Photon-counting versus Dual-Source CT of Congenital Heart Defects in Neonates and Infants: Initial Experience.

Background Photon-counting CT (PCCT) has been shown to improve cardiovascular CT imaging in adults. Data in neonates, infants, and young children under the age of 3 years are missing. Purpose To compare image quality and radiation dose of ultrahigh-pitch PCCT with that of ultrahigh-pitch dual-source CT (DSCT) in children suspected of having congenital heart defects. Materials and Methods This is a prospective analysis of existing clinical CT studies in children suspected of having congenital heart defects who underwent contrast-enhanced PCCT or DSCT in the heart and thoracic aorta between January 2019 and October 2022. CT dose index and dose-length product were used to calculate effective radiation dose. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were calculated by standardized region-of-interest analysis. SNR and CNR dose ratios were calculated. Visual image quality was assessed by four independent readers on a five-point scale: 5, excellent or absent; 4, good or minimal; 3, moderate; 2, limited or substantial; and 1, poor or massive. Results Contrast-enhanced PCCT (n = 30) or DSCT (n = 84) was performed in 113 children (55 female and 58 male participants; median age, 66 days [IQR, 15-270]; median height, 56 cm [IQR, 52-67]; and median weight, 4.5 kg [IQR, 3.4-7.1]). A diagnostic image quality score of at least 3 was obtained in 29 of 30 (97%) with PCCT versus 65 of 84 (77%) with DSCT. Mean overall image quality ratings were higher for PCCT versus DSCT (4.17 vs 3.16, respectively; P < .001). SNR and CNR were higher for PCCT versus DSCT with SNR (46.3 ± 16.3 vs 29.9 ± 15.3, respectively; P = .007) and CNR (62.0 ± 50.3 vs 37.2 ± 20.8, respectively; P = .001). Mean effective radiation doses were similar for PCCT and DSCT (0.50 mSv vs 0.52 mSv; P = .47). Conclusion At a similar radiation dose, PCCT offers a higher SNR and CNR and thus better cardiovascular imaging quality than DSCT in children suspected of having cardiac heart defects. © RSNA, 2023.

cAMP Imaging at Ryanodine Receptors Reveals ß2-Adrenoceptor Driven Arrhythmias.

[Figure: see text].

Microplegia in paediatric hearts.

INTRODUCTION: A basic prerequisite for a good surgical outcome in heart surgery is optimal myocardial protection. However, cardioplegia strategies used in adult cardiac surgery are not directly transferable to infant hearts. Paediatric microplegia, analogous to Calafiore cardioplegia used in adult cardiac surgery, offers the advantage of safe myocardial protection without haemodilution. The use of concentration-dependent paediatric microplegia is new in clinical implementation. MATERIAL AND METHODS: Paediatric microplegia has been in clinical use in our institution since late 2014. It is applied via an 1/8 inch tube of a S5-HLM roller pump (LivaNova, Italy). As cardioplegic additive, a mixture of potassium (K) 20 mL (2 mmol/mL potassium chloride 14.9% Braun) and magnesium (Mg) 10 mL (4 mmol/mL Mg-sulphate Verla® i. v. 50%) is fixed into a syringe-pump (B. Braun, Germany). This additive is mixed with arterial patient blood from the oxygenator in different flowdependent ratios to form an effective cardioplegia. TECHNIQUE: After microplegia application of initially 25 mmol/L K with 11 mmol/L Mg for 2 min, a safe cardioplegic cardiac arrest is achieved, which after release of the coronary circulation, immediately returns to a spontaneous cardiac-rhythm. In the case of prolonged aortic clamping, microplegia is repeated every 20 min with a reduction of the application dose of K by 20% and Mg by 30% (20 mmol/L K; 8.5 mmol/L Mg) and a further reduction down to a maintenance dose (15 mmol/L K; 6 mmol/L Mg) after additional 20 min. SUMMARY: The microplegia adapted to the needs of paediatric myocardium is convincing due to its simple technical implementation for the perfusionist while avoiding haemodilution. However, the required intraoperative interval of microplegia of approx. 20 min demands adapted intraoperative management from the surgeon.

