Exceptional case of aortic atresia associated with atypical double aortic arch

We present the unique case of atretic aortic valve associated with hypoplastic ascending aorta and double aortic arch of unusual configuration supplying retrograde blood flow to the coronary arteries

Significant adhesion reduction and time saving in pediatric heart surgery with 4DryField PH: A retrospective, controlled study

Adhesions formation after surgery for congenital heart defects can complicate follow-up procedures due to bleeding from detached adhesion bands, injury to cardiac structures or large vessels, all of which do prolong operation times. The problem is enhanced by the fact that detached adhesions are predilection sites for new adhesions setting off a downward spiral. 4DryField® PH gel barrier has demonstrated high efficacy in reducing postoperative adhesions in general surgical and gynecological studies. This retrospective controlled study of 22 patients evaluates whether these positive results can be confirmed in pediatric cardiac surgery. Adhesions were scored from photographs of follow-up interventions by an independent cardiac surgeon blinded to group assignment. The publication provides not only score numbers but also original photographs of all sites for better traceability and transparency. In addition, timesaving due to reduced adhesions was evaluated. Results show a significantly reduced adhesion score for the 4DryField® group. Importantly, this resulted in a significantly shorter period between skin incision and start of cardiopulmonary bypass. In addition, timesaving due reduced adhesion formation was evaluated. The use of 4DryField® was safe, although higher doses per kg were used than in adults

Absent left main coronary artery in a case of pulmonary atresia-intact ventricular septum and right ventricle-dependent coronary circulation

Right ventricle-dependent coronary circulation coexisting with left main coronary atresia in the setting of pulmonary atresia-intact ventricular septum is rare. In the case described, the left coronary artery (LCA) origin from the aorta could not be found on conventional angiography or cardiac magnetic resonance imaging. During surgery, multiple LCA branches originating from the finger-like continuum of the primitive right ventricular sinusoidal network were observed. A Damus-Kaye-Stansel anastomosis and an aortopulmonary shunt operation were performed. Shunt takedown and a bidirectional Glenn anastomosis followed at 3 months of age. At 18 months follow-up, the child is thriving with stable hemodynamics and a saturation of 85%. Awareness about this rare coronary artery anomaly is necessary to prevent catastrophic consequences. The challenges, complications, and lessons learned while treating this rare variant are discussed

Risk Factors and Outcome of Pulmonary Artery Stenting After Bidirectional Cavopulmonary Connection (BDCPC) in Single Ventricle Circulation

After bidirectional cavopulmonary connection (BDCPC) central pulmonary arteries (PAs) of single ventricle (SV) patients can be affected by stenosis or even closure. Aim of this study is to compare SV patients with and without PA-stent implantation post-BDCPC regarding risk factors for stent implantation and outcome. Single center, retrospective (2006-2021) study of 136 SV consecutive patients with and without PA-stent implantation post-BDCPC. Patient characteristics, risk factors for PA-stent implantation and PA growth were assessed comparing angiographic data pre-BDCPC and pre-TCPC. A total of 40/136 (29%) patients underwent PA-stent implantation at median (IQR) 14 (1.1-39.0) days post-BDCPC. 37/40 (92.5%) underwent LPA-stenting. Multiple regression analysis showed single LV patients to receive less likely PA-stents than single RV patients (OR 0.41; p = 0.05). Reduced LPA/BSA (mm/m2) and larger diameter of neo-ascending aorta pre-BDCPC were associated with an increased likelihood of PA-stent implantation post-BDCPC (OR 0.89, p = 0.03; OR 1.05, p = 0.001). Stent re-dilatation was performed in 36/40 (89%) after 1 (0.8-1.5) year. Pulmonary artery diameters pre-BDCPC were lower in the PA-stent group: McGoon (p < 0.001), Nakata (p < 0.001). Indexed pulmonary artery diameters increased equally in both groups but remained lower pre-TCPC in the PA-stent group: McGoon (p < 0.001), Nakata (p = 0.009), and Lower Lobe Index (p = 0.003). LPA and RPA grew symmetrically in both groups. Single RV, larger neo-ascending aorta, and small LPA pre- BDCPC are independent risk factors for PA-stent implantation post-BDCPC. Pulmonary artery diameters after PA-stent implantation and stent re-dilatation showed significant growth together with the contralateral side, but the PA-system remained symmetrically smaller in the stent group

Transannular bovine jugular vein monocusp for the reconstruction of severe right ventricular outflow tract obstruction

