The report by Danetz, Donofrio and Embrey1 provides an interesting long term follow-up on the first patient diagnosed by me with what is now known as Noonan syndrome. In 1960, when this patient was seen, cardiac ultrasound was unavailable and left-sided cardiac catheterization uncommon. He had severe pulmonary stenosis, and did well following surgery. Unfortunately, his follow-up was not adequate and left-sided obstruction was not recognized until he became symptomatic some thirty years later. Early reports stressed the dysplastic pulmonary valve, with pulmonary stenosis as a common finding.2 By 1972, the report of Ehlers et al.3 brought attention to the left-sided involvement in the Turner phenotype.

Ventricular late potentials, and dispersion of the QT interval, are markers for risk of ventricular arrhythmias. Normal values for these parameters are well established in adults, but may not apply for children. This study has investigated the age dependency of signal averaged electrocardiographic parameters and QT dispersion in 111 normal children aged from 5 days to 16 years. The results indicate that parameters change with age: duration of filtered QRS and low amplitude (< 40 μV) terminal signal increase with age, especially in the younger patients. Filtered QRS is 88.9 ± 7.87 ms in infants, and increases to 108.7 ± 8.51 in teenagers (p<0.001). Low amplitude terminal signals are 17.0 ± 3.44 ms in infants, and 24.5 ± 5.64 ms in teenagers (p< 0.001). Root mean square of the last 40 ms decreases with age, but remains stable after the age of 10 years (122.4 ± 33.30 μV in infants, 60.9 ± 31.27 in teenagers,p<0.001). QT dispersion, on the other hand, does not change significantly with age. The mean value for the whole group is 36 ± 13.7 ms. A weak but significant correlation exists between QT dispersion and filtered QRS. Thus, age must be taken into consideration when interpreting signal-averaged electrocardiograms, but not when measuring QT dispersion.

In recent years, percutaneous placement of stents has been used as an alternative to surgery or balloon angioplasty for the treatment of adults with peripheral pulmonary arterial stenosis. This therapy has also been proposed for children, but questions still remain about its indications in this group of patients. We describe here the results of intravascular placement of stents in a group of 29 patients, with a mean age of 12±7 (range 3–31) years and weighing 35 ±19 (range 11–74) kg. All were affected by postsurgical or congenital isolated pulmonary arterial stenosis, and have now been followed for 38 ±19 (range 6–65) months. The early hemodynamic results have been excellent, with a significant reduction of the pulmonary arterial systolic pressure, the systolic pressure gradient, and the ratio of systolic pressures in the pulmonary and systemic circuits, and with a significant increase of the diameter of the stented vessels in all the patients. Of the 29 patients, 24 have been recatheterized 18 ± 10 months after the procedure, demonstrating the stability of the results, with a low incidence of late restenosis, this seen in only 1 patient (2°). Lung perfusion scanning, performed in 17 patients each year after the follow-up catheterization, has showed that the results are maintained at long-term follow-up (51 ±9 months).

Double outlet left ventricle is an extremely rare anomaly. Until recently, the diagnosis was usually established by angiography or at postmortem. There are only a few reports describing the echocar-diographic findings in this lesion, and as far as we know, no report showing the anatomy as well as the velocity and pattern of flow by color Doppler echocardiography. The patient reported here underwent surgery at the age of four years, when an aortic homograft was placed between the right ventricle and the pulmonary trunk. This biventricular repair had to be changed into a Fontan-type procedure, 15 years later since the hypoplastic right ventricle did not grow adequately.

Isomerism of the atriums has often been described with various complex cardiac malformations. As far as is known, a case of ‘ventricular isomerism’ has never been recorded. Described is a specimen where, on the basis of morphological criterions, there are two right ventricles.

We describe a complex case of obstruction of the left ventricular outflow tract in one of Dr. Noonan's original patients. Intraoperative findings revealed pathology at the valvar, subvalvar and supravalvar positions. Patients with Noonan syndrome are traditionally described as having right-sided cardiac pathology. Review of the literature revealed left-sided lesions to occur in a substantial number of these patients. We therefore suggest the routine employment of cardiac ultrasonography in all patients with Noonan syndrome with attention directed toward left-sided pathology, as well as the frequent pulmonary valvar pathology.

Informed medical care, appropriate maternal and fetal monitoring, and cooperation among experienced obstetricians, anesthesists cardiologists, particularly during delivery and the early puerperium associated with psychological preparation of the mother, were the bases of the successful management of a pregnant 28-year-old woman at high risk because of the Eisenmenger reaction.

Described are the clinical, cross-sectional echocardiographic, electrocardiographic and angiographic findings, together with the results at autopsy, in a 5-month old infant with a common arterial trunk associated with an atrioventricular septal defect. As far as is known, this is only the 13th such case to be reported in the literature. A particularly unusual feature of this case was an intact atrial component of the atrioventricular septal defect.

Over the past decade, the technique of radiofrequency ablation has evolved substantially. Currently, most forms of cardiac arrhythmias seen in children can be treated with good long-term results and low risk of adverse outcome. Curative arrhythmia treatment with this technique, however, is still uncommon in neonates and infants. Reported here is our experience in the management of an 8-week-old with drug-resistant permanent junctional reciprocating tachycardia.

A two-month old male infant with the rare occurrence of double outlet left ventricle, subpulmonary ventricular septal defect and pulmonary hypertension is presented. The infant was managed temporarily with banding of the pulmonary trunk, with a favorable result, and is scheduled for definitive intraventricular repair.

Hypoplastic left heart syndrome is one of the most therapeutically challenging congenital cardiac defects. It accounts for as many as 1.5% of all congenital heart defects, but is responsible for up to one quarter of deaths in neonates with heart disease.1 The management of hypoplastic left heart syndrome is controversial. Two surgical options exist:2,3 the Norwood procedure, is a three stage repair in which the morphologically right ventricle is converted to function as the systemic ventricle. Alternatively, orthotopic transplantation can be performed. Although both surgical options have had improved outcomes, the prognosis for long-term survival is guarded, with a five year survival for either approach reported to be in the region of 50–60%. In this review, I explore the evidence for a genetic etiology for the “classic” hypoplastic left heart syndrome, defined as mitral and/or aortic atresia with hypoplasia of the left ventricular cavity and the other left-sided structures.