[Advances in the surgical treatment of hypoplastic left-heart syndrome]. / Ontwikkelingen in de chirurgische behandeling van het hypoplastisch linkerhartsyndroom.

Hypoplastic left-heart syndrome is a congenital heart malformation that is classified as a univentricular disorder (hearts with only one functioning ventricle). Treatment was first developed in 1977, but a marked improvement in survival was not seen until the last decade. This was due primarily to a better understanding of how to surgically and medically manipulate the balance between pulmonary and systemic circulation. Special attention should be given to cerebral protection before, during and after surgery and to the follow-up of neurocognitive behaviour.

[Results of extracorporeal membrane oxygenation following cardiac surgery in 16 children in the St Radboud University Medical Centre, 1996-2004]. / Resultaten van extracorporele membraanoxygenatie na een hartoperatie bij 16 kinderen in het Universitair Medisch Centrum St Radboud, 1996-2004.

OBJECTIVE: Description of the results with extracorporeal membrane oxygenation (ECMO) in infants with cardiorespiratory failure after open-heart surgery. DESIGN: Prospective observational study. METHOD: Data on the indications, survival, and complications were collected from all infants treated with ECMO for cardiorespiratory failure after open-heart surgery in the period 1996-2004 in the St Radboud University Medical Centre in Nijmegen, the Netherlands. RESULTS: 17 ECMO treatments were carried out in 16 paediatric patients: 7 girls and 9 boys. All patients had severe circulatory failure, sometimes combined with respiratory insufficiency. The patients' average age was 8 months (range: 1 day-3 years and 10 months). The average duration of treatment was 137 hours (range: 8-433). In 12 of the 17 ECMO treatments, the patient was successfully weaned from ECMO and 7 patients were discharged home. 5 patients died in hospital after successful ECMO treatment. 10 of the 16 patients died. A functional univentricular heart was associated with a high mortality. Haemorrhage, renal insufficiency and; sepsis were the most frequent complications. Major causes of death were sepsis, multi-organ failure and pulmonary hypertension.

Giant rhabdomyoma of the right ventricle.

A giant intrathoracic mass causing foetal dysrhythmias, polyhydramnios and foetal hydrops necessitated a caesarean section in a male infant of 35 weeks gestation. Despite the benign histology of cardiac rhabdomyomas and the observation of spontaneous regression, there may be significant associated morbidity and mortality, especially in neonates. There is a high incidence of associated tuberous sclerosis.

Report of the Dutch experience with the Ross procedure in 343 patients.

OBJECTIVE: Limited information is available on outcome after autograft aortic valve replacement, in particular with respect to the durability of the autograft and of the allograft used to reconstruct the right ventricular outflow tract. A retrospective follow-up study of all patients who underwent a Ross procedure in the Netherlands since 1988 was done to obtain an overview of the Dutch experience with this procedure. METHODS: From 1988 to January 2000, 348 Ross procedures were performed in nine centers in the Netherlands. Pre-operative, peri-operative and follow-up data from 343 patients in seven centers (99% of all Dutch autograft patients) were collected and analyzed. RESULTS: Mean patient age was 26 years (SD 14, range 0-58) and male/female ratio was 2.1. Bicuspid valve or other congenital heart valve disease was the most common indication for operation. The root replacement technique was used in 95% of patients and concomitant procedures were done in 12%. Hospital mortality was 2.6% (N=9). Mean follow-up was 4 years (median 3.8, SD 2.8, range 0-12.5). Overall cumulative survival was 96% at 1 year (95% confidence interval (CI) 94-98%) and 94% at 5 and 7 post-operative years, respectively (95% CI 91-97%). At last follow-up, 87% of the surviving patients was in New York Heart Association (NYHA) class I. Independent predictors of overall mortality were pre-operative NYHA class IV/V and longer perfusion time. Autograft reoperation had to be performed in 14 patients and reintervention on the pulmonary allograft in 10 patients. Freedom from any valve-related reintervention was 88% at 7 years (95% CI 81-94%). CONCLUSIONS: The Dutch experience with the Ross procedure is favorable, with low operative mortality and good mid-term results. Although both the autograft in aortic position and the allograft in the right ventricular outflow tract have a limited durability, this has not yet resulted in considerable reoperation rates and associated morbidity and mortality.

