Rare case of total anomalous pulmonary venous return into the right atrium in situs solitus.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein's ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

Case report: Cardiac arrest after radiofrequency ablation in a 76-year-old male.

RATIONALE: Previous studies have found that the main treatment of sinus arrest is pacemaker treatment. It is rare to have 12 s of sinus arrest after radiofrequency ablation, and whether a permanent pacemaker is implanted immediately in this case is not described in the guidelines. PATIENT CONCERNS: A 76-year-old male patient with persistent atrial fibrillation (AF) developed sinus arrest lasting 12 s in the early morning of the fourth day after using radiofrequency ablation for pulmonary vein isolation. DIAGNOSIS: The patient was diagnosed with AF and sinus arrest. INTERVENTIONS: The patient received cardiopulmonary resuscitation, intravenous injection of atropine 1 mg, and intravenous infusion of isoproterenol 1mg and immediately recovered consciousness thereafter. Approximately, 1.5 h later, the patient underwent surgery to install a temporary pacemaker in the right femoral vein. OUTCOMES: The patient had repeated episodes of sinus arrest after the implantation of a temporary pacemaker. After 3 weeks, the patient stabilized and was discharged. The patient was followed up for 1 year and did not experience any recurrence of sinus arrest or AF. LESSONS: We consider the potential for postoperative myocardial edema, injury to the sinoatrial node during the procedure, propafenone poisoning, and autonomic dysfunction as contributors to the occurrence of sinus arrest after radiofrequency ablation. When sinus arrest occurs after radiofrequency ablation, we can choose the appropriate treatment according to the patient's condition.

Cardioneuroablation for pediatric patients with functional sinus node dysfunction and paroxysmal atrioventricular block.

INTRODUCTION: Severe transitory episodes of bradycardia with subsequent syncope in children are common, and generally portend a benign prognosis. Rarely, patients may experience prolonged asystolic episodes secondary to significant sinus pauses (SP) or paroxysmal atrioventricular block (AVB). Cardioneuroablation (CNA) is a catheter-based intervention, used to identify and ablate the epicardial ganglionated plexi (GP), which results in disruption of the vagal-mediated parasympathetic input to the sinus and atrioventricular node. OBJECTIVE: Describe the methodology and role of CNA for treatment of pediatric patients with functional AVB or SP. METHODS: This is a single-center, case series study. Patients with SP or AVB, 21 years of age or younger, who underwent CNA between 2015 and 2021 were included. CNA was performed via anatomically guided and high-frequency stimulation methods. RESULTS: Six patients were included. The median age was 18.9 years (range 12.3-20.9 years), 33% female. Two patients had prolonged SP, two had paroxysmal AVB, and two had both SP and AVB. Four patients had prior syncope. The median longest pause was 8.9 s (range 3.9-16.8) with 11 total documented pauses (range 2-231) during the 6 months pre-CNA. Post-CNA, the median longest pause was 1.3 s (range 0.8-2.2) with one documented SP after termination of atrial tachycardia at the 3-month follow-up. At 6 months, the median longest pause was 1.1 s (0.8-1.3) with 0 documented pauses. No patients had syncope post-CNA. CONCLUSION: CNA may be an effective alternative to pacemaker implantation in pediatric patients with syncope or significant symptoms secondary to functional SP or AVB.

Clinical presentation, management, and survival in dogs with persistent atrial standstill in the United Kingdom.

