The Diagnostics and Management of Bronchopulmonary Sequestration: An International Survey among Specialized Caregivers.

BACKGROUND: Our objective was to explore the treatment preferences for bronchopulmonary sequestration (BPS) among an international group of specialized caregivers. METHODS: Sixty-three participants from 17 countries completed an online survey concerning the diagnostics, treatment, and follow-up. Recruitment took place among members of the Collaborative Neonatal Network for the first European Congenital Pulmonary Airway Malformation Trial Consortium and through the Association for European Pediatric and Congenital Cardiology working group database. RESULTS: Most of the 63 participants were pediatric surgeons (52%), followed by pediatric pulmonologists (22%), and pediatric cardiologists (19%). The majority (65%) treated more than five cases per year and 52% standardly discussed treatment in a multidisciplinary team. Half of the participants (52%) based the management on the presence of symptoms, versus 32% on the intralobar or extralobar lesion localization. Centers with both surgical and interventional cardiac/radiological facilities (85%) preferred resection to embolization in symptomatic cases (62 vs. 15%). In asymptomatic cases too, resection was preferred over embolization (38 vs. 9%); 32% preferred noninterventional treatment, while 11% varied in preference. These treatment preferences were significantly different between surgeons and nonsurgeons (p < 0.05). Little agreement was observed in the preferred timing of intervention as also for the duration of follow-up. CONCLUSIONS: This survey demonstrates a variation in management strategies of BPS, reflecting different specialist expertise. Most centers treat only a handful of cases per year and follow-up is not standardized. Therefore, management discussion within a multidisciplinary team is recommended. Recording patient data in an international registry for the comparison of management strategies and outcomes could support the development of future guidelines. LEVEL OF EVIDENCE: Level IV.

Indications for stenting of coarctation of the aorta in children under 3 months of age.

INTRODUCTION: Coarctation of the aorta in children under 3 months of age is usually treated surgically. However, there are clinical scenarios in which stenting of native or recurrent coarctation may become necessary in this age group. CASE REPORTS: Four cases illustrate possible indications: left ventricular dysfunction increasing the operative risk, thrombus formation after coarctation surgery, patient size (i.e. in premature babies), and retrograde arch obstruction after hybrid palliation of hypoplastic left heart syndrome. In all babies, coarctation stenting was carried out successfully without complications. CONCLUSION: Coarctation stenting can be carried out safely in small children. Usually, the stent has to be removed or redilated later. Results are encouraging.

Gradient over Melody valve in left AV valve position reduces with beta blockade.

The Melody valve, designed for implantation into the pulmonary outflow tract, can also be used to treat the pathology of atrioventricular (AV) valves. Increasing gradients are seen as an indication for re-dilating the valve. Our case demonstrates the heart rate dependency of the gradient across a Melody implanted in the left AV valve position in an infant. Beta blockers were used to lower both heart rate and gradient.

Correlation of echocardiographic and angiographic measurements of the pulmonary valve annulus in pulmonary stenosis.

OBJECTIVES: The pulmonary valve (PV) annulus is routinely measured angiographically in PV stenosis prior to balloon dilation. We sought to establish whether this radiation exposure is justified, or whether echocardiographic measurements prior to the procedure are sufficient to guide balloon selection. BACKGROUND: Previous studies have found a strong correlation between echocardiographic and angiographic measurements of the PV annulus. However, error of measurement and its implication for procedural practice has not been explored. METHODS: A total of 90 procedures in 84 patients were analyzed, at a median age 7.6 months (range 1 day to 14.2 years). The contemporaneous echocardiographic and angiographic measurements were recorded, and the original echocardiograms were re-measured in the 72 available cases by two independent reviewers. RESULTS: There was a good correlation between the two measurement methods (R(2) = 0.87). However, the echocardiographic PV measurements were smaller on average, with a significant variation in that discrepancy (mean ratio 0.941 (±0.16)). There was no significant reduction in error if extreme measurements (PV annulus z-score <-3) were excluded (P = 0.09), or if the reviewed echocardiographic measurements were used (P = 0.58). CONCLUSIONS: There is an unacceptable discrepancy between the measurement techniques: 95% of patients are predicted to have an echocardiographic measurement error between -37% and +26%. Therefore, there is no correction factor that could be employed to allow safe selection of balloon size, and balloon pulmonary valvoplasty without angiographic PV measurement cannot be advocated.

