Jochen Grohmann

Prospective electrocardiography-triggered CT angiography of the great thoracic vessels in infants and toddlers with congenital heart disease: Feasibility and image quality

PURPOSE To investigate feasibility and image quality and to calculate radiation dose estimates for computed tomography angiography (CTA) of the great thoracic vessels in infants and toddlers with congenital heart disease (CHD) using end-systolic prospective electrocardiography-triggered sequential dual-source data acquisition. METHODS This study was institutional review board approved; informed consent was obtained. Twenty children (age 1.2±1.1 years) underwent 22 prospective ECG-triggered sequential dual-source CTA examinations (Somatom Definition, Siemens) with tube current (250 mAs/rot) centered at 250 ms past the R-peak in the cardiac cycle (end-systole). Tube voltage was set to 80 kV. Image quality was evaluated by two readers independently using a four-point grading scale (4=excellent, 1=non-diagnostic). Radiation dose estimates were calculated from the dose-length-product (DLP). RESULTS All CT images showed diagnostic image quality (mean score 3.67±0.67, κ=0.85). Stair-step artifacts were present in one and breathing artifacts in 4 patients, with neither impairing diagnostic image quality. Mean heart rate (bpm) was 107.6±12.1 (76-130), mean heart rate variability (bpm) was 2.5±2.0 (1-9). Mean scan length (mm) was 90.7±22.7 (50-134). Mean estimated effective dose was 0.32±0.11 mSv. CONCLUSION Prospective ECG-triggered sequential dual source CTA is feasible in infants and toddlers with CHD, thereby allowing almost motion-free imaging of the great thoracic vessels with the benefit of a low radiation dose.

Aneurysms of the Pulmonary Artery

Pulmonary artery (PA) aneurysms (PAAs) are rare and infrequently diagnosed. Deterling and Clagett1 discovered 8 cases of PAAs in 109 571 consecutive postmortem examinations. PAAs generally occurred in a younger age group than aortic aneurysms with an equal sex incidence.2 Eighty-nine percent of all PAAs were located in the main PA, whereas only 11% were located in the pulmonary branches.3 When affecting the PA branches, PAAs in the left PA were more common than in the right PA.1 An aneurysm is defined as a focal dilatation of a blood vessel involving all 3 layers of the vessel wall. Pseudoaneurysms, on the other hand, do not involve all layers of the arterial wall but possess a higher risk of rupture. In computed tomography, the upper limit for adults of the main PA diameter is 29 mm, and the upper limit of the interlobar PA is 17 mm.4 Therefore, Nguyen et al5 describe a PAA as a focal dilatation of the PA beyond its maximal normal caliber. In contrast, Brown and Plotnick6 define a PAA as a PA with a diameter exceeding 40 mm, distinguishing between an ectasia of the PA and a true PAA. However, both definitions do not relate the PAA threshold to body dimensions or to the diameters of other vessels. In our center, the upper limit of the main PA diameter (29 mm) was defined as a PAA. In case of a PAA in children, the PAA size was compared with the normal values according to the method of Kampmann et al.7 In high-risk patients, the diameter of the PAA was indexed to the body weight according to patients presenting with an aneurysm of the aorta. Various origins of PAA have been described, allowing us to differentiate among congenital causes, …

Trans-catheter closure of the native aortic valve with an Amplatzer® Occluder to treat progressive aortic regurgitation after implantation of a left-ventricular assist device

We report on a patient with ischaemic dilated cardiomyopathy, who developed progressive regurgitation of his native aortic valve after implantation of a left-ventricular assist device (LVAD, HeartMate II). Increasing regurgitant volume led to reduced effective cardiac output and worsening of symptoms. To overcome aortic regurgitation, we successfully closed his aortic valve minimally invasively using the Amplatzer(®) P.I. Muscular VSD Occluder. This led to instant haemodynamic stabilisation. We observed significant residual regurgitation through the Occluder during the initial phase, which led temporarily to increased haemolysis and subsequently to worsening of kidney function; once the haemolysis ceased, we noted a very good interventional and clinical result at short-term follow-up.

Kawasaki Disease in Germany: A Prospective, Population-based Study Adjusted for Underreporting.

Background: National estimates of Kawasaki disease (KD) incidence often do not include incomplete cases (diagnosed based on only laboratory or echocardiographic criteria), and/or they rely on retrospective case reports and data registries where underreporting is known to be a problem. Methods: We conducted a prospective nationwide KD surveillance study in children younger than 5 years through the hospital-based German Pediatric Surveillance Unit (ESPED). We accounted for underreporting through applying capture–recapture methodology in 2 federal states using hospital discharge records with KD International Statistical Classification of Diseases and Related Health Problems 10th revision code (ie, M30.3). KD diagnosis (complete and incomplete) was established according to the American Heart Association criteria, 2004. Results: Incidence of KD, corrected for underreporting, was 7.2 of 100,000 in children younger than 5 years in Germany. Underreporting to ESPED was estimated at 37%–44%. Overall, 315 validated KD cases were reported. Of the 64 (20%) incomplete cases, 58% (37/64) were detected through echocardiographic findings and 42% (27/64) through laboratory criteria alone. Incomplete cases were younger than complete cases (1.2 vs. 2.0 years, P = 0.0001) and had more coronary aneurysms (43% vs. 11%, P = 0.0001). Conclusions: A substantial number of incomplete KD cases were diagnosed based on the laboratory and echocardiographic criteria only. This was particularly the case in relation to infants younger than 1 year—an age group known to have an increased risk of developing coronary aneurysms. In addition, we found a high rate of underreporting to national Pediatric Surveillance Units. We suggest that improved surveillance and development of better diagnostic tests remain a high priority.

