Diagnosis and surgical approach of congenital Gerbode defect: A case report.

INTRODUCTION: The Gerbode defect is an infrequent cardiac anomaly, with an incidence below 1 % in current worldwide literature. It consists of a communication between the left ventricle and right atrium in the membranous septum with consequential hemodynamical and structural heart changes and can present as either congenital or acquired. The concomitant affectation of the tricuspid valve poses its difficulty as a diagnostic and therapeutical challenge. PRESENTATION OF CASE: This case report presents a 27-year-old patient with an incidental finding of congenital Gerbode defect with hemodynamical repercussion during surgical treatment for multidisciplinary management in the context of open thoracic trauma. DISCUSSION: This defect has been infrequently described in the literature, and although several classification methods have been proposed, its diagnosis is challenging and must be considered when faced with nonspecific cardiac systems. CONCLUSION: It reports an infrequent congenital heart defect posing as traumatic or postoperative, generating a challenging diagnosis and successful surgical treatment given to the patient using a multidisciplinary approach to further broaden scientific literature on such an underrepresented pathology.

Chromatinopathies: insight in clinical aspects and underlying epigenetic changes.

Chromatinopathies (CPs), a group of rare inborn defects characterized by chromatin state imbalance, have evolved from initially resembling Cornelia de Lange syndrome to encompass a wide array of genetic diseases with diverse clinical presentations. The CPs classification now includes human developmental disorders caused by germline mutations in epigenes, genes that regulate the epigenome. Recent advances in next-generation sequencing have enabled the association of 154 epigenes with CPs, revealing distinctive DNA methylation patterns known as episignatures.It has been shown that episignatures are unique for a particular CP or share similarities among specific CP subgroup. Consequently, these episignatures have emerged as promising biomarkers for diagnosing and treating CPs, differentiating subtypes, evaluating variants of unknown significance, and facilitating targeted therapies tailored to the underlying epigenetic dysregulation.The following review was conducted to collect, summarize, and analyze data regarding CPs in such aspects as clinical evaluation encompassing long-term patient care, underlying epigenetic changes, and innovative molecular and bioinformatic methodologies that have been devised for the assessment of CPs. We have also shed light on promising novel treatment options that have surfaced in recent research and presented a synthesis of ongoing clinical trials, contributing to the current understanding of the dynamic and evolving nature of CPs investigation.

Autoimmune hemolytic anemia, a rare disease in newborns: a case report.

Autoimmune hemolytic anemia (AIHA) is a type of hemolytic anemia in which autoantibodies attack the membrane antigens of red blood cells, causing cell rupture (lysis). Hemolysis stimulates compensatory RBC production by boosting erythropoietin levels; however, this response is often insufficient to restore normal hemoglobin blood levels, resulting in anemia. It is a rare disease, with an annual incidence of one case in every 80 000 live births. Infants of any age can be affected, though neonatal incidence is unusual. Here, the authors report a rare case of AIHA in the neonatal period with concomitant atrial septal defect, ventricular septal defect, and patent ductus arteriosus. Case presentation: A one-hour-old male neonate weighing 3 kg who was born at 38 weeks of gestation presented to the pediatric department with the complaint of respiratory distress. Examination revealed obvious respiratory distress with subcostal and intercostal recessions and a continuous grade 2 murmur at the left upper chest; the liver was palpable 1 cm below the right subcostal margin with a palpable splenic tip. Laboratory investigations were ordered, which showed hemoglobin was decreasing continuously and bilirubin was raised, suspecting AIHA. A positive blood culture, tachycardia, tachypnea, and a raised leukocyte count showed that the baby was in sepsis. The baby improved clinically, and the complete blood count showed improved Hb. Cardiac examination findings and a second-grade continuous murmur at the left upper chest were further investigated through echocardiography, which showed a grade 2 atrial septal defect, a muscular ventricular septal defect, and a patent ductus arteriosus. Clinical discussion: Childhood AIHA is a rare and underrated disease that differs from the adult form. The disease's initial manifestation and subsequent course are both poorly understood. It affects mostly young children, and a high prevalence (21%) is found in infants. In some patients, there is a genetic predisposition to the development of this disease, and there is underlying immune deregulation in more than half of the cases, necessitating long-term homogeneous multidisciplinary follow-up. It is of two types, primary and secondary, and according to the study conducted in France, AIHA is associated not only with other autoimmune diseases but with some systemic diseases as well, like neurological, digestive, chromosomal abnormalities, and cardiac diseases, as in our case. Conclusion: There is a scarcity of data on clinical management and treatment strategies. More research should be done to know the environmental factors that can trigger the immune response against red blood cells. Moreover, a therapeutic trial is essential for a better outcome and helps prevent serious complications.