Transfontanellar Contrast-enhanced US for Intraoperative Imaging of Cerebral Perfusion during Neonatal Arterial Switch Operation.

Background Brain injury and subsequent neurodevelopmental disorders are major determinants for later-life outcomes in neonates with transposition of the great arteries (TGA). Purpose To quantitatively assess cerebral perfusion in neonates with TGA undergoing arterial switch operation (ASO) using transfontanellar contrast-enhanced US (T-CEUS). Materials and Methods In a prospective single-center cross-sectional diagnostic study, neonates with TGA scheduled for ASO were recruited from February 2018 to February 2020. Measurements were performed at five time points before, during, and after surgery (T1-T5), and 11 perfusion parameters were derived per cerebral hemisphere. Neonate clinical characteristics, heart rate, mean arterial pressure, central venous pressure, near-infrared spectroscopy, blood gas analyses, ventilation time, time spent in the pediatric intensive care unit, and time in hospital were correlated with imaging parameters. Analysis of variance or a mixed-effects model were used for groupwise comparisons. Results A total of 12 neonates (mean gestational age, 39 6/7 weeks ± 1/7 [SD]) were included and underwent ASO a mean of 6.9 days ± 3.4 after birth. When compared with baseline values, T-CEUS revealed a longer mean time-to-peak (right hemisphere, 4.3 seconds ± 2.1 vs 17 seconds ± 6.4 [P < .001]; left hemisphere, 4.0 seconds ± 2.3 vs 21 seconds ± 8.7 [P < .001]) and rise time (right hemisphere, 3.5 seconds ± 1.7 vs 11 seconds ± 5.1 [P = .002]; left hemisphere, 3.4 seconds ± 2.0 vs 22 seconds ± 7.8 [P = .004]) in both cerebral hemispheres during low-flow cardiopulmonary bypass and hypothermia (T4) for all neonates. Neonate age at surgery negatively correlated with T-CEUS parameters during ASO, as calculated with the area under the flow curve (AUC) during wash-in (R = -0.60, P = .020), washout (R = -0.82, P = .002), and both wash-in and washout (R = -0.79, P = .004). Mean AUC values were lower in neonates older than 7 days compared with younger neonates during wash-in ([87 arbitrary units {au} ± 77] × 102 vs [270 au ± 164] × 102, P = .049]), washout ([15 au ± 11] × 103 vs [65 au ± 38] × 103, P = .020]) and both wash-in and washout ([24 au ± 18] × 103 vs [92 au ± 53] × 103, P = .023). Conclusion Low-flow hypothermic conditions resulted in reduced cerebral perfusion, as measured with transfontanellar contrast-enhanced US, which inversely correlated with age at surgery. Clinical trial registration no. NCT03215628 © RSNA, 2022 Online supplemental material is available for this article.

Association of Lymphatic Abnormalities with Early Complications after Fontan Operation.

BACKGROUND: Increased central venous pressure is inherent in Fontan circulation but not strongly related to Fontan complication. Abnormalities of the lymphatic circulation may play a crucial role in early Fontan complications. METHODS: This was a retrospective, single-center study of patients undergoing Fontan operation from 2008 to 2015. The primary outcome was significant early Fontan complication defined as secondary in-hospital treatment due to peripheral edema, ascites, pleural effusions, protein-losing enteropathy, or plastic bronchitis. All patients received T2-weighted magnetic resonance images to assess abdominal and thoracic lymphatic perfusion pattern 6 months after Fontan completion with respect to localization, distribution, and extension of lymphatic perfusion pattern (type 1-4) and with application of an area score (0-12 points). RESULTS: Nine out of 42 patients developed early Fontan complication. Patients with complication had longer chest tube drainage (mean 28 [interquartile range [IQR]: 13-60] vs. 13 [IQR: 2-22] days, p = 0.01) and more often obstructions in the Fontan circuit 6 months after surgery (56 vs. 15%, p = 0.02). Twelve patients showed little or no abnormalities of lymphatic perfusion (lymphatic perfusion pattern type 1). Most frequently magnetic resonance imaging showed lymphatic congestion in the supraclavicular region (24/42 patients). Paramesenteric lymphatic congestion was observed in eight patients. Patients with early Fontan complications presented with higher lymphatic area score (6 [min-max: 2-10] vs. 2 [min-max: 0-8]), p = 0.001) and greater distribution and extension of thoracic lymphatic congestion (type 3-4: n = 5/9 vs. n = 1/33, p = 0.001). CONCLUSION: Early Fontan complication is related to hemodynamic factors such as circuit obstruction and to the occurrence and extent of lymphatic congestion.

