Walter Knirsch

[Isolated myocardial noncompaction in childhood-case report and review of the literature].

The diagnostic work-up for the first syncopal episode of a 14-year-old female adolescent revealed the morphology of an isolated myocardial noncompaction on echography. Angiography and biopsy of the left ventricle confirmed the diagnosis. Despite a reduced shortening fraction (FS 21%) and frequent premature atrial beats, there were no further cardiac or extracardiac symptoms. After establishment of therapy with β-blockade, digitalis, angiotensinconverting enzyme inhibition and acetylsalic acid, the follow-up over 24 months was good; the implantation of an automatic implantable cardioverter-defibrillator is planned. Bei einer 14-jährigen jugendlichen Patientin wurde im Rahmen einer Synkopenabklärung in der Echokardiographie morphologisch das Bild einer isolated myocardial noncompaction nachgewiesen. Durch eine linksventrikuläre Angiographie und eine Herzmuskelbiopsie konnte diese Diagnose bestätigt werden. Neben einer verminderten linksventrikulären Verkürzungsfraktion (FS 21%) und zahlreichen supraventrikulären Extrasystolen zeigten sich keine weiteren kardialen oder extrakardialen Auffälligkeiten. Unter entsprechender medikamentöser Therapie (ACE-Hemmer, Digitalis, β-Blocker und ASS) war die Patientin über einen Nachbeobachtungszeitraum von 24 Monaten beschwerdefrei, die Implantation eines AICD ist geplant.

Aortic arch enlargement and coarctation repair through a left thoracotomy: significance of ductal perfusion

OBJECTIVE To analyse the technique of neonatal aortic arch enlargement without cardiopulmonary bypass through a left posterior thoracotomy, as an adjunct to extended resection for Coarctation and severe arch hypoplasia. METHODS Ten neonates with coarctation, severe arch hypoplasia and a persistent ductus arteriosus (PDA) were subjected to arch repair through a left posterior thoracotomy. Nine of these patients had associated significant intracardiac anomalies; three of them received pulmonary artery (PA) banding. After exclusion from circulation, the roof of the intervening arch between left carotid and left subclavian was enlarged using a patch. After adequate reperfusion, a classic resection and extended end-to-end anastomosis was performed. Median age and weight were 5.5 (1-10) days and 3.3 (2.2-4.1) kg respectively. The median preoperative arch diameter was 1.07 (0.75-1.32) mm/kg body weight. RESULTS All patients could be successfully operated with this approach. The non-ischaemic and ischaemic aortic clamp times were 40 (15-68) and 23 (18-32) min, respectively. The median postoperative arch diameter achieved was 1.43 (1.06-1.46) mm/kg body weight. None of the patients had significant gradient early postoperatively. Two patients with recurrent stenosis were successfully treated with balloon dilatation (1) or surgery with cardiopulmonary bypass (CPB) (1). One patient has a corrected gradient of 16 mmHg in the proximal arch which is being observed. The remaining patients are free from stenosis at a median follow-up of 30.1 (13.2-57.8) months. CONCLUSIONS Use of PDA for lower body perfusion allows complex reconstruction of the arch without incurring lower body ischaemia. The extended resection could then be performed without excessive stretch. This modification saves these patients from undergoing a complex arch reconstruction with CPB in the early neonatal period.

Closure of restrictive ventricular septal defects through a right axillary thoracotomy.

OBJECTIVE To report the midterm results of an alternative minimally invasive approach for closure of ventricular septal defects (VSD) through a muscle-sparing minithoracotomy at the right midaxillary line. MATERIAL AND METHODS From September 2003 to December 2005, 22 patients (median age, 5 years; range, 1.5-12 years) underwent a right lateral axillary thoracotomy for closure of a perimembranous VSD. Special features of the approach included an incision between the anterior and posterior axillary fold, a muscle-sparing preparation over the fourth intercostal space, and the establishment of cardiopulmonary bypass with inguinal and superior vena cava cannulation. The ascending aorta was cross clamped and cardioplegic arrest was instituted. The VSDs were all approached through a right atriotomy and closed directly (17 patients) or with a patch (5 patients). Three patients underwent concomitant aortic valve repair. RESULTS There was no mortality or significant surgical morbidity. Median cross-clamp and cardiopulmonary bypass times were 46 and 104 min, respectively. Follow-up was complete and ranged from 2 to 22 months (median, 20 months). All patients were in sinus rhythm. Echocardiography revealed no residual defects with competent aortic and tricuspid valves. The length of the incision ranged from 4 to 6 cm, was away from the mammary gland, and remained entirely covered by the adducted arm. All children recovered right shoulder function within days, and cosmetic results were very satisfying. One patient developed iliac artery stenosis following inguinal artery cannulation. CONCLUSION Closure of perimembranous VSDs in older children can be safely performed through a right axillary thoracotomy without compromising the accuracy of the repair. The cosmetic results are excellent.

