Echocardiographic diagnosis of a case with giant right atrial aneurysm

Right atrial aneurysm (RAA) is a very rare anomaly. Rarer still is its association with atrial septal defect (ASD). We reported a case of a 42-year-old woman with giant RRA and secundum type ASD detected by means of transthoracic echocardiography.     

Interatrial septal aneurysm. Echocardiographic diagnosis

Interatrial septal aneurysm is a rare abnormality and can now be diagnosed by echocardiography. We report the case of a 52 year old woman in whom this condition was diagnosed after an embolic cerebrovascular accident. M mode recordings showed a linear echo in the left atrial cavity in early and mid systole. The aneurysm was directly visualised by 2D echo as a hemispherical bulge in the mid portion of the interatrial septum, which was mobile and had a to-and-fro motion between the two atria in relation to the different phases of the cardiac cycle. The diagnosis was confirmed by angiography, and at surgery. A feature of this case was the close correlation between echocardiographic, angiographic and operative findings. The pathogenesis of this type of aneurysm remains conjectural as does its role in the production of cerebral embolism, the evidence for which was circumstantial in the absence of other demonstrable causes and in the light of previously reported cases.     

Echocardiographic diagnosis of right atrial thromboembolism

In a patient with congestive cardiomyopathy who died suddenly from right ventricular thromboembolism, antemortem two-dimensional echocardiography demonstrated a right atrial mass with acoustic characteristics suggestive of a thromboembolus. These characteristics included an elongated structure that assumed multiple dynamic forms and lack of an intracardiac attachment point. Because of the high likelihood of right ventricular impaction of a cardiac embolus, an aggressive therapeutic approach to such lesions is advised.     

Echocardiographic diagnosis of right atrial tamponade

We report the case of a 27-year-old man who developed isolated right atrial tamponade eight weeks following aortic valve replacement. The diagnosis was made by two-dimensional and contrast echocardiography and was subsequently confirmed by cardiac catheterization and surgery. Right atrial tamponade presents a unique conglomeration of clinical, hemodynamic, and echocardiographic features. Constant awareness of this entity is necessary to make a timely diagnosis.     

Percutaneous closure of an atrial septal defect in an infant with shone's syndrome

Rarely is closure of the atrial septal defect indicated in infants. Yet, an atrial septal defect in the presence of congenital mitral stenosis may cause significant congestive heart failure in infancy. We present the case of an infant with Shone's syndrome, left ventricular hypoplasia, and a large ostium secundum atrial septal defect who developed pulmonary overcirculation following repair of coarctation of the aorta. The infant underwent cardiac catheterization, hemodynamic assessment, and successful percutaneous closure of the atrial septal defect. The patient improved dramatically following the intervention. Follow‐up echocardiograms have demonstrated growth of mitral valve annulus diameter and left ventricular dimensions. Atrial septal defect closure in this small infant was associated with immediate improved clinical status, and improved growth of the left heart structures. © 2012 Wiley Periodicals, Inc.     

Percutaneous transhepatic stenting of a restrictive atrial septal communication in hypoplastic left heart syndrome

The postnatal survival of patients with congenital cardiac defects such as hypoplastic left heart syndrome (HLHS) is dependent on the patency of the ductus arteriosus and the presence of an unrestrictive atrial septal defect (ASD). We report a six week old infant with HLHS and tricuspid valve regurgitation with restrictive ASD. Transfemoral balloon atrial septostomy provided temporary relief but further attempts were not possible. A transhepatic venous approach to stent the atrial communication was technically successful. This approach may be the only access for certain infant cardiac catheterization interventions, permitting the use of a large delivery system (≥ 8 Fr). © 2014 Wiley Periodicals, Inc.     

Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect

A severely restrictive atrial septal defect (ASD) in neonates with hypoplastic left heart syndrome (HLHS) results in pulmonary venous hypertension, pulmonary edema, and intractable hypoxia. Between January 1983 and June 1998, 21 of 355 neonates presenting with HLHS (5.9%) underwent cardiac catheterization at median age 1 day (range 0 to 25), for creation or enlargement of a restrictive or absent interatrial communication. One patient died during preliminary angiography. Three underwent blade septostomy with 2 procedure-related deaths, and 1 had balloon atrial septostomy (BAS); all 4 died before surgical intervention. Fifteen underwent Brockenbrough atrial septoplasty with transatrial needle puncture and serial balloon dilations of the new ASD, 5 after unsuccessful BAS. The most recent patient had a stent placed across the atrial septum after transatrial needle puncture. In the 16 patients treated with septoplasty or stent, oxygen saturation increased from 50 +/- 4% to 83 +/- 2% (p <0.0001) and transatrial pressure gradient decreased from 16 +/- 1 to 6 +/- 1 mm Hg (p <0.0001). One patient died awaiting transplantation, supportive care only was requested in 1, and 14 underwent stage 1 palliation. Eight of 14 (57%) survived to hospital discharge. Six of 7 (86%) survived bidirectional Glenn and the 3 who have undergone fenestrated Fontan are alive. In neonates with HLHS, a restrictive ASD resulting in profound cyanosis demands urgent intervention. BAS is frequently unsuccessful and blade septostomy has high mortality. Pulmonary venous hypertension can be adequately relieved by Brockenbrough atrial septoplasty or stenting, allowing stabilization before reconstructive surgery or while awaiting transplant.     

Atrial septal interventions in patients with hypoplastic left heart syndrome

Objectives: To report an institutional experience performing percutaneous atrial septal interventions in patients with hypoplastic left heart syndrome (HLHS). Background: The success of the Hybrid approach in palliating patients with HLHS is crucially dependant on relieving any significant interatrial restriction. Data on transcatheter interventions to relieve atrial septal restrictions in patients with HLHS are limited. Methods: We retrospectively reviewed 67 transcatheter atrial septal interventions that were performed between July 2002 and September 2007 in 56 patients with HLHS. The median weight was 3.35 kg. About 10.7% of patients had an intact atrial septum. Balloon atrial septostomy (BAS) was used in 77.6% of procedures, additional techniques in 35.8% of procedures. Patients were divided into those with standard atrial septal anatomy (group A, n = 33) and those with complex atrial septal anatomy (group B, n = 23). Results: The mean trans‐septal gradient was reduced significantly from 7 mm Hg to 1 mm Hg with the median time to discharge being 3.5 days. Major adverse events were seen in 8.9% of procedures, whereas minor adverse events occurred in 26.8% of procedures. Adverse events were significantly less common in patients with standard atrial septal anatomy, compared to those with complex atrial septal anatomy (25.6% versus 50.0%). About 19.6% patients required repeated atrial septal interventions. Survival up to and including Comprehensive stage II palliation was 73% group A, and 57% in group B. Conclusions: With utilization of appropriate techniques and equipment, atrial septal interventions in HLHS can be performed successfully in virtually all patients. Complex atrial septal anatomy is technically challenging and has a higher incidence of procedural adverse events. In the majority of patients, standard BAS can be performed safely, and is usually the only intervention required to achieve adequate relief of atrial septal restriction until Comprehensive stage II palliation. © 2008 Wiley‐Liss, Inc.     

Accidental detection of a giant right atrial aneurysm in an asymptomatic infant

We present a 4-month old infant boy accidentally detected to have cardiomegaly on a chest radiograph, and on further investigation discovered to have a giant right atrial aneurysm and a secundum atrial septal defect. Congenital right atrial aneurysm or diverticulum is a rare anomaly, usually presenting with atrial or supraventricular tachycardia. Previously reported patients did not have any associated congenital heart defect. Although asymptomatic our patient needs close follow-up, and surgical resettion of the aneurysm and closure of the atrial septal defect in view of potential complications.     

Transcatheter closure of atrial septal defects in children with a hypoplastic right ventricle.