Reduction of exposure to plasticizers in stored red blood cell units.

INTRODUCTION: Plastic can be toxic and hazardous to an organism's health, but it is being widely used in our daily lives. Di-2-ethylhexyl-phthalate is the most common plasticizer in medical devices made of polyvinylchloride and is commonly found in soft bags storing red blood cell units. Di-2-ethylhexyl-phthalate and its degradation product mono-2-ethylhexyl-phthalate can migrate into human body fluids, for example, blood and tissues. The aim of the study was to assess the concentration of plasticizers in red blood cell units according to storage time and after mechanical rinsing using a cell salvage device. METHODS: Levels of di-2-ethylhexyl-phthalate and mono-2-ethylhexyl-phthalate were analysed in 50 unwashed red blood cell units using liquid chromatography coupled with tandem mass spectrometry. In addition, phthalate concentrations were measured before and after mechanical rinsing in six more washed red blood cell units with storage times ranging between 36 and 56 days. A linear regression model was determined by the daily increase of di-2-ethylhexyl-phthalate and mono-2-ethylhexyl-phthalate in the stored red blood cell units subject to their storage time (range = 4-38 days), and the effect of mechanical rinsing on their phthalate concentration was calculated. RESULTS: A linear correlation was found between storage time of unwashed red blood cell units and the concentration of di-2-ethylhexyl-phthalate (p < 0.001) or mono-2-ethylhexyl-phthalate (p < 0.001). Stored red blood cell units older than 14 days had significantly higher concentrations of both contaminants than red blood cell units of shorter storage time (p < 0.001). Mechanical rinsing in washed red blood cell units attained a reduction in the di-2-ethylhexyl-phthalate and mono-2-ethylhexyl-phthalate concentration by a median of 53% (range = 18-68%; p = 0.031) and 87% (range = 68-96%; p = 0.031), respectively. CONCLUSION: Leaching of di-2-ethylhexyl-phthalate and mono-2-ethylhexyl-phthalate into red blood cell units depends on the duration of storage time. Plasticizers can be significantly reduced by mechanical rinsing using cell salvage devices, and thus, red blood cell units can be regenerated with respect to chemical contamination.

Multisite measurement of regional oxygen saturation in Fontan patients with and without protein-losing enteropathy at rest and during exercise.

BACKGROUND: Protein-losing enteropathy (PLE) is a severe complication of Fontan circulation with increased risk of end-organ dysfunction. We evaluated tissue oxygenation via near-infrared spectroscopy (NIRS) at different exercise levels in Fontan patients. METHODS: Assessment of multisite NIRS during cycle ergometer exercise and daily activities in three groups: Fontan patients with PLE; without PLE; patients with dextro-transposition of the great arteries (d-TGA); comparing univentricular with biventricular circulation and Fontan with/without PLE. Renal threshold analysis (<65%;<55%;<45%) of regional oxygen saturation (rSO2) was performed. RESULTS: Fontan patients showed reduced rSO2 (p < 0.05) in their quadriceps femoris muscle compared with biventricular d-TGA patients at all time points. rSO2 in renal tissue was reduced at baseline (p = 0.002), exercise (p = 0.0062), and daily activities (p = 0.03) in Fontan patients with PLE. Renal threshold analysis identified critically low renal rSO2 (rSO2 < 65%) in Fontan patients with PLE during exercise (95% of monitoring time below threshold) and daily activities (83.7% time below threshold). CONCLUSION: Fontan circulation is associated with decreased rSO2 values in skeletal muscle and hypoxemia of renal tissue solely in patients with PLE. Reduced rSO2 already during activities of daily life, might contribute to comorbidities in patients with Fontan circulation, including PLE and renal failure.

