Abstract Hepatic venous blood has been thought to play some role as a vasoactive agent in the development of pulmonary arteriovenous fistulas in patients with congenital heart disease. During the last 15 years, we have observed pulmonary arteriovenous fistulas in 3, and systemic arteriovenous fistulas in 2, patients from our 16 cases of left isomerism. During the same period, neither pulmonary nor systemic arteriovenous fistulas were detected among 50 patients with right isomerism. Pulmonary arteriovenous fistulas had developed in the absence of surgery in 1 of the patients. Both pulmonary and systemic fistulas were detected in an another patient, in whom the hepatic venous blood bypassed the pulmonary circulation. The level of somatostatin, which is known to reduce splanchnic blood flow, was high in the systemic venous blood of this patient. Although the mechanism of development of the fistulas has yet to be clarified, we should be aware that not only pulmonary, but also systemic arteriovenous fistulas can be found in patients with left isomerism, even prior to any surgical intervention.

Device closure of oval fossa atrial scptal defects with the Amplatzer Septal Occluder was performed in 26 patients ranging in age from 0.89 to 60.44 years. In eight additional patients no device implant was performed because of the presence of multiple defects or because the defect was of a size unsuitable for closure with the devices currently available. The strectched diameter of the defects that were closed ranged from 4 to 23 mm (mean 14±5.4 mm) and device sizes ranged from 4 to 24 mm. Two devices were unstable, of which one embolized to the right atrium after release. Both devices were retrived at the same procedure. One of these parients subsequently underwent a successful device closure of his defect using a larger (24-mm) device. Three patients had multiple defects, which were successfully closed with a single device. At 1-month follow-up 23/26 (88%) and at 3-month follow-up 22/24 (92%) patients had complete closure of their defects, while two had residual shunts. One further patient who had complete closure of his defect at 1-month post-implant had his device removed and his atrial septal defect patched surgically 8 weeks after device closure. This was done as a result of the development of a vegetation affecting the device after an episode of septicaemia, which was not relate to the cardiac problems. There was no procedure-related morbidty or martality and all patients remain well at the present time.

Advances in diagnosis, medical management and surgical intervention have improved the longevity and quality of life for children with congenital heart disease. Despite this, research studies specifically examining the psychosocial concerns of adolescents and young adults with congenital heart disease are few. To explore the subjective experiences and dilemmas of this population during the transition from adolescence to young adulthood, we interviewed, using a semi-structured protocol, a convenience sample of nine adolescents and young adults. Using analytic procedures inherent in Grounded Theory methodology, seven themes were identified: the dilemma of normality; dilemmas in disclosure; dilemmas in strategies for management of illness; the challenge of social integration versus social isolation; the challenge of dependence versus independence; the challenge of uncertainty; and strategies for coping. An understanding of these experiences by health professionals can be beneficial in helping this clinical population as they grow up and face the challenges of an uncertain, yet promising, future.

We sought to evaluate the saftey and efficacy of acure and chronic treatment with verapamil infanta with hypertrophic cardimyopathy. Prior studies have shown an improvement in adluts with hypertrophiuc cardiomyophathy who were treated with verapamil. Acutely, it reduced the degree of left ventricular outflow tract abstruction. Chronic therapy was associated with an improvement in sysmptoms ans incresed long-term survial. To date, no data are available on the efficacy of this drug in infants with hypertrophic cardiomyopathy. We evaluated prospectively the safety and efficacy of verapamil in infants. The acute and chronic effects of verapamil on infants with an echocardiographic diagnosis of hypertrophic cardiomyopathy were evaluated at a single institution between 1980 and 1994, with long-term follow-up available until 1996. Acute effects of an intravenous bolus of 0.1 mg/kg and infusion at 0.007 mg/kg/min were evaluated, where possible, in the cardiac catheterization laboratory. Oral verapamil at 3–5 mg/kg/day was started after catheterization. Follow-up included serial clinical, echocardiographic and Holter monitoring. A total of 22 patients were studied, 17 having a presumed diagnosis of primary hypertrophic cardiomyopathy including three with Noonan's syndrome. Acute infusion of the drug was well tolerated by all, without adverse electrophysiological effects. Haemodynamic effects were consistent with a negative inotropic action with significant falls in cardiac index (4.6±1.2 to 4.1±0.9 l/min/m2), systolic blood pressure (88±16 to 82±14 mmHg) and gradient across the left ventricular outflow tract (nine patients 48.2±30.4 to 28.4±24.1 mmHg). End-diastolic pressure was unchanged (14.0±6.8 to 13.9±4.7 mmHg). Three patients with primary hypertrophic cardiomyopathy died (two while being treated). In the group with primary hypertrophic cardiomyopathy continuing with long-term treatment, follow-up revealed regression in two, progression in three (two died) and stability in 10. For those treated, there was a trend towards improvement in clinical status. Verapamil is well tolerated acutely in infants with hypertrophic cardiomyopathy. The outcome was considerably better in these patients compared with prior reports, though careful long-term assessment is needed.