Neurodevelopmental profiles of children with congenital heart disease at school age

Objectives To assess 6‐year neurodevelopmental outcomes in a current cohort of children with congenital heart disease (CHD) who underwent cardiopulmonary bypass surgery (CPB), and to determine risk factors for adverse outcomes. Study design Outcomes were examined in 233 prospectively enrolled children with CHD (including 64 with a recognized genetic disorder) who underwent CPB between 2004 and 2009. Follow‐up assessment included standardized neurologic, motor, and cognitive tests. Variables were collected prospectively, and multiple regression analysis was performed to determine independent risk factors for adverse outcome. Results The mean patient age at assessment was 6.3 years (range, 5.1–6.8 years). IQ was lower in children with a genetic disorder (median, 55; range, 17–115) compared with children without a genetic disorder (median, 95; range, 47–135; P < .001). Cognitive and motor performance also were lower in children without a genetic disorder compared with the norm (P < .01 for both). The prevalence of children without a genetic disorder performing below ‐2 SD (IQ 70) was higher than the norm (5.3% vs 2.3%; P = .008), and the prevalence of poor motor performance (<10th percentile) ranged from 21.2% to 41.1% (P < .01 for all). Significant independent risk factors for poor neurodevelopmental outcome included a genetic disorder, longer length of intensive care stay, lower birth weight, postoperative seizures, and lower socioeconomic status. Conclusions Current cohorts of children with CHD undergoing CPB show favorable outcomes but remain at risk for long‐term neurodevelopmental impairments, particularly those with a genetic disorder and a complicated postoperative course. Close neurodevelopmental surveillance is necessary to provide early therapeutic support.

Postoperative Amplitude-Integrated Electroencephalography Predicts Four-Year Neurodevelopmental Outcome in Children with Complex Congenital Heart Disease

OBJECTIVES To evaluate the predictive value of pre- and postoperative amplitude-integrated electroencephalography (aEEG) on neurodevelopmental outcomes in children operated for congenital heart disease (CHD). STUDY DESIGN Prospectively enrolled cohort of 60 infants with CHD who underwent cardiac surgery with cardiopulmonary bypass in the first 3 months of life. Infants with a genetic comorbidity were excluded. aEEG was assessed for 12 hours pre- and 48 hours postoperatively. Background pattern was classified by the use of standard categories, and the presence of seizures and sleep-wake cycles (SWCs) was noted. Outcome at 1 and 4 years of age was assessed with standardized developmental tests. RESULTS Preoperatively, infants either showed continuous normal voltage (n = 56) or discontinuous normal voltage (n = 4). Postoperatively, abnormal background pattern (flat trace, burst suppression, or continuous low voltage) was detected in 7 (12%), discontinuous normal voltage in 37 (61%), and continuous normal voltage in 16 (27%) infants. Nineteen infants (32%) did not return to normal SWCs within the recording period. Seizures were detected in 4 infants preoperatively and in another 4 postoperatively. After we controlled for surgical and postoperative risk factors, abnormal postoperative background pattern and lack of return to SWCs independently predicted poorer intelligence quotient at 4 years (P = .03 and P = .04 respectively) but was not related to motor outcome. CONCLUSION aEEG is a useful bedside tool that helps to predict outcome in infants undergoing open-heart surgery for CHD. Abnormal postoperative background pattern and lack of return to SWCs are markers for subsequent impaired cognitive development.

Interventional Treatment of a Symptomatic Neonatal Hepatic Cavernous Hemangioma Using the Amplatzer Vascular Plug

Percutaneous intervention is one treatment option for symptomatic hepatic hemangioma in infants. We report the case of a newborn (birth weight 4060 g) with a large hepatic cavernous hemangioma, which presented early with high cardiac output failure due to arteriovenous shunting and signs of incipient Kasabach-Merritt syndrome. We performed a successful superselective transcatheter coil embolization of three feeding arteries on the seventh day of life. Because of remaining diffuse very small arteries causing a relevant residual shunt, additional occlusion of the three main draining veins was necessary with three Amplatzer vascular plugs. Cardiac failure resolved immediately. Without any additional therapy the large venous cavities disappeared within the following months. The tumor continues to regress in size 8 months after the intervention.