Caudal regression syndrome: Postnatal radiological diagnosis with literature review of 83 cases.

Caudal regression syndrome (CRS) is a rare congenital disorder characterized by arrest of caudal spinal growth and associated with wide spectrum multisystemic anomalies. Herein, we presented a case of a newborn baby who did not pass meconium due to imperforated anus and was referred to the pediatric surgeon for urgent diverting loop colostomy. The conventional X-ray, abdominal ultrasound and abdominal pelvic magnetic resonance imaging (1.5 T) at 2-month-old age revealed right kidney agenesis, sacrococcygeal agenesis, vertebral bodies dysraphism and the spinal cord ends at D12-L1 with anterior and posterior bands of the terminating filaments. The diagnosis of CRS was confirmed. Through this case report, we hope to draw attention to this rare syndrome and the wide range of associated anomalies, also to consider this syndrome on the top of differential diagnosis list once the newborn has anorectal malformation mainly imperforated anus.

An Analysis of Associated Conditions and the Relationship Between the Severity of Hand Manifestations With That of the Forearm in Ulnar Longitudinal Deficiency.

PURPOSE: A deeper investigation of medical and musculoskeletal conditions in patients with ulnar longitudinal deficiency (ULD) is needed. The association between the severity of the manifestations of ULD in the hands and forearms has not been firmly established. The purpose of this study was to describe the medical and musculoskeletal conditions associated with ULD and examine the relationship between hand and forearm anomalies. METHODS: The Congenital Upper Limb Differences registry was queried for all patients with a diagnosis of ULD, as defined by the Oberg-Manske-Tonkin classification system, between 2014 and 2020. The patients' demographic information, medical and musculoskeletal comorbidities, radiographs, and clinical images were reviewed. The participants were classified using the Bayne, Cole and Manske, and Ogino classification systems. RESULTS: Of 2,821 patients from the Congenital Upper Limb Differences registry, 75 patients (2.7%) with ULD (14 bilateral), with 89 affected extremities, were included. Hand anomalies were present in 93% of the patients. Approximately 19% of the patients had an associated medical comorbidity, and 20% of the patients had an associated musculoskeletal condition. Cardiac anomalies were present in 8.0% of the patients, and 12% of the patients had a lower extremity abnormality. Radial head dislocation was observed in 13 of 18 patients with Bayne type II or III ULD compared with 8 of 43 patients with other types of unilateral ULD. There was a significant positive association among the Bayne and Ogino, Bayne and Cole/Manske, and Ogino and Cole/Manske classification systems in patients with unilateral ULD. CONCLUSIONS: Associated medical and musculoskeletal conditions are common in patients with ULD, of which cardiac and lower extremity abnormalities are most frequently observed. There is a significant positive association between the severity of forearm anomalies and that of hand anomalies in patients with unilateral ULD. All patients with ULD should undergo a thorough cardiac evaluation by their pediatrician or a pediatric cardiologist. TYPE OF STUDY/LEVEL OF EVIDENCE: Symptom prevalence study III.

Pre-operative 2-D transthoracic echocardiographic diagnosis with intra-operative findings of children with structural heart diseases: A comparative analysis.