OBJECTIVES: To investigate the clinical and echocardiographic presentation of dogs with persistent atrial standstill (PAS), identify variables measured at first presentation that could predict their survival, and document the progression of the disease after pacing. MATERIALS AND METHODS: Retrospective study of medical records of dogs diagnosed with PAS at three referral hospitals of the United Kingdom over seven years. RESULTS: Twenty-six dogs were diagnosed with PAS during the study period. Median age of the population was three years (range: seven months-12.5 years). The most common clinical sign was syncope (14/26). Twenty-four dogs received artificial pacemakers (PM). Major complications after PM implantation were observed in four dogs (four/24). Serial echocardiographic examinations showed that cardiac dimensions of PAS dogs with left atrial or left ventricular dilation at first presentation did not return to reference range after pacing. Further dilation of the cardiac chambers, recurrence of congestive heart failure (CHF), or development of new episodes of CHF were documented in seven, four, and 10 PAS dogs, respectively, despite pacing. Median survival time for cardiac-related deaths after PM implantation was 1512 days (18-3207). Neither CHF nor echocardiographic variables at presentation predicted survival after PM implantation in PAS dogs. CONCLUSIONS: Persistent atrial standstill (PAS) is an uncommon bradyarrhythmia, occurring in young adult dogs. Affected dogs were often presented with syncope. Whilst syncope resolved, cardiac remodeling persisted after PM implantation. Long-term survival was favorable after PM implantation and was not predicted by congestive status or cardiac chamber size at first presentation.

An unusual cause of acute coronary syndrome: thrombosis of right coronary artery to right atrium fistula.

Coronary arterial fistulae are rare, but it is one of the most common coronary artery anomalies. Most of the cases are asymptomatic in younger patients unless it is large and of haemodynamic significance. The incidence of thromboembolic complications usually increases with age. We report a case of a young male in his early 20s presenting with central chest pain. Coronary computed tomographic angiography revealed acute coronary syndrome due to a fistula between right coronary artery and right atrium occluded by thrombus. After discussion with coronary and congenital heart multidisciplinary team, a consensus was agreed that we should manage him conservatively with anticoagulant and antiplatelet therapy and a 3-month follow-up strategy that included repeating cardiac imaging. After a year, his anticoagulation and antiplatelet medication was discontinued.

Idiopathic dilatation of the right atrium. Case report and literature review.

Idiopathic dilatation of the right atrium is a rare malformation characterized by disproportionate growth of the right atrium in the absence of a cause that justifies it. We present the case of a patient with idiopathic dilatation of the right atrium diagnosed since fetal life.

La dilatación idiopática de la aurícula derecha (DIAD) es una malformación poco frecuente caracterizada por un crecimiento desproporcionado de la aurícula derecha en ausencia de una causa estructural o funcional que la justifique. Presentamos el caso de una paciente con DIAD con diagnóstico desde la etapa prenatal.

Idiopathic enlargement of the right atrium masking left atrial aneurysm in a neonate.

An idiopathic enlargement of the right atrium is an extremely rare cardiac malformation. There are no established guidelines for the management of this disease, especially concerning medical versus surgical therapeutic approach and the timing for an operation. We report in this case about a neonate that first was treated conservatively until the age of 5 month and finally got an operative resection of the aneurysm. After surgery, unexpected complications occurred. A second aneurysm in the left atrium was demasked. Furthermore, a progressive dilatation of both atrial chambers after resection required regular follow-up and ongoing evaluation of treatment.

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.

Anomalous systemic venous connection to left atrium in a heart with usual atrial arrangement.

Anomalous systemic venous connection to left atrium is rare anomaly. Previously published cases described this anatomy in patients with left isomerism. Depending on the size of the atrial septal defect, patients usually present with varying degrees of cyanosis and right heart hypoplasia. Here, we report a case of anomalous systemic venous connection to left atrium in a newborn with the usual atrial arrangement.

Midline crossing right pulmonary veins with dual drainage to the left atrium.

We report the exceptional case of transcatheter treatment of a partial anomalous pulmonary venous drainage of the right lung to the innominate vein and dual drainage to the left atrium.

A black box congenital heart defect: Anomalous superior vena cava drainage into the left atrium.

A 25-year-old pregnant woman with low oxygen saturation due to unclear congenital heart disease was admitted. During work-up the rare anomaly of superior vena cava drainage to the left atrium was diagnosed by transthoracic bubble test with agitated saline. The pathology was later confirmed by contrast multislice computed tomography angiography after delivery.