Difficulties generated by the small, persistently patent, arterial duct.

Over recent years, echo-Doppler cardiography has shown that a small, sometimes silent, arterial duct exists in more patients than previously recognized. To know the incidence of an arterial duct subsequent to therapy, we studied retrospectively our patients undergoing open-heart surgery and surgical or catheter closure. Three groups of patients were studied: those with patency of the duct subsequent to open heart surgery without any sign of patency before or during surgery, those with persistent duct after surgical ligation and those with persistent patency after attempted catheter occlusion with the Rashkind device. In the first group (of 431 children) four (0.9%) had persistence of this duct, of which three were silent. In the second group, patency persisted in four of 100 patients (4%), three being silent. In the last group there were five persisting shunts, three producing no murmur, in 30 patients (17%). We compared our results with those reported in the literature and conclude that echo-Doppler cardiography is needed to detect persistent shunting across a duct after therapy, since most of the residual ducts in this study were silent. This means that clinical findings alone cannot be relied upon, and careful echo-Doppler cardiography is essential. Also, the process of closure of a persistent duct by surgical ligation or transcatheter intervention is no guarantee of success. The risk of infective endocarditis is important in such persistent ducts and, at present, it is unknown either for a small, silent duct or in a persistent duct that remains open after attempted transcatheter closure, but now is in association with a foreign body.

Surgical treatment of a fistula between the right pulmonary artery and the left atrium: presentation of two cases and review of literature.

OBJECTIVE: A direct communication between the pulmonary artery and the left atrium is a rare anomaly. On the basis of two cases of our own and a literature review of 49 cases, we focus on clinical presentation, anatomy, diagnosis, and the role of surgery. METHODS: Two cases of a fistula between the right pulmonary artery and the left atrium are described in a girl of 4 years and a boy of 15 years. Both presented with unexplained cyanosis. Diagnosis was made on echocardiography and angiography. The fistula was ligated using extracorporeal circulation in the first case and not in the second case. RESULTS: The surgical results were successful with resolution of the cyanosis. CONCLUSIONS: In newborns, urgent surgery may be necessary. In other patients, early elective surgical correction should be performed to prevent complications, especially systemic and cerebral emboli, cerebral abscesses, and rupture of aneurysmal fistulas. Complete cure can be achieved by ligation and possible division or by intracardiac repair.

[Chronic central cyanosis in children; always an indication for further diagnosis]. / Chronische centrale cyanose bij kinderen; altijd een reden voor nadere diagnostiek.

A girl and a boy, both aged 4 years, had displayed a blue discolouration of the skin for several years. In the girl, electrocardiography and roentgenography of the chest revealed no abnormalities; in the boy, the cardiac murmur was attributed to an insignificant ventricular septal defect. Further examinations were performed only when the children developed sleeping problems and decrease of exercise tolerance, respectively. In both, a right-left shunt was discovered caused by a direct communication between the right pulmonary artery and the left atrium, and tetralogy of Fallot, respectively. Both patients' condition improved after operation. Chronic central cyanosis in a child constitutes an indication for consultation of a paediatric cardiologist.

Coarctation developing after arterial switch repair for transposition of the great arteries.

We describe 2 patients with simple transposition of the great arteries in whom coarctation of the aorta developed after uncomplicated arterial switch operation. Both patients showed no symptoms or signs of this coarctation at the time of arterial switch operation.

Transesophageal echocardiography in pediatric patients: preliminary results.

