In all patients undergoing cardiac surgery, the effective delivery of oxygen to the tissues is of paramount importance. In the patient with relatively normal cardiac structures, the pulmonary and systemic circulations are relatively independent of each other. In the patient with a functional single ventricle, the pulmonary and systemic circulations are dependent on the same pump. As a consequence of this interdependency, the haemodynamic changes following complex palliative procedures, such as the Norwood operation, can be difficult to understand.

Comparison of the newly created surgical connections to a simple set of direct current electrical circuits may help the practitioner to successfully care for the patient. In patients undergoing complex palliations, the pulmonary and systemic circulations can be compared to two circuits in parallel. Manipulations of variables, such as resistance or flow, in one circuit, can profoundly affect the performance of the other circuit. A large pulmonary flow might result in a large increase in the saturation of haemoglobin with oxygen returning to the heart via the pulmonary veins at the expense of a decreased systemic flow. Accurate balancing of these parallel circulations requires an appreciation of all interventions that can affect individual components of both circulations.

The purpose of our paper is to assess the prognostic value of balloon occlusion as a test for patients with various types of functionally single ventricle after different palliative surgical procedures who were candidates for further modifications of a cavo-pulmonary operation. In all patients, there were at least two sources of pulmonary arterial supply.

Our hypothesis was that, using balloon occlusion temporarily to produce changes in the haemodynamic situation, we could simulate the potential state subsequent to surgical palliation. We used balloon occlusion in 19 patients during diagnostic catheterisation, followed by interventional and or surgical procedures as considered necessary. This test was applied for temporary closure of the pulmonary valve in 8 patients, and a Blalock-Taussig anastomosis in 11 patients.

Surgical treatment of cardiac defects in infants born with extremely low weight is sometimes required during the neonatal period. Optimal timing of these operations has yet to be clarified. With this in mind, we reviewed our experience of surgical treatment for 29 infants born with extremely low weight between 1994 and 2001. The main surgical procedures were ligation of a patent arterial duct in 26, a Brock procedure in 2, and ligation of an aorto-pulmonary window in 1 infant. The age at operation ranged from 5 to 57 days, with a median of 30 days, and weighed from 506 to 902 g, with a median of 710 g. There were no deaths. For the 2 infants undergoing the Brock procedure, the reduced systemic blood flow also necessitated closure of the arterial duct. For almost all the 26 infants with a patent arterial duct, indomethacin was given as the initial therapy, but the duct had not closed completely. Increased symptomatology just before the operation due to reduced systemic blood flow, such as decreased cerebral blood flow, decreased urine output, and intestinal ischemia, mandated the earlier surgical ligation (r = −0.576, p = 0.004). The youngest infant needed an infusion of catecholamines perioperatively to maintain stable hemodynamic conditions (r = 0.554, p = 0.003). In 4 infants, including the youngest 2, steroids were administered intravenously just after the ligation. Our results suggest that reduced systemic blood flow is the main indication of surgical repair in infants born with extremely low weight. Even for one in whom the supply of pulmonary blood is dependent on the arterial duct, early reconstruction of the pulmonary arterial pathways, using the Brock procedure, followed by ligation of the duct, is required. Acute adrenal insufficiency should not be overlooked just after the surgery, particularly in the youngest patients.

Background: In adults with heart failure, neurohormonal overstimulation is related to the progression of the disease, and influences prognosis. β-blockers, which modulate neurohormonal activation, now play an essential role in the pharmacological management of heart failure in adults, but their use in children is very limited. Patients and Methods: To investigate the effects of carvedilol administration on neurohormonal activation and left ventricular function, carvedilol was added to standard treatment for heart failure in 9 patients with dilated cardiomyopathy due to heart muscle disease. Standard treatment has been in place for at least 1 month. The protocol consisted in a baseline evaluation to assess neurohormonal activation, and echocardiographic evaluation of left ventricular function. This was followed by a final evaluation at 12 months from carvedilol loading. Carvedilol was started at 0.05 mg/kg/day, and increased every two weeks until the target dose of 0.8 mg/kg/day was reached. Results: Carvedilol administration was associated with a significant reduction in plasma norepinephrine (p = 0.00001), dopamine (p = 0.0001), aldosterone (p = 0.00001) and activation of the renin-angiotensin system (p = 0.0006). Similar reductions in vanilmandelic and homovanillic acid were noted. After 12 months, a positive remodeling took place, with significant reductions in end-diastolic (p = 0.004) and end-systolic diameters (p = 0.009), and an increase in left ventricular ejection fraction (p = 0.001). No adverse effects needing reduction or interruption in the dosage were noted in the run-in phase, nor in the period of maintenance. Conclusion: Carvedilol is a safe complement to standard therapy for heart failure in children, allowing a significant reduction of neurohormonal activation with evident benefits on both ventricular function and the clinical condition.