Vein of Galen Aneurysmal Malformation with Anomalous Right Superior Vena Cava to the Left Atrium Leading to Atypical Clinical and Echocardiographic Findings.

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM.

The anomalous drainage of the inferior vena cava into the left atrium.

BACKGROUND: Anomalies in systemic venous return most commonly involve a persistent left supe-rior vena cava draining into the left atrium. Anomalous drainage of the inferior vena cava (IVC) into the left atrium is a rare congenital vascular disorder. The diagnosis was confirmed as anoma-lous drainage of the right superior pulmonary vein and large atrial septal defect following echo-cardiography. Anomalous drainage of the inferior vena cave was confirmed with computed tomog-raphy (CT). We report a rare combination of drainage of the inferior vena cava associated with atrial septal defect (ASD) and partial anomalous pulmonary venous return. CASE: A 14-year-old girl was referred to our hospital for the evaluation of palpitations, hypoxia, exertional dyspnea, and cyanosis. Transthoracic echocardiography (TTE) revealed a large sinus venosus ASD and anomalous right superior pulmonary venous return. A cardiac CT demonstrated IVC drainage to the left atrium and an anomalous right superior pulmonary vein draining into the right atrium. CONCLUSIONS: In older patients with cyanosis, further imaging methods together with TTE will be useful in detecting additional cardiac anomalies. Patients with inferior vena cava opening to the left atrium are different from caval type ASD`s and should be surgically repaired using a patch. Corrective surgery involves repositioning of the interatrial septum via a patch.

Atresia of the coronary sinus ostium with persistent left superior caval vein.

We describe a case of a 33-year-old woman with congenitally corrected transposition where computed tomography angiography incidentally detected ostial atresia of the coronary sinus with dilatation of the terminal parts of the middle cardiac vein and great cardiac vein and retrograde drainage of the coronary sinus into the persistent left superior caval vein, the intercommunicating vein, then to the right superior caval vein, and ultimately into the right atrium.

Intramedullary spinal cord abscess associated with right-to-left shunt via right superior vena cava draining into left atrium: A case report.

RATIONALE: Intramedullary spinal cord abscess (ISCA) is a rare but treatable bacterial infection of the central nervous system, and the etiology in no less than 40% of the cases is cryptogenic. Although a few cases of ISCA in individuals with a right-to-left shunt (RL shunt) have been reported, only few arguments focused on the association between RL shunt and ISCA have been provoked. The right superior vena cava (RSVC) draining into the left atrium (LA) is an uncommon systemic venous anomaly that results in an RL shunt, and this anomaly causes several types of neurological complication such as stroke or brain abscess. We report the first case of ISCA associated with RSVC-LA RL shunt. PATIENT CONCERNS: A 36-year-old man developed progressive paraparesis, dysuria, and spontaneous pain in the lumbar region and lower extremities. Spinal magnetic resonance imaging revealed an intramedullary lesion extended from Th12 to L2 with ring-shaped gadolinium enhancement. Cerebrospinal fluid (CSF) study exhibited a marked pleocytosis, and CSF culture grew Streptococcus intermedius. Cardiovascular computed tomography angiography identified RSVC-LA RL shunt, which caused transient acute cardiac syndrome due to air embolus. DIAGNOSES: The patient was diagnosed with ISCA associated with an RSVC-LA RL shunt. INTERVENTIONS: The patient was treated with a combination of intravenous administration of meropenem and vancomycin in a daily dose of 6 and 2.5 g, respectively, followed by intravenous administration of ampicillin in a daily dose of 750 mg. The intravenous antibiotic therapy was continued for 37 days. OUTCOMES: A favorable neurological outcome was obtained by the intravenous antibiotic therapy, and recurrence of infection was prevented by continuous oral antibiotic therapy for 18 months. LESSONS: With a literature review of ISCA associated with RL shunt, we insist that screening for RSVC-LA is beneficial to patients who are diagnosed with cryptogenic ISCA as its identification leads to appropriate preventive therapy.

