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Double-outlet right atrium is an extremely rare congenital cardiac defect, in which the right atrium drains into both ventricles. Here in, we present a peculiar case involving an 8-year-old female with double-outlet right atrium characterized by three distinct atrioventricular valves and a membranous ventricular septal defect. This is associated with right atrium to left ventricular flow through the accessory atrioventricular valve while maintaining adequate size of the right ventricle. Additionally, we include a comprehensive review of the literature to underscore the variability in clinical presentation and management strategies., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Congenital heart disease (CHD) remains the number one birth defect worldwide. Pulse oximetry screening (POS) is a widely used CHD screening modality effective in detecting critical lesions. This study is aimed at assessing the accuracy and cost-effectiveness of POS in a cohort of term well-babies admitted to a regular nursery in a tertiary care center., Methods: We reviewed the charts of term babies admitted to our regular nursery over a period of one year. The results of POS and the findings of echocardiography were collected. Similarly, we explored the records of our fetal echocardiography program to identify the fetuses screened for CHD during the same period., Results: 900 term babies were born and admitted to newborn nursery at our center, and 69 fetuses were evaluated by our fetal cardiology team during the study period. None of our term babies had a positive POS at birth or 24 hours of age. However, 56 babies had a cardiac echo before hospital discharge due to suspicious findings on physical examination or a family history of CHD. A simple noncritical CHD was noted in 10 of them. Additionally, 53 babies underwent echocardiography within the first five years of life; a simple CHD was noted in 6 of them. In parallel, 21 of our fetuses were found to have CHD: 16 simple CHD and 5 critical CHD (CCHD)., Conclusion: Despite its cost-effectiveness and efficacy in screening for CCHD, POS is suboptimal for detecting simple CHD. In the absence of a proper prenatal screening and fetal echocardiography program, POS remains a cost-effective modality for detecting CCHD., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2024 Ziad R. Bulbul et al.)

We herein report on a series of four patients presented to our tertiary care centre with features of multisystem inflammatory syndrome in children and cardiac involvement. Two of our patients had recent exposure to a COVID-19-positive patient, one had recent documented infection, and another had no known positive contact. All the patients were tested positive for severe acute respiratory syndrome coronavirus 2 immunoglobulin G antibody at the time of presentation. All of them fulfilled the diagnostic criteria according to the World Health Organization Centers for Disease Control or the British guidelines for MIS-C (fever for ≥3 days, multisystem involvement (at least two), elevated markers of inflammation, and no other alternative diagnosis).Cardiac involvement was variable ranging from isolated ectasia of the coronary arteries to full-blown pancarditis such as severe biventricular dysfunction, multi-valvar involvement, and pericardial effusion.All our patients received intravenous immunoglobulin (2 g/kg), methylprednisolone, and aspirin and some required inotropic support and ICU admission.Remarkably, all our patients showed significant improvement in their cardiac disease within a few days as evident on serial echocardiographic evaluation. However, we stress the need for long-term follow-up as one of our patients demonstrated mild LV myocardial scarring as evident by gadolinium late enhancement on a cardiac MRI.

Background and Objectives: Experience with alteplase in pediatric patients is limited and recommendations are extrapolated from adult data. Comprehensive guidelines on the management of thromboembolic events in this group are lacking. We assessed the efficacy and safety of alteplase (recombinant tissue plasminogen activator) in the management of intracardiac and major cardiac vessel thrombosis in pediatric patients., Methods: All pediatric patients, 14 years of age and younger, with intracardiac or major cardiac vessel thrombus who were treated with alteplase from 1997 to 2004 at our tertiary care institute were identified through the pharmacy database. Patient data were retrospectively evaluated for the efficacy and safety of altepase., Results: Five cases were eligible out of nineteen who received alteplase. Patient ages ranged from 40 days to 13 years. The initial dose of alteplase ranged from 0.3 to 0.6 mg/kg followed by a continuous infusion in three patients with a dosage range between 0.05 and 0.5 mg/kg/hr, while intermittent infusion was used in the other two patients. The duration of therapy ranged from 2 to 4 days. By the end of the treatment, two patients had complete resolution of thrombus and one had partial resolution. Two patients failed to respond and had "old" thrombus. Major bleeding events were reported in three patients. The rest had minor bleeding events., Conclusion: Alteplase may effectively dissolve intracardiac thrombi, particularly when freshly formed. Continuous infusion for a long duration appears to be associated with an increased risk of major bleeding. Optimal dose and duration of infusion are still unknown.