Transesophageal echocardiography was attempted in 59 pediatric patients with congenital heart disease in the operating room (n = 33) or during heart catheterization or in the intensive care unit (n = 26). Six different commercially available transducers were used with diameters ranging from 7 to 15 mm. Age ranged from 1 day to 16 years, and body weight ranged from 3.7 to 65 kg. Objectives of the study were to determine (1) minimum body weight in which transesophageal echocardiography with various probes is possible, (2) additional diagnostic value, and (3) potential applications. In three cases (one surgical and two nonsurgical) the probe could not be inserted. Minimum body weight was 17 to 20 kg for probes with a diameter of greater than or equal to 13 mm and approximately 12 kg for the 11 mm probes. A 7 mm probe, on the other hand, could be inserted easily in all patients (including a neonate) in whom transesophageal echocardiography was attempted. In 11 of 56 patients, additional diagnostic information was obtained. Thus, transesophageal echocardiography is feasible in the pediatric age group provided that special probes are used in small children. Additional diagnostic information can be obtained, and the technique is of value during cardiac surgery or balloon interventions for evaluation of the efficacy of the procedure and for monitoring ventricular function.

Anomalous origin of the left coronary artery from the pulmonary artery: repair by aortic reimplantation.

From 1980 to 1990, 12 patients (mean age 2.5 years, range 5 months to 9 years) with anomalous origin of the left coronary artery from the pulmonary artery were treated surgically. Five infants were operated upon in the first year of life because of persistent symptoms of congestive heart failure. In all cases, a two-coronary system was constructed by direct aortic reimplantation of the anomalous vessel with no deaths early or late over a follow-up period of up to 10 years. The technique of reimplantation was facilitated by transection of the main pulmonary artery. One patient with severe mitral regurgitation underwent, in addition, a mitral annuloplasty. A pulmonary valvotomy was performed in another patient with associated pulmonary stenosis. Three patients are receiving medication. The reimplanted anomalous left coronary artery was patent in each reevaluated patient (10/12). Left ventricular function improved considerably in all cases. Patients with symptoms should undergo repair soon after diagnosis. Direct aortic reimplantation should be technically feasible in even the smallest infant. Operative mortality is related to preoperative conditions and severity of ischemic damage of the myocardium.

An asymmetric bulging of the chest as a leading sign of a cardiac fibroma in a child.

A 17-month-old child with an asymmetrical bulging of the chest due to an intramural fibroma of the heart is described. The clinical symptoms that may be caused by cardiac tumours are discussed. To detect these tumours several investigations can be performed, of which echocardiography is the most specific. Surgical resection of these tumours should be attempted, even if a complete resection is not possible.

An infant with the scimitar syndrome and pulmonary hypertension; successful surgical intervention.

The case of an infant with the scimitar syndrome and pulmonary hypertension is presented. After surgical intervention with ligation of the abnormal arterial supply to the hypoplastic lung, the pulmonary hypertension disappeared. This was proven by cardiac catheterization after the operation. The abnormal drainage from the right lung into the inferior vena cava was not corrected. The child has had no complaints since except for recurrent pulmonary infections.

End-tidal carbon dioxide concentration as an indicator of pulmonary blood flow during closed heart surgery in children. A report of two cases.

The value of the continuous monitoring of end-tidal carbon dioxide concentration as an indicator of the acute intraoperative interruption of pulmonary blood flow, in patients with pre-existing restrictions of pulmonary blood flow undergoing closed heart surgery, is illustrated by two case reports. Decreases in end-tidal carbon dioxide concentration often appeared several minutes before the changes in systemic haemodynamics.

Ventricular exclusion during Fontan operation: an evolving technique.

