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Although 2-dimensional, Doppler, color flow echocardiography is accepted as a safe diagnostic method to guide the surgical treatment of certain heart defects, cardiac catheterization remains mandatory for patients with tetralogy of Fallot. Based on the excellent diagnostic correlation between echocardiography and angiocardiography, a policy of echo-guided primary repair of uncomplicated, selected cases of tetralogy of Fallot was introduced at Ospedale Bambino Gesu'. In the last 5 years, of 139 patients who had surgery for tetralogy of Fallot, 105 underwent primary repair. In 56,patient (53.3%), surgery was guided only by echocardiography (group I). In the remaining 49 patients (46.7%) (group II), unclear imaging of cardiovascular anatomy (n = 23), or echo-suspected malformations (n = 26) promoted cardiac catheterization. The 2 groups did not differ in age and weight at surgery. Intraoperative examination did not show diagnostic errors in patients of group I; cardiac catheterization and surgery confirmed the suspected associated anomalies in 16 of 26 patients of group II (38.4% false-positive). Echocardiography showed an overall sensitivity of 100% and specificity of 85% for detection of associated malformations. In conclusion, echocardiography proved to be adequate for selection of patients with uncomplicated forms of tetralogy of Fallot for primary repair.

An alternative strategy for left ventricular (LV) venting during short-term mechanical circulatory support is use of Impella axial-flow pump. We present our transcarotid Impella 2.5 implantation technique using a polytetrafluoroethylene graft, in two children with acute heart failure treated primarily with venoarterial ECMO. The venoarterial extracorporeal membrane oxygenator and Impella support were maintained for 5 and 17 days, respectively. Transcarotid Impella implantation might be an alternative and feasible option in pediatrics patients affected by severe LV failure, as a bridge to decision or bridge to candidacy. Potentially, the Impella 2.5 device provides less invasive support for children with heart failure.

AbstractIntroduction:Some authors advocate the use of a dedicated formula to predict the Fontan pressure starting from pre-Fontan catheterisation data. This paper aims at testing the predictive value of the mentioned formula through a retrospective clinical study.Methods and Results:Pre-Fontan catheterisation data and Fontan pressure measured at the completion were retrospectively collected. Pre-Fontan data were used to calculate the predicted pressure in the Fontan system. The predicted values were compared to the Fontan pressure measured at the Fontan completion and with the needs for fenestration. One hundred twenty-four Fontan patients were retrospectively enrolled (At Fontan: median age 30.73 [24.70–37.20] months, median weight 12.00 [10.98–14.15] kg). Fontan conduit was fenestrated in 78 patients. A poor correlation (r2= 0.05128) between the measured and predicted data for non-fenestrated patients was observed. In the case of Fontan-predicted pressure <17.59 mmHg, the formula identified a good short-term clinical outcome with a sensitivity of 92%.Conclusion:The proposed formula showed a poor capability in estimating the actual pressure into the Fontan system and in identifying patients needing fenestration. As the pressure into the Fontan system is determined by multiple factors, the tested formula could be an additional data in a multi-parametric approach.

Vascular events in patients with coarctation of the aorta have been extensively reported and account for the majority of morbidity and mortality in untreated patients. The exact mechanism for this association is not completely understood and may include acquired anomalies or congenital abnormalities of intracranial vessel. Here we report a case of intracranial internal carotid artery dissection with subsequent formation of acquired large carotid aneurysm in a child with severe systemic hypertension and coarctation of the aorta. Endovascular aneurysm exclusion was pursued and it was able to control this potentially lethal complication. This case supports the notion of acquired nature of intracranial vessel abnormalities and underscores the clinical role of interventional neuroradiology in a subset of patients with congenital heart disease.