Background: We sought to identify the prevalence of bicuspid pulmonary valve among patients with transposition of the great arteries undergoing the arterial switch operation and evaluate functional integrity of that valve in the neoaortic position., Methods: Between October 1985 and December 2001, 391 patients had an arterial switch operation for transposition and its variants. Perioperative information and follow-up data were available for 342 patients. The serial echocardiograms of patients with bicuspid pulmonary valve were reviewed. The neoaortic valve was serially assessed, focusing on aortic insufficiency, annulus diameter, and pressure gradients., Results: Twenty-four patients (7%) had a bicuspid pulmonary valve. Age at operation was 5 days to 12 years. Two patients were lost to follow up, and 22 patients had mean follow-up of 5.3 years (range, 2 months to 13 years), of which 21 patients were alive and 1 died late. At least two postoperative echocardiogram reports were available on 19 patients. Seven patients had no neoaortic regurgitation, and 10 had trivial regurgitation. Severe aortic regurgitation developed in 1 patient with endocarditis and in another with repair of Taussig-Bing anomaly. Neoaortic valve size indexed to body surface area showed an increase in annular diameter over time proportional to somatic growth. No significant valve stenosis developed., Conclusions: Encountering a bicuspid pulmonary valve at the time of an arterial switch operation is not uncommon. The integrity of a bicuspid pulmonary valve in the neoaortic position is maintained at a mean follow-up of 5.3 years. We believe that the presence of a bicuspid pulmonary valve is not a contraindication to an arterial switch operation.

Ketamine and midazolam are commonly used in children undergoing cardiac catheterization. However, there is controversy regarding the safety of administering these agents in the absence of an anesthesiologist. We retrospectively reviewed pediatric cardiac catheterization procedures at our institution between 1996 and 1997. A total of 154 patients (0.3-192 months) underwent a total of 205 procedures. They received ketamine (n = 79, 1.05 +/- 0.88 mg/kg/hr), midazolam (n = 35, 0.14 +/- 0.09 mg/kg/hr), or both (n = 91; ketamine, 1.13 +/- 0.84 mg/kg/hr; midazolam, 1.57 +/- 1.03 mg/kg//hr). In 18.5% of patients there were complex cardiac lesions. Mean procedure time was 79 +/- 36.2 minutes. Pre- and postprocedure systolic and diastolic mean blood procedure 72 +/- 14 and 68 +/- 12 mmHg, respectively. Pre- and postprocedure O2 saturation was 93.19 +/- 8.72 and 93.63 +/- 8.3, respectively. One patient required intubation, and 15% required oxygen therapy. The mortality rate was zero. The anesthesiologist's assistance was requested by the cardiologist in 21 procedures (group A) and not requested in 184 procedures (group B). The two groups were not different in relation to the drug used (p = 0.283) or the complexity of the cardiac lesions (p = 0.051). However, there was significant difference between the two regarding the need for supporting drugs (3/21 vs 3/184, p = 0.02) or oxygen treatment (7/21 vs 26/184, p = 0.014). No patients in group B required intubation, whereas 14% and 1.6% required oxygen therapy and supporting drugs, respectively. We conclude that low-dose ketamine and midazolam can be administered safely to most pediatric patients by the cardiologist, who can safely predict the need for an anesthesiologist.

We report two siblings with isolated ectopic hearts. Neither child had associated congenital diseases. To the best of our knowledge, this is the first reported familial occurrence of ectopic hearts.

We sought to determine if a higher dose of heparin would reduce arterial complications in patients weighing 10 kg or less undergoing cardiac catheterization to investigate congenital heart disease. Sixty patients were given either 100 (group A) or 150 (group B) IU x kg(-1) of heparin in a double-blinded randomized manner. Initial arterial access was established using a 4F cannula in all patients. Mean activated clotting time measured 20 minutes following heparin administration was significantly lower in group A than in group B (199 versus 251 seconds). Only 3 out of 60 patients (5%) required treatment for loss of femoral pulse. The age, weight, activated clotting time, length of catheterization procedure, time taken to establish arterial access, and the duration of arterial cannulation were comparable between the groups. Weight under 4 kg, age under 1 month, and cannula size larger than 4F were identified as independent risk factors for the development of arterial complications. Arterial access using a 4F cannula is a safe procedure in children weighing 10 kg or less. The incidence of significant arterial complications is low, and they do not appear to be preventable by a higher dose of heparin.