A hitherto unreported combination of pulmonary stenosis, single coronary artery anomaly, and coronary sinus to left atrial communication.

BACKGROUND: We report a hitherto unreported combination of pulmonary stenosis, single coronary artery anomaly and coronary sinus to left atrial communication. Our case highlights the important value of coronary computed tomographic angiography and transthoracic echocardiography for the diagnosis of such anomalies and guidance for proper management. METHODS AND RESULTS: A 64-year-old male presented chest tightness and shortness of breath for 2 days. Transthoracic echocardiography revealed a thickened pulmonary valve leaflet and subvalvular outflow tract stenosis, colour flow Doppler showed a significant accelerated blood flow in the pulmonary artery cavity originating from the subvalvular outflow tract, continuous wave Doppler revealed the transpulmonary valvular pressure gradient of 63mmHg. Computed tomographic angiography image reveals thickened pulmonary valve leaflets and subvalvular outflow tract stenosis, single coronary artery anomaly and levoatriocardinal vein. The patient underwent percutaneous pulmonary valve balloon dilatation, the post-procedural course was uneventful. DISCUSSION: Pulmonary stenosis can occur as part of more congenital cardiac malformations or as rare primary isolated pulmonary stenosis, which includes the valvular, sub-valvular, or supra-valvular pulmonary stenosis. Single coronary artery anomalies are very rare, anomalous right coronary artery originates from proximal to mid-left anterior descending coronary artery is one such single coronary artery anomaly, in most cases, it is asymptomatic, diagnosed incidentally, and a benign entity has a better prognosis except if the right coronary artery is passing between the aorta and pulmonary artery. This course of the right coronary artery anomaly is malignant. Coronary sinus to left atrial communication includes a direct or indirect communication. The direct communication is described as a partial or complete absence of the roof between the coronary sinus and left atrium, as it is well known as the unroofed coronary sinus syndrome. The indirect communication is an anomalous bridging vein communicating the coronary sinus to the left atrium, which can be distinguished from classical unroofed coronary sinus syndrome. The venous collateral channel communication between the coronary sinus to the left atrium by a bridging vein is also categorized as a variant type of unroofed coronary sinus syndrome. Understanding coronary venous variations has significant clinical implications particularly in the realm of electrophysiology. The anatomical variations can have important consequences for procedures such as biventricular pacing and trans-coronary vein ablations. CONCLUSION: Pulmonary stenosis combined with single coronary artery anomaly and bridging vein communication between the coronary sinus and the left atrium is an extremely rare. Coronary computed tomographic angiography and transthoracic echocardiographyplay an important role the diagnosis of such anomalies and guidance for clinical Treatment.

A sudden infant death associated with atrial septal aneurysm and abnormality of the connecting site between the inferior vena cava and right atrium.

An atrial septal aneurysm (ASA) is a rare cardiac anomaly characterized by varicose bulgingofthe atrial septum (oval fossa) into the left or right atrium. Pathogenesis and clinical significance of ASA are controversial. We report an autopsy case of a huge undiagnosed ASA with abnormality of the connecting site between the inferior vena cava and the right atrial ostium in a 2-month-oldJapanesefemale who died suddenly and unexpectedly. She was born at 36 weeks 4 days (body weight 3,110 g). No abnormality was detected during pregnancy or delivery. The postnatal growth was normal with no cardiac problem detected at the 1-month checkup. The ASA bulged off in a mass to the left atrium (width, 0.8 cm; excursion ratio, 53%), reaching close to the inflow site of the right pulmonary vein, with dilation of the pulmonary vein. The connecting site between the inferior vena cava and the right atrium was atypically located 1.6 cm away from the atrioventricular groove. Although most cases of ASA in an infant resolve physiologically as the infant grows, the infant in the present case is thought to have had an exceptional pathological ASA, possibly causing supraventricular arrhythmia. The abnormality of the connecting site between the inferior vena cava and the right atrium might have affected the development and continuation of the ASA.