Objective: Modern surgical techniques now offer the potential for reconstructing rather than replacing the mitral valve in those patients who require an operation during childhood. The purpose of this study was to assess the outcome in our centre following mitral valvar surgery in children. Methods: Between 1992 and 2002, 51 consecutive children, with a mean age of 12.5 ± 3.5 years, underwent surgical treatment of mitral valvar stenosis and/or insufficiency in the Cardiovascular Surgical Department of Istanbul University. Of the children, 20 underwent repair, whilst we replaced the valve in 31 patients. In one, we inserted a pulmonary valvar autograft in mitral position. Results: There were neither early nor late deaths in those who underwent mitral valvar repair. Similarly, there were no early deaths in those in whom the valve was replaced, but two patients died late, while six outgrew their prosthetic valves and required insertion of a further valve within 10 years of their first operations. Conclusion: Our experience shows that procedures designed to repair the mitral valve are effective and reliable when performed in children, with low mortality, and a lower incidence of reoperation when compared with those having replacement of the mitral valve.

Objective: To heighten the awareness of pediatricians and pediatric cardiologists to aortic dissection, a potentially dangerous medical condition. Methods: We reviewed the charts of 13 patients, seen in four medical centers, who suffered acute or chronic aortic dissection over the period 1970 through 2000 whilst under the age of 25 years. Results: There were seven male and six female patients, with the mean age at diagnosis being 12.1 years, with a range from one day to 25 years. Congenital cardiac defects were present in five patients, and Marfan syndrome in four. In three of the patients with congenital cardiac defects, aortic dissection developed as a complication of medical procedures. In three patients, dissection followed blunt trauma to the chest. We could not identify any risk factors in one patient. The presenting symptoms included chest pain in four patients, abdominal pain and signs of ischemic bowel in two, non-palpable femoral pulses in one, and obstruction of the superior caval vein in one. Angiography and magnetic resonance imaging were the main diagnostic tools. Overall mortality was 38%. Only six patients had successful surgical outcomes. Conclusion: Due to the rarity of aortic dissection a high index of suspicion is required to reach the diagnosis in a timely manner. It should be considered in young patients complaining of chest pain in association with Marfan syndrome, anomalies of the aortic valve and arch, and chest trauma.

Congenital disorders of glycosylation are a group of inherited metabolic multisystem disorders characterized by defects in the glycosylation of proteins and lipids. In most cases, neuromuscular disease is present. The purpose of this study was to characterize the cardiological aspects in this disorder.

From the literature, we identified six children with congenital disorders of glycosylation associated with cardiac disease. We then screened for cardiovascular manifestations 20 patients diagnosed with congenital disorders of glycosylation at our own institution.

Of the 6 patients identified in the literature, 4 had hypertrophic cardiomyopathy, while in the other 2 the cardiac diagnosis was unclear. The mean age at cardiac diagnosis was 5 months, with a range from 34 weeks to 24 months. Of the patients, five had died at a mean age of 3.5 months, with a range from 1.5 to 6 months, with one documented cardiac death. Three of our 20 patients (15%) had coexistent cardiomyopathy, and in three additional patients presenting with cardiomyopathy we made the diagnosis of a congenital disorder of glycosylation. In our cohort, dilated cardiomyopathy was found in two-thirds of the patients, with hypertrophic cardiomyopathy in the other third. The mean age at cardiac diagnosis was 19 months, with a range from 0.5 to 84 months. Of these patients, two died in infancy at a mean age of 4 months, specifically at 1.5 and 7 months, due to cardiac disease, with one dying suddenly. The remaining four patients are alive with minor to severe cardiac dysfunction.

We conclude that congenital disorders of glycosylation have to be considered in the differential diagnosis of children presenting with cardiomyopathy, and that all patients with congenital disorders of glycosylation should be screened for an associated cardiomyopathy. Cardiac involvement contributes significantly to morbidity and mortality, and probably to sudden cardiac death in this disorder.