AbstractObjectiveTo determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification.MethodsRetrospective study of 41 consecutive children affected by dilated cardiomyopathy ? aged 0?14 years; median 33.4 plus or minus 49.25 ? between 1993 and 2008. We reviewed the medical history to determine age at diagnosis, family history, previous viral illness, aetiology, symptoms and signs at presentation, treatment, and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. We also carried out a metabolic evaluation including blood lactate, pyruvate, carnitine, amino acids, urine organic acids, assessment of respiratory chain enzymes, and analysis of histopathological material. Survival curves were constructed by the Kaplan?Meier method.ResultsFollow-up ranged from 10 days to 162 months ? median 45.25 plus or minus 41.15 months. Freedom from death or cardiac transplantation was 68.3% at 5 years. The primary end-point of death/cardiac transplantation was associated with the need for intravenous inotropic support. A trend towards a poorer prognosis was found for age at diagnosis of more than 5 years and for a metabolic aetiology of dilated cardiomyopathy. For the children affected by cardiomyopathy as part of a multi-system involvement, mortality was 50%.ConclusionsIn children, dilated cardiomyopathy is a diverse disorder with outcomes that depend on cause, age, and cardiac failure status at presentation. Overt cardiac failure at presentation is a major prognostic factor for death or cardiac transplantation. Older age at presentation and metabolic aetiology may be associated with a poorer prognosis.

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.

OBJECTIVES

Cardiac angiotensin AT1receptors have been found in several animal species. In-vitro studies performed on cardiac preparations have shown that angiotensin II (ANG II) exerts a positive inotropic effect; however, in-vivo results have allowed no definitive conclusion to be drawn. The reasons behind these controversial results remain unknown, and could originate both from different experimental conditions and from the techniques used to assess myocardial contractility.

The effects of isoflurane versus halothane on cardiac contractility were evaluated by two-dimensional (2D) transthoracic echo cardiography in rabbits. The relationship between the left ventricular end-systolic wall stress (LVESWS) and the velocity of heart rate corrected circumferential fibre shortening (Vcfc) was used. Arterial blood pressure, heart rate, left ventricular pressure and transthoracic 2D echocardiographic data were determined at 1 MAC (minimum alveolar concentration) of halothane or isoflurane, both with 50% nitrous oxide. Drug-induced changes in pre-and afterload were performed in all study animals to assess the left ventricular contractile response over a wide range. LVESWS and Vcfc Were calculated on videotape recorded M-mode tracings.Mean heart rate and arterial blood pressure were not significantly different between the two groups. Myocardial contractility under isoflurane/nitrous oxide anaesthesia was significantly higher than under halothane/nitrous oxide anaesthesia at 1 MAC. The results of the present study confirm data obtained from humans and other animal species and suggest that, in rabbits, myocardial contractility is best preserved by inhalation of isoflurane. Isoflurane should therefore be preferred over halothane, especially in cases of prolonged anaesthetic procedures.

