Case report: Successful anesthesia management of noncardiac surgery in a patient with single atrium.

Background: Single atrium is very rare congenital cardiac anomaly in adults. The prognosis of patients with single atrium is very poor, with 50% of patients dying owing to cardiopulmonary complications in childhood. Herein, we focused on anesthesia management for noncardiac surgery in patients with single atrium. Case presentation: A 58-year-old male with a history of bilateral varicocele underwent laparotomy for high-position ligation of the spermatic vein. The patient also had a history of single atrium, atrial fibrillation, chronic heart failure, pulmonary hypertension (PH), and complete right bundle branch block (CRBBB). Given the significant complications associated with general anesthesia in patients with PH, we preferred to use low-dose epidural anesthesia for this patient. Transthoracic echocardiography was used to assess cardiac function before and during surgery and guide perioperative fluid therapy. To limit the stress response, we used a regional nerve block for reducing postoperative pain. Furthermore, we used norepinephrine to appropriately increase the systemic vascular resistance in response to the reduction of systemic vascular resistance caused by epidural anesthesia. Conclusion: Low-dose epidural anesthesia can be safely used in patients with single atrium and PH. The use of perioperative transthoracic echocardiography is helpful in guiding fluid therapy and effectively assessing the cardiac structure and function of patients. Prophylactic administration of norepinephrine before epidural injection may make it easier to maintain the patient's BP.

Mesocolic hernia, a case series.

INTRODUCTION AND IMPORTANCE: Paraduodenal hernias are difficult to diagnose due to their unusual presentation. Herein, five new cases are added to the literature. CASE PRESENTATION: Four male and one female child complained of paraduodenal hernias, two on the right side and three on the left side. The intestinal part that herniated inside the hernia sac was also malrotated in four patients. One patient had Meckel's diverticulum with a herniated intestine. One infant had extrahepatic biliary disease, a single atrium, polysplenia, intestinal malrotation, and a left paraduodenal hernia. Exploratory labarotomy was done for reduction of the intestine, reorientation, and repair of hernia orifices. CLINICAL DISCUSSION: Paraduodenal hernia is a component of malrotation. Cautious dissection of the hernia orifice is required to keep away from injuries to the inferior mesenteric vein or left colic artery in the course of the restoration of the left paraduodenal hernia. Also, the superior mesenteric vessels may be injured in the course of the restoration of the right paraduodenal hernia. CONCLUSION: There is a correlation between the occurrence of PDH with malrotation. The diagnosis of malrotation can be made with an ultrasound abdomen; however, it is true that ultrasound cannot make a confirmed diagnosis in all patients. Once the diagnosis of a mesocolic hernia has occurred, surgical repair is mandatory by closure of the defect.

Situs Inversus Partialis With a Wandering Spleen Having a Single Atrium: A Rare Tale of Survival.

This case report of a rare condition involving situs inversus partialis, wandering spleen, and a single atrium. Situs inversus partialis is a congenital developmental defect in which the abdominal or thoracic organs are reversed to the opposite side of the body across the sagittal plane. The case report highlights the congenital developmental anomaly and the diagnostic and management complexities associated with this condition. The patient in this case has survived to the age of 24, despite the presence of a single atrium. In the existing literature, situs inversus is a known congenital condition, but partial situs inversus is less common. A wandering spleen is also a rare condition characterized by splenic hypermobility. The combination of situs inversus partialis, a wandering spleen, and a single atrium is particularly unusual and has limited reported cases. Therefore, this research contributes to the existing literature by providing a unique case report and highlighting the challenges associated with diagnosis and management in such cases.

A 78-year-old man diagnosed with single atrium as congenital heart disease.