Objectives: This study was designed to identify predictors of residual defects following deployment of the Amplatzer device to close septal defects within the oval fossa. Methods: Between February 1997 and February 2000, we used the Amplatzer device to close defects in the oval fossa in 89 patients. Of these patients, 18 (20%) had residual defects. At 6 or 12 months following placement of the device, 13 defects (14.6%) had persisted. We evaluated several variables derived from clinical features, transesophageal echocardiography and catheterization to establish predictors for residual shunting. Results: Multivariate analysis identified a shorter superior rim of less than 8 mm (Odds ratio = 10.1; 95% confidence intervals = 2.64–38.72; p = 0.001), and a smaller interatrial septum in the 30-degree transesophageal echocardiographic plane of less than 30 mm (Odds ratio = 5.5; 95% confidence intervals = 1.17–26.14; p = 0.03) as independent predictors of residual defects. When the analysis was repeated defining only those 13 patients with persisting residual defects at 6 or 12 months as failures, a short superior rim (p = 0.004) remained a predictor for residual shunting. Conclusions: Defects with a short superior rim and smaller interatrial septum in the 30-degree transesophageal echocardiographic plane independently and additively predict an increased probability of residual shunting following closure of defects in the oval fossa using the Amplatzer device.

Aerosolized iloprost is now used as a therapeutic option in the treatment of pulmonary hypertension. We report on the administration of this derivative of prostacycline in treating severe pulmonary hypertension of the newborn. The combination of iloprost instilled endotracheally and inhaled was chosen as a last attempt at treatment in a critically ill patient who did not respond to advanced conventional treatments, including high frequency oscillation and inhalation of nitric oxide. The use of iloprost converted permanently the right-to-left shunting, leading to a substantial improvement in oxygenation.

A 17-year-old girl with Turner's syndrome underwent two cardiac operations due to severe mitral stenosis with pulmonary hypertension, caused by a parachute-like mitral valve. The anomaly was associated with persistence of the left superior caval vein, which drained to the coronary sinus, and non-compaction of the left ventricular myocardium. The association of these lesions is rare in patients with Turner's syndrome.

An 11-year-old female, in apparent good health, was referred to our Institute because of exercise-induced left bundle branch block detected during a Master's step test performed to obtain the clearance for competitive sport. The tests that we performed to investigate the possible causes were all negative. We suspect, however, that the left bundle branch block is the initial sign of cardiomyopathy. If our suspicions are correct, close follow-up is important to chart the possible progression of the disease.

We report a patient with Down's syndrome referred to our Department of Echocardiography for evaluation after an episode of tachyarrhythmia. We diagnosed Ebstein's malformation. As far as we know, this association has been reported previously on only three occasions. We review these previous cases, describe our echocardiographic techniques, and discuss our experience.

We describe a patient with advanced perinatal acquired immunodeficiency syndrome who had early clinical manifestation of severe dilated cardiomyopathy with congestive heart failure. The picture was completely reversed after six years treatment and follow-up, and the child is now doing well at the age of seven, with normal left ventricular dimension and contractility as shown by echodopplercardiography. To the best of our knowledge, this is the first reported case of full recovery from cardiomyopathy in children with perinatally acquired infection by the human immunodeficiency virus.

A young woman with hypertrophic cardiomyopathy presented with intractable chest pain due to a myocardial bridge over the left anterior descending artery, producing severe compression during systole. Percutaneous intracoronary stenting was performed. The patient, however, developed severe and diffuse restenosis within 30 days of the procedure. Myotomy may provide a more effective treatment option for such patients with symptomatic myocardial bridging.

At 32 weeks, a fetus was found to be hydropic with congenitally complete heart block in an asymptomatic mother who was positive to anti-Ro antibodies. Maternal therapy with oral salbutamol was successful in prolonging gestation for a period of 3 weeks so that preterm delivery was delayed until the 35th week of gestation. Following treatment with inotropes and diuretics, a permanent transvenous pacemaker was implanted at 8 weeks of age. The child is now thriving at 20 months of age.

We report two instances of transient isolated right-sided myocardial hypertrophy in patients with an intact ventricular septum, normal thickness of the posterior wall of the left ventricle, and normal ventricular function, diagnosed by echocardiography on the third day of life. The two neonates, born at 36 and 38 weeks gestation respectively, had perinatal distress. Both were diagnosed as having isolated right ventricular hypertrophy with mild pulmonary hypertension, which disappeared in both cases within 8 weeks without any specific therapy. Though the cause of the ventricular hypertrophy remains unclear, we believe that it is the consequence of remodeling of pulmonary vasculature secondary to acute perinatal distress, resulting in persistent pulmonary hypertension and producing pressure overload on the right ventricle, and hence right ventricular hypertrophy. The finding of early and transient right ventricular hypertrophy, with normal left-sided structures and normal ventricular function, has thus far failed to gain attention in the paediatric cardiologic literature.