We present an unusual case of criss-cross heart with supero-inferior ventricles, double outlet of the right ventricle, restrictive ventricular septal defect, severe infundibular stenosis and “absent” pulmonary valve who underwent successful surgical repair. Complete preoperative diagnosis was established with cross-sectional echocardiography combined with colour Doppler and angiocardiography, which facilitated successful surgical correction.

This study compared anxiety, fears, depression and behavioural problems as occurring in children with congenital heart disease, comparing them with samples of normal children. It further considered the influence of maternal anxiety, as well as analyzing a subgroup of children with cyanotic forms of congenital heart disease to determine if they were at higher risk than acyanotic children for the problems identified.

Tetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiogra phy alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restroration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2–12.5 years after surgery (median 4.9 years, mean 5.9 ± 3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.

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.

We report three cases of pulmonary venous stenosis developing after repair of totally anomalous pulmonary venous connection. In each case, we used steroids as a therapeutic measure following re-operation. In two cases, we were successful in preventing significant re-stenosis. The third patient rapidly developed stenosis (mean pulmonary wedge pressure approximately 20 mmHg at cardiac catheterisation) after stopping steroids. This patient subsequently died after a third operation. The remaining patients continue to do well.

We describe a recipient of an orthotopic cardiac transplant who developed severe ventricular dysfunction following an episode of sepsis. He had normal cardiac biopsies and responded to treatment with thiamine. Causes other than rejection should be considered in patients who have received a cardiac transplant and who present in cardiac failure.

Muscular ventricular septal defects were diagnosed by echocardiography in 97 neonates within 7 days of birth. In 82 of the neonates (84.5%), the defect was solitary, while 15 had multiple defects. The solitary defects was located at mid-septal, apical, anterior and inlet locations in 42 (51.2%), 21 (25.6%), 14 (17.1%) and 5 (6.1%) neonates, respectively. Multiple defects occurred in the apical, anterior and mid-septal areas. The diameter of the solitary defects ranged from 1 to 6 mm (2.3 ± 0.8 mm), while the multiple lesions were 1 to 4 mm in diameter (2.1 2.3 ± 0.8 mm 0.8 mm) in 28 instances in which they could measured. It proved possible to follow 79 of the patients for period of 10 to 13 months. The defects closed spontaneously in 56 (84.8%) of 66 patients with a single defect, and in 7 (53.8%) of 13 of those with multiple defects (P<0.05). For the solitary defects, the position and size were factors determining the likelihood and speed of closure. Defects located at the apical septum, or defects larger than 4 mm in diameter, closed slowly and at a later stage. Echocardiography is an useful technique in establishing of natural history of muscular ventricular septal defects encountered in neonates.

We report two cases with persistence of the hepatic venous complex, underdevelopment of the inferior caval vein and azygos continuation to the superior caval vein. We suggest that the hepatic venous complex is a true congenital malformation rather than the result of passive enlargement of venous channels subsequent to high venous pressures. Preoperative diagnosis is important in patients with complex congenital heart disease undergoing palliative venous redirection procedures if a postoperative right-to-left shunt and cyanosis are to be avoided.

Pericardiocentesis is usually an easy and uncomplicated procedure when guided by cross-sectional echocardiography, but an abnormal intracardiac or extrapericardial position of the puncture system can occur, especially in children. Injection of echo contrast medium through the puncture needle is a very sensitive, quick, easy and harmless procedure which can be performed at the bedside in all cases in which doubt remains concerning the location of the needle. Prompt enhancement of signal intensity of the peri-cardial fluid validates the correct intrapericardial position, and permits visualization of the tip of the needle. Absence of echodense formations excludes an intrapericardial position. We recommend the use of echo contrast medium during pericardiocentesis whenever blood is aspirated through the needle, or if there is any doubt concerning its location.

Formation of thrombus in the heart is a rare event in children. Genetic risk factors determined for thrombophilia with additional exogenous risk factors may, none the less, promote cardiac thrombosis. We report on a boy without predisposing cardiac disease, heterozygous for resistance to activated protein C, who developed multiple thrombuses attached to the mitral valve which led to recurrent arterial throm boembolism. In addition this patient showed enhanced levels of lipoprotein (a).

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

Either arrangement of the abdominal vessels or the morphology of the bronchial tree are usually used to predict atrial arrangement. We report a patient in whom, in the setting of normal physiology as seen with mirror-imaged venous return, and due to disparity between the arrangement of the abdominal vessels (suggestive of right isomerism) and thoracic arrangement (indicative for left isomerism), atrial arrangement could not be inferred. Direct echocardiographic imaging of both appendages revealed left isomerism.

A 41-day-old boy was transferred to our department with severe congestive heart failure. Digital subtraction counter current aortography, and antegrade aortography, revealed coarctation of a persistent right fifth aortic arch, stenosis of the origin of an aberrant left subclavian artery, and sequestration of the lower lobe of the right lung.

As his heart failure seemed to be caused not only by pressure overload to the left ventricle following coarctation, but also by the volume load associated with the shunting effect of the pulmonary sequestration, we performed transcatheter balloon dilation of the coarctation and the origin of the aberrant subclavian artery, together with embolization of the aberrant pulmonary artery. His heart failure responded dramatically to these procedures.

We report a case of a fetus presenting intermittent ventricular tachycardia during midgestation, whose mother had long QT syndrome. The arrhythmia disappeared spontaneously, and the electrocardiogram of the baby after birth showed prolonged QTc. As far as we are aware, this is the first case in which ventricular tachycardia was documented during fetal life in a baby with long QT syndrome.

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.

In three children with coronary arterial fistulas, two extending from the right coronary artery to the right atrium and one from the left circumflex coronary artery to the right ventricle, multiplanar cine magnetic resonance imaging was able accurately to demonstrate dilation of the involved coronary artery, the tortuous nature of the dilated fistulas, blood flow within the fistulas, and luminal narrowing. The technique provides a new adjunct for management of patients, especially young children, with coronary arterial fistulas.

A 10-year-old girl required replacement of the aortic valve because of massive regurgitation caused by Kawasaki disease at the age of 3 months. Postoperatively, left ventricular function and mass improved remarkably, with reduction of both hypertrophy and interstitial fibrosis. During a follow-up period of 10 years, she has been free from any cardiac event.