First experience with Tolvaptan for the treatment of neonates and infants with capillary leak syndrome after cardiac surgery.

BACKGROUND: Postoperative fluid management in critically ill neonates and infants with capillary leak syndrome (CLS) and extensive volume overload after cardiac surgery on cardiopulmonary bypass is challenging. CLS is often resistant to conventional diuretic therapy, aggravating the course of weaning from invasive ventilation, increasing length of stay on ICU and morbidity and mortality. METHODS: Tolvaptan (TLV, vasopressin type 2 receptor antagonist) was used as an additive diuretic in neonates and infants with CLS after cardiac surgery. Retrospective analysis of 25 patients with CLS including preoperative and postoperative parameters was performed. Multivariate regression analysis was performed to identify predictors for TLV response. RESULTS: Multivariate analysis identified urinary output during 24 h after TLV administration and mean blood pressure (BP) on day 2 of TLV treatment as predictors for TLV response (AUC = 0.956). Responder showed greater weight reduction (p < 0.0001), earlier weaning from ventilator during TLV (p = 0.0421) and shorter time in the ICU after TLV treatment (p = 0.0155). Serum sodium and serum osmolality increased significantly over time in all patients treated with TLV. CONCLUSION: In neonates and infants with diuretic-refractory CLS after cardiac surgery, additional aquaretic therapy with TLV showed an increase in urinary output and reduction in bodyweight in patients classified as TLV responder. Increase in urinary output and mean BP on day 2 of treatment were strong predictors for TLV response.

Multicenter Experience With Mechanical Circulatory Support Using a New Diagonal Pump in 233 Children.

Technological innovations in pediatric extracorporeal life support circuits can reduce system-related complications and may improve patients' outcome. The Deltastream DP3 (Medos Medizintechnik AG, Stolberg, Germany) is a novel rotational pump with a diagonally streamed impeller that can be used over a broad range of flows. We collected patient data from seven pediatric centers to conduct a retrospective cohort study. We examined 233 patients whose median age was 1.9 (0-201) months. The DP3 system was used for cardiopulmonary support as veno-arterial extracorporeal membrane oxygenation (ECMO) in 162 patients. Respiratory support via veno-venous ECMO was provided in 63 patients. The pump was used as a ventricular assist device in eight patients. Median supporting time was 5.5 (0.2-69) days and the weaning rate was 72.5%. The discharge home rate was 62% in the pulmonary group versus 55% in the cardiac group. Extracorporeal cardiopulmonary resuscitation was carried out in 24 patients (10%) with a survival to discharge of rate of 37.5%. About 106 (47%) children experienced no complications, while 33% suffered bleeding requiring blood transfusion or surgical intervention. Three patients suffered a fatal cerebral event. Renal replacement therapy was performed in 28% and pump or oxygenator exchange in 26%. Multivariable analysis identified system exchange (OR 1.94), kidney failure (OR 3.43), and complications on support (OR 2.56) as risk factors for dismal outcome. This novel diagonal pump has demonstrated its efficacy in all kinds of mechanical circulatory and respiratory support, revealing good survival rates.

A new approach of three-dimensional guidance in paediatric cath lab: segmented and tessellated heart models for cardiovascular interventions in CHD.