Background: Structural heart disease is a major cause of morbidity and mortality in children. Echocardiography is accepted as the first line cost-effective diagnostic modality for pre-operative assessment of children with structural heart diseases. Two-dimensional transthoracic echocardiography (2-D TTE) may be the only diagnostic tool in a resource-poor environment where further investigations may be very expensive and not readily available. Aim: The aim of the study is to determine the degree of accuracy of pre-operative 2-D echocardiographic diagnosis with eventual surgical (intra-operative) findings among children with structural heart diseases with a view to audit the echocardiographic diagnoses and final surgical diagnoses among the patients in the University of Nigeria Teaching Hospital Ituku-Ozalla Enugu, a tertiary cardiothoracic center in Enugu, South-east Nigeria. Patients and Methods: 2-D TEE (GE Model) diagnosis of all the children that had cardiac surgery at University of Nigeria Teaching Hospital (UNTH) Ituku/Ozalla Enugu over a 3-year period was studied. All the patients had at least two echocardiographic sessions and results were recorded in a proforma. Surgical findings were obtained from post-operative surgical notes. Intra-operative findings were compared with 2-D TTE findings. Data were analyzed using SPSS version 20. The degree of accuracy was expressed as percentages. The relationship between the sensitivity of 2-D TTE and intra-operative findings as ascertained using sensitivities and positive predictive values. Results: There were 55 pediatric cardiac operations performed within the period under review. There were 22 males and 23 females, the age range was from 8 months to 17 years. Fifty-two (94.5%) were due to congenital heart diseases, whereas three (5.5%) were due to acquired heart diseases. Echocardiographic findings were the same as surgical findings in all isolated PDAs (100%), Isolated ASDs (100%), Mitral valve regurgitation three (100%), but missed out PDA as an associated finding in a case of sub-aortic VSD (7.7%) and an ASD in a case of TOF (5.9%), congenital absence of tricuspid valve was also missed as a component of complex cardiac anomaly one (1.1%). These omissions however did not change the surgical approach and outcome. Pre-operative echocardiographic diagnoses and eventual surgical diagnoses were largely concordant. The sensitivity of 2-D TTE and intra-operative findings is 94.5%, positive predictive value is 94.5%, and the false negative rate is 5.5%. Conclusion: Echocardiography is a veritable diagnostic tool in the pre-operative evaluation of children with structural heart diseases. Continuous training and re-training are key in skill development and capacity building in resource-poor countries.

Transcatheter management of adult aortic coarctation with "Railway" technique.

Endografting for atretic coarctation is technically feasible to avoid the risks of open surgery. It requires a strategic and structured endovascular approach such as the "Railway" technique for safe and successful restructuring of complete aortic atresia and avoiding rupture or bleeding.

Simultaneous transthoracic intervention for multiple cardiac defects in children.

BACKGROUND: Transthoracic intervention for isolated congenital heart disease (CHD) has been well tested for its technological feasibility and is increasingly used in clinical practice. We aimed to present our experience in simultaneous transthoracic intervention for multiple cardiac lesions in a series of pediatric patients. METHODS: Between March 2015 and December 2019, 20 patients with multiple CHD were referred to this study; mean age was 18.8±8.6 (range, 4-36) months. The transthoracic echocardiography (TTE) diagnosis was atrial septal defect (ASD) and perimembranous ventricular septal defect (pmVSD) in 7 patients, patent ductus arteriosus (PDA) and ASD in 6, pmVSD and PDA in 2, pmVSD and valvular pulmonary stenosis (PS) in 2, ASD and PS in 2, and doubly committed subarterial VSD (dcsVSD) and PS in 1 patient. These patients underwent simultaneous transthoracic interventions with transesophageal echocardiography guidance. The procedure sequence was PS→VSD→PDA→ASD. Electrocardiography and TTE were scheduled at discharge and follow-ups. RESULTS: All patients were occluded successfully without any thoracotomy conversion. Operation time was 56-120 (mean, 75±13) minutes. A 1.5-2.0-cm median sternum incision was performed in 6 ASD&PDAs, 2 ASD&PSs, and 1 dcsVSD&PS. In 11 other patients, a 1.5-2.0-cm incision in the inferior sternum was made and the chest closed with a drain. There were no serious complications before discharge and at follow-up. CONCLUSIONS: Simultaneous transthoracic intervention for multiple cardiac defects in children is feasible with good short-term outcomes. For different lesions, the appropriate surgical incision and operational sequence can render the intervention minimally invasive and safer.

Thyroid disrupting effects of low-dose dibenzothiophene and cadmium in single or concurrent exposure: New evidence from a translational zebrafish model.