The efficacy and safety of device closure of atrial defects in children with complex congenital heart disease and a hypoplastic right ventricle have not been detailed. The objective of this study was to determine the clinical impact and outcomes of a staged surgical catheter-based management strategy. A retrospective analysis of 17 children with a hypoplastic right ventricle who had undergone cardiac catheterization and attempted device occlusion of an atrial defect was undertaken. Clinical data, anatomical diagnoses, previous surgeries, and interventions were noted. The clinical course and echocardiographic findings were compared before and after defect closure (6 +/- 4 months of follow-up). Nine children had pulmonary atresia with an intact ventricular septum, six had Ebstein's anomaly of the tricuspid valve, and two had isolated right ventricular hypoplasia. The mean age at defect closure was 6.6 +/- 3.4 years. Under general anesthesia, closure of the atrial defect was undertaken after initial temporary test occlusion. The Qp:Qs ratio before closure was 1.1 +/- 0.4. Oxygen saturation improved in all children from 91% +/- 1% to 98% +/- 1% (P < 0.0005). Mean right atrial pressure increased from 9 +/- 3 to 11 +/- 4 mm Hg after closure, but the difference was not statistically significant (P = 0.2). Follow-up revealed an increased right ventricular length (P = 0.009) and Z-score (P = 0.02), although no significant increase in the diameter of the tricuspid valve annulus was observed in children with pulmonary atresia. Right ventricular systolic pressures or systolic function did not change significantly during follow-up (P = 0.5 and 0.29, respectively). Exercise tolerance improved in all children and clinical evidence of right ventricular decompensation was absent. Catheter closure of atrial defects in children with a hypoplastic right ventricle is well tolerated, safe, and effective as a treatment strategy in the staged management.     

Intervention in the critically ill neonate and infant with hypoplastic left heart syndrome and intact atrial septum

Neonates that present with hypoplastic left heart syndrome (HLHS) and intact atrial septum (IAS) pose a major management problem for the pediatric cardiac team. They are critically ill newborns with profound hypoxemia and acidosis that require immediate attention. Controversy exists as to the most appropriate management strategy. In one series where a primary and emergent surgical-staged reconstructive procedure was performed, the in-house hospital mortality was 65% and the overall survival was 17%. With equal abysmal results, transcatheter creation of an atrial septal defect (ASD) using conventional balloon atrial septostomy (BAS) with or without the combination of blade atrial septotomy had an unacceptable high risk of cardiac perforation leading to tamponade and death. However, using more modern transcatheter techniques of transseptal perforation of the atrial septum followed by progressive and serial balloon septoplasty, creating an ASD, significantly reduced the risk of the procedure. In one series, 16 consecutive neonates underwent this type of interventional procedure without procedural mortality. The management strategy of creating an ASD in the catheterization lab followed by Stage I reconstructive surgical repair 3-5 days after the initial catheterization procedure improved the in-house survival to 57%. Unfortunately, there continues to be significant attrition of these patients undergoing Stage II and III reconstructive repair, which supports cardiac transplantation as an alternative strategy. There have been echocardiographic and histopathologic studies of these neonates, and an important echo classification of left atrial morphology has been described with perhaps some prognostic implication. In addition, autopsy specimens have demonstrated significant "arterialization" of the pulmonary venous architecture that likely dooms the patient with single ventricle physiology to a poor outcome. Future improvement in transcatheter techniques and materials offer promise in palliating these critically ill neonates. The concept of radiofrequency energy perforating catheters has great merit and may reduce the risk of cardiac perforation as compared with the rigid and long transseptal needle. Echocardiographic imaging at the time of entry through the IAS may improve the safety as well. The novel concepts of "butterfly" or "dog-bone" stents placed across the atrial septum creates a precisely sized ASD that may be more conducive to effectively lower left atrial hypertension, yet avoids excessive pulmonary blood flow associated with large atrial communications. In addition, new materials, such as the Cutting Balloon Catheter, may offer promise in creating ASDs in these patients. A more aggressive approach would be to consider intrauterine fetal transcatheter opening of the IAS using modified techniques that have been attempted for left ventricular outflow tract obstruction. Unfortunately to date, the results of attempted relief of aortic valve stenosis have been extremely poor. Finally, we as interventionalists need to continue to improve our skills to help in the complex management of these critically ill neonates and infants. Only through continued efforts of the entire cardiac team of intensivists, cardiologists, cardiothoracic surgeons, and interventionalists will our management strategy be defined to maximize the future outcome in this group of patients.     

Echocardiographic diagnosis of the right tri-atrial heart

In this report we describe the two-dimensional echocardiographic features of a case of cor triatriatum dexter. In this situation the right atrium is divided by a membrane in two chambers. Considering the anatomic characteristics of this case we added a new type in the classical classification proposed by Doucette et al.