Leadless pacemaker implantation in dextrocardia with situs viscerum inversus: A case report and literature review.

Leadless pacemaker implantation (LPI) has fewer device complications and reduced chance of infection compared to conventional pacemakers. Dextrocardia with situs viscerum inversus (DC+SVI) is a rare condition, which seldom leads to cardiac complications. However, its presence poses a challenge to operators in cardiac procedures. LPI reports in DC patients are scarce. We report a case of LPI in a DC+SVI patient, followed by a brief but comprehensive literature review.

VACTERL Association with Situs Inversus Totalis: A Unique Combination.

BACKGROUND: VACTERL association is a sporadic, nonrandom series of congenital malformations diagnosed by the presence of three or more of the following: vertebral malformations, anal atresia, cardiac defects, tracheoesophageal fistula, renal malformations, and limb malformations. Situs inversus totalis (SIT) and esophageal malformations are rarely associated. This is the first reported case in North America of VACTERL association with SIT. IMPLICATIONS FOR PRACTICE: Respiratory distress in the term infant requires full exploration of all possible causes because the etiology may be far more complex than routinely diagnosed respiratory distress syndrome. This particular case demonstrates physical exam findings and supportive imaging that would be observed in infants with VACTERL association and with SIT, highlighting considerations when, rarely, both occur simultaneously.

Portal vein embolisation in a patient with situs inversus.

Portal vein embolisation (PVE) is a well-established technique used for patients who require major hepatic resections without sufficient volume of future remnant liver (FRL). We describe a case of PVE in a patient with situs inversus. Computed Tomography (CT) 4 weeks after the procedure demonstrated significant hypertrophy of the FRL. However, the surgical procedure was aborted due to signs of extrahepatic progression.

[Situs inversus totalis: a case report]. / Situs inversus totalis: reporte de un caso.

The situs inversus totalis is a rare condition associated with multiple congenital malformations, the most common heart defects and is associated with more complex syndromes such as Kartagener. Most of the time, the defect is found after a physical examination for other reason. We reported the case of a patient diagnosed on the first day of extra-uterine life. It has been the first case reported in the state of Guerrero, with several prenatal history of interest. Our aim is to emphasize that early diagnosis completely changes the future healthcare. Reviewing the literature, it does not have any algorithm on searching protocol of congenital malformations and management attention on common pathologies.

El situs inversus totalis es una entidad rara asociada con múltiples malformaciones congénitas, las más frecuentes son las cardiacas; asimismo, se asocia a síndromes más complejos como el de Kartagener. En la mayoría de las ocasiones, la entidad es un hallazgo tras un examen físico por alguna otra razón. Presentamos el caso clínico de un paciente diagnosticado en el primer día de vida extrauterina; siendo este el primer caso reportado en el estado de Guerrero, con numerosos antecedentes prenatales de interés. Nuestro objetivo es enfatizar que el diagnóstico temprano modifica por completo la atención médica futura. Al revisar la literatura no se cuenta con algoritmo alguno sobre el protocolo de búsqueda de malformaciones congénitas, así como sobre el manejo de atención en patologías comunes.

Jalili syndrome presenting with situs inversus totalis and keratoconus: the first case in the Indian subcontinent.

Jalili syndrome (JS) (MIM#217080) is a rare genetic disorder characterized by the comorbid appearance of cone-rod dystrophy (CORD) and amelogenesis imperfecta (AI). JS is an autosomal recessive inherited disorder caused by different mutations, all with a linkage at achromatopsia locus 2 q11 on the metal transporter gene CNNM4. The case report presented here describes JS with distinct phenotypic variations such as situs inversus totalis (SIT) along with additional ophthalmic findings such as keratoconus and ectopia lentis. It is the first case of JS reported from the Indian subcontinent, affecting a male patient of Muslim faith from an area having high fluoride levels in the ground water. A positive history of consanguineous marriage among his family members of past generations was also evident.

Primary ciliary dyskinesia and neonatal respiratory distress.

