In recent years, post-operative intensive care of the child with congenital cardiac disease has placed an emphasis on earlier weaning from mechanical ventilation. We describe our experience of postoperative fast-tracking of children undergoing cardiac surgery during a charitable mission in Venezuela, where resources and equipment were severely limited. During our stay, 11 children, with a median age of 2 years, underwent total correction of tetralogy of Fallot. The median duration of ventilation was 2.5 hours, and all patients were extubated within 12 hours of surgery. Effective analgesia was achieved without the need for continuous intravenous infusions of opiates. This experience shows that early extubation can safely be carried out in well-selected patients after surgery to correct congenital cardiac malformations. This allows faster throughput of patients, and helps provide an efficient and cost-effective service.

In this review, we describe the experience from 13 European centres using the CardioSEAL and Starflex double umbrella devices to close interatrial communications within the oval fossa (so-called ‘stcundum’ defects). Between October 1996 and April 1999, the procedure was attempted in 334 patients with a mean age of 12 years and a mean weight of 44kg. The mean measured stretched diameter of the defect was 15 mm. In the overall group, the defect was solitary in 245 patients (73%), multiple in 21 (6%), associated with an aneurysm of the flap valve in 15 (5%), was represented by patency of the oval foramen in 44 (13%), and was a fenestration in a Fontan repair in 9 (3%). In all patients, the devices were inserted under general anesthesia, using fluoroscopic and transesophageal echocardiographic control. Implantation was achieved in 325 (97,3%). The device embolized within either a few minutes or a few hours in 13 patients (4%). Of these, uncomplicated surgical repair was undertaken in 10, while the device was retrieved in 3 using catheters and a second device was successfully implanted. Residual shunting was detected immediately after the procedure in 41% of the patients, with the incidence decreasing to 31% at discharge, 24% at 1 month, 21% at 6 months, and 20.5% at one year. During the period of follow-up, elective surgical repair became necessary in two patients, due to malposition of the device in one, and late embolization in the other. Fractures of arms were seen in 6.1%, most commonly with the largest devices. All those with fractured arms of the device were asymptomatic, and no clinical complications related to the fractures were observed. There were no arrythmias, endocarditis, valvar distortion, thromboembolic events, or other complications. After one year of follow-up, clinical success, defined as complete closure of the defect or presence of only a trivial leak, had been obtained in 92.5% of the patients. We conclude, therefore, that these devices produce excellent results when used to close defects of small to moderate size. Results are less than optimal, or else complications ensure, when attempts are made to close very large defects.

Intermittent functional closure of a patent duct has been reported both clinically and echocardio-graphically. We describe the case of a 6-year-old with intermittent complete closure of a patent duct occurring during attempted transcatheter closure. Recurrence of clinical and echocardiographic signs prompted restudy. A patent duct of moderate size was demonstrated and successfully occluded by coil embolization. The interventional aspects of this unusual condition are discussed.

Intrapericardial teratoma is a rare but recognised cause of respiratory distress in neonates. Patients often present with the compressive effects of the mass within the thorax. Prompt diagnosis should be followed swiftly by surgical resection. We report an unusual case of intrapericardial teratoma in a neonate presenting with collapse of the lung which was successfully treated by surgery.

Severe mechanical haemolysis occurred in an 11-month-old boy after implantation of the new Amplatzer duct occluder. Temporary balloon occlusion of the aortic ampulla was performed 4 days after the initial procedure leading to prompt abolition of the haemolysis.

Aortic dissection is one of several complications that can be seen late after repair of aortic coarctation. We report an unusual presentation of this complication, and review the pertinent literature.

We present an example of corrected transposition with twisted discordant atrioventricular connections in which both fetal and postanatal echocardiograms were obtained. We correlate the fetal echocardiograms with the postanatal echocardiograms, placing emphasis on the echocardiographic clues to the diagnosis which were present in fetal life. The diagnosis should be suspected when the cardiac chambers and greatarteries show an unexpected spatial relationship for the given segmental connections, and when the axes of opening of the atrioventricular valves are not parallel.

We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.

We describe a profoundly retarded infant girl with multiple anomalies caused by trisomy 13. Due to heart failure, which was resistant to medical treatment, we closed successfully a ventricular septal defect at three months of age. She died at 10 months of age. Despite the short survival, we believe that the patient benefitted significantly from the surgical repair of her cardiac defect.