Thyroid hormones (THs) are major regulators of biological processes essential for correct development and energy homeostasis. Although thyroid disruptors can deeply affect human health, the impact of exogenous chemicals and in particular mixture of chemicals on different aspects of thyroid development and metabolism is not yet fully understood. In this study we have used the highly versatile zebrafish model to assess the thyroid axis disrupting effects of cadmium (Cd) and dibenzothiophene (DBT), two environmental endocrine disruptors found to be significantly correlated in epidemiological co-exposure studies. Zebrafish embryos (5hpf) were exposed to low concentrations of Cd (from 0.05 to 2 µM) and DBT (from 0.05 to 1 µM) and to mixtures of them. A multilevel assessment of the pollutant effects has been obtained by combining in vivo morphological analyses allowed by the use of transgenic fluorescent lines with liquid chromatography mass spectrometry determination of TH levels and quantification of the expression levels of key genes involved in the Hypothalamic-Pituitary-Thyroid Axis (HPTA) and TH metabolism. Our results underscore for the first time an important synergistic toxic effect of these pollutants on embryonic development and thyroid morphology highlighting differences in the mechanisms through which they can adversely impact on multiple physiological processes of the HPTA and TH disposal influencing also heart geometry and function.

The assessment of patients with situs inversus and corrected transposition of the great arteries.

INTRODUCTION: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect. There are different subgroups according to the location of the heart in the thorax, apical position and situs. OBJECTIVES: The purpose of this study was to assess pediatric patients with situs inversus (SI) ccTGA (SI-ccTGA), a rare subgroup of this condition, in detail. METHODS: The records of patients with SI-ccTGA followed between January 1, 2010 and January 1, 2019 in our clinic were analyzed retrospectively. Demographic features, associated cardiac defects, arrhythmias and follow-up data were recorded. RESULTS: Twenty-one out of 120 ccTGA patients had SI. The median age was 30 months (4 days-18 years). There were hemodynamically significant associated lesions in 85.7% (n=18) of the patients. A large ventricular septal defect was found in 16 patients (76.2%), severe pulmonary stenosis in 11 (52.4%), pulmonary atresia in six (28.5%), and severe tricuspid regurgitation in two (9.5%). Eleven out of 21 patients had biventricular physiology and eight had single-ventricle physiology. Bidirectional cavopulmonary anastomosis followed by a hemi-Mustard-Rastelli operation were planned for the remaining two patients. Twelve out of 18 patients with associated defects (66.6%) were operated and surgery was planned for three more patients (16.6%). The remaining three patients were scheduled for clinical follow-up. Arrhythmias developed in two (9.5%) patients on follow-up; ablation was performed in one of them and pacemaker implantation followed by cardiac resynchronization therapy was performed in the other. Two patients died during follow-up, one after a central shunt operation and the other preoperatively due to pneumonia and sepsis. CONCLUSION: SI-ccTGA is not a mirror image of situs solitus ccTGA (SS-ccTGA) due to important anatomic and physiologic differences between them. SI-ccTGA patients have a lower risk of tricuspid valve regurgitation than SS-ccTGA patients. The timing of clinical presentation of these patients mainly depends on the type and severity of the associated lesions, as in all subtypes of ccTGA.

Performance of the ADAPT-Treated CardioCel® Scaffold in Pediatric Patients With Congenital Cardiac Anomalies: Medium to Long-Term Outcomes.