BACKGROUND AND OBJECTIVE: Primary ciliary dyskinesia (PCD) is a rare inherited disease affecting motile cilia lining the respiratory tract. Despite neonatal respiratory distress as an early feature, diagnosis is typically delayed until late childhood. Our objective was to identify characteristics that differentiate PCD from common causes of term neonatal respiratory distress. METHODS: This was a case-control study. Patients with PCD born after 1994 attending a regional PCD clinic who had a history of neonatal respiratory distress (n = 46) were included. Controls (n = 46), term neonates with respiratory distress requiring a chest radiograph, were randomly selected from hospital birth records and matched on gender, birth month/year, and mode of delivery. Multiple logistic regression was used to determine the association between neonatal characteristics and PCD diagnosis. The diagnostic performance of the best predictive variables was estimated by calculating sensitivity and specificity. RESULTS: PCD cases required more oxygen therapy (39 cases, 29 controls, P = .01), longer duration of oxygen therapy (PCD mean = 15.2 days, control mean = 0.80 days, P < .01), had later onset of neonatal respiratory distress (PCD median = 12 hours, control median = 1 hour, P < .001), and higher frequency of lobar collapse and situs inversus (PCD = 70% and 48% respectively, control = 0% for both, P < .001). Situs inversus, lobar collapse, or oxygen need for >2 days had 87% (95% confidence interval: 74-94) sensitivity and 96% (95% confidence interval: 85-99) specificity for PCD. CONCLUSIONS: When encountering term neonates with unexplained respiratory distress, clinicians should consider PCD in those with lobar collapse, situs inversus, and/or prolonged oxygen therapy (>2 days).

Lung cancer in situs inversus totalis (SIT)--literature review.

We present 21 studies of cases of lung cancer in patients with situs inversus totalis (SIT) published worldwide. The first case was described in 1952. Thirteen patients were from Japan, 4 from Eastern Europe, including 2 Polish cases from the authors` center (Department of Thoracic Surgery, Pomeranian Medical University in Szczecin, Poland), 2 from Western Asia, 1 from the U.S. and 1 from Australia. Male patients (20/21) as well as left-sided lung cancer cases (14/21) and squamous cell carcinoma cases (8/21) dominated in the entire group. Thirteen patients underwent surgical treatment. There were 10 left-sided and 3 right-sided surgical interventions with uneventful intra- and postoperative course. Explorative thoracotomy was performed in one case only on the right side. Upper lobectomy was performed in 5 cases, pneumonectomy in 3 cases, lower bilobectomy and middle lobectomy in one case and lower lobectomy in two cases. Surgery was performed through thoracotomy in 10 cases, VATS-assisted approach in two cases and sternotomy in one case. Descriptions of the surgical anatomy confirmed mirror image of the anatomy in all cases and were consistent with the preoperative CT images. Preoperative diagnosis was discussed including the role of 3-D reconstruction of CT for improving perioperative safety in this group of patients. In conclusion, lung cancer/SIT cases despite inversed but regular anatomy can be operated on radically as cases with normal anatomy with preservation of intraoperative security level.

Primary angioplasty and later elective multivessel stenting in a patient with dextrocardia: a case report and literature review.

Dextrocardia is a rare congenital anomaly with a prevalence of 1 in 10,000 births. The incidence of coronary artery disease in such patients is thought to be similar to that of the general public, however, patients are seldom seen during routine clinical practice and at the cardiac catheterization laboratory. Patients with this condition and acute myocardial ischemia may pose challenges at presentation, clinical and diagnostic findings and at cardiac catheterization. In this report, a male patient presenting with acute inferior myocardial infarction and three-vessel coronary artery disease was managed successfully with emergency primary angioplasty and later elective multivessel stenting. This is the first combined primary and later multivessel stenting to be reported in a single patient with dextrocardia and situs inversus. The clinical findings, details of the procedures and literature review will be discussed.

Total situs inversus: a rare anomaly presenting to a chiropractic teaching clinic.