We present a case of tetralogy of Fallot associated with Scimitar syndrome. The patient was an 11-month old female who underwent successfully total repair of her lesion, including rerouting of the anomalous pulmonary vein to the left atrium. The diagnosis was suspected from the chest x-ray and echocardiography, and confirmed by angiography. To the best of our knowledge only 2 additional cases have previously been reported.

Three patients with patent ductus arteriosus and moderate aortic stenosis had a marked reduction in aortic valve gradient following transcatheter ductal occlusion. The hemodynamic effects of an aortopulmonary shunt on the severity of left ventricular outflow obstruction and the implications on intervention are discussed.

This study was performed to evaluate the efficacy of transcatheter coil closure of the patent ductus arteriosus in comparison to our experience with the Rashkind umbrella device. Transcatheter coil closure of the patent ductus arteriosus has been reported with encouraging results. We present our experience with ducti up to 5.0 mm in diameter and report the short-term follow-up. We compare the results with our previous experience with the Rashkind umbrella device. Seventy-one patients underwent transcatheter coil closure. Median age was 3.1 years, and median weight was 13.6 kg. Mean ductus diameter was 2.0 +/- 1.1 mm. These were compared with 105 patients who underwent transcatheter closure using a single Rashkind umbrella device. The median age was 3.2 years and the median weight was 14.0 kg. The mean ductus diameter for this group was 2.1 +/- 0.6 mm. The ductus murmur in the coil group disappeared in all patients. Immediate (< or = 24h), complete closure was achieved in 89% of the coil group as compared to 71% for the Rashkind umbrella device group (P < 0.005). Closure rate for the coil group was 97% at the 6-month follow-up, vs. 82% for the Rashkind umbrella device group at the 6-12 month follow-up (P < 0.05). In almost all patients requiring more than one coil, the ductus was crossed serially from the aortic end. All patients with ductus diameter > or = 3.0 mm required two or more coils. Eleven coils in six patients embolized to the pulmonary arteries. All coils except one were retrieved with subsequent successful foil placement. Sixty-seven patients (94%) in the coil group were discharged in < or = 24 h. Transcatheter closure of the patent ductus arteriosus using multiple coils is a more effective technique than the Rashkind umbrella closure and has excellent short-term results. This can be performed safely as an outpatient procedure.

We report the immediate results and the short-term follow-up in a group of selected patients with coarctation of the aorta who underwent endovascular stent implantation. Balloon-expandable stents were implanted in 6 patients (mean age 19.8 +/- 5.1 years) with coarctation of the aorta (4 recurrent and 2 native) who underwent a total of 7 procedures (6 implantation and 1 further expansion). The systolic peak pressure gradient was decreased from 36.7 +/- 16.9 to 13.3 +/- 23.2 mm Hg (P < 0.005). There was a 66% increase in the mean coarctation diameter from 9.3 +/- 1.7 to 15.6 +/- 3.1 mm (P = 0.001) with the ratio of the coarctation to descending aorta diameter, measured at the level of the diaphragm, increasing from 0.49 +/- 0.1 to 0.81 +/- 0.2 (P < 0.005). The dilatation was successful in expanding the stent to an acceptable diameter in 5 of 6 patients. One patient underwent successful further expansion of a stent implanted 22 months previously. There were no immediate complications during balloon expansion and stent implantation. One patient suffered a femoral arterial bleed requiring surgical repair. There was one unrelated death. All patients were hypertensive (systolic blood pressure > 140 mm Hg) prior to stent implantation. At mean follow-up of 8 months, 3 patients are normotensive. There was no recurrence of coarctation, aortic dissection, or aneurysm formation in the patients in whom stent implantation was successful. These findings indicate that balloon-expandable stent implantation for coarctation of the aorta in selected patients is a safe and effective alternative approach for relieving the obstruction with a low complication rate and no recoarctation at short-term follow-up.