Effectiveness of simulator-based echocardiography training of noncardiologists in congenital heart diseases.

BACKGROUND: Congenital heart diseases (CHD) are responsible for substantial morbidity and mortality in neonates. The preliminary diagnosis often is made by noncardiologists. For this reason, there is a huge demand of training in echocardiography of CHD. This is difficult to achieve due to limited resources of specialized centers. OBJECTIVE: The goal of this study was to investigate the training effect of the echocardiography simulator EchoCom on trainee's ability to diagnose CHD. DESIGN/METHODS: We enrolled 10 residents for simulator-based training in echocardiography of CHD. All participants were instructed on the simulator's basic handling and had one hour to scan the first 9 datasets information (ventricular septal defect, atrial septal defect, atrioventricular septal defect, Tetralogy of Fallot, transposition of great arteries, congenital corrected transposition of great arteries, common arterial trunk, hypoplastic left heart syndrome, normal anatomy) and establish a diagnosis. No help was given except for support regarding simulator related issues. Afterward, 2 rounds of structured simulator based echocardiography training focused on echocardiographic anatomy, spatial orientation, standard views, and echocardiographic anatomy of different CHD followed. All participants completed a standardized questionnaire containing 10 multiple-choice (MC) questions focusing on basic theoretical knowledge in echocardiographic anatomy and common CHD. RESULTS: Almost all of the residents invited from the affiliated children's hospital had little (20%) or no experience (80%) in echocardiography of CHD. Their Pretest and Posttest scores showed significant improvement for both, MC test and performance test, respectively. CONCLUSIONS: Our study showed that simulator-based training in echocardiography in CHD could be very effective and may assist with training outside the scope of CHD.

Long-term results after repair of complete atrioventricular septal defect with two-patch technique.

BACKGROUND: Surgical management of patients with complete atrioventricular septal defect (AVSD) has advanced over the last decades. Definitive early surgical repair for AVSD has become the treatment of choice at many centers. This trend has contributed to the recent decline in postoperative mortality and good long-term results. METHODS: We reviewed long-term results of 100 consecutive patients with complete AVSD undergoing definitive early repair with a two-patch technique and complete cleft closure operated on between June 1999 and June 2009. Valve performance, mortality, morbidity, and indications for reoperation were retrospectively studied. RESULTS: Median age at operation was 3.8 months (range, 15 days to 4.7 years); median weight was 5.0 kg (range, 3.0 to 19 kg). Follow-up was 99% complete (mean 58 months; range, 1 to 120 months). Early definitive repair was performed in all patients who initially presented to our institution over the study period. There was no perioperative, in-hospital mortality, or late mortality. At the latest follow up, left atrioventricular valve (LAVV) regurgitation was absent or trace in 80 patients (80%), mild to moderate in 15 patients (15%), and moderate to severe in 4 patients (4%). Right AV valve regurgitation was none or trace in 86 patients (86%), mild to moderate in 11 patients (11%), and moderate to severe in 2 patients (2%). Actuarial freedom from reoperation for LAVV dysfunction at 1, 3, 5, and 10 years was 98%, 95%, 94%, and 94%, respectively. Actuarial freedom from reoperation for left ventricular outflow tract obstruction at 1, 3, 5, and 10 years was 100%, 99%, and remained constant by 99% at 5 and 10 years. CONCLUSIONS: Definitive early repair for complete AVSD can be performed with excellent results. The two-patch technique with complete cleft closure is a safe and reproducible surgical method that can achieve very low mortality and stable long-term outcomes, even in neonates.

Differential effects of the site of permanent epicardial pacing on left ventricular synchrony and function in the young: implications for lead placement.

AIMS: To analyse left ventricular (LV) synchrony and function with respect to the epicardial pacing site in the young. METHODS AND RESULTS: Left ventricular function and synchrony (M-mode, speckle tracking) were evaluated during mid-term follow-up in 32 children with complete non-surgical (n = 15) or surgical (n = 17) atrioventricular block (structural heart disease in 21/32) paced from LV apex (n = 19), right ventricular (RV) apex (n = 7), and RV free wall (n = 6), respectively. Data are in the following order: LV apical, RV apical, and RV free wall pacing. Septal to posterior wall motion delay (SPWMD) = median 0, 69, and 136 ms (P < 0.001), septal to lateral mechanical delay = 54 +/- 29, 73 +/- 24, and 129 +/- 70 ms (P = 0.001), apical to basal mechanical delay = 96 +/- 37, 106 +/- 50, and 79 +/- 18 ms (P NS), and LV ejection fraction (LVEF) = 57 +/- 9, 49 +/- 12, and 33 +/- 10% (P < 0.001), respectively. Left ventricular ejection fraction correlated negatively with SPWMD (R(2) = 0.454, P < 0.001) and septal to lateral mechanical delay (R(2) = 0.320, P < 0.001) but not with apical to basal mechanical delay. Right ventricular free wall pacing (P = 0.014) and SPWMD (P = 0.044) were negative multivariable predictors of LVEF. CONCLUSION: Compared with other sites, LV apical pacing preserves septal to lateral LV synchrony and systolic function and may be the preferred epicardial pacing site in the young.