To evaluate the influence of the size of the defect and the age of surgical repair on left ventricular mechanics, including geometry, shape, diastolic and systolic function as well as myocardial contractility, we used cross-sectional echo-Doppler to study 20 patients (12 males, 8 females) who had undergone successful surgical closure of a ventricular septal defect. The patients were divided in two groups, corrected early and late, on the basis of the degree of left-to-right shunting (ratio of pulmonary to systemic output of greater or less than 2.5/1) and the age at the surgical repair (older or younger than 2 years of age). The group undergoing early correction included 11 patients, mean age 7.1 ± 1.8 years (range 4.2–11.8 years) having surgery at mean age of 1.3±0.6 years for a large ventricular septal defect (mean ratio of pulmonary to systemic output of 3.1/1; range 3.4–2.7/1) with a mean postoperative follow-up 4.6±1.9 years. The group of nine patients undergoing late correction had a mean age of 11.3±4.9 years (range 6.7–17.2 years), with a later surgical repair (mean age 4.7±2.7 years) for a moderate-sized ventricular septal defect (mean pulmonary/systemic output ratio 2.1/1; range 2.3–1.7) and a mean postoperative follow-up of 7±4.2 years. Each group of surgically repaired patients was compared with a control group matched for age, body surface area and gender. No significant differences were found between the normal controls and those undergoing early correction for any assessed functional index regarding left ventricular geometry (normalized volumes and mass for body surface area, mass/volume and thickness/radius ratios), shape (long axis–short axis ratio), diastolic (mitral and pulmonary venous flow patterns) and systolic (fractional shortening and rate-corrected mean velocity of circumferential fibre shortening) function. In addition, the data points for each patient for the rate-corrected mean velocity of circumferential fibre shortening to end-systolic stress relationship were within the 95% confidence limits of normal, suggesting normal left ventricular contractility. On the other hand, the patients undergoing surgery at a later age showed a persistent increase of the normalized left ventricular end-diastolic volume and mass, with an higher mass/volume ratio and reduced end-systolic stress compared with normal controls. Furthermore, left ventricular shape (long axis–short axis ratio) was abnormal at end-diastole but with its normal values at end-systole. Our data suggest that, in the presence of a large ventricular septal defect, early successful surgical repair <2 years of age results in complete recovery of left ventricular mechanics in the postoperative follow-up. In ntrast, surgical closure at >2 years of age, even for a moderately sized ventricular septal defect, deleteriously affects postoperative left ventricular geometry and shape. Since prolonged volume overload may be detrimental to myocardial function, earlier surgical repair should be recommended.

Malta is a small island with minimal changes in its population, making it an ideal location for epidemiological and historical studies dealing with congenital heart malformations. Ventricular septal defect was studied retrospectively from 1930 to 1994. A sharp and significant decline in age at diagnosis was found, predating echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence of this lesion as seen at birth, particularly of minor defects. The prevalence of ventricular septal defect from 1990 to 1994 was 3.85/1000 live births, with 3.O3/1OOO not needing surgery and 0.83/1000 requiring operative intervention. Half the defects closed spontaneously. The majority of ventricular septal defects overall, and those spontaneously closing, were muscular defects. The overall prevalence at birth was significantly higher than that reported in recent studies using similar methodologies, implying that the reported rate is more likely to be the true prevalence at birth of clinically detectable defects.