OBJECTIVE: The purpose of this article is to describe a patient with a previously undiagnosed rare anomaly, total situs inversus (TSI), and discuss the range of clinical presentations and complications associated with this and other situs anomalies. An emphasis is directed toward educating the clinician concerning the potential diagnostic challenges posed by patients with TSI and the need for patient education. CLINICAL FEATURES: The patient was a 26-year-old white woman who presented to a chiropractic teaching clinic for a scoliosis evaluation and underwent full-spine radiography. The cardiac silhouette and gastric air bubble were evident on the right side. INTERVENTION AND OUTCOME: Radiography including a 2-view chest and 1-view abdomen series was performed. Examination showed a complete mirror image presentation of the cardiothoracic and visceral organs. The patient was informed of her diagnosis and associated complications and counseled concerning how typical health complaints may present atypically in patients with situs anomalies. The patient has not experienced complications associated with the TSI. CONCLUSION: Total situs inversus is a rare autosomal recessive anomaly. This article highlights the classification and nomenclature of situs anomalies and their clinical presentations and complications. In addition, the importance of thorough patient education and counseling in those with situs anomalies is emphasized.

Paralysis in the left phrenic nerve after living-donor liver transplantation for biliary atresia with situs inversus.

A 7-month-old boy with biliary atresia accompanied by situs inversus and absent inferior vena cava (IVC) underwent living-donor liver transplantation (LDLT). Because a constriction in the recipient hepatic vein (HV) was detected during the preparation of the HV in LDLT, a dissection in the cranial direction and a total clamp of the suprahepatic IVC was performed, and the suprahepatic IVC and the graft HV were anastomosed end-to-end. Postoperatively, atelectasis in the left upper lobe and ventilator failure accompanied by an elevation of the left hemidiaphragm were observed and mechanical ventilation was repetitively required. Paralysis in the left phrenic nerve was diagnosed by chest radiograph and ultrasonography. In our patient, conservative treatment was administrated, because weaning him from mechanical ventilation was possible a few days after intubation and the ventilator function was expected to be improved with growth. The disease course was good, and he was discharged from the hospital at 78 days after LDLT. Complications of paralysis in the phrenic nerve after cadaveric liver transplantation have been reported to be high. Although using a conventional technique during the reconstruction of the HV may injure the phrenic nerve directly, use of the piggyback technique with preservation of the IVC is rare. Even if LDLT was undertaken, a dissection of the HV or a total clamp of the suprahepatic IVC as a conventional technique can directly injure the phrenic nerve. Therefore, a dissection of the HV or a total clamp of the suprahepatic IVC at the reconstruction of the HV in LDLT should be carefully performed, and the possibility of paralysis in the phrenic nerve should be considered in patients with a relapse of respiratory symptoms and an elevation of the hemidiaphragm after LDLT.

Combined endovascular and surgical treatment of carotid body tumor in a patient with thoracic situs solitus.

BACKGROUND: Chemodectomas of the head and neck are tumors that originate from the neural crest. The authors report a case of carotid body tumor with chronic hypoxia secondary to congenital cyanogenic cardiac malformation, radiologically and surgically treated. METHODS AND RESULTS: A 37-year-old woman presented 1-year history of a slow-growing right lateral-cervical swelling. Radiologic examination led to a suspicion of carotid body tumor. Her history revealed thoracic situs solitus and a complex congenital heart disease. She was hospitalized for a selective angiography of the cervical vessels. Twenty-four hours later, the tumor was completely removed under local anesthesia. One year later, there were no signs of recurrence, and the patient showed a correct hemodynamic compensation. CONCLUSIONS: The choice of surgical resection with selective preoperative embolization, which induced the obliteration of the feeder vessels, devascularizing the tumor and avoiding significant intraoperative bleeding, was successful.

Coronary angioplasty via the radial approach in an individual with dextrocardia.

We present the first published case of coronary angioplasty via the radial artery approach in an individual with situs inversus. The technical issues involved are discussed.