Background: A Phase II Clinical Trial reviewed the performance (morbidity and calcification) of the tissue-engineered ADAPT® bovine pericardial scaffold (CardioCel®) in pediatric patients (n = 30) with congenital cardiac defects. In that study, CardioCel® demonstrated no graft-related morbidity and mortality in 25 patients, over 12 months. Five patients died due to non-graft-related events. Echocardiography revealed hemodynamically stable repairs with no calcification of the scaffold. Magnetic resonance imaging (MRI) at 12 months in 10 patients confirmed the absence of calcification. These patients were followed up for further up to 10 years. We present the results of this retrospective review of these patients that were followed for further medium to long-term (median 7.2 years, 25%: 3.6 years 75%: 9.25 years) postoperatively in these patients. Methods: Between April 2008 and September 2009, CardioCel® was implanted in 30 patients with congenital cardiac defects. Efficacy measures included graft-related mortality, morbidity and haemodynamic abnormalities. Calcification was assessed by standard 2D-M mode echocardiography and MRI at 12 months. Medium to long-term assessment included routine clinical assessments and echocardiography. Results: Median age at surgery was 18 months (27 days-13 years). Twenty-five patients (142 patient years) were followed for up to 10 years. The 10-year survival rate is estimated as 86.9% (95% CI 71.4-100.0%) over the entire follow-up period. One patient was lost to follow-up. No graft-related mortality was encountered up to a median follow-up of 7.2 years. Two patients died (pacemaker complications >5 years and arrhythmia >7 years postoperatively). No graft failure, thromboembolic events, infections or device-related reinterventions were recorded. Non-significant residual leaks occurred in 3 patients. Echocardiography demonstrated the absence of calcification in all implants. Conclusion: The tissue-engineered ADAPT® bovine pericardial scaffold demonstrated excellent medium to long-term performance (up to 10 years) when used as a scaffold for repair of congenital cardiac defects in children. Durability, acellularity, biostability and non-calcifying potential of CardioCel® makes it a very attractive tissue for congenital cardiac repair procedures.

External validation of a new classification of spinal lipomas based on embryonic stage.

OBJECTIVE: Contention exists regarding appropriate classification and management of spinal lipomas (SLs). Given the heterogeneity of SLs, omissions and overlap between surgically incomparable groups exist in conventional classification systems. The new classification of spinal lipoma (NCSL) recently proposed by Morota et al. delineates morphology by embryological pathogenesis and the resultant operative difficulty. Here, the authors aimed to validate the NCSL by applying it to patients who had been operated on at their institution. METHODS: All children who had undergone resection for SL between 2014 and 2018 were included in this analysis. MRI studies were independently reviewed and classified by three adjudicators. Baseline characteristics, inter-adjudicator agreement, coexisting anomalies and/or malformations, and postoperative outcomes and complications were analyzed. RESULTS: Thirty-six patients underwent surgical untethering for SL: NCSL type 1 in 5 patients (14%), type 2 in 14 patients (39%), type 3 in 4 patients (11%), and type 4 in 13 patients (36%). All classification was agreed on first assignment by the adjudicators. Radical or near-radical resection, which was attempted in all patients, was always possible in those with type 1 and 4 SL, but never in those with type 2 and 3 SL. Neurological stabilization and/or improvement were observed in all patients at the last follow-up. CONCLUSIONS: The NCSL was found to be a logical and reproducible system to apply in this SL population. All cases were successfully classified with a high degree of inter-assessor agreement. Widespread establishment of a commonly adopted and clinically useful classification system will enable clinicians to improve patient selection as well as discussion with patient representatives during the decision-making process.

Rescue Cardiac Surgeries After Pediatric Catheter-Based Interventions: A Ten-Year Retrospective Study.

BACKGROUND: Catheter-based interventions for treating congenital cardiac defects have gained wide acceptance as they reduce the risks associated with surgery. However, these procedures are associated with some complications, such as the embolization of devices or stents and the injury of surrounding structures, which might need a rescue surgical intervention. METHODS: Data from 25 patients who needed rescue surgery following interventional catheterization between January 2008 and January 2018 were retrospectively collected and statistically analyzed to review the decision and timing of surgery as well as the surgical techniques and results. RESULTS: There were 24 cases of rescue surgery after device embolization, including 9 cases of atrial septal defect closure, 8 cases of patent ductus arteriosus closure, 4 cases of pulmonary artery balloon dilatation with stenting, 3 cases of muscular ventricular septal defect closure, and 1 case of right ventricular outflow tract injury during balloon valvuloplasty. Median age was 4 years (range, 2 months to 12 years). All rescue surgeries were done via median sternotomy. The mean time interval between the decision to remove the device surgically and the actual surgical procedure was 75 ± 14 minutes. There were no reported cases of postoperative complications or mortality among the patients who underwent surgery. CONCLUSION: Our single-center experience confirms that early rescue cardiac surgery to correct adverse events after pediatric transcatheter interventions is safe and effective. Surgical strategies should be tailored according to the situation in each case.