We report our experience using the buttoned device to close defects within the oval fossa and probe-patent oval foramens, comparing the findings with those obtained with the Amplatzer septal occluder. from 1992 to 1997, we used the buttoned device to close defects in 73 consecutive patients, 64 with defects in the oval fossa and nine with patent foramens. We compared this experience with a further series of 62 patients seen from 1997 to 1999 in whom the Amplatzer septal occluder was used. Successful implantation was achieved in three-quarters of those with septal defects in whom the buttoned device was used, in all of those in whom the buttoned device was used for patent foramens, and in nine-tenths of those in whom closure was attempted using the Amplatzer occluder. Immediate surgery was needed in 3 patients in whom a buttoned device was used, one because of embolization and two with residual shunts and a straddling device. Similar immediate surgery was needed to retrieve one embolized Amplatzer occluder. During follow-up, surgery was needed in a further 7 patients, all having had insertion of a buttoned device, because of atrial perforation in one and a significant residual shunt in the remainder. At late follow-up, the rate of complete occlusion was 69% in the patients in whom the buttoned device was used to close a septal defect, 100% when the buttoned device was used for patent foramens, and 95% in those treated with the Amplatzer occluder. Our experience shows that the Amplatzer occluder produced a significantly higher rate of occlusion for larger defects, and with a shorter fluoroscopy time than the buttoned device. The Amplatzer septal occluder, therefore, is our preferred device for closure of defects within the oval fossa.

We discovered supraventricular tachycardia with advanced hydrops in the setting of normal cardiac anatomy at 26 weeks of gestation which resolved successfully following administration of digoxin and flecainide to the mother. A female baby was born after a premature rupture of the membranes at 30.6 weeks. The neonate was in sinus rhythm, showed a progressive regression of right ventricular insufficiency, but developed signs of acquired pulmonary valvar and subvalvar stenosis at 2 months of age.

We describe our treatment of a premature baby born weighing 1400 g with severe aortic stenosis, with a gradient of 80 mmHg across the valve. Efforts to advance a 6 mm angioplasty catheter into the stenotic aortic valve via the left ventricle failed. Anterograde angioplasty, instead, was performed using two 4 mm coronary angioplasty catheters. Six months subsequent to the intervention, the pressure gradient measured 25 mmHg, and there was no hemodynamically significant aortic insufficiency.

We describe a rare instance of isolated pulmonary regurgitation caused by a dysplastic pulmonary valve which was detected prenatally. Fetal echocardiography demonstrated severe pulmonary regurgitation, and progressive cardiomegaly because of right ventricular volume overload. After birth, conservative therapy was successful in alleviating the pulmonary vascular resistance, and the pulmonary regurgitation gradually decreased.

We describe an unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant. The infant is asymptomatic 18 months after the second surgery. The very rare cardiac involvement, and the early relapse of the inflammatory pseudotumor, to the best of our knowledge, is a unique combination.

The inflammatory myofibroblastic tumor, as known as a pseudotumor or plasma cell granuloma is an uncommon reactive lesion with unknown aetiology. It is found most commonly in the lung and a number of visceral organs, such as the spleen, liver, ileum, salivary glands, urinary bladder, larynx or brain or in the retroperitoneum and lymph nodes. To our knowledge only 9 cases have been published of such tumor arising within the heart.1–6

We present a case of tetralogy of Fallot associated with Scimitar syndrome. The patient was an 11-month old female who underwent successfully total repair of her lesion, including rerouting of the anomalous pulmonary vein to the left atrium. The diagnosis was suspected from the chest x-ray and echocardiography, and confirmed by angiography. To the best of our knowledge only 2 additional cases have previously been reported.

Injury to the spinal cord injury with paraplegia, is a rare complication of surgical repair of aortic coarctation recognized immediately post-operatively. We present the case of a 41-year-old male undergoing surgery for restenosis at the site of a repair. Intra-operatively, he suffered inadvertent injury to an intercostal arterial branch during isolation of the aorta below the graft. Over the following months, he developed unusual symptoms involving the legs and genitourinary tract which, only after extensive investigations, were attributed to ischemic damage to the spinal cord related to the surgery. We suspect that similar syndromes reflecting injury to the spinal cord injury may be unrecognized following surgical repair of coarctation.

An infant developed severe desaturation within a few hours of construction of a modified Blalock-Taussig shunt. Echocardiography revealed that the shunt had become occluded, and this was confirmed angiographically. At catheterisation, therefore, we passed a 0.014”percutaneous transluminal coronary angioplasty wire through the occluded shunt into the right pulmonary artery and then dilated the shunt successfully using a 5 mm coronary angioplasty balloon. Six weeks later, the shunt remained patent.

An aortic valve with four leaflets is a rare congenital malformation. It is usually diagnosed unexpectedly at autopsy, during replacement of the aortic valve, or by aortic angiography. Transthoracic cross-sectional echocardiography, however, can also delineate the structure of the aortic valve, and demonstrate the presence of valves with four leaflets

We describe a neonate with critical coarctation of the aorta. Since treatment with Prostaglandin El had failed to reopen the arterial duct, and surgery was deemed to be associated with an unacceptably high risk in this unstable neonate, it was decided to perform balloon dilation of the coarctation as a palliative procedure to stabilize the patient. Balloon angioplasty failed to reduce the pressure gradient across the coarctation, so a stent was implanted retrogradely as an emergency procedure. Subsequently, the stented segment was resected surgically and end-to-end anastomosis created without complications at seven months of age.