Cor Triatriatum Sinister With a Decompressing Levoatriocardinal Vein: A Rare Association.

Levoatriocardinal vein has rarely been described with cor triatriatum. We report a case of a newborn with respiratory distress who was found to have cor triatriatum sinister with a decompressing levoatriocardinal vein on transthoracic echocardiography. The pulmonary venous confluence received all pulmonary veins and drained to the proximal left atrial chamber through significant communication. Moreover, the cor triatriatum membrane separated the left atrial body into proximal and distal left atrial chambers with a restrictive opening in the membrane, causing severe flow limitation to the cardiac output, severe pulmonary arterial hypertension, and significant right ventricular dilatation. In addition, a sizeable levoatriocardinal vein decompressed the pulmonary venous confluence drained superiorly to the left innominate vein. She had a successful surgical repair by resectioning the cor triatriatum membrane and ligating the levoatriocardinal vein. The patient had improved pulmonary arterial pressures and right ventricular dimensions at the one-month follow-up. The case highlights the rare association of the levoatriocardinal vein and cor triatriatum, and its presence signifies severe obstruction at the level of cor triatriatum.

The Effectiveness of Leadership Interventions on Cardiologists' Performance Using Benchmarking as a Tool.

BACKGROUND: Aligned with the Health Sector Transformation Strategy of Saudi Vision 2030, the study analyzed the attitudes and behaviors of cardiologists toward change and identified factors that could either facilitate or hinder the success of leadership interventions. At our cardiac center, the cath lab department is at the forefront of operations, accounting for 80% of the procedures. Our team members may not be fully equipped with the necessary attitudes and behaviors to drive successful improvement projects. Therefore, our top priority is ensuring they remain productive and engaged throughout the process. This is especially crucial because 60% of our budget is allocated to the cath lab department. OBJECTIVES: The study aimed to assess the effectiveness of a leadership intervention on cardiologists' performance in terms of safety, speed, and cost. The research analyzed the behavior and attitude of cardiologists towards change and encouraged progress and collaborative learning between doctors, using benchmarking as a tool. Besides, the study sought to determine the contribution of the interventions used to overall efficiency in performing interventions. This case study focuses on four main aspects of the program. First, it aims to explore an innovative approach to improving the PSCCQ cath lab for patients. Second, it assesses the collective effort of all participants involved in the program. Third, it analyzes the program results and compares them with those of international experiences. And finally, it examines the program's potential benefits for our patients. METHODS: The study's objectives were evaluated through qualitative analysis of in-depth interviews and quantitative data analysis of three variables in the cath lab: radiation dose, time, and inventory. The reason for using mixed methods was to comprehensively understand the same concept from different angles. RESULTS: According to the study, participants improved the safety and effectiveness of our cath lab by reducing the radiation dose and its cost. The study revealed a 52% decrease in the radiation dose for diagnostic cases and an 11% decrease for interventional cases. Similarly, the cost of the radiation dose decreased by 28% for diagnostic cases and 11% for interventional cases. During the observation, it was noted that the participants were highly engaged and willing to adapt to the situation. Some even viewed it as an opportunity for personal growth and improvement in the cath lab. However, they stressed the significance of awareness as a crucial element in improving their behavior and reinforcing it as the foundation for maintaining progress. Furthermore, the study revealed that collaborative work among the participants could have been more optimal. CONCLUSION: The study concludes that implementing innovative improvements to the cath lab was a necessary yet complex undertaking. Participants were more inclined to embrace the changes when they were easily understandable and motivating. The study recommends the appointment of a change agent, the establishment of benchmarks, and the creation of a collaborative working environment between leaders and staff. Above all, the leader should support and sponsor the change to facilitate the transition at various levels.

The Burden of Neonatal Referrals on a Pediatric Cardiology Service: A Local Center Experience.