Background: Peripheral pulmonary artery stenosis is a relatively frequent anomaly responsible for right ventricular hypertension, significantly affecting the quality of life and the long‐term prognosis of patients with congenital heart disease. Balloon angioplasty has been used to relieve pulmonary artery stenosis, but immediate and long‐term results have not been completely satisfactory. Conversely, stent implantation seems a promising and more effective technique for the treatment of this vascular anomaly. Methods: Between April 1987 and September 1997, indication for interventional cardiac catheterization has been estabilished for 150 patients. Balloon angioplasty was performed in 120 patients (162 procedures) and stent implantation in 60 patients (79 stents). In 30 patients, both procedures have been carried out. Indication was based on the presence of right ventricular hypertension (right ventricular/systemic peak systolic pressure ratio ≥ 0.5), right ventricular dysfunction, or a markedly unbalanced pulmonary blood flow, especially after bidirectional cavopulmonary anastomosis. Criteria for technical success of balloon dilation were defined as a ≥ 50% increase in the predilation diameter, a ≥ 50% decrease in the transstenotic pressure gradient, or a ≥ 20% decrease in the right ventricular‐to‐aortic pressure ratio. Mean balloon/stenosis diameter ratio was 3.0 ± 0.7 (range 1.8–4.2). Time between primary surgery/balloon angioplasty and stent implantation was 18 ± 7 months (range 8–21 months). Stainless steel balloon‐expandable endovascular stents were maximally dilated to 12–18 mm of diameter, and high‐pressure dilations were performed to further expand the stent, if necessary. Results: After balloon angioplasty, the diameter of the stenotic segment increased from 3.1 ± 0.3 to 5.1 ± 1.4 mm (P < 0.01), the gradient across the stenotic segment dropped from 51 ± 19 to 28 ± 8 mmHg (P < 0.01), and the right ventricular/systemic peak pressure ratio decreased from 0.81 ± 0.20 to 0.65 ± 0.15 (P < 0.05). No significant difference between procedures with low‐pressure and high‐pressure dilation was recorded. Success rates using the above‐mentioned criteria could be assessed as high as 60%. After stent implantation, the diameter of the stenotic segments increased from 3.5 ± 1.2 to 13.2 ± 3.4 mm (P < 0.01), the pressure gradient across the stenotic segment dropped from 53 ± 30 to 13 ± 10 mmHg (P < 0.01), and the mean right ventricular/systemic pressure ratio decreased from 0.84 ± 0.2 to 0.40 ± 0.15 (P < 0.01). Immediate overall success for balloon angioplasty was achieved in 73 of 120 patients compared with 58 of 60 patients submitted to stent implantation (60% vs 97%, P < 0.001). The complication rate was 6.9% (5/162 procedures) in the balloon angioplasty group and 1.2% (1/79 implants) in the stent group. Restenosis has been recorded in 25% of the balloon group patients, in respect to 2% of the stent group. Conclusions: Peripheral pulmonary stenosis (PPS) is an important factor conditioning outcome of patients with congenital heart defects. Balloon angioplasty may be technically effective, but it often fails if clinical criteria and impact on patient's outcome are considered. In our experience, the use of high‐pressure balloons did not change the success rate of balloon dilation. Stent implantation seems to offer a better and more definitive approach for the relief of PPS.

An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure.

• OBJECTIVE. —To investigate the overall occurrence of congenital heart disease in 103 pedigrees with a proband affected with atrioventricular canal. DESIGN. —Family study of patient series. SETTING. —Department of Pediatric Cardiology, Bambino Gesu' Hospital, Rome, Italy. PARTICIPANTS. —One hundred three consecutive patients with atrioventricular canal and normal karyotype and their first- and second-degree relatives. INTERVENTIONS. —None. MEASUREMENTS/MAIN RESULTS. —In 12 pedigrees (11.7%), one or more relatives had concordant or discordant congenital heart disease. Congenital heart disease occurred in four (1.9%) of the 206 parents of probands, in four (3.6%) of the 111 siblings, and in five (0.8%) of the 644 uncles or aunts. None of the grandparents had congenital heart disease. Atrioventricular canal affected several family members in five pedigrees. CONCLUSIONS. —Familial aggregation of atrioventricular canal in several pedigrees requires careful collection of family histories, accurate cardiologic evaluation of the first-and second-degree relatives of the probands, and fetal echocardiography in all pregnant women in at-risk families.(AJDC. 1993;147:1295-1297)

Single trunk anomalous origin of both coronary arteries from the pulmonary artery is a rare congenital cardiac anomaly. We report on 2 cases of its association with aortic coarctation, the diagnosis of which in living patients is very difficult. We think that the possibility of this anatomic arrangement should always be considered in patients with isolated aortic coarctation whose clinical condition seems impaired rather than improved after an apparently successful coarctectomy.

In conclusion, pulmonary balloon valvuloplasty is a useful, safe, and probably definitive procedure in the treatment of isolated PS in patients <1 year of age. Because RV pressure overload can also be effectively reduced in neonates and infants, with a low incidence of complications and low risk of restenosis, we see no reason to delay valvuloplasty until a later stage of life.

AbstractDerivatives of Imidazoline usually act to stimulate peripheral alpha2 receptors causing vasoconstriction. In young children, however, they can also stimulate alpha2receptors in the cardiovascular and central nervous systems, possibly causing cardiovascular, neurological, and respiratory depression. These medications do not require medical prescriptions, so often parents use them, bypassing paediatricians. We report here a case of cardiovascular and neurological depression induced by oxymetalzoline in a toddler.