Walter J. Duncan

Two-dimensional echocardiographic identification of hemitruncus: Anomalous origin of one pulmonary artery from ascending aorta with the other pulmonary artery arising normally from right ventricle

Two-dimensional echocardiography (2DE) was performed on a 2300 gm newborn who presented with congestive heart failure. Clinical examination suggested left-to-right shunt with pulmonary hypertension. Cardiac catheterization and angiography demonstrated origin of the right pulmonary artery (RPA) from the aorta. Retrospectively, this feature (anomalous RPA) had been present on 2DE. A second patient aged 2 years had evidence of ventricular septal defect with large left-to-right shunt early in life. Gradual development of infundibular stenosis and clinical cyanosis ensued. 2DE showed subaortic ventricular septal defect with infundibular stenosis. Cardiac catheterization and angiography confirmed tetralogy of Fallot with origin of the left pulmonary artery (LPA) from the aorta. Retrospective review of 2DE showed the anomalous LPA arising from the side of the ascending aorta.

Catecholamine cardiomyopathy: An unusual presentation of pheochromocytoma in children

CARDIOMYOPATHY may be primary or secondary. Primary cardiomyopathy is unrelated to systemic disease or diseases in other organs, whereas secondary cardiomyopathy is associated with other disease complexes or etiologic agents? We report two patients with pheochromocytoma, a catecholamine-se creting tumor, who presented to The Hospital for Sick Children, Toronto, with cardiomyopathy.

Space-occupying lesions of the myocardium: Role of two-dimensional echocardiography in detection of cardiac tumors in children

Four patients with space-occupying myocardial lesions were studied using electronically focused high-resolution two-dimensional echocardiography. In two patients with clusters of high-intensity echoes at various depths on the epicardial surface, histology confirmed the diagnosis of lymphosarcoma, showing patchy, malignant infiltrates of different thickness. The remaining two patients had very different features: the lesions appeared to be intrinsic to the posterior left ventricular wall. One patient who had arrhythmias and a small encapsulated noncontractile mass had a histologically proved rhabdomyoma. In the second patient fibroma was histologically confirmed. In these two patients the position of the lesions was confirmed by angiocardiography. Two-dimensional echocardiography greatly improved delineation of space-occupying cardiac masses and is an accurate method for confirming the presence of such lesions.

Neurological complications of balloon angioplasty in children

SummaryWe report on the occurrence of cerebrovascular accidents as a possible complication of balloon angioplasty in children. The first patient underwent balloon angioplasty for aortic stenosis and subsequently developed a right temporoparietal infarct in the vascular territory of the right middle cerebral artery. The second patient developed a right temporoparietal infarct also in the vascular territory of the right middle cerebral artery following balloon angioplasty of native coarctation of the aorta. This experience suggests that, in addition to the previously reported complications of balloon angioplasty, the occurrence of a potential cerebrovascular accident must be considered in the risk-benefit analysis of this procedure.

Diagnosis of fetal ductus arteriosus aneurysm: importance of the three‐vessel view

To present four cases of ductus arteriosus aneurysm (DAA) detected by fetal echocardiography and highlight the value of the three‐vessel view in the diagnosis of DAA.

Pulmonary atresia with intact ventricular septum and antenatal left ventricular failure

Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.

Clipped Tube Fenestration After Extracardiac Fontan Allows for Simple Transcatheter Coil Occlusion

BACKGROUND Expensive devices are increasingly used to close a patent fenestration after a modified Fontan operation. We report our 5-year institutional experience of clipped tube fenestration after extracardiac Fontan operation, which allows for simple transcatheter coil occlusion. METHODS We retrospectively reviewed 30 children, median age of 4.0 years (range, 2.4 to 8.8 years) who underwent extracardiac Fontan operation between May 1996 and May 2001, and were fenestrated using a 4- to 8-mm diameter clipped tube graft. RESULTS Ten children had a patent fenestration occluded by transcatheter placement of 15 detachable coils (5- to 8-mm diameter). Aortic oxygen saturations increased on average by 5.5% (2% to 14%) and mean pressures in the Fontan circuit by 2.5 mm Hg (0 to 3 mm Hg). Four had immediate complete occlusion angiographically and 6 had trivial residual shunt, but complete occlusion by echocardiography at follow-up. There have been no immediate complications, late coil embolizations, thromboembolic events, or documented hemolysis within a follow-up after coil implantation of 1.7 years (0.4 to 4.5 years). Spontaneous fenestration closure was documented in 8 patients at cardiac catheterization and 9 patients by echocardiography with consistent improvement in resting transcutaneous oxygen saturation. Two children with a patent fenestration have been considered inappropriate for closure, and there was one early surgical death. There have been no complications related to the tube fenestration modification within a follow-up postoperation of 2.6 years (0.1 to 5.5 years). CONCLUSIONS Clipped tube fenestration after extracardiac Fontan operation is a useful surgical modification that allows for simple transcatheter coil occlusion.

The atrioventricular junction in the univentricular heart: A two-dimensional echocardiographic analysis

SummaryThe atrioventricular junction of 40 patients with univentricular heart was evaluated by two-dimensional echocardiography. The apical 4 chamber view optimally imaged the atrioventricular junction, and allowed determination of the type of atrioventricular connection: double inlet, common atrioventricular orifice, and absent right or left atrioventricular connection. When double inlet to 1 ventricle was demonstrated, the 4 chamber view allowed immediate comparison of the form and function of the right and left atrioventricular valves. Because anomalies of the atrioventricular valves frequently complicate the univentricular heart, two-dimensional echocardiographic assessment is a most important adjunct to the preoperative investigation of these patients.

Cardiac strangulation following epicardial pacemaker implantation: A rare pediatric complication

OBJECTIVES The aim of our study was 2-fold: to determine the incidence of cardiac strangulation (CS) and to develop a clinical pathway to aid in the diagnosis and prognosis of CS. In <2 years, 2 cases of CS occurred in our institution, which caused much alarm and led to the studys objectives. METHODS All patients who underwent implantation of an epicardial pacemaker from January 1992 to March 2012 were included. There were no exclusion criteria. Health records were used to locate all subjects and gather all retrospective data. Prospectively, subjects without a chest radiograph from the previous 2 years were approached for imaging. RESULTS This study included 86 patients retrospectively, and 84 patients prospectively. There was a 2.3% incidence, and a 1.2% mortality, related to CS. A pattern of posterior looping of the ventricular lead was seen in radiographs of both CS-diagnosed patients. Five variables were significantly associated with an outcome of CS (P = .0153). CONCLUSIONS Our data indicate that the 2 cases of CS were not caused by a lack of follow-up but by a lack of consistent imaging for diagnosis. This conclusion is supported by the 8 cases of CS found in the English-language literature. If the patient is age ≤6 months at the time of implantation, particular attention should be given to the placement of leads and follow-up.

Cardiac strangulation from epicardial pacemaker: early recognition and prevention.

Cardiac strangulation from epicardial pacemaker leads is a rare event that can be difficult to recognise and can cause serious complications such as cardiac failure or death. We describe a 3-year-old girl who received an epicardial pacing system as a neonate for complete congenital cardiac block and developed cardiac strangulation from the leads. The clinical presentation modes are reviewed and technical aspects for lead and generator positioning are discussed.