situs inversus in animals, while Fabricius first characterized it in humans. Although the specific cause is uncertain, there is evidence of autosomal recessive and X-linked inheritance. Before seeking treatment for an unrelated ailment, many people with SIT are unaware of their distinct anatomy, as in our case. The presented case is a 30-year-old female patient from Central Ethiopia, presented to Hakim Gizaw Teaching Hospital outpatient department of medicine with the complaint of right-sided anterior chest pain for five days. Clinically, the apical beat was heard in the right 5th intercostal space. After undergoing an electrocardiogram (ECG), echocardiogram, chest X-ray, and abdominal ultrasound, she was diagnosed with situs inversus totalis. The clinical implications of SIT encompass challenges in diagnosis and procedures, potential congenital cardiac abnormalities, considerations for organ transplantation, and clinical decision-making, particularly in emergency scenarios. Key Clinical Messages. This case highlights the clinical implications of dextrocardia with situs inversus totalis, emphasizing the importance of awareness for accurate diagnosis, procedural challenges, and informed clinical decision-making. Understanding this rare condition is crucial for providing appropriate medical care and to navigate potential complications in affected individuals. [ABSTRACT FROM AUTHOR]

The extracardiac Fontan procedure is the final common pathway for palliation of patients with univentricular physiology. The procedure is typically performed on cardiopulmonary bypass with or without fenestration using an adequate-sized polytetrafluoroethylene graft as the extracardiac conduit. On a few occasions, some patients may develop narrowing of their conduit with or without calcifications, which is often managed by various transcatheter techniques. Rarely, some of these conduits must be replaced surgically. In the current report, we present an alternative to standard surgical replacement of the calcified Fontan conduit in a patient with dextrocardia. [ABSTRACT FROM AUTHOR]

We present the case of a 74-year-old man with dextrocardia and situs inversus who presented with non-ST-elevation acute myocardial infarction. The patient underwent successful coronary angiography without requiring percutaneous coronary intervention or coronary artery bypass grafting. We discuss the patient’s clinical characteristics, electrocardiography findings, diagnosis, and treatment, and review the relevant literature.

Introduction: Dextrocardia is rare in the general population and may be associated with significant additional cardiac malformations. It is commonly associated with additional cardiac malformations. In this report, we have described the follow-up of a patient with Situs inversus dextrocardia and cyanogen complex cardiopathy in a 16-year-old Albanian male. The male patient born on 2007 in Albania, was referred to our ambulatory at 6 months of life by pediatrician cause of cyanosis and cardiac murmur. It was performed the echo Color Doppler examination, with the conclusion: situs inversus dextrocardia, unique ventricle, pulmonary arterial atresia. On 2008, a diagnostic catheterization was performed. The medico-chirurgical consultation has decided to leave the boy in natural history with a periodic follow–up. On 06.2009 in one of the routine examinations, there was make evidence of hypertrophy of the unique ventricle associated with arteria hypertension. From that time the patient is under medical treatment with periodic monitoring. Conclusions: The regular follow up of complex cyanogen congenital heart disease improve health care towards risqué target group. In heave desaturations patient the hypertension must be valuated as secondary complication of primary problem.

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

Situs inversus totalis (SIT) with dextrocardia is a rare congenital anomaly that poses a surgical challenge. This case report presents the first known case of a totally 3D endoscopic mitral valve replacement (MVR), which was performed in a 75-year-old woman with SIT and severe functional mitral regurgitation. Despite the anatomical complexity, the procedure was successfully completed using a simplified three-port system and a 3D endoscope by requiring careful preoperative planning and intraoperative adaptation to the mirrored anatomy of SIT. This case report demonstrates the feasibility and potential benefits of totally endoscopic MVR in patients withSIT. [ABSTRACT FROM AUTHOR]

A letter published in the Archives of Gynecology & Obstetrics explores the potential association between COVID-19 infection and fetuses with mirror-image dextrocardia. The study conducted an epidemiological investigation to analyze the incidence of mirror-image dextrocardia in pregnant women and fetuses before and after the lifting of COVID-19 control measures in China. The findings suggest that there is a significant increase in the incidence of mirror-image dextrocardia in fetuses after the lifting of COVID-19 restrictions, with all pregnant women with this condition having a history of SARS-CoV-2 infection. The study proposes that maternal fever caused by the virus may interfere with the normal development of the embryonic heart. However, further research is needed to understand the specific mechanism. [Extracted from the article]

Congenital birth defects of brachy/polysyndactyly class can occur as isolated defects or as a part of syndromes and should raise suspicion of associated heart defects. Poland syndrome is one such congenital condition with its characteristic features. Although rare, it can significantly impact a patient’s quality of life due to its physical and psychological effects. This case report aims to contribute to the limited body of literature by detailing the clinical presentation, diagnosis, and cardiac defects associated with Poland’s syndrome.

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. [ABSTRACT FROM AUTHOR]

The article discusses a case of a 25-year-old primigravida with a fetus showing mirror-image dextrocardia and thoracic-abdominal discordance, detected through fetal echocardiography. Three-dimensional high-definition live tissue virtual dissection, including HD-live tissue rendering and 3D/4D spatiotemporal image correlation HD-live color rendering, was utilized to comprehensively analyze the complex cardiac morphology.

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Key Clinical Message Situs inversus totalis is a rare diagnosis, but the likelihood of experiencing myocardial infarction is presumed to be comparable to the general population average. In individuals exhibiting situs inversus with suspected myocardial infarction, ECG recording, including right precordial leads, is crucial for diagnostic assessment. Coronary angiography and intervention should be performed with standard equipment using inverted maneuvers and radiographic projections.

We report a successful percutaneous mitral balloon commissurotomy via left transhepatic venous access in a 42-year-old female patient with dextrocardia, situs inversus totalis, and inferior vena cava interruption. fWe also discuss the revisions required for optimal trans-septal approach from the left transhepatic vein.

Background: Kartagener's syndrome, a rare autosomal recessive genetic disorder, is characterized by primary ciliary dyskinesia (PCD), resulting in defective cilia function in the respiratory tract and fallopian tubes. Case presentation: This case report discusses a 23-year-old female with Kartagener's syndrome, bronchiectasis, and cardiac involvement, who presented with shortness of breath, cough, and syncope. Notably, she received home oxygen therapy but became exhausted, leading to loss of consciousness. Clinical examination revealed prominent heart sounds and abnormal lung findings. Laboratory results indicated leukocytosis, and an ECG confirmed dextrocardia and cardiac abnormalities. Doppler studies identified mitral and tricuspid regurgitation along with severe pulmonary arterial hypertension. Antibiotics were administered for coagulase-negative Staphylococcus infection. The patient improved with a treatment regimen, including oxygenation and nebulization. Regular follow-up and patient education were emphasized. Conclusion: This case underscores the complexity of Kartagener's syndrome and the importance of a multidisciplinary approach in managing its respiratory and cardiac manifestations. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Situs inversus totalis is a rare diagnosis, but the likelihood of experiencing myocardial infarction is presumed to be comparable to the general population average. In individuals exhibiting situs inversus with suspected myocardial infarction, ECG recording, including right precordial leads, is crucial for diagnostic assessment. Coronary angiography and intervention should be performed with standard equipment using inverted maneuvers and radiographic projections. [ABSTRACT FROM AUTHOR]