Over the years, management of critical pulmonary stenosis in young infants has evolved from surgical reconstruction of the right ventricular outflow tract and closed pulmonary valvotomy to transcatheter balloon valvoplasty. Our study aimed at evaluating how the changing policy for management had affected the immediate and long term outcomes of babies with this cardiac lesion. Interventions were made in 34 infants at a median age of 8.5 days (2–90 days). Reconstruction of the right ventricular outflow tract reconstruction was performed in 10 patients, closed pulmonary valvotomy in 13, and balloon valvoplasty in 11. Initial procedure-related mortality was 50%, 15% and 0% respectively. Multivariate analysis revealed transannular patching of the right ventricular outflow tract, and male sex, to be significant factors for death. For the 27 survivors, the ratio of right ventricular to systemic systolic pressure decreased from 1.6 ± 0.3 to 0.3 ± 0.2 after reconstruction of the outflow tract, 1.8 ± 0.5 to 0.8 ± 0.4 after closed valvotomy, and 1.8 ± 0.6 to 0.9 ± 0.3 after balloon valvoplasty. The decrease was significantly greater after patch reconstruction (p=0.025) that required no further reinterventions. The overall rate of reintervention for the survivors was 37% (10/27). The freedom from reintervention after closed valvotomy was 82%, 64% and 51% at 1, 5 and 10 years respectively. The figure remained at 78% at both 1 and 5 years (p=0.66) after balloon valvoplasty. The higher reintervention rate for closed valvotomy corresponded to the significantly greater residual gradient across the pulmonary valve noted on follow-up (p=0.01). Reinterventions included balloon dilation (n=6), reconstruction of the outflow tract (n=4), and 1 each of ligation of an arterial duct and systemic-pulmonary arterial shunting. The risk factor for reintervention was a hypoplastic right ventricle. In conclusion, transcatheter balloon valvoplasty appears to be the optimum initial approach in view of its low mortality, efficacy at relieving the obstruction, and low rate of reintervention.

The bidirectional Glenn and Fontan procedures are empirically performed as interim and definitive procedures in children with a functionally single ventricle. The optimal stage of palliation, nonetheless, remains unknown. During childhood, growth is a fundamental measure of response to therapy. Growth may be influenced by the degree of cyanosis, the volume load on the ventricle, and cardiac performance. Thus, the weight and stature of children with a functionally single ventricle who underwent a bidirectional Glenn procedure or a Fontan procedure were studied to determine the effect of each intervention on growth. Z scores for weight and stature were retrospectively determined prior to palliation, at yearly intervals for 4 years, and from long-term measurements until 18 years of age in all patients with at least 2 years of observation following palliation. Growth was evaluated in 54 patients with a bidirectional Glenn procedure, and 65 patients with a Fontan procedure. The Z scores for weight were improved after each method of surgical palliation. Stature, however, was improved only following the bidirectional Glenn procedure. Growth was impaired in patients who developed protein losing enteropathy. Weight improved only during the initial 2 years after the Fontan procedure in patients who had a surgical fenestration. Over the long-term, patients who underwent a Fontan procedure were more likely to have a Z score less than −2.0 for weight and stature than patients who underwent only a bidirectional Glenn procedure. Late mortality and the incidence of heart transplantation were increased in patients who experienced a decrease in their rate of growth, defined as a negative change of more than one Z score in weight or stature, following the Fontan procedure. In conclusion, at moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure. A decrease in the rate of growth is associated with a poor prognosis following the Fontan procedure.

We describe two cases of left juxtaposition of the atrial appendages in which fetal echocardiograms provided a clue to the diagnosis. Both cases were associated with complex cyanotic congenital heart disease. The clue to the diagnosis was found at the three-vessel view. Abnormal vascular spaces were seen on the left side of the cross-sections of the great arterial trunks.

Ventriculo-coronary arterial communications are rare congenital heart defects which have been explained traditionally on the basis of abnormal persistence of such communications found in the normal developing heart. Recent studies, however, have suggested that these embryonic communications might be an incidental finding rather than a normal feature. Thus, it has been suggested that congenital ventriculo-coronary communications do not represent remnants of normal embryonic vessels, but rather represent acquired lesions. In the present study, hearts were constructed in embryonic chicks in which the coronary vasculature was almost completely derived from a quail-donor. After immunohistochemical staining of the quail-derived coronary endothelium, chimeric hearts were analysed with respect to the presence of embryonic ventriculo-coronary communications, and with respect to the origin of these structures from either coronary arteries or endocardium. The results demonstrate the normal presence of ventriculo-coronary communications in avian embryonic hearts. They show, furthermore, that these structures are of coronary endothelial origin. The findings are in accord with the traditional view on the pathogenesis of congenital ventriculo-coronary communications. The roles of elevated ventricular pressure, abnormal remodelling of the developing myocardium, and of abnormal growth of the coronary vasculature are discussed relative to the pathogenesis of congenital ventriculo-coronary communications.

The perigraft reaction is an unusual complication found in patients in whom a modified Blalock Taussig shunt has been created using a polytetrafluoroethylene graft. We found that, in two infants, consistent laboratory findings during such a perigraft reaction were hypofibrinogenemia, increased levels of thrombin-antithrombin III complex, prothrombinfragment 1 and 2 and products of degradation of fibrin. Normalization of the levels of fibrinogen produced resolution of the perigraft reaction.

Severe mechanical haemolysis occurred in an 11-month-old boy after implantation of the new Amplatzer duct occluder. Temporary balloon occlusion of the aortic ampulla was performed 4 days after the initial procedure leading to prompt abolition of the haemolysis.

We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.

We describe an unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant. The infant is asymptomatic 18 months after the second surgery. The very rare cardiac involvement, and the early relapse of the inflammatory pseudotumor, to the best of our knowledge, is a unique combination.

The inflammatory myofibroblastic tumor, as known as a pseudotumor or plasma cell granuloma is an uncommon reactive lesion with unknown aetiology. It is found most commonly in the lung and a number of visceral organs, such as the spleen, liver, ileum, salivary glands, urinary bladder, larynx or brain or in the retroperitoneum and lymph nodes. To our knowledge only 9 cases have been published of such tumor arising within the heart.1–6

We report a newborn male who presented with severe central cyanosis on the third day of life. Partial thrombotic obstruction of the pulmonary trunk secondary to Antithrombin III (homozygous defect of heparin binding site) deficiency was subsequently diagnosed. Surgical thrombectomy, and infusions of Antithrombin III concentrate, led to a successful outcome. We postulate that intrauterine thrombosis occurred to give this unusual presentation.

In a hemodynamically compromised neonate with complete transposition and intact ventricular septum (concordant atrioventricular and discordant ventriculo-arterial connections), it became necessary to control severe congestive heart failure. The arterial switch operation had been delayed because of intracranial bleeding and subsequent coagulopathy. The cardiac failure was reversed by surgical banding of the patent arterial duct. The arterial switch was performed successfully one week later.