Single atrium is a rare congenital heart disease characterized by complete absence of the interatrial septum. It occurs as an isolated cardiac defect, or as a part of skeletal, muscular, ophthalmologic, and vascular malformations to signify congenital syndromes. A 78-year-old male patient presented with generalized body swelling of 2 weeks duration. He had associated shortness of breath, orthopnea, productive cough, and palpitation. Upon physical examination, blood pressure = 150/75 mmHg, pulse rate = 50 bpm, respiratory rate = 24 bpm, and T° = 36.7 °C. He had signs of pleural effusion on left lung field. Cardiovascular examination revealed mean heart rate of 50 beats/min with irregularly irregular pulse rhythm, raised jugular venous pressure, and pansystolic murmur at left lower sternal border. He had tender hepatomegaly, ascites, and pitting leg edema. Chest X-ray showed cardiomegaly, prominent pulmonary trunks, and left-sided pleural effusion. Electrocardiography revealed atrial fibrillation, bifascicular block (right bundle branch block and left anterior fascicular block) with mean heart rate of 50 beats/min. Two-dimensional transthoracic echocardiography showed complete absence of interatrial septum (single atrium) without atrioventricular defect and interventricular communication. Diagnosis of heart failure secondary to congenital heart disease (single atrium) with atrial fibrillation and bifascicular block was made based on clinical and imaging evaluation. Well-tolerated symptoms of a single atrium until late adulthood could be explained by the presence of streaming or incomplete mixing of blood within the atrium, in which the more oxygenated blood is directed to the systemic circuit.

Atrial fibrillation ablation in a single atrium with inferior vena cava interruption.

Common atrium (CA), also called three-chambered heart, is one of the rare congenital anomalies, defined by a complete absence of the atrial septum, eventually associated with malformation of the atrioventricular (AV) valves. We report the case of a 57-year-old woman with CA complicated with Eisenmenger syndrome and inferior vena cava interruption, who suffered from symptomatic persistent atrial fibrillation (AF). She underwent an initial successful pulmonary vein isolation procedure. A repeat procedure for perivalvular atrial flutter was complicated with inadvertent complete AV block, due to unusual AV node location in this challenging anatomy.

An adult female patient with single atrium and single ventricle undergoing appendectomy: A case report.

Appendicitis is one of the common diseases, and appendectomy is one of the most commonly performed procedures. Single atrium and single ventricle are rare heart diseases, and very few patients survive to adulthood. We report a patient with single atrial and single ventricles undergoing appendectomy with transverse abdominis plane block and dexmedetomidine sedation anesthesia with smooth postoperative appendectomy.

Three-Chambered Heart (Single Atrium) - A Rare Entity.

Single atrium (SA) is one of the rare congenital anomalies in which there is a complete absence of the atrial septum without an endocardial cushion defect associated with the absence of malformation of the atrioventricular (AV) valves. The term "common atrium" is used to denote the condition where there is a complete absence of the atrial septum or it is represented by a small strand of tissue present at the superior atrial wall of the common chamber, absence of interventricular communication, and accompanying AV cushion defect. Our patient demonstrated typical echocardiographic features of three-chambered heart (SA), which is a rare entity.

Single Atrium and Miscarriages.

Background A single atrium is a very rare heart condition, in which the inter atrial septum is missing. These congenital heart defects usually are surgically corrected after birth. Case Presentation We present a successful surgical repair of a single atrium (SA) in a 27-year-old woman, who has a complete missing of the atrial septum, without coexisting valvular pathology. The SA diameter was 9.97 × 6.18 cm and the Systemic blood flow/pulmonary blood flow (Qp/Qs) was 4.1 due to the single atrium. Surgical correction consisted of creating a new atrial septum, using a double-velor patch. At first- and second-year follow up, the patient was in a very good condition and without any symptoms present. We consider that the diagnoses of single atrium, especially in child bearing women, should be done in appropriate time, to avoid miscarriages, preterm births and other complications associated. Conclusion There are many case reports that show good surgical repair of single atrium and without late complications. In our case, we have obtained a very good surgical repair, without AV bundle damage and no residual shunt demonstrated on intraoperative transesophageal echocardiography.