BACKGROUND: Congenital heart disease (CHD) is a common occurrence in live births, with some exhibiting critical congenital heart disease; therefore, cardiology services should be available around the clock to ensure timely diagnosis and management. This study aims to describe the workload and the need for pediatric cardiac services in a maternity hospital for newborn referrals. Moreover, the study describes the indications for neonatal cardiology consultations. METHODS: The prospective cohort study was conducted over four months, from January to April 2022, in the Prince Sultan Cardiac Center Al Qassim region of Saudi Arabia. Prince Sultan Cardiac Center's pediatric cardiology department provides cardiac services to the Maternity and Children Hospital Buraidah Al Qassim. Out of the total 2,606 live births during the study period, the cardiology team evaluated 352 neonates. Neonates less than 30 days of age who were born in the maternity hospital were enrolled in the study. The outborn babies referred from other centers as suspected congenital heart disease for whom a cardiac evaluation was done were excluded. In addition, babies assessed in the emergency room and born elsewhere were excluded. Only new consultations have been considered, excluding follow-up consultations. STATISTICAL ANALYSIS: Data about patients' demographic, clinical and echocardiographic findings were recorded on Google Forms and converted to a Google spreadsheet. The Google spreadsheet's inbuilt statistical software was used for analysis. Categorical data were presented as percentages, and numerical data as median and range. RESULTS: The cardiology team evaluated 352 neonates from 2,606 live births over four months, accounting for 13.5 per 100 live births. The median weight was 2.8 kilograms, with a 0.5-4.3 kilogram range. Males comprised 187 (53%), and females comprised 165 (47%). Moreover, full-term, preterm, and post-term accounted for 236 (67%), 113 (32%), and 3 (0.8%) of patients, respectively. The common indications for neonatal cardiac referral were respiratory distress 60 (17%), infants born to diabetic mothers 50 (14%), abnormal fetal echocardiogram 49 (13.9%), family history of abortion or neonatal death 31 (8.8%), and congenital anomalies 30 (8.5%). Systolic murmur was the commonest clinical finding that prompted cardiology referrals 82 (23.2%), followed by desaturation 38 (10.7%) and dysmorphic features 31 (8.8%). Among the congenital cardiac defects, an isolated atrial septal defect (ASD) was seen in 66 (18.5%), isolated patent ductus arteriosus in 50 (14.2%), and ventricular septal defect in 21 (5.9%). Moreover, 13 (4.4%) lesions were critical CHDs. Finally, 27 (7.6%) had a diagnosis of pulmonary hypertension. CONCLUSION: Knowing the burden of neonatal cardiac assessment on pediatric cardiology services in any maternity center may help the healthcare authorities to allocate resources and optimize the delivery of cardiac services among the neonatal population. Properly allocating pediatric cardiologists to the needed centers may optimize neonatal cardiac services. Moreover, it may decide on the number of pediatric cardiologists that need to be trained each year to meet the requirements of neonatal cardiac services.

Comparison of Demographic, Clinical, and Echocardiographic Features Between Complete and Incomplete, and Early and Late Presenters of Kawasaki Disease: A 10-Year Single-Center Experience.

INTRODUCTION:  The diagnosis of Kawasaki disease (KD) is based mainly on clinical findings and supported by laboratory tests. Complete KD fulfills the main clinical criteria, while incomplete KD includes patients with fewer main criteria and compatible laboratory or echocardiographic findings. The study compares the demographic, clinical, laboratory, and echocardiographic parameters between the complete and incomplete KD and early and late presenters. Moreover, it describes the coronary manifestations of the study population. METHODOLOGY: A retrospective review of all patients admitted with a diagnosis of KD during the period from January 2010 to September 2020 was conducted. Clinical presentation, laboratory features, echocardiographic observations, and follow-up data were examined. Moreover, the patients were further classified as early presenters (presented within 10 days of fever onset) and late presenters (presented after 10 days of disease onset). A comparison between complete and incomplete KD and early and late presenters was performed for demographic, clinical, and echocardiographic features. RESULTS: A total of 76 patients were admitted with a diagnosis of KD. The median age of presentation was 28 months, with a range of five to 144 months, and the median timing was seven days, with a range of one to 30 days. The median follow-up period was six weeks, with a range of one to 192 weeks. Complete KD was present in 38 patients (50%), and 38 (50%) had incomplete KD. Skin manifestations, oral mucosal changes, skin desquamation, conjunctivitis, and lymphadenopathy were present more in patients with complete KD than incomplete ones. Complete and incomplete diseases did not differ regarding coronary artery lesions. Of the patients, 53 (70%) presented 10 days or less after the onset of fever, and 23 (30%) presented after the 10th day of disease onset. Comparison between early and late presenters revealed significantly greater mucus membrane changes and lymphadenopathy manifestations among the early presenters and coronary artery lesions among the late presenters. CONCLUSION: The clinical features of KD should prompt early referral for evaluation, echocardiography, and early administration of intravenous immunoglobulin to prevent coronary artery complications. The complete form of Kawasaki does not have more frequent coronary artery lesions than the incomplete form. Additionally, late presenters may be at increased risk for coronary artery abnormalities than early presenters.

