Constrictive pericarditis is a uncommom disease in children. We have now encountered pericardial thickening as the cause of severe constrictive physiology in two patients, one also having haemodynamic features of restrictive cardiomyopathy. Both patients, who had refractory ascites and evidence of increased systemic venous pressure, underwent Doppler echocardiography, cardiac catheterisation, and magnetic resonance imaging. Resonance imaging failed to show any thickning of the pericardium, but cardiac catheterisation revealed diastolic equalisation of pressures in all four chambers, with only mild elevation of pulmonary pressure in the first patient, but nearly equalisation of diastolic pressure, and a very high pulmonary arterial pressure with a difference of 7 mm Hg between the end diastolic pressures in the two ventricles in the second patient. Doppler revealed a restrictive pattern of mitral inflow, with high E and small A velocities and a short deceleration time. The clinical background did not suggest pericardial disease in either of the patients. We conclude that a careful search is needed to uncover constrictive pericarditis when there is no previous disease which may suggest late pericardial constriction. The haemodynamic features of restrictive cardiomyopathy can co-exist with pericardial restriction, and differentiation between the two entities is critical in view of the diverse management and prognosis of the two conditions.

Data on long-term follow-up for closure of so-called secundum type“ atrial septal defects within the oval fossa using recently developed devices are limited, and results focused on presence of residual shunting. The purpose of our study was to report the experience from a single center establishing the effectiveness of transcatheter closure in patients with various types of defect other than those located centrally within the oval fossa.

A total of 72 patients was included in this study. On transesophageal echocardiography, the size of the defects varied from 6 to 18 mm, with estimation of the stretched diameter from 11 to 21 mm. The ratio of stretched diameter to the extent of the residual septum ranged from 0.28 to 0.54. Mean follow-up was 30.5+ 7.4 months, with a range from 13 to 42 months. The rate of closure using devices with diameters from 28 to 40 mm increased from 80% immediately after implantation to 93% in the 57 patients examined 24 months after implantation. For further analysis, we compared the 44 patients with a solitary, centrally located, defect to 28 having morphological variations, including superiorly located defects with deficient superior and aortic rims, multifenestrated and aneurysmal defects, or isolated additional defects. There was no incidence of formation of thrombus, sustained atrial arrhythmia, or infective endocarditis. Residual shunting was not influenced by location or morphology of the defects, but increased with size, stretched diameter, and the ratio of pulmonary to systemic flows. Serial transthoracic echocardiographic findings revealed malposition of one right-sided superior arm of the device in 8 patients, while protrusion of one leftsided arm onto the right atrial aspect was observed in 3 patients. Fluoroscopy showed fatigue fracture of a single arm in 7 patients (9.7%) within the first 6 months after implantation.

These results demonstrate that transcatheter closure with the non self-centering double umbrella device was effective and safe on medium-term follow-up, and could be extended to defects within the oval fossa having various morphologies. Residual shunting resolved with time, and was not related to either morphology or the position of the device.

An 8-year-old girl, without any previous medical history, presented with a first short syncope. Physical examination was unremarkable. Transthoracic echocardiography revealed a thin-walled, echo-free cystic structure adjacent to the posterior wall of the left ventricle, and compressing it moderately. Other echocardiographic findings were normal. Both computed tomography and magnetic resonance imaging suggested a simple pericardial cyst, but during surgery we found an epicardial cyst with partial involvement of the circumflex branch of the left coronary artery. Cardiopulmonary bypass was necessary for successful resection of the cyst, leaving behind only the small area in continuity with the coronary artery.

A 6-year-old boy presented with endocarditis of the tricuspid valve caused by penicillin resistant pneumococcus. He had no history of congenital cardiac disease. The likely portal of entry for the organism was the middle ear.

In an 11-year old boy with a large coronary arteriovenous fistula between the left coronary artery and the right atrium, we achieved successful closure using a 10–8 Amplatzer Duct Occluder introduced from the right internal jugular vein. Angiography 6 weeks later showed complete occlusion of the fistula, with normal opacification of the left coronary arterial system. This technique may be used as an alternative to coils and surgery in selected patients.

A young woman with surgically treated tetralogy of Fallot with pulmonary atresia, with multiple aorto-pulmonary collateral arteries, developed an arteriovenous fistula subsequent to transvenous insertion of an automatic implantable defibrillator via the left subclavian vein. The fistula extended between this vein and a systemic-to-pulmonary collateral artery arising from the subclavian artery. Unilateral peau d'orange, and painful congestion of the left arm and breast, ensued. These were cured by coil embolization of a fistula-related aneurysm.