Dual-source CT diagnosis of single atrium / 中国医学影像技术

Objective To investigate the value of ECG-gated dual-source computed tomography angiography (DSCTA) in the diagnosis of single atrium (SA).Methods DSCTA was performed in 29 patients with SA.The classification diagnosis was made,and the complicated deformities were observed.Results Among 29 patients,atrial situs solitus was found in 14 (14/29,48.28％),atrial situs inversus was found in 6 (6/29,20.69％) and atrial situs ambiguous was found in 9 (9/29,31.03％,including right atrial isomerism in 8 and left atrial isomerism in 1 patient).The patients complicated with 39 kinds,totally 228 deformities.The bronchi and lung structures were found in the same position as atrium in 23 patients (23/29,79.31％),including atrial situs solitus in 13,right atrial isomerism in 7 and atrial situs inversus in 3 patients.Conclusion DSCTA can accurately diagnose SA and associated abnormalities.The features of bronchus and lung structures are helpful to identifying left and right atrial appendage.

An infant with truncus arteriosus with situs inversus with single atrium: A case report.

Truncus arteriosus is a rare heart defect comprising <1% of congenital cyanotic heart disease. Rarely it may be associated with single ventricle, but association with single atrium is very rare. We report a case of truncus arteriosus (Van Praag type A2) with single atrium and single ventricle. .

Crossed pulmonary arteries associated with single atrium in an adult: a case report.

BACKGROUND: Crossed pulmonary arteries or single atrium is a rare form of cardiovascular anomaly. In previous studies, the anomalies are detected in infant or early adolescence, and infrequently seen in adult population. CASE PRESENTATION: We presented a case of the coexistence of two congenital anomalies in a 44-year-old woman who remained well tolerated and undiscovered until adulthood. Physical examination showed a grade III systolic murmur at the cardiac apex, and a grade II/III systolic murmur at left 2-3 intercostal space. An echocardiography revealed absence of atrial septal tissue. Dual-source CT angiography was performed for further evaluation of the great vessel. Except an enlarged single atrium, the imaging showed that the origination of the left pulmonary artery from the pulmonary trunk was superior to that of the right pulmonary artery. The branch pulmonary arteries then crisscrossed as they coursed to their respective lungs. The findings were illustrated by the right heart catheterization and then confirmed at surgery. CONCLUSIONS: To our knowledge, this is the first case report of crossed pulmonary arteries with single atrium as the only additional cardiac anomaly in an adult. Knowledge of this rare combination will be helpful in the differential diagnosis of congenital heart disease and assist the surgeon in treatment planning.

Single ventricle and single atrium with anomalous hepatic venous drainage and azygos continuation of inferior vena cava in an adult patient.

In a 37-year-old patient, ultrasonography showed and CT confirmed the presence of single ventricle, single atrium, azygos continuation of inferior vena cava, anomalous hepatic vein drainage, and persistent left superior vena cava. Such a constellation of cardiovascular anomalies is exceptionally diagnosed in adulthood.

Defecto del canal auriculoventricular, aurícula única y atresia tricuspídea como parte de un caso de síndrome de Ellis-Van Creveld / Atrioventricular canal defect, single atrium and tricuspid atresia as part of a case of Ellis-Van Creveld syndrome

El síndrome de Ellis-Van Creveld (SEVC) o displasia condroectodérmica se debe a una mutación de transmisión autosómica recesiva en el brazo corto del cromosoma 4 y afecta múltiples órganos. Descrito como una tétrada clásica de condrodisplasia, displasia ectodérmica, polidactilia y defectos cardíacos congénitos, sólo se conoce a partir de informes y series de casos. Se describe el caso de un varón de 3 meses, sin antecedentes familiares de importancia, que presentó un cuadro de condrodisplasia, labio superior fusionado al paladar, simpolidactilia posaxial bilateral en las manos, displasia del desarrollo de las caderas, tórax estrecho con costillas cortas y compromiso cardíaco. El presente caso sería la primera comunicación del SEVC en el Perú.(AU)

Ellis-Van Creveld Syndrome or chondrectodermal dysplasia is produced by an autosomal recessive inheritance secondary to mutation in the short arm of chromosome 4. The syndrome affects multiple organs. It is described as a clinical tetrad that involves chondrodysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. It is only known from reports and case series. We present a three months old male, without relevant family history, who presented chondrodysplasia, upper lip merged to palate, bilateral sinpolydactyly in the hands, developmental dysplasia of the hip, narrow chest with short ribs, and heart defects. This case is the first report of EVC in Peruvian literature.(AU)