The Impact of Integration of a Genetic Clinic Into a Pediatric Cardiac Unit.

BACKGROUND: Previously published studies suggest that genetic or environmental causes can be observed in 20-30% of congenital heart disease (CHD) patients, which include aneuploidy, single gene defects, pathological copy number variations, and de novo autosomal dominant and recessive inheritance. Moreover, the genetic background of childhood cardiomyopathies (CMs) has not been elucidated well. OBJECTIVE: The study highlights the value of genetic assessment in diagnosing and family counseling for CHD and pediatric CM patients referred to the genetic clinic in a pediatric cardiology department. METHODS: The study involved patients less than 18 years of age attending the cardiogenetic clinic in the pediatric cardiology department between December 2010 and February 2019. The following patient categories who had genetic evaluation were included: CHD in the presence of a syndromic phenotype, patients with CHD having extracardiac congenital anomalies or delayed development, hypertrophic and dilated CM patients, patients with dilated aortic root and ascending aorta, significant CHD in siblings or first-degree relatives, suspected channelopathies; and interrupted aortic arch abnormalities. RESULTS: A total of 285 patients were evaluated in the cardiogenetic clinic. The mean age was 20.2 months, with a range of 0-168. Females and males constituted 153 (53.7%) and 132 (46.3%), respectively. The most common cause of referral to the genetic clinic was the presence of CM (N=134 (46.3%)): hypertrophic CM in 24% and dilated CM in 20% of cases. Seventy-six patients (26.7%) had positive genetic results. The most common genetic abnormality was familial infantile hypertrophic CM-causing gene ELAC2 in 19 (23.5%) cases. CONCLUSION: It may be beneficial for any pediatric cardiology unit to provide an established genetic clinic. Using a genetic clinic will enhance understanding of CHD pathophysiology, family education, and genetic counseling. Agreement on a well-written protocol and the way forward to specify what congenital heart conditions require genetic investigation should be clarified.

Can Echocardiography Aid in Diagnosing Vascular Rings?

Even though cardiac computed tomography and magnetic resonance imaging are the gold standard for evaluating the aortic arch in the context of vascular rings in children, echocardiography is usually the first-line modality. The echocardiographic evaluation of the aortic arch in the context of vascular rings in children has received little attention. This article details the step-by-step echocardiographic assessment of the aortic arch in vascular ring patients.

Radiation Dose Exposure to Patients During Transcatheter Patent Ductus Arteriosus Closure via the Arterial Route.

