Clinical Presentation and Therapy of Total Anomalous Pulmonary Venous Return.

Total anomalous pulmonary venous return (TAPVR) is rare (accounting for about 1% of all CHD) and can occur as a single lesion or in combination with other types of CHD (such as heterotaxy or HLHS). TAPVR is defined as an abnormal connection where all pulmonary veins do not drain into the left atrium but into the right atrium either directly or through a vein that is connected to the right atrium. TAPVR can be divided into four anatomic groups (Fig. 32.1): (1) supracardiac (about 55%), (2) cardiac (about 30%), (3) infracardiac (about 13%), and (4) mixed (very rare). In addition, it can be divided into two physiological types: nonobstructed and obstructed. Embryologically, all pulmonary veins usually connect to a pulmonary venous confluence that connects to the left atrium. If this connection does not occur, the pulmonary venous confluence connects to a systemic vein instead.

Aorto-left atrial fistula secondary to aortic infective endocarditis in a dog with a bicuspid aortic valve.

An 11-year-old male neutered American bulldog was presented for evaluation of thrombocytopenia, acute onset of ataxia, and vomiting. A new murmur was auscultated on physical examination. Transthoracic echocardiographic examination revealed a bicuspid aortic valve, vegetative lesions on the aortic valve, and continuous shunting from the aortic root to the left atrium through an aorta to left atrial fistula. The dog was euthanized due to its guarded prognosis and critical condition. Pathological examination confirmed presence of a bicuspid aortic valve, aorto-left atrial fistula, and aortic infective endocarditis. Antemortem blood culture revealed two unusual organisms: Achromobacter xylosoxidans and Fusobacterium mortiferum.

Prominent crista terminalis mimicking a right atrial mass: a systematic literature review and meta-analysis.

The crista terminalis is an anatomical structure localized on the posterolateral wall of the right atrium (RA). We performed a systematic review of the literature and meta-analysis concerning cases of unusual prominent crista terminalis mimicking RA mass. Moreover, we described the differential diagnosis of cardiac masses with the use of echocardiography, computed tomography, and cardiac magnetic resonance (CMR). We also emphasize the potential importance of this structure in electrophysiological procedures, including its role in exaggerated arrhythmias. Prominent crista terminalis may be a potential obstacle during invasive cardiac procedures or catheter ablation target. In analyzed cases, the crista terminalis was often erroneously interpreted as pathologic and at first confused with a thrombus or tumor during transthoracic echocardiography examination. The correct final diagnoses were mostly made with used transesophageal echocardiography or CMR. The most important imaging findings suggestive of prominent crista terminalis rather than tumor were a similar echogenicity/intensity with adjacent myocardium, the location on posterolateral wall of the RA, the phasic change in size, and no enhancement after contrast injection. We describe up to date and detailed imaging features for the differential diagnostics of selected intracardiac masses using various imaging techniques, including multimodality cardiac imaging. Familiarity with the anatomy and the imaging findings of the prominent crista terminalis will reduce misdiagnosis and avoid additional tests and unwarranted clinical interventions, while in patients considered for invasive cardiac procedures it might increase their efficacy and safety.

Congenital right atrial diverticulum in a kitten.

A four-month-old domestic shorthair cat with no clinical signs was referred for further examination of a heart murmur. An echocardiogram revealed marked right atrial dilation, extending into the left hemithorax. Computed tomography angiography was conducted to investigate further, which revealed a balloon-shaped, contrast-filled cavity on the cranial and left side of the chest that connected to the right atrium through a narrow passage. This was diagnosed as a congenital right atrial diverticulum. Treatment included clopidogrel to reduce the risk of thrombus formation. Two months after the initial diagnosis, castration surgery was successfully performed without complications. The cat died suddenly at home 10 months after diagnosis. To the authors' knowledge, this is the first right atrial diverticulum reported in a cat.

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.

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.