Angiotensin-converting Enzyme Inhibitors in Pediatric Patients with Mitral Valve Regurgitation—Case-control Study and Review of the Literature

OBJECTIVES To investigate the effectiveness of chronic administration of angiotensin-converting enzyme inhibitors in pediatric patients with mitral valve regurgitation. DESIGN, SETTING, PATIENTS, INTERVENTIONS This was a case-control study of all echocardiograms of patients with moderate-to-severe mitral valve regurgitation, who underwent chronic administration of angiotensin-converting enzyme inhibitors between January 1990 and December 2006 at a single center. OUTCOME MEASURES Echocardiographic parameters (left ventricular end-diastolic diameter, left ventricular posterior wall diameter, interventricular septum diameter, left atrium to aortic root diameter ratio, grade of mitral valve regurgitation, shortening fraction) were analyzed before and during therapy with angiotensin-converting enzyme inhibitors in 12 patients and compared with 12 patients without medications after one month and one year. RESULTS Twenty-four consecutive pediatric patients (median age of 7 years with a range 1 month-16 years) with moderate-to-severe mitral valve regurgitation were included. Data are given as standard deviation scores (z-scores) derived from body-surface-adjusted normal values. During angiotensin-converting enzyme inhibition left ventricular end-diastolic diameter decreased from mean z-score 2.04 to 1.66 (after 1 month) and to 1.73 (after 1 year), while left ventricular posterior wall diameter decreased from 0.25 to 0.12 (after 1 year), respectively. Shortening fraction, interventricular septum diameter, grade of mitral valve regurgitation, and left atrium to aortic root diameter ratio remained stable. CONCLUSIONS In this case-control study of patients with moderate-to-severe mitral valve regurgitation effectiveness of angiotensin-converting enzyme inhibition on left ventricular dimensions and function after 1 month and 1 year is limited. Reviewing the literature, the lack of long-term follow-up studies with large patient cohorts and controversial study-results in adults require a prospective long-term multicenter follow-up study in pediatric patients.

Regional microstructural and volumetric magnetic resonance imaging (MRI) abnormalities in the corpus callosum of neonates with congenital heart defect undergoing cardiac surgery

The purpose of the study is to investigate the structural development of the corpus callosum in term neonates with congenital heart defect before and after surgery using diffusion tensor imaging and 3-dimensional T1-weighted magnetic resonance imaging (MRI). We compared parallel and radial diffusions, apparent diffusion coefficient (ADC), fractional anisotropy, and volume of 5 substructures of the corpus callosum: genu, rostral body, body, isthmus, and splenium. Compared to healthy controls, we found a significantly lower volume of the splenium and total corpus callosum and a higher radial diffusion and lower fractional anisotropy in the splenium of patients presurgery; a lower volume in all substructures in the postsurgery group; higher radial diffusion in the rostral body, body, and splenium; and a higher apparent diffusion coefficient in the splenium of postsurgery patients. Similar fractional anisotropy changes in congenital heart defect patients were reported in preterm infants. Our findings in apparent diffusion coefficient in the splenium of these patients (pre and postsurgery) are comparable to findings in preterm neonates with psychomotor delay. Delayed maturation of the isthmus was also reported in preterm infants.

Catheter Interventional Closure of a Large Right Pulmonary Artery–to–Left Atrial Communication in a Neonate

Right pulmonary artery–to–left atrial communication is a rare congenital vascular malformation that results in a right-to-left shunting. This report describes the case history of a neonate with a large right pulmonary artery–to–left atrial connection resulting in cyanosis and severe heart failure who underwent successful early catheter interventional therapy. In the neonate, this lesion can be diagnosed accurately using transthoracic echocardiography. Closure of the communication can be achieved even in infants via percutaneous interventional catheterization with a low procedure-related risk and a good midterm follow-up result.

Role of sevoflurane in organ protection during cardiac surgery in children: a randomized controlled trial.

OBJECTIVES The protective effects of volatile anaesthetics against ischaemia-reperfusion injury have been shown in vitro, but clinical studies have yielded variable results. We hypothesized that, in children, sevoflurane provides superior cardioprotection after cardiac surgery on cardiopulmonary bypass (CPB) compared with totally intravenous anaesthesia (TIVA). METHODS In this randomized controlled, single-centre study, 60 children with cyanotic and acyanotic heart defects undergoing elective cardiac surgery under CPB (RACHS-1 1-3) were randomized to sevoflurane or TIVA (midazolam <6 months of age, propofol >6 months of age). The primary end-point was the postoperative peak cardiac troponin I/T (cTnI/T). Perioperative cardiac function (as determined by brain-type natriuretic peptide, echocardiography and postoperative vasopressor/inotrope requirements), short-term clinical outcomes (duration of intubation, intensive care unit and hospital length of stay), postoperative inflammatory profile, and pulmonary, renal and liver function were defined as secondary end-points. Analysis of variance was used for statistical analysis. RESULTS There was no statistically significant difference in postoperative peak troponin values or any of the secondary end-points. In the subgroup of acyanotic patients under 6 months, sevoflurane led to significantly lower postoperative troponin levels compared with midazolam [reduction of 54% (95% confidence interval 29-71%, P = 0.002)], without any differences in secondary outcome parameters. CONCLUSIONS Sevoflurane did not provide superior myocardial protection in our general paediatric cardiac surgical population. In children under 6 months, however, sevoflurane might be beneficial in comparison with midazolam. The conditioning effects of sevoflurane in specific paediatric subgroups need to be further investigated.