The modified Fontan operation, which consists of ventricular exclusion by obliteration of the right atrioventricular ostium, requires a technique that provides firm and permanent occlusion while preserving the atrioventricular node and bundle. Ten patients underwent such an operation. In 5 of them, closure of the right atrioventricular ostium was achieved by suturing a patch into the valve leaflets; in the other 5, the ventricle was excluded by a patch sutured into the right atrial wall above the annulus fibrosus. None of the patients who underwent this procedure experienced heart block postoperatively. However, three instances of late patch dehiscence occurred, each in the group of patients in whom the patch had been attached to the valve leaflets. We conclude that both patching techniques are safe as far as the atrioventricular node and bundle are concerned but that permanent occlusion is better obtained by implanting the patch into the atrial wall. Therefore, we prefer to use this technique.

2D echocardiographic diagnosis of total anomalous pulmonary venous connection of the infradiaphragmatic type.

In two newborn infants with infradiaphragmatic total anomalous pulmonary venous connection the common pulmonary vein was visualized with 2D echocardiography. The transducer was placed in the subcostal region so as to image the descending aorta and the vertebral column. Apart from the aorta and the inferior vena cava a third vessel was identified, lying anterior to the aorta, the proximal part lying behind the left atrium, the distal part curving anterior in the liver, the distal end being distended. These findings are highly characteristic and diagnostic for total anomalous pulmonary venous connection of the infradiaphragmatic type.

Early extubation of the trachea after open heart surgery for congenital heart disease. A review of 3 years' experience.

The results of early extubation after open heart surgery for congenital heart disease in 209 consecutive patients have been reviewed. No patient younger than 3 months of age, 52% of those between 3 and 12 months, and 88% of those older than 12 months had the tracheal tube removed in the operating theatre. Four patients required reintubation of the trachea, three because of respiratory difficulty and one because of cerebral oedema. There were two deaths in the extubated group. Twelve patients had PaO2 values less than 8.0 kPa after operation, despite adequate oxygen therapy. In four of these, this was related to persistent intracardiac shunting. It is concluded that early extubation after open heart surgery for congenital heart disease has minimal risk in carefully selected patients.

The Fontan procedure in the absence of the interatrial septum. Failure of its principle?

The Fontan principle, defined as a procedure in which the right ventricle is bypassed in order to convey desaturated venous blood from the right atrium to the lungs, is presently applied for a wide variety of congenital heart malformations including those in which there is no suitable ventricular pumping chamber. Recently, the procedure has also been advocated for complex malformations that require atrial septation or intra-atrial rerouting. The present report evaluates our experience in four patients with such complex malformations. Three had a complete form of atrioventricular septal defect with double-outlet right ventricle and one patient had left atrioventricular valve atresia. The common atrium was morphologically right in two patients and morphologically left in one. In each of these instances anomalous pulmonary venous connections were present, together with abnormal systemic venous connections. The results were unsatisfactory. Three of the four patients died. The only survivor had no pulmonary or systemic venous abnormalities. Severe impairment of pulmonary blood flow was one of the most important postoperative complications. The findings suggest that the complexities of rerouting the blood within the atria play an important role. The excess proportion of prosthetic material to atrial myocardium may result in excessive loss of contractile atrial myocardium and, probably equally significant, in a complex intra-atrial geometry of pathways that may cause a critical pressure gradient. The results clearly show that with an expanding horizon of the application of the Fontan principle, new pitfalls may arise which presently appear to be the prevailing factors limiting its success.

Right and left isomerism: the cardiac surgeon's view.

We have treated by palliative or corrective surgery 12 patients with the complex cardiovascular anomalies associated with right isomerism (asplenia syndrome) or left isomerism (polysplenia syndrome). A systemic-pulmonary artery shunt was performed in 4 patients, with 2 early deaths. Anatomical intracardiac repair was successfully done in 2 patients, while an orthoterminal repair using a modified Fontan procedure was attempted in 5 patients, only 1 of whom survived. A 1-year-old girl with diffuse pulmonary arteriovenous fistulas associated with left isomerism was not considered a good candidate for operation after an exploratory sternotomy. The surgical options and technical problems related to the complex intracardiac morphology of these developmental syndromes are presented.