Pseudoachondroplasia is an autosomal dominant variant of osteochondroplasia that results in mild to severe short-limb dwarfism and early-onset of osteoarthrosis. It has been linked to results from mutations in the gene for cartilage oligomeric matrix protein. We describe a 4-year-old boy with pseudoachondroplasia who also had prolapse of the mitral valve. To the best of our knowledge, this association has not previously been reported.

Congenital absence of aortic valvar leaflets is a rare and fatal variant of the hypoplastic left heart syndrome. We describe a recent patient seen at our institution with this lesion, illustrating a combined echocardiographic and angiographic approach that delineates both anatomy and physiology. The early mortality experienced in previous reports, as well as unsuccessful surgical palliation in our case, should promote further discussion regarding the optimal treatment.

We describe the finding of unsuspected aortic coarctation, discovered by catheterisation at 4 months, in a neonate first daignosed on the fourth day of life as having tetralogy of Fallot with pulmonary atresia and major aorto-to-pulmonary collateral arteries. This previously unreported combination contravenes the established theory of patterns of flow during fetal life, and presented difficult issues in clinical management.

We describe a three-year-old boy who presented with recurrent expectoration of bronchial casts six months following creation of a fenestrated lateral tunnel Fontan circulation for pulmonary atresia with intact ventricular septum. Cardiac catheterization demonstrated elevated central venous pressure with two areas of stenosis within the Fontan circuit, specifically at the junction of the right superior caval vein and the right pulmonary artery, and between the atrial baffle and the right superior caval vein. Insertion of Palmaz stents in these areas resulted in a reduction in central venous pressure, and a transient reduction in production of casts. Eight weeks after catheterization, however, he produced further casts, which resulted in hypoxia, respiratory arrest and death. We reviewed the autopsied specimens obtained from patients with the Fontan circulation over an eleven-year period at our institution in order to ascertain the prevalence of subclinical production of bronchial casts. We found no casts in the thirteen patients examined. Hemodynamic assessment is vital in all patients who develop this syndrome, and should be the primary focus of investigation, rather than solely directing efforts at lysis of casts.

The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy.

Endocarditis due to group B streptococcus is very rare in infants, and may be associated with significant morbidity and mortality. Review of the literature reveals only a single reported case of an infant with this type of streptococcal endocarditis involving the mitral valve. This infant had underlying congenital heart disease, and died shortly after catheterization. We now report group B streptococcal endocarditis occurring in an infant with a structurally normal heart who was treated successfully by replacement of the mitral valve.

Isolated noncompaction of ventricular myocardium is a rare cardiomyopathy, presumed to originate from a developmental abnormality in the evolution of the heart, and resulting in sponge-like myocardium. Isolated ventricular noncompaction can present with a variety of symptoms, but usually includes heart failure. The diagnosis is often made by echocardiography, which reveals a very distinct image of the myocardium, with many deep, confluent recesses and dense trabeculations. We encountered such findings in a moribund neonate presenting with cardiogenic shock with extremely low shortening fractions. After treatment with intravenous and oral cardiotonics, coupled with afterload reduction, we were able to optimize the balance between ventricular filling and myocardial contractility, resulting in markedly improved cardiac function as judged clinically, and as measured by echocardiography. As far as we know, this is one of the youngest patients yet reported to have a good recovery.

Tissue harmonic imaging improves the quality of images in adults with poor acoustic windows, but its utility in children has yet to be evaluated. The use of fundamental and harmonic imaging were compared in 70 children with poor echocardiographic windows. Scores were higher for harmonic in comparison to fundamental imaging for all views. Harmonic imaging improves the visualization of cardiac structures over fundamental imaging in children with poor echocardiographic windows.

We describe the development and exacerbation of protein-losing enteropathy after relocating to an environment at an altitude of 3695 feet in El Paso, Texas, in a patient who had undergone a Fontan operation. This report should heighten awareness to the possibility of such patients developing protein-losing enteropathy at high-altitude, with hypoxemia-induced pulmonary vasoconstriction, and subsequent elevation of central venous pressure, the most likely underlying mechanism.

We report our experience of aortic valvar replacement subsequent to the arterial switch procedure, incorporating the Lecompte maneuver, in patients with complete transposition. The anterior wall of the neoaorta was incised in continuity with the posterior wall of the pulmonary trunk. The aortic root was reconstructed with or without augmentation using a patch after the operative procedures to the aortic valve. With this approach, the neoaortic root could readily be exposed without extensive dissection, and valvar replacement was safely achieved. These patients experienced no postoperative complications.