Reversal of Fetal Heart Block in Antibody-Positive Mother After Hydroxychloroquine and Dexamethasone.

Maternal autoantibody-related complete heart block in the fetus is considered irreversible. During prenatal care for a 25-year-old nulliparous Hispanic woman with newly diagnosed nephrotic-range proteinuria and positive anti-nuclear antigen antibody, complete fetal heart block with a ventricular rate of 60 beats per minute was detected on a fetal echocardiogram at 28-week gestation. A small pericardial effusion and ascites were noted consistent with fetal hydrops. Dexamethasone and hydroxychloroquine were initiated. Fetal rhythm improved to Mobitz type 1 second-degree heart block, with a ventricular rate of 91 beats per minute. The fetus was born prematurely at 34-week gestation with second-degree heart block which improved to first-degree heart block prior to hospital discharge. First-degree heart block persisted at 2 years of age with a P-R interval of 185 ms. Transplacental treatment with dexamethasone and hydroxychloroquine was associated with sustained reversal of complete heart block to sinus rhythm.

Vein of Galen Aneurysmal Malformation with Anomalous Right Superior Vena Cava to the Left Atrium Leading to Atypical Clinical and Echocardiographic Findings.

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM.

Mechanical tricuspid valve replacement in hypoplastic left heart syndrome: An institutional experience.

Objective: Atrioventricular valvar regurgitation in patients with single ventricles is associated with worse outcomes. Valve repair or replacement has been undertaken in an attempt to reduce mortality and morbidity. Current data on valve replacement in single ventricle patients are limited and derived from heterogenous populations. We describe our experience with repair and replacement of the tricuspid valve in children undergoing single ventricle palliation for hypoplastic left heart syndrome. Methods: We included 27 patients with hypoplastic left heart syndrome with at least moderate tricuspid regurgitation who underwent valve intervention between 2007 and 2021 at our institution; charts were retrospectively reviewed for data. Results: Eleven patients (73% male) underwent valve replacement (median age, 3 years). Preoperative ventricular systolic function was normal in 10 patients (91%). Median follow-up postoperatively was 4 years with no early mortality, 1 (9%) late mortality, and 1 heart transplant (9%). Morbidity consisted of complete heart block in 1 patient (9%), with no important hemorrhagic or thrombotic events. Among survivors of replacement (n = 9), ventricular function was preserved in all (n = 8) who had normal function preoperatively and improved in the remaining patient. Sixteen patients underwent valve repair (median age, 4 months) with no early mortality, 8 (50%) midterm mortalities, and 2 heart transplants (12%). Conclusions: Tricuspid valve replacement is a feasible option in hypoplastic left heart syndrome with significant tricuspid regurgitation, with favorable outcomes in the intermediate follow-up. When undertaken in the setting of normal function, ventricular function may be preserved in up to 80% of patients. Long-term follow-up is needed.

Role of Cross-Sectional Imaging in Pediatric Interventional Cardiac Catheterization.

Management of congenital heart disease (CHD) has recently increased utilization of cross-sectional imaging to plan percutaneous interventions. Cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) imaging have become indispensable tools for pre-procedural planning prior to intervention in the pediatric cardiac catheterization lab. In this article, we review several common indications for referral and the impact of cross-sectional imaging on procedural planning, success, and patient surveillance.

Congenital nephrotic syndrome in a Hispanic Guatemalan newborn associated with a NPHS1 variant: A case report.

Congenital nephrotic syndrome (CNS) is an autosomal recessive disorder usually detected in the first 3 months of life when the syndromes effects manifest, including edema and a failure to gain weight. A baby boy was admitted to the Neonatal Intensive Care Unit for prematurity (35 weeks) with unremarkable maternal prenatal laboratory tests. The patient had persistent systemic hypertension, hypoproteinemia, hypoalbuminemia and nephrotic range proteinuria. CNS was diagnosed, and genetic testing showed a homozygous variant, c.3024A>G (AGA>AGG) in exon 22 of the nephrin locus. Bioinformatics analysis suggested the genetic condition was likely a result of malfunctional DNA binding sites of transcription factors FOXL1 and FOXC1.

Fly control to prevent diarrhoea in children.