Criss-cross heart with double-outlet right ventricle, subpulmonary ventricular septal defect, and bicuspid pulmonary valve.

Criss-cross heart (CCH) is an extremely rare complex congenital heart malformation. It accounts for less than 0.1% of congenital heart diseases. Here, we describe a unique case of CCH with double-outlet right ventricle, huge subpulmonary ventricular septal defect, bicuspid pulmonary valve, and right-hand aortic arch. The anatomic features were observed with echocardiography, and the diagnosis was confirmed at surgery. Many variations of CCH have been described. The present case expands the spectrum of this entity and may provide new insight into this complex anatomy.

Descripción anatómica, clínica y sobrevida de los pacientes con atresia pulmonar que han sido llevados a angioplastia con stent en el ductus arterioso persistente en el periodo 2009-2014 / Anatomical and clinical description and survival of patients with pulmonary atresia subjected to angioplasty with ductal stent in the period 2009-2014

Resumen Objetivo: describir las características anatómicas, la evolución clínica y la sobrevida de los pacientes con atresia pulmonar que han sido llevados a angioplastia con stent en ductus arterioso persistente y determinar si existen diferencias significativas en la saturación al inicio y final del procedimiento y a las 6, 12 y 24 horas posprocedimiento. Materiales y métodos: estudio analítico, descriptivo tipo cohorte. La población de estudio se tomó de las bases de datos del servicio de Cardiología pediátrica de una institución especializada en la ciudad de Bogotá, Colombia. Se incluyeron pacientes con diagnóstico de atresia pulmonar sometidos a angioplastia, en tanto que se excluyeron aquellos con cirugía cardiovascular previa. El análisis estadístico se realizó en STATA® 12,0, mediante estadística descriptiva, curvas de Kaplan-Meier y ANOVA de medidas repetidas. Resultados: se identificaron 237 pacientes con atresia pulmonar. Se seleccionaron 30 de los cuales se excluyeron 5. En total se construyó una cohorte de 25 pacientes que fueron llevados a angioplastia, 52% con atresia pulmonar con comunicación interventricular. Edad promedio 12,2 días, peso 2.698 g, diámetro del DAP 3,8 mm (DE = 1,1), índice de McGoon media 1,35. El implante del stent fue exitoso en todos los pacientes; sin embargo 2 fallecieron en la sala de procedimientos y uno durante las 6 horas siguientes. Las complicaciones más frecuentes fueron paro cardiorrespiratorio (n = 2), trombosis del stent (n = 2) y bacteriemia (n = 2). La mortalidad a 30 días fue del 28% (n = 7); durante el tiempo total fue del 36% (n = 9). Por medio del ANOVA no se encontraron diferencias significativas en los promedios de saturación en los cinco tiempos de interés. Conclusiones: la atresia pulmonar es una patología compleja que requiere intervención invasiva para el mantenimiento de la capacidad circulatoria de los pacientes. Este estudio muestra que a pesar de una intervención efectiva, la mortalidad a los 30 días es elevada. No se encontraron diferencias en los tiempos de saturación, lo que sugiere que mantiene la saturación de los pacientes de una forma no distinta que con la prostaglandina E (PGE1) pero garantiza la permeabilidad del ductus.

Abstract Objective: the aim of this study is to describe the anatomical characteristics, the clinical progression, and survival of patients with pulmonary atresia subjected to angioplasty with a stent in patent ductus arteriosus (PDA), and to determine if there are significant difference in the saturation at the beginning and the end of the procedure, and at 6, 12, and 24 hours after the procedure. Materials and methods: An analytical and descriptive cohort study was conducted on a population taken from the database of a Paediatric Cardiology Department of a specialised institution in the city of Bogota, Colombia. Patients with pulmonary atresia subjected to angioplasty were included, and those with previous cardiovascular were excluded. The statistical analysis was performed using STATA® 12.0, by means of descriptive statistics, Kaplan-Meier curves and repeated measurements ANOVA. Results: A total of 237 patients with pulmonary atresia were identified. Of the 30 that were selected, 5 were excluded, leaving a total of 25 patients that were subjected to angioplasty. Interventricular communication was observed in 52%. The mean age was 12.2 days, with a mean weight of 2.698 g, a mean PDA diameter of 3.8 mm (SD = 1.1), and a mean McGoon index of 1.35. Although the stent implant was successful in all patients, two died in the procedures room, and another one during the following 6 hours. The most common complications were, cardiorespiratory arrest in 2 patients, thrombosis of the stent in 2, and bacteraemia in two patients. The mortality at 30 days was 28% (7), and 36% (9) during the total time. Using ANOVA, no significant differences were found in the saturation means at the five times of interest. Conclusions: Pulmonary atresia is a complicated condition that requires invasive intervention in order to maintain the circulatory capacity of the patients. This study shows that, despite an effective intervention, the 30-day mortality is elevated. No differences were, found in the saturation times, which suggest that saturation is maintained in the patients in a way not unlike that with prostaglandin E (PGE1), but ensures the patency of the ductus.