OBJECTIVE Severe right ventricular outflow tract obstruction in tetralogy of Fallot and variants necessitates the use of transannular patch in a significant proportion of children undergoing repair. We have used a Contegra monocusp together with delamination of native leaflet tissue in order to create a functioning pulmonary valve. METHODS In total, 18 (2017-2022) consecutive Contegra monocusp implantations were included. Median age and weight were 3.65 [2.00; 9.43] months and 6.12 [4.30; 8.22] kg, respectively. Nine of 18 patients had undergone palliation. Native pulmonary leaflet tissue was recruited to create a single posterior cusp. Contegra monocusp selection was based on the goal to achieve a neoannulus of Z value ≈ 0. Monocusp sizes implanted were 16 [14; 18] mm. Patch plasty of left pulmonary artery (LPA) (9), right pulmonary artery (RPA) (2), and both LPA-RPA (5) were often performed. RESULTS All patients survived the operation and were discharged home in good health. Median ventilation time and hospital stay were 2 [1; 9] and 12.5 [9; 54] days, respectively. Follow-up duration was 30.68 [3.47; 60.47] months and 100% complete. One patient with well-corrected right ventricular outflow tract died 9.4 months postoperatively, possibly of aspiration. One child with membranous pulmonary atresia needed reoperation (conduit insertion) at 3.5 months of follow-up. Five needed catheter interventions: supravalvar stent (2), LPA stent (3), and RPA stent (1), most of them in the earlier half of the experience. Pulmonary annulus changed from preoperative -3.91 [-5.98; -2.23] to -0.10 [-1.44; 1.92] at discharge; growing proportionally to -0.13 [-3.52; 2.73] at follow-up. Kaplan-Meier freedom from composite dysfunction was 79.25 (95% confidence interval, +13.68%, -31.44%) at 36 months. CONCLUSIONS Recruitment of native leaflets, optimal Contegra monocusp, and commissuroplasty provide an easily replicable technique for achieving a competent, proportionally growing neopulmonary valve. Longer follow-up is needed to determine its impact on delaying a pulmonary valve replacement

Risk Factors and Outcomes of Children with Congenital Heart Disease on Extracorporeal Membrane Oxygenation-A Ten-Year Single-Center Report.

For children born with congenital heart defects (CHDs), extracorporeal life support may be necessary. This retrospective single-center study aimed to investigate the outcomes of children with CHDs on extracorporeal membrane oxygenation (ECMO), focusing on various risk factors. Among the 88 patients, 36 (41%) had a single-ventricle heart defect, while 52 (59%) had a biventricular defect. In total, 25 (28%) survived, with 7 (8%) in the first group and 18 (20%) in the latter. A p-value of 0.19 indicated no significant difference in survival rates. Children with biventricular hearts had shorter ECMO durations but longer stays in the intensive care unit. The overall rate of complications on ECMO was higher in children with a single ventricle (odds ratio [OR] 1.57, 95% confidence interval [CI] 0.67-3.7); bleeding was the most common complication in both groups. The occurrence of a second ECMO run was more frequent in patients with a single ventricle (22% vs. 9.6%). ECMO can be effective for children with congenital heart defects, including single-ventricle patients. Bleeding remains a serious complication associated with worse outcomes. Patients requiring a second ECMO run within 30 days have lower survival rates

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

Bridging with Veno-Arterial Extracorporeal Membrane Oxygenation in Children: A 10-Year Single-Center Experience

Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) is frequently used in children with and without congenital heart disease (CHD). This study, of a single-center and retrospective design, evaluated the use and timing of V-A ECMO in a pediatric cohort who underwent V-A ECMO implantation between January 2009 and December 2019. The patients were divided into a pre-/non-surgical group and a post-surgical group. Among the investigated variables were age, gender, weight, duration of ECMO, ECMO indication, and ventricular physiology, with only the latter being statistically relevant between the two groups. A total of 111 children (58 male/53 female), with a median age of 87 days (IQR: 7-623) were supported using V-A ECMO. The pre-/non-surgical group consisted of 59 patients and the post-surgical group of 52 patients. Survival at discharge was 49% for the pre-/non-surgical group and 21% for the surgical group (p = 0.04). Single-ventricle physiology was significant for a worse outcome (p = 0.0193). Heart anatomy still has the biggest role in the outcomes of children on ECMO. Nevertheless, children with CHD can be successfully bridged with ECMO to cardiac operation

Risk Factors and Outcomes of Children with Congenital Heart Disease on Extracorporeal Membrane Oxygenation-A Ten-Year Single-Center Report

For children born with congenital heart defects (CHDs), extracorporeal life support may be necessary. This retrospective single-center study aimed to investigate the outcomes of children with CHDs on extracorporeal membrane oxygenation (ECMO), focusing on various risk factors. Among the 88 patients, 36 (41%) had a single-ventricle heart defect, while 52 (59%) had a biventricular defect. In total, 25 (28%) survived, with 7 (8%) in the first group and 18 (20%) in the latter. A p-value of 0.19 indicated no significant difference in survival rates. Children with biventricular hearts had shorter ECMO durations but longer stays in the intensive care unit. The overall rate of complications on ECMO was higher in children with a single ventricle (odds ratio [OR] 1.57, 95% confidence interval [CI] 0.67-3.7); bleeding was the most common complication in both groups. The occurrence of a second ECMO run was more frequent in patients with a single ventricle (22% vs. 9.6%). ECMO can be effective for children with congenital heart defects, including single-ventricle patients. Bleeding remains a serious complication associated with worse outcomes. Patients requiring a second ECMO run within 30 days have lower survival rates

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