BACKGROUND: Optimal imaging is essential for catheter-based interventions in CHD. The three-dimensional models in volume-rendering technique currently in use are not standardised. This paper investigates the feasibility and impact of novel three-dimensional guidance with segmented and tessellated three-dimensional heart models in catheterisation of CHD. In addition, a nearly radiation-free two- to three-dimensional registration and a biplane overlay were used.Methods and resultsWe analysed 60 consecutive cases in which segmented tessellated three-dimensional heart models were merged with live fluoroscopy images and aligned using the tracheal bifurcation as a fiducial mark. The models were generated from previous MRI or CT by dedicated medical software. We chose the stereo-lithography format, as this promises advantage over volume-rendering-technique models regarding visualisation. Prospects, potential benefits, and accuracy of the two- to three-dimensional registration were rated separately by two paediatric interventionalists on a five-point Likert scale. Fluoroscopy time, radiation dose, and contrast dye consumption were evaluated. Over a 10-month study period, two- to three-dimensional image fusion was applied to 60 out of 354 cases. Of the 60 catheterisations, 73.3% were performed in the context of interventions. The accuracy of two- to three-dimensional registration was sufficient in all cases. Three-dimensional guidance was rated superior to conventional biplane imaging in all 60 cases. We registered significantly smaller amounts of used contrast dye (p<0.01), lower levels of radiation dose (p<0.02), and less fluoroscopy time (p<0.01) during interventions concerning the aortic arch compared with a control group. CONCLUSIONS: Two- to three-dimensional image fusion can be applied successfully in most catheter-based interventions of CHD. Meshes in stereo-lithography format are accurate and base for standardised and reproducible three-dimensional models.

Accuracy and Specific Value of Cardiovascular 3D-Models in Pediatric CT-Angiography.

Computed tomography (CT)-angiography is routinely performed prior to catheter-based and surgical treatment in congenital heart disease. To date, little is known about the accuracy and advantage of different 3D-reconstructions in CT-data. Exact anatomical information is crucial. We analyzed 35 consecutive CT-angiographies of infants with congenital heart disease. All datasets are reconstructed three-dimensionally using volume rendering technique (VRT) and threshold-based segmentation (stereolithographic model, STL). Additionally, the two-dimensional maximum intensity projection (MIP) reconstructs two-dimensional data. In each dataset and resulting image, measurements of vascular diameters for four different vessels were estimated and compared to the reference standard, measured via multiplanar reformation (MPR). The resulting measurements obtained via the STL-images, MIP-images, and the VRT-images were compared with the reference standard. There was a significant difference (p < 0.05) between measurements. The mean difference was 0.0 for STL-images, -0.1 for MIP-images, and -0.3 for VRT-images. The range of the differences was -0.7 to 1.0 mm for STL-images, -0.6 to 0.5 mm for MIP-images and -1.1 to 0.7 mm for VRT-images. There was an excellent correlation between the STL-, MIP-, VRT-measurements, and the reference standard. Inter-reader reliability was excellent (p < 0.01). STL-models of cardiovascular structures are more accurate than the traditional VRT-models. Additionally, they can be standardized and are reproducible.

Aortic Arch Reconstruction in Neonates with Biventricular Morphology: Increased Risk for Development of Recoarctation by Use of Autologous Pericardium.

BACKGROUND: The aim of this study was to analyze risk factors promoting development of recoarctation (Re-CoA) in neonates who survived aortic arch repair from an anterior approach. METHODS: Fifty consecutive neonates with biventricular morphology and ductal-dependent lower body perfusion who were discharged home following aortic arch repair with cardiopulmonary bypass between 2000 and 2012 were retrospectively reviewed. Arch anatomy was either interruption (n = 10) or hypoplasia with coarctation (n = 40). Aortic arch reconstruction was performed by using patch material (bovine pericardium, n = 30, homograft, n = 10, or glutaraldehyde-treated autologous pericardium, n = 7), and three patients underwent direct end-to-side anastomosis. Antegrade cerebral and continuous myocardial perfusion was performed in 39 and 21 patients, respectively. Kaplan-Meier freedom from Re-CoA was calculated. Morphologic and perioperative data indicating increased risk of Re-CoA by univariate analysis were included in multivariate Cox regression analysis. RESULTS: Mean follow-up was 5.3 ± 4.1 years. Re-CoA occurred in 13 patients and was treated successfully by balloon dilatation (n = 6) or surgery (n = 7). Freedom from Re-CoA after 1 and 5 years was 83 ± 5 and 79 ± 6%, respectively. Two patients died early after surgical repair of Re-CoA. The use of autologous pericardium for aortic arch augmentation was the only independent risk factor for development of Re-CoA (hazard ratio: 4.3 [95% confidence interval: 1.2-16.1]; p = 0.028). CONCLUSION: Re-CoA following neonatal aortic arch surgery can be treated by balloon dilatation or surgery, if adequate. In this study, the risk for development of Re-CoA was independently increased by the use of autologous pericardium during initial arch repair.