BACKGROUND: Diarrhoeal disease accounts for millions of child deaths every year. Although the role of flies as vectors of infectious diarrhoea has been established, fly control is not often mentioned as an approach to decrease childhood diarrhoea. Theoretically, fly control for decreasing diarrhoea incidence can be achieved by intervening at four different levels: reduction or elimination of fly breeding sites; reduction of sources that attract houseflies; prevention of contact between flies and disease-causing organisms; and protection of people, food, and food utensils from contact with flies. OBJECTIVES: To assess the impact of various housefly control measures on the incidence of diarrhoea and its related morbidity and mortality in children under five years of age. SEARCH METHODS: We searched electronic databases including the Cochrane Central Register of Controlled Trials (CENTRAL) in the Cochrane Library, MEDLINE, Embase, CINAHL, and LILACS, from database inception to 24 May 2018. We also searched trial registries for relevant grey literature and ongoing trials. We checked the references of the identified studies and reviews. We did not apply any filters for language, publication status (published, unpublished, in press, and ongoing), or publication date. SELECTION CRITERIA: We planned to include randomized controlled trials (RCTs), quasi-RCTs, and controlled before-and-after studies that studied the effect of fly control on diarrhoea in children under five years of age. DATA COLLECTION AND ANALYSIS: Two review authors extracted the data and independently assessed the risk of bias in the included study. We planned to contact study authors for additional information, where necessary. We assessed the certainty of the evidence using the GRADE approach. MAIN RESULTS: We included one cluster-RCT (491 participants) conducted in Pakistan that evaluated insecticide spraying in the first two years and baited fly traps in the third year. Insecticide spraying reduced the fly population (house index) in the intervention group during the four months of the year when both flies and cases of diarrhoea were more common, but not at other times. On average, this was associated with a reduction in the incidence of diarrhoea in the first year (illustrative mean episodes per child-year in the intervention group was 6.3 while in the control group was 7.1) and second year of the intervention (illustrative mean episodes per child‒year in the intervention group was 4.4 while in the control group was 6.5; rate ratio (RaR) 0.77, 95% confidence interval (CI) 0.67 to 0.89, low-certainty evidence). In the third year of the intervention, the baited fly traps did not demonstrate an effect on the fly population or on diarrhoea incidence (RaR 1.15, 95% CI 0.90 to 1.47, low-certainty evidence). AUTHORS' CONCLUSIONS: The trial, conducted in a setting where there were clear seasonal peaks in fly numbers and associated diarrhoea, shows insecticide spraying may reduce diarrhoea in children. Further research on whether this finding is applicable to other setting is required, as well as work on other fly control methods, their effects, feasibility, costs, and acceptability.

Diagnostic paradox: Ruptured aneurysm of sinus of Valsalva simulating tricuspid valve endocarditis.

Rupture of aneurysm of sinus of Valsalva into the right atrium mimicking tricuspid valve endocarditis is a rare presentation. We review a case of spontaneous rupture of aneurysm of sinus of Valsalva into the right atrium presenting as a murmur. Transthoracic echocardiogram showed a mobile mass that appeared to be attached to the tricuspid valve leaflet with moderate tricuspid regurgitation suggestive of tricuspid valve endocarditis. The diagnosis was confirmed as spontaneous rupture of noncoronary sinus in to the right atrium by transesophageal echocardiogram. Patient recovered completely after surgical repair.

On-table Extubation after Open Heart Surgery in Children: An Experience from a Tertiary Care Hospital in a Developing Country.

BACKGROUND: Recent advances in various disciplines of medicine have significantly changed the courses following cardiac surgery in children. On-table extubation (OTE) after open heart surgery in children is evolving. OBJECTIVE: To assess the rate of postoperative complications in children extubated on table after open heart surgery. DESIGN: This is a retrospective, descriptive study. SETTING: Operating room (OR) then admitted to the pediatric intensive care unit (PICU). PATIENTS: All pediatric patients (between 0 and 18 years) undergoing open heart surgery between January 2011 and June 2013. INTERVENTION: On-table extubation. OUTCOME MEASURES: Rates of immediate postoperative complications, i.e., re-intubation, significant bleeding, low cardiac output syndrome, and arrhythmia in PICU, were assessed. Data are presented as frequencies and mean ± standard deviation. RESULTS: A total of 82 patients were included. Mean age at time of operation was 7.25 ± 6.6 years. Fifty-three percent (n = 44) were <5 years old and 64% (n = 53) were men. Ventricular septal defect (47%, n = 39) was the most common lesion, followed by atrial septal defect (36%, n = 30), and tetralogy of Fallot (15%, n = 12), which were repaired. Cardiopulmonary bypass and aortic cross clamp time were 72.3 ± 34.2 and 47.3 ± 27.8 minutes, respectively. The mean inotrope score was 2.66 ± 3.53. There was no mortality in the cohort, whereas 97.8% (n = 80) had no complications during PICU stay. One patient (1.1%) required re-intubation for respiratory failure and one patient (1.1%) had arrhythmia that was medically managed. The mean length of PICU stay was 1.77 ± 0.985 days. CONCLUSION: On-table extubation in children after open heart surgery was feasible and safe in selected group of patients. There was no major complication observed in the PICU.

Cost of care in a paediatric intensive care unit of a tertiary-care university hospital of Pakistan.

