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Abstract. We compared the left ventricular Tei index measured by tissue Doppler imaging (TDI) to that obtained by pulsed Doppler (PW) in patients with congenital heart disease. In 40 consecutive [...]

Abstract. This study aimed to quantify paradoxical interventricular septal motion (PSM) among 20 patients following tetralogy of Fallot (TOF) repair without severe pulmonary regurgitation and 20 age-matched normal subjects. PSM [...]

Coronary artery lesions represent rare conditions in pediatric congenital heart disease and mainly include coronary artery stenoses (CAS) or coronary artery fistulae (CAF). Due to the small vessel size, pediatric percutaneous coronary interventions (PCI) are demanding and studies concerning long-term results are missing. In this retrospective study, we analyzed indications, procedural details, and post-procedural outcomes in pediatric patients who underwent PCI in our institution. For CAS treatment, procedural success was defined as efficient coronary revascularization with a significant improvement of coronary perfusion. CAF treatment was considered successful, when no residual shunt was detectable. From 1995 to 2020, 32 pediatric patients aged ≤ 18 years received interventional treatment for CAS (n = 24/32) or CAF (n = 8/32). Reasons for CAS were post-surgical (n = 15/24) or post-transplant (n = 9/24). Interventional treatment strategies included coronary angioplasty (20/43), stent placement (10/43), and a combination of both (13/43). In-hospital mortality occurred in 6/24 patients and late mortality in 5/24 patients leading to an overall 5-year survival of 62.5%. Early mortality mainly occurred due to post-ischemic myocardial failure. CAF occlusion was performed using coil embolization (n = 3), placement of vascular plugs (n = 3), a combination of both (n = 1), or a combination of coil embolization and a covered stent (n = 1). Treatment of coronary fistulae was successful in all patients with excellent post-procedural results and no follow-up death. PCI in pediatric patients with congenital heart disease can be performed safely and effectively. However, the overall 5-year survival probability of patients with CAS is reduced due to severe ischemic myocardial damage., (© 2021. The Author(s).)

Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). In this study, we performed a detailed hemodynamic and hepatic assessment to analyze the incidence and spectrum of FALD and its association with patients' hemodynamics. From 2017 to 2019, 145 patients underwent a detailed, age-adjusted hepatic examination including laboratory analysis (FibroTest ® , n = 101), liver ultrasound (n = 117) and transient elastography (FibroScan ® , n = 61). The median patient age was 16.0 years [IQR 14.2], and the median duration of the Fontan circulation was 10.3 years [IQR 14.7]. Hemodynamic assessment was performed using echocardiography, cardiopulmonary exercise capacity testing and cardiac catheterization. Liver ultrasound revealed hepatic parenchymal changes in 83 patients (70.9%). Severe liver cirrhosis was detectable in 20 patients (17.1%). Median liver stiffness measured by FibroScan ® was 27.7 kPa [IQR 14.5], and the median Fibrotest ® score was 0.5 [IQR 0.3], corresponding to fibrosis stage ≥ 2. Liver stiffness values and Fibrotest ® scores correlated significantly with Fontan duration (P 1  = 0.013, P 2  = 0.012). Exercise performance was significantly impaired in patients with severe liver cirrhosis (P = 0.003). Pulmonary artery pressure and end-diastolic pressure were highly elevated in cirrhotic patients (P 1  = 0.008, P 2  = 0.003). Multivariable risk factor analysis revealed Fontan duration to be a major risk factor for the development of FALD (P < 0.001, OR 0.77, CI 0.68-0.87). In the majority of patients, hepatic abnormalities suggestive of FALD were detectable by liver ultrasound, transient elastography and laboratory analysis. The severity of FALD correlated significantly with Fontan duration and impaired Fontan hemodynamics. A detailed hepatic assessment is indispensable for long-term surveillance of Fontan patients.

In August 2010, the Nit-Occlud ® Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud ® Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.2 years, mean weight 28.82 kg), 81 perimembranous VSDs (48 with aneurysm), 30 muscular VSDs, mean procedure time was 121.1 min, and mean fluoroscopy time was 26.3 min. Short- and midterm term follow-up was possible in 100/102 patients, there was 1 embolization and 1 explantation after 24 months. Immediate complete closure occurred in 49 of 101 patients (48.5%), trivial residual shunt was present in 51 (50.0%), closure rate was 95% after 6 months and 97% after 1 year. Out of the 102 patients, there were 2 severe complications (1.8%) (1 severe hemolysis, 1 embolization) and 8 moderate/transient (=7.2%) including 1 transient AV block. During a mean follow-up period of 31.3 months (range 24-48) and a total follow-up time of 224.75 patient years, no further problems occurred. VSD closure with the Nit-Occlud ® Lê VSD coil is feasible and safe with a minimal risk of severe side effects. The long-term effects and safety require further clinical follow-up studies.

Propofol is a short-acting, intravenously administered hypnotic agent which is used in procedural sedation in children. Propofol is known to decrease systemic vascular resistance, arterial blood pressure and can lead to desaturations and decreased systemic perfusion in children with cardiac shunting. This may result in a reduction in cerebral blood flow and oxygenation. Near-infrared spectroscopy (NIRS) can monitor cerebral tissue oxygenation in the frontal neocortex. The objective of our study was to measure the changes in cerebral oxygen and blood supply after Propofol infusion in children with congenital heart disease. Propofol infusion may reduce cerebral oxygenation in children with congenital heart disease. The study group consisted of 32 children (f:m = 18:14), with median age of 49 (5-112) months and median weight of 15 (5-34) kg. We performed NIRS derived continuous measurement of cerebral oxygenation and cardiac output using Electrical velocimetry for 5 min before and after sedation with Propofol (1-2 mg/kg i.v.) for cardiac catheterization. Simultaneously, non-invasive arterial blood pressure and transcutaneous oxygen saturation were measured. Propofol sedation led to a significant decrease in mean arterial pressure (79 ± 16 vs. 67 ± 12 mmHg) (p = 0.01) and cardiac index (3.2 ± 0.8 vs. 2.9 ± 0.6 ml/min/m(2)) (p = 0.03). In contrast, cerebral tissue oxygenation index, increased significantly from 57 ± 11 to 59 ± 10 % (p < 0.05). Sedation with Propofol increased cerebral tissue oxygenation despite a decrease in cardiac index and arterial blood pressure. This may be caused by a decreased oxygen consumption of the sedated brain with intact cerebral auto-regulation.