Midterm postoperative outcomes of different types of surgical reconstruction of sinus venosus atrial septal defects with anomalous pulmonary venous connection: The Results of Prospective Cohort Study.

Background and Aims: Sinus venosus atrial septal defects (SVASDs) constitute a substantial part of atrial septal defects and are usually characterized by anomalous pulmonary venous connection (APVC), causing complications like sinus node dysfunction and arrhythmias. Several surgical approaches are used for treating SVASDs in pediatric patients, including single- and two-patch techniques. The study aimed to prospectively evaluate and compare the safety and efficacy of these two methods with different follow-up periods. Methods: Ten patients aged 1-8 years with SVASDs and partial APVC were enrolled in the study at Bhanubhai and Madhuben Patel Cardiac Centre, Karamsad, India, between December 2018 and October 2021. The single-patch (sandwich-patch) technique was used in two patients, whereas the two-patch (dual-patch) technique with autologous pericardium was used in seven. Safety was assessed as the absence of complications in the follow-up periods of 6 months, 1, and 2 years, whereas efficacy was estimated by the preserved sinus rhythm and the development of arrhythmias. Electrocardiographic and echocardiographic methods were used to evaluate both parameters. Results: No deaths, reoperations, pulmonary vein, and superior vena cava (SVC) stenosis or phrenic nerve palsy were observed among the 10 patients in the three follow-up periods. Sinus rhythm was arrested in two of the seven patients who underwent two-patch repair, whereas no rhythm disturbances occurred in those who underwent single-patch repair. Conclusion: Both techniques used in SVASD repair with autologous pericardium proved to cause the smaller rate of complications in midterm postsurgical phase. However, there is a potentially great risk of the development of sinus node malfunction after the application of the two-patch technique. Therefore, methods avoiding sinus node interference are preferred in patients with partial APVC involving SVC.

Evaluation of right ventricular performance in patients with postoperative congenital heart disease using Doppler tissue imaging and cardiopulmonary bypass indices: A prospective cohort study.

Background and Aims: Postoperative cardiac outcomes after intracardiac repair (ICR) are determined by numerous factors whereas right ventricle (RV) dysfunction is considered essential for them, as only few studies attempted to evaluate it postsurgically. RV's function is supposed to be the strong prognostic factor for patients diagnosed with congenital heart defects; therefore, assessing it is the main objective of the study. Methods: This is a prospective single-centered cohort study performed on 50 pediatric patients with congenital heart disease (CHD) who underwent ICR between January 2019 and January 2022. All patients underwent echocardiographic assessment of RV function via tricuspid annular plane systolic excursion (TAPSE) and fractional area change (FAC) at 1, 24, and 48 h. After surgery, where pre- and postoperative RV pressure, cardiopulmonary bypass (CPB), and aortic cross-clamp (ACC) time were assessed. Similarly ventilation intensive care unit (ICU) and hospital stay times and mediastinal drainage were also monitored. Results: The mean ± standard deviation for pre- and postoperative RV pressure was 49.1 ± 16.12 and 42.7 ± 2.9 mmHg, respectively, whereas that for pre- and postoperative pulmonary artery pressure was 30.4 ± 2.6 and 24.2 ± 12.9 mmHg, with p value of <0.002 and <0.001, respectively. The mean ± standard deviation of CPB and ACC times was 120.92 ± 74.17 and 78.44 ± 50.5 min accordingly, while those for mean ± standard deviation of ventilation time, mediastinum chest drainage, ICU and hospital stays were 30.36 ± 54.04, 43.78 ± 46.7 min, 5.9 ± 4.01 h, were 30.36 ± 54.0, 43.78 ± 46.7 min, 5.9 ± 4.01 and 10.3 ± 4.83 h, respectively. Conclusions: RV dysfunction plays the important role in longer recovery and intraoperative time, while its effect is mostly transient. The use of TAPSE and FAC methods is valuable in the evaluation of postoperative outcomes, and the former proved to be more effective.

Perioperative Management of Sickle Cell Disease in Complex Congenital Cardiac Surgery: A Compilation of Two Cases.

Preoperative exchange transfusion is frequently recommended in patients with homozygous sickle cell anemia (homozygous SS) who undergo cardiopulmonary bypass to reduce the concentration of circulated sickle hemoglobin. The information regarding the ideal level of sickle hemoglobin for sickle cell disease (SCD) patients who require surgery is still divergent in the literature. We present the successfully managed cases of two children aged 11 months and three years with homozygous SS who underwent cardiopulmonary bypass for double-outlet right ventricle and cor-triatriatum sinistrum, respectively. In both cases, we performed preoperative blood and exchange transfusion, as well as strict intraoperative invasive monitoring. We also maintained normothermia, avoided hypoxia and acidosis, and offered effective pain management.

Bilateral pheochromcytomas presenting as shock: A rare case report.

Pheochromocytomas are rare chromaffin cell tumors of adrenal medulla (90%) that secrete catecholamines. Among children, the average age of presentation is 11-13 years, with a male preponderance of 2:1. Symptoms may be caused by catecholamine overproduction, local pressure, or metastasis. Sustained hypertension is the most common symptom. Elevated circulating catecholamines can cause cardiovascular alterations such as coronary vasospasm, ventricular and supraventricular arrhythmias, and dilated cardiomyopathy, precipitating in cardiogenic shock. We present a rare case of cardiogenic shock as the initial presentation of a bilateral pheochromocytoma in a child.

Perioperative Management of Scimitar Syndrome Mimicking Total Anomalous Pulmonary Venous Return Posing a Diagnostic Dilemma: A Compilation of Two Cases.

