René Höhn

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, …

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.

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

Long-Term Follow-Up on Health-Related Quality of Life After Mechanical Circulatory Support in Children.

Objective: The objective of this study was to evaluate health-related quality of life in long-term survivors of mechanical circulatory support after acute cardiopulmonary failure. Design: Prospective follow-up study. Setting: Single-institutional in a center for congenital heart disease and pediatric cardiology. Patients: Fifty patients who underwent 58 mechanical circulatory support therapies in our institution from 2001 to 2012. Median age was 2 (0–213) months, and median supporting time was 5 (1–234) days. Indication groups: 1) extracorporeal life support in low cardiac output: 30 cases (52%); 2) extracorporeal cardiopulmonary resuscitation: 13 cases (22%); 3) extracorporeal membrane oxygenation in acute respiratory distress syndrome: four cases (7%); and 4) ventricular assist devices: 11 cases (19%). Interventions: Health-related quality of life was measured using standardized questionnaires according to the age group and completed by either parent proxies in children under 7 years old or the survivors themselves. Measurements and Main Results: Fifty percentage of the patients were discharged home, and 22 long-term survivors (44%) were studied prospectively for health-related quality of life. Median follow-up period was 4.5 (0.3–11.3) years. Median age at follow-up was 5 (0.6–29) years old. Nineteen long-term survivors filled in the health-related quality of life questionnaires and were classified into three age groups: 0–4 years (n = 7): median health-related quality of life score, 69 (59–86) points; 4–12 years (n = 7): median health-related quality of life score, 50 (48–85) points; older than 12 years (n = 5): median health-related quality of life score, 90 (80–100) points. Conclusion: Long-term survivors’ health-related quality of life as reported by their parents is lower than that of healthy children. However, the self-assessed health-related quality of life of the patients older than 12 years in our group is comparable to a healthy control population.

Closure of patent foramen ovale defects using GORE® CARDIOFORM septal occluder: Results from a prospective European multicenter study

The GORE® CARDIOFORM Septal Occluder (GSO) is a novel device designed for rapid and effective closure of patent foramen ovale (PFO) which has distinctive features making it suitable for a broad spectrum of anatomical variations. We report the procedural and 6 months follow‐up results of the first prospective, multicenter study using GSO.

Closure of patent forman ovale defects using GORE® CARDIOFORM septal occluder

The GORE® CARDIOFORM Septal Occluder (GSO) is a novel device designed for rapid and effective closure of patent foramen ovale (PFO) which has distinctive features making it suitable for a broad spectrum of anatomical variations. We report the procedural and 6 months follow‐up results of the first prospective, multicenter study using GSO.

No-React® Injectable BioPulmonic™ valves re-evaluated: discouraging follow-up results

OBJECTIVES The No-React® Injectable BioPulmonic™ valve (BioIntegral) was introduced for minimally invasive off-pump replacement of the pulmonary valve almost 10 years ago. We present our mid- to long-term follow-up results. METHODS We conducted a retrospective analysis of all 7 patients treated at our institution at the median age of 9 (range 1-24) years. The children underwent cardiac catheterization when worsening strain on the right heart was suspected after examining their medical history and/or observing significant changes on echocardiography. RESULTS After a median follow-up period lasting 5.2 (range 0.7-6.7) years, all patients presented the indication for recatheterization, particularly because the maximum instantaneous velocity measured by Doppler had revealed systolic gradients of a median 63 (dP 18-74) mmHg across the right ventricular outflow. Catheterization confirmed severe stenosis in 2, and moderate stenosis together with moderate insufficiency in 4 patients. We observed two principal failure mechanisms: technical problems resulting from poor alignment to the right ventricular outflow tract and structural problems leading to neointimal proliferation even in cases with appropriate prosthesis positioning. At median of 5.7 (0.7-7) years after implantation, 6 of the 7 patients underwent valve rereplacement. Redo surgery was necessary in 3, and percutaneous valve-in-valve implantation in the remaining 3 patients. Histological analysis of two explanted valves confirmed significant neointima proliferation and thickened valve cusps leading to stenosis of the graft. CONCLUSIONS These mid-term results after implantation of the No-React® Injectable BioPulmonic™ valve are disappointing. Graft failure was mainly due to neointimal formation and valve malposition.

Implementing and Assessing a Standardized Protocol for Weaning Children Successfully From Extracorporeal Life Support: STANDARDIZED WEANING CHILDREN FROM ECLS

Extracorporeal life support (ECLS) weaning is a complex interdisciplinary process with no clear guidelines. To assess ventricular and pulmonary function as well as hemodynamics including end-organ recovery during ECLS weaning, we developed a standardized weaning protocol. We reviewed our experience 2 years later to assess its feasibility and efficacy. In 2015 we established an inter-professional, standardized, stepwise protocol for weaning from ECLS. If the patient did not require further surgery, weaning was conducted bedside in the intensive care unit (ICU). Most of the weaning procedures are guided via echocardiography. Data acquisition began at baseline level, followed by four-step course (each step lasting 10 min), entailing flow-reduction and ending 30 min after decannulation. Moreover, data from the preprotocol era are presented. Between May 2015 and 2017, 26 consecutive patients (18 male), median age 177 days (2 days-20 years) required ECLS with median support of 4 (2-11) days. Excluding eight not weanable patients, 21 standardized weaning procedures were protocolled in the remaining 18 children. Our generally successful protocol-guided weaning rate (with at least 24-h survival) was 89%, with a discharge home rate of 58%. Practical application of the novel standard protocol seems to facilitate ECLS weaning and to improve its success rate. The protocol can be administered as part of standard bedside ICU assessment.