Regurgitation of the atrioventricular valves after corrective surgery for complete atrioventricular septal defects - comparison of different surgical techniques.

OBJECTIVE: Different surgical approaches have been used to repair complete atrioventricular septal defects (AVSD). Regurgitant atrioventricular valves (AV-valves) are common after surgery. We compared different surgical techniques with respect to long-term postoperative AV-valve regurgitation. METHODS: In 69 patients with complete AVSD, three different surgical techniques were applied: Single-patch, two-patch, and modified techniques. The left-sided AV-valve cleft was surgically closed in all patients. RESULTS: A comparison of the results of the different techniques showed no difference in the degree of AV-valve regurgitation on either the right or the left side. The average degree was mild on both sides. Only one patient needed reoperation for severe left-sided AV-valve regurgitation. CONCLUSION: The different surgical techniques used for the correction of AVSD do not have a major bearing on the degree of AV-valve regurgitation.

Heart transplantation for isolated noncompaction of the left ventricle in an infant.

Isolated noncompaction of the left ventricular myocardium is a rare cardiomyopathy typically showing a "spongy" myocardium on ultrasound. We report on the ultrasonic and pathomorphological characteristics of an infant who, at the age of 40 days, was treated by heart transplantation for isolated noncompaction. Noncompaction should be suspected in newborns with otherwise unexplained cardiomyopathy and a "spongy" left ventricle. However, ultrasonic and pathological findings may be much less pronounced at this age than later in life.

Low weight in congenital heart surgery: is it the right way?

OBJECTIVE: There is ample evidence that premature and low birth weight children have a poor outcome after congenital heart surgery. The aim of the present study was to characterize the perioperative factors which significantly influence the outcome of these babies following cardiac surgery, and to clarify whether the RACHS-1 and the Aristotle score are compatible for this complex kind of heart surgery. METHODS: During the past 10 years, 108 children with a body weight of less than 3000 g were operated, including 43 premature babies. Mean weight at operation was 2.5 +/- 0.5 kg, mean age was 36.8 +/- 55 days. Fifty percent of the treatments were categorized into risk groups 4 and 6 and 54 % belonged to the complexity levels 3 and 4. Eighty-one operations (75 %) were performed using extracorporeal circulation. RESULTS: The 30 day mortality rate was 18 %, and the overall mortality rate was 30 %. The correlation between mortality rates and risk groups was significant ( p < 0.001). Other significant factors were preoperative acidosis ( p = 0.026), preoperative catecholamine support ( p < 0.001), prolonged ICU stay (> 7 days) after operation ( p < 0.001), and postoperative infection ( p = 0.019). In addition, X-clamp time ( p = 0.029) and palliative procedures ( p < 0.001) were significant factors for poor outcome. CONCLUSION: The results demonstrate that the mortality for correction of congenital heart defects in children weighing less than 3000 g depends on several factors. The risk groups of the RACHS-1 study and the complexity levels of the Aristotle score are useful tools to assess preoperative risk.

A decade of staged Norwood palliation in hypoplastic left heart syndrome in a midsized cardiosurgical center.

Hypoplastic left heart syndrome (HLHS) is a challenge for the pediatric cardiologist and the surgeon. It is generally assumed that the postoperative outcome after surgery for congenital heart disease is influenced by the institutional size. We present the results of 43 patients with true HLHS (situs solitus and atrioventricular and ventriculoarterial concordance) referred for operation between 1992 and 2002 in our center. Two children had atrioseptostomy: one died soon after the operation, and the other one was transplanted successfully but died at the age of 6 months following acute rejection. The remaining 41 underwent Norwood I palliation, 21 stage II palliation, and 10 stage III palliation. Early mortality was 29% after stage I operation, 4.7% after stage II palliation, and 0% after stage III operation. Overall mortality was 39% after stage I, 9.5% after stage II, and 10% after stage III operation. Low birth weight was associated with a higher mortality (p < 0.05). Mortality declined with increasing experience, comparable to the results of very large cardiosurgical centers with many more patients. The quality of surgery and perioperative management in smaller pediatric cardiosurgical centers can reach the level of very large centers.