Objective This study aims to evaluate the radiation dose for transcatheter patent ductus arteriosus (PDA) via the arterial route and compare it with previously published benchmarks. Background Exposure to radiation in the catheterization lab can cause skin injury and cancer in the long run, especially in pediatric patients with complex heart conditions, which necessitate serial catheterizations. Therefore, measuring the patient radiation dose and establishing a benchmark for each cardiac interventional procedure is essential. Material and methods In this prospective study, 34 patients with transcatheter patent ductus arteriosus closure via an arterial route were included. Patients who had silent PDAs, no left heart dilatation and PDA size of less than 2mm had PDA closed via an arterial route. All the study group patients received an Amplatzer duct occluder II-additional size device (St. Jude Medical Corp, St. Paul, MN) using biplane flat-panel fluoroscopy equipment adjusted in accordance with the pediatric parameters. Patients' dose area product, air kerma and fluoroscopy time were recorded in the catheterization lab and finally compared with internationally published reference data. Results Of 73 patients who had transcatheter patent ductus arteriosus closure between April 2021 to December 2021, 34 patients who had a PDA closure via an arterial route were enrolled. The median age and weight were 11.5 (4-168) months and 10.5 (6-31) kg. Twenty-one (61.8%) were males, and 13 (38.2%) were males. The median radiation dose parameters were as follows: air kerma 11 (3-42) milliGray, dose area product 131 (33-443) microGray per m2, median dose area product indexed to weight 12 (1-48) microGray m2 per kg, fluoroscopy time 2 (2-4) min and frame rate 15 (7.5-15) frames per second. Due to many factors, our radiation dose parameters were less than internationally published reference values for transcatheter PDA closure. Conclusion Patient selection, detailed pre-catheterization echocardiography and procedure planning are essential for accomplishing device closure of PDA with a significant reduction in radiation dose. Hemodynamic assessment in the catheterization lab is unnecessary for most PDA patients. Additionally, a next-generation imaging platform equipped with flat-panel detectors and adjusted for pediatric settings and a fluoro recording option can be used to reduce radiation exposure.

Catheter Closure of Clinically Silent Patent Ductus Arteriosus Using the Amplatzer Duct Occluder II-Additional Size: A Single-Center Experience.

Objectives Transcatheter closure is the treatment of choice for most patent ductus arteriosus (PDA) in infants, children, and adults. However, there is a controversy regarding transcatheter closure of clinically silent PDAs. Some authors favor device closure to eliminate the lifelong risk of infective endarteritis while others recommend avoiding PDA closure in such patients. The study describes our experience of closing the silent PDAs using the Amplatzer duct occluder II-additional size (ADO II-AS) (St. Jude Medical Corp, St. Paul, MN). Materials and methods From April 2018 through March 2021, 52 consecutive pediatric patients aged 18 years and less with clinically silent PDA who had transcatheter closure at our center were enrolled. Patients were excluded if they had clinically detected PDAs; had surgical ligation of PDA with no residual shunt; had left heart dilatation on echocardiography; or moderate-sized PDAs closed with ADO II-AS. In addition, patients with an innocent murmur or murmur due to an associated lesion were included. This study was retrospective, and all of the 52 patients underwent PDA device closure using ADO II-AS. Results Fifty-two consecutive patients were enrolled with a median age of 17 months, range (97-2.5) 94.5 months. Mean weight was 11.29 kilogram, range (24.8-3.5) 21.3 kilogram, and mean follow-up was 13.5 months, range (29-0) 29 months. Thirty-one (59.6%) were females, and 21 (40.4%) were males. The mean procedure time was 30.6 min, range (60-10) 50 min, and mean fluoroscopic time was 5.5 min, range (28-1.7) 26.3 min. The mean volume of contrast given was 9.1 milliliter, range (30-4) 26 milliliter. Forty-five (45; 88.2%) patients had immediate closure of PDA. No patients had anesthetic or vascular complications; however, two patients had procedural complications. Device placement was unsuccessful in one patient with Downs syndrome. The mean follow-up for our patients was 13.5 months, range (29-0) 29 months; the patients were asymptomatic at the follow-up, and none of the patients had any residual leak. None of the patients showed coarctation or left pulmonary artery stenosis at the latest follow-up. Conclusion The usefulness of catheter-based therapy for silent PDA is less well-established by current evidence. Further studies are needed to justify the intervention solely based on the premise that the silent duct is a substrate for infective endarteritis; however, our reason to close silent PDA was to do so primarily because of social reasons. This study found that device closure of silent PDA is safe and effective using an ADO II-AS device with minimal risk of embolization and a low residual shunt rate. Coils have been used to close small PDAs, however, with higher rates of embolization and device malpositioning. We believe ADO-II AS offers an advantage of safety and efficacy over coils. In addition, the study highlights the advantage of using an ADO II-AS device, which can be delivered via a four French delivery system with no arterial complications.