Extubation in the Operating Room After Fontan Procedure: Does It Make a Difference?

Early extubation appears to have beneficial effects on the Fontan circulation. The goal of this study was to assess the impact of extubation on the operating table in comparison with extubation during the first hours after Fontan operation (FO) on the early postoperative course. Between 2013 and 2016, 114 children with a single ventricle heart malformations (mean age, 3.8 ± 2.3 years) underwent FO: 60 patients were extubated in the operating room (ORE) and 54 in the intensive care unit (ICUE) in the median time of 195 min (range 30-515 min) after procedure. Pre-, peri-, and postoperative records were retrospectively analyzed. The hospital survival rate was 100%. One patient from the ORE group needed an immediate reintubation because of laryngospasm. The ORE group showed lower heart rate (106.5 vs. 120.3 bpm; p < 0.001) and lower central venous pressure (10.4 vs. 11.4 mmHg; p = 0.001) than patients in the ICUE group within the first 24 h after FO, as well as higher systolic blood pressure within 7 h after operation (88.6 ± 2.5 vs. 85.6 ± 2.6 mmHg; p = 0.036). The ORE children manifested significantly less pleural effusions during 48 h after FO (38.0 vs. 49.5 ml/kg; p = 0.004), received less intravenous fluid administration within 24 h after FO (54.1 vs. 73.8 ml/kg; p = 0.019), less inotropic support (9.8 vs. 12.8 h of dopamine; p = 0.033), and less antibiotics (4.7 vs. 5.8 days; p = 0.037). ICUE children manifested metabolic acidosis more frequently than the ORE group 3-4 h after FO (p < 0.05). Immediate extubation after FO in comparison with extubation in the ICU appears to be associated with improved hemodynamics and reduced application of therapeutic interventions in the postoperative course.

Minimally Invasive Cardiac Surgery Without Peripheral Cannulation: A Single Centre Experience.

BACKGROUND: A minimally invasive approach without any peripheral cannulation was used as an alternative to median sternotomy for the correction of a wide range of cardiac defects with superior results and good cosmetic outcome. METHODS: From October 2015 to March 2017, 145 patients underwent correction of congenital cardiac malformations with cardiopulmonary bypass through right anterior minithoracotomy (RAMT) with routine cannulation. The average age was 9.69±4.38years (range 2-21) and the average body surface area was 0.91±0.27square metres (range 0.5-1.7). The corrected defects included all types of atrial septal defect (ASD) and ventricular septal defects (VSD), partial atrioventricular septal defects with severe mitral regurgitation, mitral valve repair, repair of tetralogy physiology requiring outlet patch enhancement, isolated pulmonary stenosis (PS) and congenital coronary cameral fistula. RESULTS: There was no operative mortality or major morbidity. All patients were alive at the time of this follow-up. The mean cardiopulmonary bypass time was 64.75±22.28minutes (range 30-175) and mean aortic cross clamp time was 37.53±18.23minutes (range 14-135). Fifty-five (55) patients were extubated in the operating room and the remaining 90 patients were extubated within 3hours in the intensive care unit. Repair and cosmetic results were excellent in all patients. There were no conversions to full sternotomy. No neurological events were reported. CONCLUSIONS: Right anterior minithoracotomy without peripheral cannulation is safe and effective for the correction of a wide range of congenital heart defects including right ventricular outlet obstructions. The cosmetic results are satisfactory, avoiding psychosocial problems.