Cardiac transplantation in neonatal Marfan syndrome -- a life-saving approach.

Marfan syndrome is a connective tissue disease with typical clinical signs and cardiac involvement. Its appearance in the neonatal period has a bad prognosis due to incompetence of all cardiac valves with subsequent congestive heart failure. Conservative management usually fails, the children die during their first year of life. We report on a girl with neonatal Marfan syndrome who suffered from regurgitance of all cardiac valves, enlarged ventricles, and dilated great arteries. She was NYHA class IV. At the age of six months she underwent heart transplantation. To prevent aneurysm formation and dissection of the great vessels, the whole aortic arch and pulmonary trunk were replaced as well.

Heart failure in children -- mechanical assistance.

The use of mechanical circulatory support for the treatment of heart failure in the pediatric population has become increasingly important in pediatric heart surgery units; however, the treatment options for the pediatric population have not attained the same level of technological development as we have seen for the adult population. The use of mechanical assistance as a bridge to transplantation or a bridge to recovery are the main indications for mechanical circulatory support in infants and children. The problem of organ donor shortage is even worse compared to the situation in the field of adult heart transplantations. Especially in Europe however, newly developed pulsatile, paracorporeal ventricular assist devices designed for long-term assist in children have demonstrated their ability to provide excellent results beyond the abilities of extracorporeal membrane oxygenation and centrifugal pumps, which are still the mainstay of mechanical support in children worldwide. Especially in the group of the smallest patients, the use of the most appropriate form of circulatory assistance has to be carefully considered as the co-incidence of respiratory failure as well as other complex physiological situations will severely influence the outcome.

Successful long-term bridge to transplant in a 5-year-old boy with the EXCOR left ventricular assist device.

We report on a 5-year-old boy who presented with postcardiotomy failure after aortic valve replacement and had to undergo implantation of a Berlin Heart-Excor system since treatment with ECMO did not improve myocardial pump function. After a stormy postoperative course with delayed sternal closure after 9 days, the young boy finally recovered and could be fully mobilized. Until successful heart transplantation after a support interval of 77 days, he experienced no device-related infectious or thromboembolic complications.

Congenital aortic regurgitation due to absent aortic cusps and high-degree mitral stenosis.

Congenital absence of aortic cusps leads to severe aortic regurgitation. We present a newborn with this rare entity with extreme mitral stenosis. Hemodynamic features were those of hypoplastic left heart syndrome. Surgical management consisted of initial modified Norwood procedure followed by orthotopic heart transplantation.

Unusual systemic venous return with complete absence of the superior caval veins.

The absence of the right superior vena cava (SVC) is usually associated with the presence of a left SVC. This is the first report of a case of systemic venous return without the presence of either upper caval veins. The blood of the upper half of the body was collected into a vessel located on the left side of the spine behind the heart. This vessel crossed over to the right side at the level of the kidneys, draining directly into the inferior vena cava. During correction of tetralogy of Fallot, these findings were confirmed. The terminology and the bearing on interventional planning are discussed.

Early infantile epileptic encephalopathy (Ohtahara syndrome) after maternal electric injury during pregnancy: etiological considerations.

Early infantile epileptic encephalopathy (EIEE) is a polyetiologic age-dependent neurological disorder. We present two patients with EIEE whose mothers experienced electric injury during pregnancy. After the accident one mother noticed decreased fetal movements. Neither other prenatal factors nor intrapartal damage or postnatally examined structural, metabolic or infectious causes which might have been responsible for the EIEE in these children could be found. The question of electric accident during pregnancy should be considered when documenting the history of children with Ohtahara syndrome.

Ventricular aneurysm or diverticulum? Clinical differential diagnosis.

Intrathoracic ventricular aneurysms and diverticula can be differentiated by several criteria. Contractility is the only reliable parameter: aneurysms expand, whereas diverticula contract during ventricular systole.

Cervical encephalocele in a newborn--Chiari III malformation. Case report and review of the literature.

Chiari malformations are brain stem anomalies with or without spinal canal involvement. The combination of brain stem and cerebellar anomaly with a cervical cele is named Chiari III malformation. Patients with this extremely rare defect frequently present with severe neurological defects and can have a poor prognosis. Following a report on a newborn child with a Chiari III malformation that took a fatal but typical course the literature is reviewed.