A family with a new elastin gene mutation: broad clinical spectrum, including sudden cardiac death

Supravalvular aortic stenosis is associated with the Williams-Beuren syndrome, but it also occurs in a non-syndromatic congenital form. An elastin gene mutation of chromosome 7q11.23 is responsible in both cases. The vascular features are identical. These patients have a higher risk of sudden death, particularly when undergoing diagnostic or surgical procedures. We report the account of a family with a new mutation in the elastin gene. Screening over three generations revealed eight affected individuals. The cardiac and vascular malformations ranged from mild asymptomatic supravalvular aortic stenosis and isolated dysplastic atrioventricular valves to diffuse arterial hypoplasia. Two infants presented arteries affected at multiple locations, including the left coronary artery. Both died of sudden cardiac death and myocardial ischaemia, one while under general anaesthesia for cardiac catheterisation, and the other perioperatively. We discuss the pathophysiological aspects in these patients that deserve consideration before any general anaesthesia is administered.

Transcatheter closure of atrial septal defects in children and adolescents: single-center experience with the GORE® septal occluder.

Device closure of atrial septal defects (ASD II) is an alternative to surgery. ASD morphology and intracardiac relationships may influence device selection. Biocompatibility, techniques for closing large or multiple defects, and the risk of erosion are main issues in children

Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease

OBJECTIVES To determine the frequency and severity of acquired von Willebrand syndrome (AVWS) in children with stenotic congenital heart disease (CHD) before and after intervention. METHODS In this single-centre prospective observational case-control study, 50 children [median age: 26 (range, 0-175) months, bodyweight: 9.5 (2.2-7.5) kg] underwent catheter interventions or cardiac surgery. A total of 26 children with high stenosis [mean gradient: 80 (range, 52-130) mmHg] represented the stenosis group and 24 without relevant stenosis (<20 mmHg) served as the control group. von Willebrand factor (VWF) was analysed with respect to quantity and function before and after corrective or palliative intervention. RESULTS Demographic data were comparable. The stenosis group had more surgical and the control group more interventional procedures (P = 0.025). Before intervention, 13 patients from the stenosis group (50%) showed a significant reduction in VWF-multimers compared with no patients in the control group (P <0.001). Collagen binding capacity (VWF:CB) was lower in the stenosis group [0.5 (0.2-2.6) U/ml vs 0.8 (0.4-2.1) U/ml, P <0.05), as was the collagen binding capacity to antigen ratio (VWF:CB/Ag) [0.8 (0.4-1.4) U/ml vs 1 (0.4-1.7) U/ml, P <0.001). After intervention, VWF parameters normalized rapidly within the first 24 h after the procedure and showed no group differences. {VWF:CB [1.7 (0.6-3.7) vs 1.7 (0.6-4.2) U/ml, P = n.s.], VWF:CB/Ag [1.1 (0.5-2.9) vs 1.2 (0.7-2.2), P = n.s} Time in the intensive care unit, respirator time, duration of stay and bleeding before and after intervention were not significantly different. CONCLUSIONS AVWS is detected in half of the children with high intra- or extracardiac stenoses and resolves completely after surgical or interventional repair. Even when undergoing surgery on cardiopulmonary bypass, excessive surgical site bleeding was not detected in our study patients.

A new breakable stent for recoarctation in early infancy: Preliminary Clinical Experience

Transcatheter treatment of aortic coarctation (CoA) via stent implantation has become an established treatment option depending on the patients age and CoA type.

Coil-occlusion of the left ventricle as emergency treatment in failing stage I palliation for hypoplastic left heart syndrome with sinusoids.

We report on a patient with hypoplastic left heart syndrome (HLHS), ventricular septal defects, and coronary sinusoids who suffered recurrent myocardial ischemic events that required cardiopulmonary resuscitation after stage I palliation. We identified the main reason to be a steal phenomenon of blood from the left coronary artery via the sinusoids into the rudimentary left ventricle and across the septal defects into the right ventricle. To limit this coronary steal phenomenon, we successfully performed transcatheter closure of the left ventricle with implantation of three Microplex Terumo®‐Coils. This led to the patients clinical stabilization. Stage II surgery took place at the age of 5 months without difficulty. In the setting of a symptomatic neonate with HLHS and ventriculocoronary connections, embolization of the left ventricle is a feasible interventional therapy.

Multicenter midterm follow‐up results using the gore septal occluder for atrial septal defect closure in pediatric patients

To assess the safety and efficacy of the Gore Septal Occluder (GSO) used for device‐closure of significant secundum‐type atrial septal defects (ASD II) focusing on pediatric patients.