A Proposed Etiology for Atrial Tachyarrhythmia in Neonates with Atrial Septal Aneurysms.

An atrial septal aneurysm (ASA) is an increasingly recognized entity that involves septal tissue significantly bulging into either atria instead of remaining in a relatively neutral position. ASAs may be described based on the length of the segment of atrial septal tissue involved as well as the distance and direction of excursion into the atria throughout the cardiac cycle. Complications associated with ASA include arrhythmias and thromboembolic events with the latter usually in the context of atrial shunting. While the presence of an ASA has been implicated in the development of atrial tachyarrhythmias, no clear mechanism has been elucidated to-date. In this case, we document one of the previously proposed mechanisms of atrial arrhythmia using echocardiography imaging.

The Use of Fecal Calprotectin Levels in the Fontan population.

The Fontan procedure was first performed in the seventies as a palliation for patients with single ventricle physiology. A feared complication after a Fontan procedure is the development of protein losing enteropathy (PLE). Systemic inflammation has a negative effect on the intestinal barrier integrity, which has supported the use of steroids in this setting. To the best of our knowledge there are no studies linking intestinal inflammation in patients with PLE after Fontan. The objective of this study was to identify the presence of intestinal inflammation measured by FC in patients with PLE after a Fontan procedure. A cross-sectional analysis was performed examining 23 stool samples from 23 Fontan patients for both Fecal alpha-1-antitrypsin (FA1AT) and FC with and without PLE. The median FC was 21 mcg/gm of stool (IQR: 15.7-241 mcg/gm of stool), and the median FA1AT was 40 mg/dL (IQR: 30-220 mg/dL). The median FC and FA1AT were significantly higher in the PLE group than in the Non-PLE group (p = 0.002 and p < 0.0001, respectively). Significantly elevated levels of FC were demonstrated in Fontan patients with PLE, which correlated with the elevated levels of FA1AT. Inversely, levels of FC in Fontan patients without suspected PLE were within the normal range. To our knowledge, this is the first study to demonstrate intestinal inflammation using FC in the setting of PLE within this cohort, and may prove to be useful as a diagnostic tool in its treatment.

Chronic Low Dose Prostaglandin and Neonatal Heart Block.

Long-term prostaglandin use is commonly associated with side effects such as cortical proliferation of the bones, hypertrophic pyloric stenosis, and soft tissue swelling of the extremities. We report a neonate with critical coarctation of the aorta, who developed second and third degree atrioventricular blocks associated with prolonged prostaglandin E1 (PGE1) infusion. Interestingly, these conduction blocks only occurred at low PGE1 dose. The rhythm disturbances resolved promptly with the discontinuation of PGE1 following surgical repair.

The use of intracardiac echocardiography during percutaneous pulmonary valve replacement.

High-quality live imaging assessment of cardiac valves and cardiac anatomy is crucial for the success of catheter-based procedures. We present our experience using Intracardiac echocardiography (ICE) during transcatheter Percutaneous Pulmonary Valve replacement (tPVR).This is a retrospective study that included 35 patients who underwent tPVR between April 2008 and June 2012. Thirty-one of these patients had the procedure performed under continuous ICE guidance. Pre-procedure transthoracic echocardiography (TTE) was obtained in all patients. ICE was performed at baseline, during the procedure, and at the conclusion of the procedure. Comparisons between the pre-procedure TTE and baseline ICE data and between post-procedure ICE data and the following day TTE were performed. Total of 35 patients had tPVR during the above-mentioned time period. Twenty-one patients received the Edwards Sapien valve and 14 patients had the Melody valve. Thirty-one patients had the procedure performed under continuous ICE guidance. The mean Pre-TTE peak gradient (PG) and Pre-ICE-PG were 45.5 ± 20 vs 33 ± 13 mmHg (p < 0.001) and the mean Pre-TTE mean gradient (MG) and Pre-ICE-MG were 27.7 ± 13 vs 21 ± 18 mmHg (p < 0.001). The mean Post-TTE- PG and Post-ICE-PG were 24.3 ± 11 vs 15.3 ± 7 mmHg (p < 0.001) and the mean of the Post-TTE-MG and Post-ICE-MG were 14.2 ± 7 vs 8.4 ± 4 mmHg (p < 0.001). There was a good correlation between peak ICE and TTE gradient at baseline and after valve placement. For the degree of pulmonary regurgitation, there was no significant difference between TTE and ICE. ICE is an important modality to guide tPVR in patients with dysfunctional homograft valve between the right ventricle and pulmonary artery and should be used to assess valve function before, during and immediately after the procedure.

Metastatic osteosarcoma to the interventricular septum: an unusual presentation in a child.

Whereas primary cardiac tumors are reported in only 0.17 % of pediatric patients, malignant cardiac tumors are even less common in children. This report presents a 10-year-old child with relapsed osteosarcoma manifesting as metastatic lesions to the interventricular septum.