Assessment of cavopulmonary connections by advanced imaging: value of flat-detector computed tomography.

OBJECTIVES: To investigate the impact of flat-detector computed tomography on the clinical assessment of patients with cavopulmonary connections, and to evaluate the obtained diagnostic accuracy and supplementary information, as well as the value of overlaid three-dimensional reconstructions on fluoroscopic images during catheter-based interventions. METHODS: We analysed 31 consecutive patients retrospectively in whom flat-detector computed tomography was used to visualise the cavopulmonary connection. We investigated patients with cavopulmonary connections either early post-operatively (first group), before converting to a total cavopulmonary connection (second group), and patients with failing total cavopulmonary connection (third group). Flat-detector computed tomography based on a single rotational angiography was used to create a three-dimensional vascular model. The clinical value of flat-detector computed tomography was evaluated using standard categories of diagnostic utility. Used contrast volume and radiation exposure were quantified. RESULTS: Within 18 months, flat-detector computed tomography was performed in 31 cases with cavopulmonary connections. The median age was 1.9 years (range 0.3-43 years). In the first group, we found anomalies in 4 out of 8 cases, which led to therapeutic or prophylactic procedures; in the second and third groups, we performed interventions in 14 out of 23 cases. The overall clinical value was always rated superior to conventional biplane angiography. The median dose area product was 91.8 microgray square metres (range 33.0-679.3 microgray square metres). The required contrast medium was 2.08 millilitres per kilogram (range 0.66-4.7 millilitres per kilogram). CONCLUSION: Flat-detector computed tomography improves the diagnostic accuracy in cavopulmonary connections and provides additional diagnostic information, which may lead to therapeutic or prophylactic procedures. Overlaid three-dimensional images on fluoroscopy facilitate and provide security for interventions.

Minimal invasive approaches for pediatric & congenital heart surgery: safe, reproducible, more cosmetic than through sternotomy, and here to stay.

Minimal invasive approaches through small thoracic incisions for the isolated repair of the most common congenital heart defects have been around for decades. However, the lack of belonging in established surgical training curriculums compared to the traditional median sternotomy, the requirement for more technical expertise and a certain learning curve, has limited their use, being routinely performed only by certain surgeons in specialized centers. More recently, through cumulated and increasingly mediatized shared experience, remote teaching potential through universally accessible surgical videos and simulation, the approach has gained traction and acceptance, and even established itself as the new norm in many centers. In this review, we present technically focused aspects of our own experience and protocols which have evolved over time, along with a brief overview of the literature pertaining to other right thoracic approaches, and some comparison to established results using the traditional median sternotomy. An increasing body of literature, produced more frequently and across all continents, seems to suggest that repairs of congenital heart defects through a minimal invasive right thoracic approach are becoming the new norm, as they are reported to be safe and reproducible, with excellent surgical results, and an obvious superior and more desirable cosmetic result. This comes at a cost of additional training and learning curve by surgeons, who are not offered the technique as part of their standard professional training curriculum.

Equal cerebral perfusion during extended aortic coarctation repair.