Defecto del canal auriculoventricular, aurícula única y atresia tricuspídea como parte de un caso de síndrome de Ellis-Van Creveld / Atrioventricular canal defect, single atrium and tricuspid atresia as part of a case of Ellis-Van Creveld syndrome

El síndrome de Ellis-Van Creveld (SEVC) o displasia condroectodérmica se debe a una mutación de transmisión autosómica recesiva en el brazo corto del cromosoma 4 y afecta múltiples órganos. Descrito como una tétrada clásica de condrodisplasia, displasia ectodérmica, polidactilia y defectos cardíacos congénitos, sólo se conoce a partir de informes y series de casos. Se describe el caso de un varón de 3 meses, sin antecedentes familiares de importancia, que presentó un cuadro de condrodisplasia, labio superior fusionado al paladar, simpolidactilia posaxial bilateral en las manos, displasia del desarrollo de las caderas, tórax estrecho con costillas cortas y compromiso cardíaco. El presente caso sería la primera comunicación del SEVC en el Perú.

Ellis-Van Creveld Syndrome or chondrectodermal dysplasia is produced by an autosomal recessive inheritance secondary to mutation in the short arm of chromosome 4. The syndrome affects multiple organs. It is described as a clinical tetrad that involves chondrodysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. It is only known from reports and case series. We present a three months old male, without relevant family history, who presented chondrodysplasia, upper lip merged to palate, bilateral sinpolydactyly in the hands, developmental dysplasia of the hip, narrow chest with short ribs, and heart defects. This case is the first report of EVC in Peruvian literature.

A 1.1Mb deletion in distal 13q deletion syndrome region with congenital heart defect and postaxial polydactyly: additional support for a CHD locus at distal 13q34 region.

13q deletion syndrome is a rare genetic disorder, especially for group 3 deletion (13q33-q34 deletion). Previously we described a patient with congenital heart defect and mental retardation and proposed that a distal 6Mb region might contain the causative gene of congenital heart defect. Here we present a new patient with congenital heart defects (CHD), hand and foot anomalies and mild mental retardation. We identified a 1.1Mb deletion at chromosome 13q34 with high resolution SNP-array BeadChips (HumanOmni1-Quad, Illumina, USA). This chromosome region contains ten annotated genes, including GRK1, TFDP1, RASA3 and GAS6. To our knowledge, this represents the smallest 13q34 deletion identified to date. Our study provides additional support that distal 13q34 deletion region might contain key gene(s) responsible for cardiac development.

Color Doppler echocardiographic characteristics of bilocular heart and associated cardiac malformations / 中华超声影像学杂志

Objective To probe into the color Doppler echocardiographic characteristics of bilocular heart (BH) and associated malformations. Methods Twenty patients of BH were examined with color Doppler echocardiography (CDE) and the characteristics were observed. All the CDE results were compared with angiocardiography and 14 were confirmed by operation. Results Nineteen cases were properly diagnosed based on the CDE characteristics,1 case were misdiagnosed as mitral atresia. CDE characteristics of BH were obvious:① Apical four-chamber view of two-dimensional echocardiography (2DE) showed total echo dropout in both the interatrial septum and the interventricular septum and disappearance of the intracardiac "cross". The common atrioventricular valve closed during systole and appeared as figure "8", named 2DE "8" sign. During diastole, the common atrioventricular valve opened to a common ventricle. ② Color Doppler flow imaging (CDFI) showed common intra-atrial flow signal entereda common ventricle through common atrioventricular valve during diastole in all patients and colorful reflux through common atrioventricular valve during systole in 15 cases. ③ Among the 20 eases, 16 of the common atria were situs solitus,4 were situs inversus; 10 of the common ventricles were type A,2 were type B and 8 were type C. According to the spatial relationship of the great arteries,there were 4 type Ⅰ ,7 type Ⅱ and 9 type Ⅲ.There were 18 pulmonary stenosis and 2 pulmonary hypertension. ④ In the presence of pulmonary stenosis, CDFI showed colorful shunt signals through pulmonary artery during systole. Conclusions BH and associated malformations have obvious echocardiographic characteristics. CDE has a specific value in diagnosing BH and associated malformations.