most paediatric cardiologists think that the optimal treatment for patients with functionally single ventricle is the relatively early construction, at from 2 to 7 years of age, of the total cavo-pulmonary circulation. this approach is based on the desire to produce an acyanotic child, such an outcome fostering the belief that the patient is cured. when using this therapeutic approach, the total connection is usually preceded by earlier creation of a partial connection, achieved by creating a bi-directional anastomosis between the superior caval vein and the right pulmonary artery at the age of from 3 to 9 months, without providing additional flow of blood to the lungs. this leads to severe cyanosis by the age of 3 years, when the flow increases in the inferior caval vein, and is the signal for early completion of the total connection. in this policy, the absence of additional flow to the lungs is justified by the fear of altering the ventricular function essential for the success of the total connection, because of the volume, and eventually the pressure, overload of the additional flow on the functionally single ventricle,which may impair its compliance and/or contractility, or be responsible for atrioventricular valvar regurgitation. these alterations in myocardial functions would then contraindicate, or increase, the mortality and morbidity of the total connection, producing deterioration in its long-term results.

We describe an infant with hypoplasia of the left heart diagnosed prenatally who, at birth, had signs of severe pulmonary venous obstruction. Echocardiography indicated normally connecting pulmonary veins, and showed a paradoxical right-to-left shunt across a patent oval foramen. Postmortem examination revealed that the obstruction was due to a divided left atrium, or cor triatriatum sinister, with an imperforate muscular diaphragm separating completely the two components of the divided atrium.

The use of a conduit of polytetrafluoroethylene placed between the right ventricle and the pulmonary arteries as source of pulmonary arterial supply during the first stage of palliation for the hypoplastic left heart syndrome has facilitated post-operative management and resulted in decreased mortality. We describe here the use of a cryopreserved saphenous vein inserted in reversed direction to create the connection between the right ventricle and the pulmonary arteries in a neonate with low birth weight undergoing the modified Norwood procedure.

We report on two children with advanced acquired immune deficiency syndrome presenting with vasculopathy involving the large vessels. Both patients had extensive involvement of the aorta and its branches. One patient presented with heart failure, and mild systemic hypertension secondary to renal arterial stenosis, while the other patient manifested with gangrene of both arms.

based on the revolutionary concept of the dispensable right ventricle, the fontan operation stands as the extreme example of physiologic repair in pediatric cardiac surgery, serving as the common surgical denominator for the entire spectrum of functionally univentricular conditions. also called ortho-terminal repair, it makes allowance for a circulatory arrangement that is in-series by effectively driving the pumpless systemic venous return directly through the pulmonary circulation. if this arrangement is to work, then two conditions must be fulfilled. first, there must not be any anatomical or functional hindrance along the whole fontan pathway, from the caval venous inflows to the aortic outflow. second, there must be a well-suited systemic pump, essentially recruited to the dual function of drawing blood from and, simultaneously, driving blood through, the lungs. this latter function, driving the blood through the lungs, takes place during the venous phase of the systemic output.

Cardiac transplantation is now a therapeutic option with good outcomes for infants and children with end-stage cardiac failure or inoperable congenital cardiac defects.1 In 2000, 356 procedures in children aged up to 18 years were reported to the United Network for Organ Sharing – International Society of Heart and Lung Transplantation Registry.2 Congenital cardiac disease was the most common indication in children less than 1 year of age, and overall was the most common diagnosis leading to cardiac transplantation in children. As a result of the groundbreaking contributions of Bailey et al.,3 transplantation is now an option for primary treatment of infants with hypoplastic left heart syndrome born in the United States of America. The availability of donor organs, however, both limits the number of children with hypoplastic left heart syndrome who can be realistically offered transplantation, and contributes significantly to morbidity, mortality, and costs. Current limitations in the identification and availability of donors would not allow transplantation to be the primary option for all children born with hypoplastic left heart syndrome. Analyses of the database of the Registry, however, indicated that only about half of donations resulted in transplantation of the heart.4 These observations provide compelling evidence of significant opportunities to improve rates of donation, and to utilize better the existing donations, with emphasis on optimizing identification of donors, expanding the number of potential donors, and better distribution of the available organs.

A right aortic arch, with a retroesophageal transverse arch and an aberrant left brachiocephalic artery, is a rare anomaly by itself. When combined with severe mitral stenosis and hypoplasia of the left ventricle, it is rarer still. We describe the diagnosis and treatment of this rare anomaly in an infant using a modification of the Norwood operation.

