Category: Paediatric Cardiology

Abstract: Patent ductus arteriosus (PDA), the most commonly diagnosed cardiovascular condition in preterm infants, is associated with increased mortality and harmful long-term outcomes (chronic lung disease, neurodevelopmental delay). Although pharmacologic […]

Abstract: Childhood undernutrition causes significant morbidity and mortality in low- and middle-income countries (LMICs). In Tanzania, the in-hospital prevalence of undernutrition in children under five years of age is approximated […]

Abstract: We aimed to compare cardiometabolic indicators in singletons and multiples at age 7 and explore the birthweight mediation effect.We studied 5431 singletons and 103 sets of multiples from Generation […]

Abstract: Few data are currently available for Kawasaki disease (KD) below 12?months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort […]

Abstract: Traditional percutaneous device closure of perimembranous ventricular septal defects (PmVSDs) is a minimally invasive technique, but can result in high radiation exposure and can result in potential arterial complications. […]

Abstract: Scimitar syndrome is a rare form of partial anomalous pulmonary venous drainage associated with pulmonary hypertension and congestive heart failure that may lead to death in the newborn infant. […]

Abstract: Acute left ventricular (LV) systolic failure as a consequence of acute severe brain injury with status epilepticus in a young infant is not common; managing such a patient on […]

Abstract: High-risk patients in the pediatric intensive care unit (PICU) contribute substantially to PICU-mortality. Complex chronic conditions (CCCs) are associated with death. However, it is unknown whether CCCs also increase […]

Streptococcus pasteurianus is a rare cause of neonatal infection, with only 3 cases reported in the USA and 18 cases reported in other countries within the past decade. Neonatal S. pasteurianus infection typically presents as meningitis. This case report describes the first neonatal case of S. pasteurianus endocarditis in the literature, in addition to a neonatal case of S. pasteurianus infection presenting as pneumonitis without meningitis. The S. pasteurianus infections in these two cases are unusual not only because of how rare this particular pathogen is, but also because of the atypical clinical manifestations.The first patient is a full-term male infant admitted to NICU at 20?h of life due to respiratory distress. He was empirically started on ampicillin and gentamicin for presumed sepsis. Laboratory analysis of cerebral spinal fluid obtained after initiation of antibiotics was suggestive of partially treated meningitis. Blood cultures came back positive for S. pasteurianus. The neonate was transitioned from ampicillin to cefepime, while gentamicin was continued. Echocardiograph showed a possible tricuspid valve vegetation concerning for endocarditis. Due to the unusual complication of endocarditis, the patient remained on IV cefepime for 28?days rather than the more conventional duration of 14-21?days reported in the literature. The baby clinically improved with no evidence of thrombi or vegetations on repeat cardiac echo. The second patient is a full-term male infant who required intubation at birth for respiratory distress. Chest X-ray findings were concerning for meconium aspiration with pneumonitis. The baby went into septic shock and was empirically started on ampicillin and gentamicin. Blood cultures came back positive for S. pasteurianus, while cerebral spinal fluid and urine cultures were negative. Ampicillin and gentamicin were discontinued after 3?days and the baby was started on cefepime and clindamycin for a total 14-day course. The baby clinically recovered and was discharged from NICU without any sequelae.These two cases highlight the importance of recognizing S. pasteurianus as a potential cause of neonatal sepsis and the importance of recognizing endocarditis and pneumonitis as possible clinical manifestations of this infection.

Marfan syndrome (MFS) is a heritable connective tissue disease caused by a defect in FBN1. The diagnosis is based on the revised Ghent criteria. The main features involve the cardiovascular, musculoskeletal, ophthalmic, pulmonary systems and facial features. Although the clinical manifestations of MFS in children are thoroughly addressed in several studies, literature on the impact of MFS on daily functioning is restricted to pediatric advice on sports and leisure participation. Therefore, the full impact of MFS on daily functioning remains unclear. The aim of this qualitative study was to explore parents’ perspectives on the impact of MFS on daily functioning of children with MFS aged 4-12?years, themselves and family regarding functional performance, activities, participation, personal and environmental factors, and disease burden.In this qualitative study parents participated in individual semi-structured interviews (n =?10) and 3 focus groups (n =?5, n =?5 and n =?6). Meetings were transcribed, and data were analyzed using thematic analysis. Meaningful concepts were coded, and concepts concerning children with MFS were linked to the International Classification of Functioning, Disability and Health for Children and Youth. Thereafter themes were identified and interpreted.Parents reported their children could not keep up with peers because of fatigue, pain and physical impairments. Children experienced participation restrictions in school, sports, play and other leisure activities. Parents reported their child as being different due to physical appearance, which provoked unsupportive attitudes. Parental burden was caused by high care needs, lack of support, a limited social life, and concerns about the child’s development. Family burden was caused by adjusted and complex family schedules, other family members with MFS, and reproductive planning decision-making, whereas family cohesiveness and caring were positively perceived factors.Parents perceived a large impact of MFS on daily functioning of their children with MFS, themselves and their family. More awareness among all professionals involved in the care of children with MFS and their families is needed so that professionals can address their support needs and provide tailored interventions, rehabilitation and/or educational programs to empower and improve daily functioning of the children, parents and family.