OBJECTIVES: Aortic coarctation with distal aortic arch hypoplasia can be effectively addressed by coarctation resection with extended end-to-end-anastomosis (REEEA). Particularly, when unilateral cerebral perfusion (UCP) is established by clamping of left-sided supra-aortic vessels, the extent of cerebral blood flow distribution during repair remains undetermined, so far. Transfontanellar contrast-enhanced ultrasound (T-CEUS) can be utilized for real-time visualization and quantitative evaluation of cerebral blood flow. This study quantitatively evaluates cerebral perfusion during REEEA by using intraoperative T-CEUS. METHODS: In a prospective study, 9 infants with open fontanelle undergoing REEEA [median age: 13 days (range 1-34) and median weight 3.1 kg (range 2.2-4.4)] were intraoperatively examined with T-CEUS at 3 consecutive time-points: before skin incision, during UCP and after skin suture. A software-based analysis of 11 parameters was used for data evaluation. Absolute and relative blood flow in contralateral hemispheres was measured in side-by-side comparison, and referenced to baseline measurements. RESULTS: No side-depend absolute or relative cerebral perfusion differences were found during REEEA, except for an increased relative 'wash-out-rate' (P = 0.0013) in favour of the right hemisphere after surgery. Compared to ipsilateral baseline levels, 'rise time' was transiently increased in right (P = 0.0277) and 'time-to-peak' in both hemispheres (right: P = 0.0403 and left: P = 0.0286), all during UCP. CONCLUSIONS: The use of T-CEUS provided evidence for homogenous distribution of contrast agent in both hemispheres during UCP. T-CEUS can be utilized for the postprocedural evaluation of cerebral perfusion during congenital cardiac surgery. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov Unique, Identifier: NCT03215628.

The value of flat-detector computed tomography during catheterisation of congenital heart disease.

OBJECTIVES: To analyse the diagnostic utility of flat-detector computed tomography imaging (FD-CT) in patients with congenital heart disease, including the value of image fusion to overlay three-dimensional (3D) reconstructions on fluoroscopic images during catheter-based interventions. METHODS: We retrospectively analysed 62 consecutive paediatric patients in whom FD-CT was used during catheterisation of congenital heart disease. Expert operators rated the clinical value of FD-CT over conventional fluoroscopic imaging. Added radiation exposure and contrast medium volume were evaluated. RESULTS: During a 12-month period, FD-CT was performed in 62 out of 303 cardiac catheterisations. Median patient age was 3.5 years. In 32/62 cases, FD-CT was used for diagnostic purposes, in 30/62 cases it was used in the context of interventions. Diagnostic utility was never rated as "misleading". It was classified as "not useful" in six cases (9.7%), "useful" in 18 cases (29.0%), "very useful" in 37 cases (59.7%) and "essential" in one case (1.6%). The median added dose-area product was 111.0 µGym(2), the required additional quantity of contrast medium was 1.6 ml/kg. CONCLUSION: FD-CT provides useful diagnostic information in most of the patients investigated for congenital heart disease. The added radiation exposure and contrast medium volume are reasonable.

Right aortic arch forming a true vascular ring: a clinical review.

OBJECTIVES: This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided ligamentum forming a complete vascular ring around the oesophagus and trachea. METHODS: A systematic database search for appropriate literature was conducted on PubMed/MEDLINE. Articles were considered relevant when providing details on the presentation, diagnosis and surgical treatment of this specific congenital arch anomaly in human beings. RESULTS: Affected patients present with respiratory and/or oesophageal difficulties due to tracheoesophageal compression. Conservative treatment might be reasonable in asymptomatic or mildly symptomatic cases; however, once moderate-to-severe symptoms develop, surgical intervention is definitely indicated. Surgery is commonly performed through a left thoracotomy or median sternotomy and includes the division of the left ductal ligamentum; if a Kommerell's diverticulum is present that is >1.5 times the diameter of the subclavian artery, then concomitant resection of the large diverticulum and translocation of the aberrant left subclavian artery is also conducted. Postoperative morbidity and mortality are low and are rather related to concomitant intracardiac and extracardiac anomalies than to the procedure itself. In a majority of patients, full resolution of symptoms is seen within months to years from the surgery. Nevertheless, there is also a subset of patients who remain with some tracheobronchial narrowing, sometimes even requiring reintervention during follow-up due to persisting or recurring symptoms. CONCLUSIONS: Overall, the surgical management of a right aortic arch forming a true vascular ring in infancy, childhood and adulthood seems relatively safe and effective in providing symptomatic relief despite some persistent tracheobronchial and/or oesophageal narrowing in some cases.