Prenatal diagnosis of isolated levocardia and a structurally normal heart: two case reports and a review of the literature.

Isolated levocardia (ILC) is a developmental abnormality involving an abnormal abdominal situs with a normal cardiac situs. This abnormality is especially rare when it is associated with a normal cardiac anatomy. The prenatal diagnoses of seven cases were reported in the English literature. This report presents two cases referred to the authors' echocardiography laboratory for maternal diabetes mellitus in case 1 and suspected dextrocardia in case 2. In both cases, ILC with a structurally normal heart was diagnosed prenatally. The child in the first case was found to have a normal inferior vena cava (IVC) prenatally. Postnatally, he was found to have intestinal malrotation with duodenal obstruction and multiple splenules. Interruption of the IVC was shown by abdominal ultrasound. The child in the second case was found to have an interrupted IVC with azygos continuation prenatally. Postnatally, intestinal malrotation with no evidence of intestinal obstruction or asplenia was detected. Neither of the cases had reported cardiac arrhythmias. Early diagnosis is crucial in these cases due to the high incidence of associated anomalies and potential life-threatening conditions. Management of patients with ILC is dictated by the associated anomalies. Long-term follow-up assessment is recommended for these patients to monitor the development of rhythm abnormalities.

Left ventricular accessory chamber: a case report and review of the literature.

Congenital left ventricular out-pouching, consisting of left ventricular aneurysms, left ventricle diverticulae, and double-chambered left ventricle, is a rare cardiac malformation. Criteria to differentiate between left ventricular aneurysm and diverticulum are defined. Reports of left ventricular accessory chamber in the medical literature are rare. In this article, we present a case of left ventricular accessory chamber diagnosed during the late prenatal period. Review of the literature, imaging modalities used for differentiation of the nature of this lesion, and management plan are discussed.

Intracardiac echocardiography for the guidance of percutaneous procedures.

Interventional cardiology has seen great advances in the past decade. A wide range of interventional procedures has been established as standard therapeutic modalities and more are yet to come. Multiple imaging modalities have been used to guide these procedures. Intracardiac echocardiography (ICE) provides an accurate imaging tool to guide the appropriate performance of many of these procedures. Early studies compared ICE as a new imaging modality to guide interventional closure of atrial communications with other more established imaging techniques, such as transesophageal echocardiography, with excellent accuracy. In this article, we discuss the value of using ICE in guiding some percutaneous interventional procedures. We also discuss the imaging protocol for using ICE to guide atrial level shunt device closure. Our experience in using ICE for guiding percutaneous valve placement is also discussed.

Pediatric cardiac interventions.

The field of pediatric cardiac interventions has witnessed a dramatic increase in the number and type of procedures performed. We review the most common procedures performed in the catheter laboratory. Lesions are divided according to their physiological characteristics into left-to-right shunting lesions (atrial septal defect, patent ductus arteriosus, ventricular septal defect), right-to-left shunting lesions (pulmonary stenosis, pulmonary atresia/intact ventricular septum), right heart obstructive lesions (peripheral arterial pulmonic stenosis, right ventricular outflow tract obstruction), and left heart obstructive lesions (aortic valve stenosis, coarctation of the aorta). In addition, a miscellaneous group of lesions is discussed.

Multiple Amplatzer septal occluder devices for multiple atrial communications: immediate and long-term follow-up results.

OBJECTIVES: We report on the feasibility, effectiveness, and long-term outcome of transcatheter closure of multiple ASD's using multiple ASO devices. BACKGROUND: Little is known about the use and long-term outcome of multiple Amplatzer Septal Occluder (ASO) devices to close multiple atrial septal defects (ASD's). METHODS: From May 1997 to June 2006, 33 patients (mean age 38.9 years and mean weight 68 kg) underwent transcatheter closure of multiple ASD's under transesophageal (TEE) or intracardiac echocardiographic (ICE) guidance. RESULTS: Unless not available, the device size chosen to be deployed was +/-2 mm larger than the stretched diameter or no more than 30-40% larger than the ICE/TEE 2D diameter. Sixty-seven devices were deployed in 33 patients. The mean diameters of the larger and smaller defects were 12.9 and 7.7 mm, and the corresponding mean balloon stretched diameters were 19.2 and 12.0 mm, respectively. The mean larger and smaller device diameters were 19.0 and 13.4 mm, respectively. The mean QP: Qs ratio was 1.8:1. The mean fluoroscopy time, and procedure time were 16.4 and 81.6 min respectively. Immediately after the procedure 15 patients had complete closure, 8 had trivial shunt, 9 had small shunt and one had large shunt. Complications included a device embolization within 24 hr and left atrial-aortic wall erosion and pericardial effusion at 2 years. The mean follow-up interval was 34.8+25.7 months. CONCLUSIONS: Device closure of multiple ASD's using multiple ASO's is safe and effective. Continued follow-up is important to assess the long-term outcome.