The low prevalence of scimitar syndrome along with its varied clinical presentation poses a diagnostic dilemma to the treating clinicians. It usually falls under a large spectrum of conditions called venolobar syndrome. Scimitar involves the partial venous drainage of the right lung to the inferior vena cava (IVC). We share our experience of two cases that were diagnosed as partial anomalous pulmonary venous connection/drainage (PAPVC/PAPVD) on echocardiography but CT scan revealed the underlying scimitar syndrome. Perioperative pulmonary arterial hypertension, intraoperative ventilation strategies for managing associated lung hypoplasia, and postoperative right ventricular dysfunction are a few challenges faced in the perioperative period.

A Novel Approach to Facilitate an Ideal Surgical Correction of Congenital Lobar Emphysema by Using Neonatal Ultrathin Bronchoscopy-Guided Endo-Bronchial Block.

This is a case report of a child who had acyanotic congenital heart disease - ventricular septal defect (VSD) and a patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension. The child underwent open-heart surgery - VSD closure with PDA ligation - and six months later was re-admitted for congenital lobar emphysema of the right middle lobe. He underwent successful right middle lobectomy of the lung six months after cardiac surgery under a one-lung ventilation technique in which application of fiber-optic bronchoscope made the surgery safer and more suitable.

A Comparison of the Pulmonary Valve, Main Pulmonary Artery, and Branch Pulmonary Artery Measurements by Echocardiography and Computed Tomography Scan.

INTRODUCTION: Congenital heart diseases (CHD) are one of the most commonly occurring congenital anomalies. Echocardiography is usually the initial investigation for suspected CHD. However, it is operator-dependent and limited by available chest windows. Multidetector computed tomography (MDCT) scan provides superior temporal and spatial resolution producing excellent cross-sectional anatomical images. MDCT is specifically helpful for pulmonary artery anomalies if not clearly visible on an echocardiogram. OBJECTIVE AND METHODS: The study aims to compare measurements of branch pulmonary arteries, pulmonary valve, and main pulmonary artery obtained from trans-thoracic echocardiography measurements and MDCT. Forty-nine patients younger than 17 years of age underwent MDCT, and an echocardiogram was included in the study. The measurements of the pulmonary valve, main pulmonary artery, and branch pulmonary arteries were measured on MDCT and echocardiogram. RESULTS: Bland-Altman analysis revealed the mean difference (95% confidence limits) in measurements of diameter between echocardiogram and MDCT for the right pulmonary artery, left pulmonary artery, pulmonary valve, and main pulmonary artery, which were -0.5 (-3.1, 2.2) mm, -0.6 (-3.3, 2.1) mm, 0.7 (-2.5, 3.9) mm, and 1.2 (-6.9, 4.5) mm, respectively. CONCLUSION:  The analysis revealed acceptable agreement in measurements of the pulmonary valve, main pulmonary artery, and branch pulmonary arteries obtained from MDCT and echocardiogram. The difference was marginally more for the main pulmonary artery compared to the pulmonary valve and branch pulmonary arteries.

Clinical Application and Multidisciplinary Assessment of Three Dimensional Printing in Double Outlet Right Ventricle With Remote Ventricular Septal Defect.

BACKGROUND: Double outlet right ventricle (DORV) with two well-developed ventricles and with a remote ventricular septal defect (VSD) may present a therapeutic challenge. Echocardiographic imaging of such complex cases does not always provide all of the information required to decide on an operative approach (biventricular or univentricular) and to design an intracardiac baffle to direct left ventricular outflow through the VSD and to the aorta for biventricular repair. A three dimensional (3D) printed model of the heart based upon data derived from computed tomography (CT) or magnetic resonance imaging (MRI) may contribute to a more complete appreciation of the intracardiac anatomy. METHODS: From April to September 2015, six consecutive patients with DORV and remote VSD underwent CT/MRI scans. Data sets from these studies were used to generate life-size 3D models using a 3D printer. We compared the assessment of 3D printed heart model findings with information obtained from echocardiography, CT, or cardiac MRI and with details of the surgeon's intraoperative direct observations when available. Quantification of the information provided by the 3D model was achieved using a unique scale that was created for the purpose of this study. The accuracy and utility of information derived preoperatively from the models were assessed. RESULTS: Six data sets from six patients were analyzed. Five data sets could be successfully used to create sandstone models using 3D printing. The five patients ranged from 7 months to 11 years of age and weighed 6.7 to 26 kg. The spatial orientation of the heart in the thorax, the relationships of the great arteries and the semilunar valves, the size and location of the VSD were well appreciated in all models, as were the anticipated dimensions and orientation of a surgically planned interventricular baffle. Three of the five patients underwent successful biventricular repair. CONCLUSION: The 3D printed models scored higher than conventional imaging, with respect to most aspects of the surface spatial orientation and intracardiac anatomy. The models are a useful adjunct in preoperative assessment of complex DORV. The unique scale helps quantify the advantages and limitations of the 3D heart models.

Hydroxyurea in sickle cell disease: drug review.

Hydroxyurea, a myelosuppressive agent, is the only effective drug proven to reduce the frequency of painful episodes. It raises the level of HbF and the haemoglobin level. It usually decreases the rate of painful episodes by 50 %. It was first tested in sickle cell disease in 1984. It also decreases the rate of ACS episodes and blood transfusions by ~50 % in adults. It was developed as an anticancer drug and has been used to treat myeloproliferative syndromes-leukemia, melanoma, and ovarian cancer. It was approved for use by FDA in adults. Side effects includes neutropenia, bone marrow suppression, elevation of hepatic enzymes, anorexia, nausea, vomiting and infertility.