We occluded a large arterial duct in a patient with atrial fibrillation, using an Amplatzer duct occluder, with no residual shunt at the end of the procedure. Follow-up revealed recanalization, which was successfully treated with a 4 mm Amplatzer ASD occluder. It is speculated the reopening of the initially occluded duct was the consequence of anticoagulation needed because of the atrial fibrillation.

The unguarded tricuspid valve is uncommon. We describe herein a fetus with a grossly dilated right ventricle and atrium, with severe tricuspid and pulmonary valvar regurgitation. The right ventricle was akinetic, and no tricuspid tissue or valvar apparatus was identified. Colour Doppler showed a highly unusual retrograde flow of blood through the right heart. The pregnancy was terminated, and necropsy examination confirmed the gross dilation of the right heart chambers, with severely dysplastic valvar tissue at the right atrioventricular junction effectively giving an unguarded orifice. There was no valvar displacement, and the left heart was normal. The fetus had a normal karyotype, albeit with absent kidneys.

Based on experience in several centers, the double switch operation has reportedly become the standard surgical therapy for congenitally corrected transposition. We report and discuss here the use of a “ventricle and a half” double switch operation performed due to the concerns raised intraoperatively because of the size of the morphologically right ventricle. Although the long-term course of such a procedure in this setting remains unknown, we submit that the proposed benefits of the double switch operation, even when used in the “ventricle and a half” configuration, may be superior to the alternatives.

Transplantation of the heart remains a viable option not only as primary treatment for hypoplastic left heart syndrome, but also for end-stage problems after the Norwood sequence of palliations. In this review, I discuss the pre-operative, intra-operative, and post-operative echocardiographic evaluation of these patients, with special emphasis on hemodynamic and functional assessment as well as surveillance for rejection and coronary arterial disease.

A young child with exertional chest pain, and an electrocardiographic pattern suggesting reversible ischaemia of the anterior ventricular wall documented by Cardiolyte stress-testing, underwent cardiac catheterization and selective coronary angiography. Although the coronary arteries were entirely normal, the recirculation phase demonstrated marked dilation of the coronary sinus, with atresia of its mouth. At surgery, the patient was confirmed to have muscular atresia at the mouth of the coronary sinus, and underwent unroofing of the coronary sinus to the left atrium, with ligation of a persistent left superior caval vein. Post-operatively, the patient continued to have persistent chest pain, albeit without inducible ischaemia on stress-testing.

We found spontaneous echoes in two teenagers with nephrotic syndrome and profound hypoalbuminemia, both having normal cardiac structure, function and output. The phenomenon disappeared after the level of albumin normalized. In one patient, all spontaneous echoes disappeared following convalescence, the level of albumin in the serum then being documented at normal levels. The second patient, who presented with profound hypoalbuminemia, was infused with human albumin because of oliguria, following which the spontaneous echoes disappeared.

We report a case of a true cleft in the anterior leaflet of the mitral valve. The cleft, however, is directed toward the ventricular septum, and the left ventricular papillary muscles have the same arrangement as seen in the setting of a common atrioventricular orifice. The atrioventricular septum is intact. This appearance reflects the presence, in this patient, of right ventricular origin of a solitary arterial trunk, so that there was no outflow tract within the left ventricle to interpose between the mitral valve and the septum.

the norwood procedure involves three separate stages of operative corrections. the first stage involves re-fashioning the pulmonary trunk into a neo-aorta so that it is possible to establish an unrestricted systemic circulation. an interpositional, or systemic-to-pulmonary arterial, shunt is then created between the neo-aorta and the pulmonary arteries to allow pulmonary perfusion and gas exchange. two of the available options for the systemic-to-pulmonary shunt are the central shunt and the right modified blalock-taussig shunt. in the setting of a central shunt, pulmonary perfusion is derived from a conduit placed between the pulmonary arterial bed and the neo-aorta whereas, in the modified blalock-taussig shunt, the conduit is interposed between one of the pulmonary arteries and the brachiocephalic artery. in subsequent stages, pulmonary perfusion is provided directly by deoxygenated blood. this is achieved by connecting, first, the superior caval vein, and then the inferior caval vein, to the pulmonary arteries. it is usually during the second stage that the systemic-to-pulmonary shunt is removed.