OBJECTIVE: To assess the cost of treatment for families of children hospitalised in paediatric intensive care unit of a tertiary care teaching hospital. METHODS: The retrospective cohort study was conducted in Aga Khan University Hospital, Karachi, and comprised record of all children admitted to the paediatric intensive care unit from January 1 to June 30, 2013. Demographic data, diagnosis at the time of admission, co-morbidity, length of stay in intensive care and outcome were recorded. The record of all hospital charges for each day the patient was cared for were also recorded. The finance department itemised the cost into major categories like pharmacy, radiology, laboratory, etc. SPSS 19 was used for statistical analysis. RESULTS: Record of 148 patients represented the study sample. Of them, 98(66%) were males. Overall median age was 2.7 yrs (interquartile range: 1 month to 16 years) and 93(62.8%) were below 5 years of age. Median length of stay was 3.5 days (range: 2-5 days) and total patient days in intensive care were 622. The median cost per admission was PKR 217,238 (range: (114,550-368,808) and mean cost per day was PKR 57,535 (43,911-85,527). The major cost distributions were bed charges PKR 8,092,080 (18.02%), physician charges PKR 6,398653(14.25%), medical-surgical supplies PKR 8,000772(17.8%), laboratory charges PKR 8,403,615(18.9%) and pharmacy charges PKR 5,852.226(13.03%). CONCLUSIONS: The cost of paediatric intensive care unit was expensive. Cost distribution was almost evenly distributed. Hence, a better admission policy is needed for resource utilisation and cost-effectiveness.

Clinical profiles and outcomes of children admitted to the pediatric intensive care unit from the emergency department.

The aim of this study was to describe clinical profiles and outcomes of children admitted directly from the Emergency Room (ER) to the Pediatric Intensive Care Unit (PICU) of academic hospital. The medical records of all children (1 month to 16 years) admitted in PICU from ER, from January 2011 to December 2012 were reviewed. Of the 26,774 patients seen in the ER during the study period, 468 (1.7%) were admitted to the PICU which constituted about 41.5% (468/1127) of all the total PICU admissions. Sixty three percent (n=294) were under-five; males were 60.9% (285), 82.3% (385) were in medical category. Neurological and respiratory illnesses were the most common groups (> 50% of all ER admissions). Multi-organ dysfunction syndrome and co-morbidity were present in 25.2% (n=118) and 23.5% (n=110) respectively. The mean length of stay was 5 ± 3.7 hours. The case-fatality rate was 20.3% (n=95) as compared to the overall PICU mortality rate of 11.9% (n=135).

Feasibility of pediatric plasma apheresis in intensive care settings.

Therapeutic plasma apheresis or exchange (TPE) in the pediatric population is technically challenging. Moreover, there is generally an apprehension in using TPE in children compared to adults. Recently, usage of TPE has evolved and is now being used in heterogenous clinical conditions. Its usefulness is classified by the American Society for Apheresis (ASFA) into various categories ranging from I to IV. The objective of this paper was to review the procedure in context of clinical indications, complications and outcomes in children. For this purpose, we retrospectively reviewed all TPE procedures performed on inpatients of 3 to 16 years of age during a 6-year period (2007-2012). A total of 130 procedures were performed on 28 patients (M : F ratio of 1:1) with median age (range) of 8.8 (4-16) years. All procedures were done using the continuous cell-separator centrifugal method. Due to organ dysfunctions, the majority of procedures (N = 26 of 28 or 92% patients) were performed in the pediatric intensive care unit. Twenty-three, four and one patient belonged to ASFA categories I, II and III, respectively. The most common indications were neurological disorders (N = 13 or 46.4%), comprised of Guillain-Barré syndrome (N = 10) and myasthenia gravis (N = 3). Hematological disorders (N = 10 or 35.7%) including thrombotic thrombocytopenic purpura-hemolytic uremic syndrome were a close second. Complete recovery was seen in 23 patients (84%). Trivial adverse effects were observed in 18/130 (13.8%) procedures. Major complications including cardiac arrest, hypotension and transfusion-related acute lung injury were observed in 5/130 or 3.8% of procedures. However, there was no procedure-related death though five patients died during treatment due to underlying pathology. In conclusion, TPE is a safe and effective option in sick children for appropriate indications. An experienced staff with sound procedural skills is imperative for successful therapy.

Benign granulomatous polyp obstructing the bronchus.

Inflammatory endobronchial polyps are rarely encountered. We report a case of a 14 years old girl with a benign granulomatous polyp originating in the bronchus. Computed tomography scan showed an intraluminal soft tissue opacity while fiberoptic bronchoscopy revealed a large soft tissue mass completely occluding the left lower lobe. Rigid bronchoscopy under general anaesthesia identified a fleshy mass in the left main stem bronchus which was excised via bronchoscopy. The histopathology showed benign polyp with non-specific inflammation. The patient's symptoms improved subsequently and no recurrence was reported till two years of follow-up.