The Maltese populartion constitutes an ideal location for epidemiological and historical studies dealing with congenital hearr malformations. Pulmonary stenosis was studied retrospectively from 1943 to 1994. A sharp, significant decline in age at diagnosis was found, which predates the introduction of echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence at birth of this lesion, patticularly of pulmonary stenosis not requiring intervention. The prevalence at birth of pulmonary stenosis from 1990 to 1994 was 1.65/1000 live births (95% CL: 1.21–2.24), with 1.11/1000 mild lesions (95% CI: 0.76–1.62) and 0.54/1000 lesions requiring intervention (95% CI: 0.31–0.92). The prevalence at birth overal was significantly higher than that reported in recent studies with similar methodologies due to the higher pickup of milder variants of pulmonary stenosis by echocardiography.Significantly more pulmonary more pulmonary stenosis was found in females than in males.

Isolation of the subclavian artery is an unusual anomaly in which the subclavian artery arises not from the aortic arch but from a pulmonary artery via an arterial duct. Such isolation most often occurs with a right aortic arch, and in lesions frequently associated with a right arc, such as tetralogy of Fallot. Since1994, we have undertaken surgery in four young infants with isolated subclavian arteries and unusual associated anomalies, including one with atrioventricular septal defect and common valvar orifice, two with interruption of a left aortic arch and one with interruption of a right aortic arch. In both patients with interrupted left arch, the isolated subclavian artery was diagnosed preperatively by echocardiography. We emphasize the significant issues.

Cognitive, functional, educational achievement and behavioural measures were employed to assess neurobehavioral status in 57 of 60 participants who were initially enrolled in the Baltimore–Washington Infant Study, and who survived surgical correction of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). Charts were reviewed to investigate the relationship between birth variables, surgical strategy and developmental outcomes. Higher preoperative weight was associated with better outcomes on the Stanford–Biner Short-term Memory subtest, while lower preoperative oxygen tension was associated with better outcomes on the Abstract/Visual Reasoning subtest and a test of Visual–Motor Integration. Longer total bypass time was associated with poor outcomes on the Short-term Memory subtests. Higher average flow rates during cooling and rewarming were associated with higher scores in the test of short term memory but poorer outcomes on a test for visual motor integration. Longer cooling times were associated with higher scores on the test for Visual–Motor Integration. Patients suffering seizures scored lower on the Stanford–Biner Composite, as well as in their tests of achievement. The data indicate that non-verbal. skills may be particularly sensitive to variations in surgical strategies employed to correct complete transposition. Overt neurological events, such as seizures, were related to global deficits in intellectual functioning. Prospective studies evaluating systemic variations in surgical procedures and attempts to prevent and manage perioperative neurological events are important for further investigation of neurodevelopmental outcomes in children surviving surgical correction.

Despite a good haemodynamic result, many children have amildly decreased arterial oxygen saturation following a total cavopulmonary connection. Our study was performed to determine possible mechanisms of right-to-left shunting in these patients. We performed elective cardiac catheterization in 19 children at a mean interval of 3.6 years following a total cavopulmonary connection. The intrapulmonary right-to-left shunt, the intracardiac right-to-left shunt and the total right-to-left shunt were calculated under mechanical ventilation with 100% oxygen. The intrapulmonary right-to-left shunt was 10.8±3.5% of the pulmonary blood flow, and the total right-to left shunt accounted for 18.9±5.2% of the systemic blood flow. The intracardiac right-to-left shunt in patients with no relevant venovenous collaterals or leaks in the atrial tunnel was calculated at 6.4±3.0% of the systemic blood flow, while the intracardiac right-to-left shunt in patients with relevant collaterials or leaks accounted for 13.0±5.9% of the systemic blood flow. Since intrapulmonary arteriovenous fistulas were not demonstrated angiographically in any of our patients, the intrapulmonary right-to-left shunt is probably due to low ratios of perfusion to ventilation in some pulmonary segments. The intracafdiac right-to-left shunt was due to leaks across the interatrial baffle, collaterals between stystemic and pulmonary veins, and to the coronary sinus draining to the pulmonary venous atrium.