Reactivation of the Bacillus Calmette-Guérin (BCG), manifesting as erythema, induration, ulceration or crust formation at a previous BCG inoculation site, is a common and highly specific feature of Kawasaki disease (KD). We report the unusual finding of BCG reactivation in an infant with laboratory-confirmed measles.A previously healthy 7-month old infant presented initially with fever, cough and coryza, and subsequently developed Koplik’s spots followed by a typical morbilliform skin rash. There was significant contact history with a household relative who had recently been diagnosed with measles. On examination, a 2.5?cm area of erythema and induration was seen at the previous BCG inoculation site, in addition to the widespread maculopapular rash. No other clinical features of KD were present. Measles virus was isolated from the throat swab and measles antibodies (IgM) were present in the serum. The patient recovered completely with oral vitamin A and supportive therapy, and had normal echocardiography examination on follow up.This case report highlights the rare finding of BCG reactivation in a child with confirmed measles infection, and suggests that this clinical manifestation may occasionally occur in children with infections or conditions other than KD.

During the first three months of pregnancy, the developing embryo may be susceptible to external and internal factors, which may lead to structural and functional congenital anomalies. The main objective of this study was to determine the prevalence of congenital anomalies in Addis Ababa and the Amhara region, Ethiopia.A descriptive cross-sectional study was conducted on children 0-17?years of age who visited the 16 selected hospitals in Addis Ababa and the Amhara Region between January 1 and July 5, 2015. The proportions of neonates, infants, and children with external and internal congenital anomalies whether the anomalies were major or minor were estimated.Out of 76,201 children, 1518 of whom 57.6% were male identified with congenital anomalies. The overall proportion of congenital anomaly was 1.99% (95% CI: 1.89-2.091) i.e., 199 per 10,000 children. The proportion of neural tube defects, orofacial clefts, masculo-skeletal system anomalies, syndrome disorders, and cardiovascular system problems were 40.3% 37.7-43, 23.3% 21.3-25.4, 23.1% 20.9-25.2, 8% 6.7-9.4, and 2.6% 1.8-3.4, with a 95% CI, respectively. The majority (72.5%) of the mothers were multigravidae; 38(2.5%) of the mothers and 32(2.1%) of the fathers had history of other children with congenital anomalies. Similarly, 20(1.3%) of the participant children’s mothers and 17(1.1%) of the fathers had familial history of congenital anomaly. Iron folate and multivitamin use by mothers during preconception and early pregnancy was found to be low.Neural tube defects, orofacial clefts, and musculoskeletal anomalies were the observed prevalent problems. Maternal illness, viral infections, and malnutrition were seen in a significant number of the mothers. Iron folate/folic acid and multivitamin use by the mothers during and before pregnancy was very low.

Cyanosis is usually associated with serious conditions requiring urgent treatment in the neonatal intensive care unit (NICU). Hemoglobin M (Hb M) disease is one type of congenital methemoglobinemia characterized by cyanosis. Among these variants, ?-globin chain mutations such as Hb M Boston present cyanosis from birth while other variants usually manifest later in life.We report a case of a male newborn with cyanosis apparent since birth. Surprisingly, his respiratory and hemodynamic status including normal arterial blood oxygen saturation was stable, but oxygen saturation on pulse oximetry did not increase after 100% supplemental oxygen was started. In addition to routine pulmonary and cardiologic evaluation, further evaluation for dyshemoglobin was conducted; ?2-globin gene sequencing showed a single-point variant causing Hb M Boston. Methemoglobin (MetHb) level estimated by co-oximetry was normal. After a 14-day stay in the NICU, the patient remained respiratory and hemodynamically stable without supplemental oxygen except for cyanosis.Hb M disease is a benign disease and does not require any treatment whereas acquired methemoglobinemia is a potentially fatal condition. Neonatologists should be aware that low oxygenation status on pulse oximetry in the face of normal arterial blood saturation values might indicate the possibility of Hb M disease in early neonatal cyanosis, irrespective of MetHb value.

Payer-type (government-sponsored health coverage versus private health insurance) has been shown to influence a variety of cardiovascular disease outcomes in adults. However, it is unclear if the payer-type impacts the response to a lifestyle intervention in children with dyslipidemia.We analyzed data prospectively collected from patients under the age of 25?years who were referred to a large regional preventive cardiology clinic from 2010 to 2016 in Massachusetts. We compared baseline high density lipoprotein cholesterol (HDL-C), triglycerides (TG), non-HDL-C, and low density lipoprotein cholesterol (LDL-C) by payer-type. Further, we analyzed the change in lipid values in response to a clinic-based multidisciplinary intervention over a nearly six-year period by payer-type with multi-variable adjusted linear regression models. We also tested for effect modifications by age, sex, race, and body mass index (BMI) category.Of the 1739 eligible patients (mean age 13?years, 52% female, 60% overweight and obese, 59% White), we found that patients with government-sponsored coverage (n?=?354, 20%) presented to referral lipid clinic with lower HDL-C (-?3.5?mg/dL [1.0], p? » Read more

Abstract: Familial hypercholesterolemia (FH), the most frequent monogenetic hereditary disorder, is underdiagnosed and undertreated. Early identification of FH is essential because of the increased risk for premature cardiovascular diseases and […]