Interruption of the ascending aorta: a hitherto undescribed lesion.

We are describing a most unusual variant of aortic arch interruption. The ascending aorta was atretic above the sinutubular junction forming a blind-ending pouch. The coronary arteries were the only vessels arising from the aortic trunk. All the brachiocephalic vessels were perfused in retrograde fashion through the arterial duct. The aortic valve itself was dysplastic, stenotic, and permitted severe regurgitation. There was no subaortic obstruction. A modified first stage Norwood palliation was performed. Due to myocardial insufficiency transthoracic extracorporeal membrane oxygenation had to be commenced at the end of surgery. Unfortunately the child died due to severe intracranial hemorrhage.

Adverse effects of Wolff-Parkinson-White syndrome with right septal or posteroseptal accessory pathways on cardiac function.

AIMS: Wolff-Parkinson-White syndrome with right septal or posteroseptal accessory pathways causes eccentric septal mechanical activation and may provoke left ventricular (LV) dyssynchrony and dysfunction. The aim of the study was to evaluate the effect of radiofrequency catheter ablation (RFA) of the accessory pathways on LV function. METHODS AND RESULTS: Retrospectively, transthoracic echocardiography and electrocardiogram recordings were analysed in 34 patients (age: 14.2 +/- 2.5 years) with right septal or posteroseptal accessory pathways prior and after (median: 1 day) successful RFA. Results prior to RFA, LV ejection fraction was decreased (<55%) in 19/34 patients (56%). After RFA, QRS duration was normalized (129 +/- 23 vs. 90 +/- 11, P < 0.0001), LV function improved (ejection fraction: 50 +/- 10 vs. 56 +/- 4%, P = 0.0005) and septal-to-posterior wall motion delay as a global measure for LV dyssynchrony decreased (110 +/- 94 vs. 66 +/- 53, P = 0.012). Longitudinal two-dimensional strain evaluated in five patients demonstrated a decrease of left intraventricular mechanical delay from 292 +/- 125 to 118 +/- 37 ms after RFA. CONCLUSION: Wolff-Parkinson-White syndrome with right septal or posteroseptal accessory pathways may cause LV dyssynchrony and jeopardize global LV function. Radiofrequency catheter ablation resulted in normalized QRS duration, mechanical resynchronization, and improved LV function. Even in the absence of arrhythmias, RFA of right septal or posteroseptal pathways may be considered in patients with significantly decreased LV function.

Sinus venosus atrial septal defect with partial anomalous pulmonary venous return: diagnosis with 64-slice spiral computed tomography at low radiation dose.

The use of computed tomography is often debatable for pediatric patients because of the associated radiation exposure. Using a state-of-the-art 64-slice spiral computed tomography unit, we demonstrate the feasibility of lowering the radiation dose without losing essential diagnostic information in a case of a 2-year-old boy with a rare congenital heart defect.

Real-time 3-dimensional echocardiography for follow-up of Waterston shunt.

A 37-year-old man presented with Eisenmenger's reaction. He had progressive cyanosis and signs of right-sided heart failure. In early childhood he was palliated with a Waterston shunt for cyanotic congenital heart disease. Two-dimensional echocardiography provided only a rudimentary view of his heart's anatomy and hemodynamics. Real-time three-dimensional echocardiography (RT3DE) was superior in visualizing both the size and the shape of the large shunt. RT3DE easily identifies and quantifies a key pathogenic factor for pulmonary hypertension with irreversible vascular damage in such patients. If applied early, RT3DE may prove to be a valuable follow-up tool.

Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study.

AIMS: Non-compaction of the left ventricular myocardium (NCVM) is reportedly exceedingly rare and associated with a high morbidity and mortality. A different genetic background has been suggested for NCVM with [non-isolated NCVM (ni-NCVM)] and without [isolated NCVM (i-NCVM)] other congenital heart defects. We prospectively evaluated both the NCVM subgroups regarding frequency of occurrence and cardiovascular complications in a paediatric population. Results In a prospective, single-centre study, 66/5220 consecutive patients (1.26%) were diagnosed (25 i-NCVM, 41 ni-NCVM). The median age was 4 years (range 0-21), the median follow-up 12 months (range 0-51). The occurrence of congestive heart failure (CHF) at follow-up was 68.0%. CHF was as frequently seen in i-NCVM and ni-NCVM patients (77.5 vs. 62.1%, P = 0.322). The occurrence of arrhythmias (20.0%) and thrombo-embolic events (13.9%) was not different between subgroups. The cardiomyopathy related mortality was 7.1%, with three and one deaths in the i-NCVM and ni-NCVM groups, respectively (P = 0.126). CONCLUSION: When prospectively evaluated, NCVM appears to have been previously under-diagnosed. Whereas arrhythmias and thrombo-embolic events were rare, CHF was frequently found. An equally aggressive anticongestive treatment regimen would seem indicated for both the NCVM subgroups.

Ross-Konno procedure in neonates: report of three patients.

The Ross-Konno procedure, applied to neonates with severe left ventricular outflow tract (LVOT) obstruction, offers a satisfactory solution in fully releasing the LVOT gradient, and in replacing the aortic valve with a pulmonary autograft with an excellent growth potential. We reported on three recent neonatal cases.