The development of pulmonary atteriovenous fistulas after bidirectional cavopuomonary operat-tions, such as the bidirectional Glenn shunt and Kawashima's procedure, has raised concern. Development of these fistulas, which are more frequent than initially thought, can represnt a limiting factor in the late outcome of these patients and may even limit the indication for these of surgery. Whether the fistulas can be reversed by transforming the surgical procedures has yet to be established. In the hope of avoiding this kind of complication, thought to be caused by the lack of passage of a hypothetical hepatic factor through the pulmonary circulation. wedevelped an inverred type of gidirectional cavopulmonary connection in which the blood coming from the liver perfuses immediately both lungs. This is made possi-ble by shuntiong via an intra-atrial tunnel the blood from the superior caval vein directly to the left atrium, and the blood from the inferior caval vein to the right branch of the pulmonary trunk (keeping its bifurcation intact). We describe findings in two patients undergoing successful surgery with this technique. Serial follow -up with cohtrast echocardiography did not show evidence of arteriovenous pulmonary fistulas.Despite our numbers being small, and the time of follow-up being limited, we believe that it is importantto document these and similar cases

Occlusion of the atrial septal defects in the oval fossa by interventional catheterization has progressed, but still has limitations. Three-dimensional (3D) echocardiography can provide unique views unavailable by cross-sectional imaging. The objective of this study was to define the clinical application of 3D echocardiography in the assessment and monitoring of transcatheter occlusion of atrial septal defects. Three-dimensional echocardiography was attempted prior to occlusion of atrial septal defects in 41 patients (median age 8.6 years). Serial cross-sectional images were acquired by multiplane transoesophageal echocardiography and displayed by means of computer reconstruction. Dynamic 3D echocardiographic images of defects in the oval fossa were obtained in 40 of 41 patients (98%). Volume-rendering demonstrated the anterosuperior rim in 36 (90%) and the inferoposterior rim in 24 (60%), but failed to reveal small additional fenestrations in six. Sizes measured by 3D echocardiography were significantly larger than those provided by cross-sectional transoesophageal echocardiography (p =;0.007), but differed little from those obtained with balloon sizing (p =; 0.6). After occlusion, 3D echocardiography showed positions of all arms of the device in 20 of 24 cases. Location of any protruding arms, or residual defects, were also clearly revealed.Three-dimensional images obtained in 12 patients during deployment of the double-umbrella device were useful in monitoring its position (single-frame) and for explaining the mechanism of protrusion. Current 3D echocardiography provides clinically relevant information for selection of patients for closure of atrial septal defects by interventional catheterization and when monitoring during implantation. Information obtained by this technique can clarify the mechanism of deployment of the device and closure of the defect, therefore influencing outcomes.

A patient with repaired tetralogy of Fallot presented with recurrent syncope and had multiple haemodynamically unstable ventricular tachycardias unresponsive to antiarrhythmic medications.Ventricular tachycardias became haemodynamically tolerated with amiodarone, procainamide and dopamine, permitting activation and entrainment mapping. Radiofrequency ablation of three tachycardia circuits was performed. Ventricular tachycardia could not be induced 1 week, and 3 and 9 months later.Radiofrequency ablation is feasible for multiple, haemodynamically unstable ventricular tachycardias in repaired tettralogy of Fallot

A 25-year-old man died 10 years after a Dacron patch was used to repair a coarctation of the aorta. Death was due to rupture of an unrecognized aneurysm at the site of the patch. After the initial operation at the age of 15 years, there had been no signs of residual or recurrent obstruction. He had no evidence of hypertension and was discharged some years later from regular hospital follow-up to the care of his general practitioner. We strongly recommend that patients who have undergone repair of aortic coarctation by patch aortoplasty should have lifelong follow-up in cardiac units with imaging facilities for monitoring aortic dilation. We would now recommend surgical intervention in the presence of progressive aortic dilation.

A male infant, aged 2 month, with Kawasaki disease had a myocardial infarction despite intravenous infusions of gamma globulin and aspirin at high dosage. He developed progressively a thin walled, dilated aneurysm of the apex of the left ventricle which became lined with thrombus despite treatment with warfarin. Another boy, aged 6 years, was noted on the 10th day of the evolution of Kawasaki disease to have developed a giant aneurysm of the main stem of the left coronary artery. Despite infusion of gamma globulin, the aneurysm remained unaltered and developed a thrombus. The thrombus resolved following treatment with warfarin, though the giant aneurysm has persisted. These two cases illustrate the serious consequences that can follow Kawasaki disease despite management optimal by current standards.