Heart failure in the young: Insights into myocardial recovery with ventricular assist device support.

BACKGROUND: Data on ventricular unloading-promoted myocardial recovery and post-weaning outcome in children is scarce. We analyzed the weaning outcome in children with heart failure (HF) supported with ventricular assist device (VAD). METHODS: A multi-institutional data on VAD implanted in 193 children and adolescents with HF between April 1990 and November 2015 was reviewed. Among them, 25 children (mean age 3.4±3.0, range, 0.058-16.3 years, 15 females) were weaned from VAD. Etiology of HF were myocarditis (n=11), dilated cardiomyopathy (DCMP) (n=7), ischemic HF (n=3), arrhythmogenic CMP (n=1), post-correction of congenital heart disease (CHD) (n=1) and acute graft failure (n=1). Mean duration of HF before VAD implantation was 59.4±3 days. RESULTS: Age, duration of HF, DCMP, cardiac arrest and duration of VAD are essential clinical characteristics to delineate who may have the potential to myocardial recovery. Echocardiographic parameters pre-implantation, during the final off-pump trial and during the post-explantation follow-ups revealed that LVEF, LVEDD and relative wall thickness (RWT) showed significant differences (P<0.001) among patients stratified by outcome to assess recovery. Presently, 21 (84.0%) of the weaned patients are alive with their native hearts 1.3-19.1 years after VAD explantation. An additional weaned patient had HF recurrence 3 months post-weaning and was transplanted. CONCLUSIONS: Post-weaning myocardial recovery and cardiac stability of children with HF from several etiologies supported with a VAD appears sustainable and durable. Young patients with short HF duration are more likely to recover. Absence of cardiac arrest, cardiac size, geometry and function may prospectively identify patients who may be likely to have myocardial recovery.

Sensitivity of High-Pitch Dual-Source Computed Tomography for the Detection of Anomalous Pulmonary Venous Connection in Infants.

PURPOSE: To evaluate the sensitivity, specificity, and interobserver reliability of high-pitch dual-source computed tomography angiography (CTA) in the detection of anomalous pulmonary venous connection (APVC) in infants with congenital heart defects and to assess the associated radiation exposure. MATERIALS AND METHODS: 78 pulmonary veins in 17 consecutively enrolled patients with congenital heart defects (6 females; 11 males; median age: 6 days; range: 1-299 days) were retrospectively included in this study. All patients underwent high-pitch dual-source CTA of the chest at low tube voltages (70 kV). APVC was evaluated independently by two radiologists. Sensitivity, specificity, positive (PPV) and negative predictive values (NPV), and interobserver agreement were determined. For standard of reference, one additional observer reviewed CT scans, echocardiography reports, clinical reports as well as surgical reports. In cases of disagreement the additional observer made the final decision based on all available information. RESULTS: Detection of APVC with high-pitch dual-source CTA revealed a good sensitivity (91 %) and specificity (99 %), with PPV and NPV of 98 % and 97 %. Interobserver agreement was almost perfect (Kappa = 0.84). The median DLP was 3.8 mGy*cm (IQR 3.3-4.7 mGy*cm) and the median radiation dose was 0.33 mSv (IQR 0.26-0.39 mSv). CONCLUSION: High-pitch dual-source CTA in infants with congenital heart defects allows for accurate and reliable assessment of APVC at a low radiation dose. KEY POINTS: · High-pitch dual-source CTA enables detection of anomalous pulmonary vein connection with high sensitivity in infants.. · Interrater reliability in the detection of anomalous pulmonary vein connection with high-pitch dual-source CTA is almost perfect.. · Radiation dose of high-pitch dual-source CTA in the cardiac examination of infants is low.. CITATION FORMAT: · Well L, Weinrich JM, Meyer M et al. Sensitivity of High-Pitch Dual-Source Computed Tomography for the Detection of Anomalous Pulmonary Venous Connection in Infants. Fortschr Röntgenstr 2021; 193: 551 - 558.