Long-Term Outcomes and Characteristics Associated With Mortality of Adult Patients Post Fontan Surgery: 27-Year Single-Center Experience.

Data on the characteristics and long-term outcomes of patients who underwent Fontan surgery and surviving into adulthood are limited. We aimed to describe our center's long-term experience with this unique patient population. Included were adult patients who had undergone Fontan surgery and were followed up at our Adult Congenital Heart Disease clinic between the years 1994 and 2021. We describe cardiac and noncardiac morbidities, medical treatment, laboratory data, echocardiographic characteristics, and all-cause mortality. The primary outcome was a composite of heart failure hospitalizations or death. A total of 107 patients who underwent Fontan surgery were followed up during the study period; 46.7% were male. The mean age at time of Fontan was 7.4 ± 6.2 years and the mean age at the last follow-up or at the time of an outcome event was 35.0 ± 8.0 years (range 21.1 to 62.8). At the last documented follow-up, 74.7% of the cohort were in New York Heart Association functional class I/II. The common morbidities included atrial arrythmias (37%) and stroke (17%). The primary outcome occurred in 17.7%. By the end of the study period, 9.3% of the patients in the cohort died. In a multivariate logistic regression analysis, controlling for gender, age, and Fontan type, worse functional class at the last follow-up (New York Heart Association III/IV vs I/II) was significantly associated with the risk of the primary outcome (odds ratio 34.57, 95% confidence interval 6.728 to 177.623, p <0.001). In conclusion, long-term outcomes of patients surviving into adulthood with a Fontan circulation is encouraging. Most of these patients achieve good functional cardiovascular status, despite the complex anatomy and a substantial burden of co-morbid conditions, specifically, atrial arrythmias and thrombotic events. Functional class was independently associated with heart failure hospitalizations and mortality.

Low Risk of Inappropriate Shock Among Pediatric Patients With an Implantable Cardioverter Defibrillator: A Single Center Experience.

Implantable cardioverter-defibrillators (ICD) are increasingly being used among the pediatric population for indications of both primary and secondary prevention. There is limited long-term data on the outcomes of pediatric patients following ICD implantation. The aim of this study was to investigate the characteristics of this population, burden of appropriate and inappropriate shock and complication rate in a large tertiary pediatric medical center. Included were children under the age of 18 years who underwent ICD implantation and had clinical follow up at our center. Data were retrospectively collected between study period 2005-2020. Primary outcome was the incidence of ICD shock appropriate and inappropriate. Secondary outcome was defining our patient population characteristics. Our cohort included 51 patients who underwent ICD implantation. Mean age at implantation was 10.9 ± 4.7 years and average follow-up time was 67 months. Diagnoses of implanted patients were: 28 (55%) patients with syndromes with risk for sudden death, cardiomyopathy in 14 patients (27%) and congenital heart disease (CHD) in 9 patients (18%). Forty-two (82%) patients had an ICD implanted for secondary prevention after experiencing a life-threatening arrhythmia and 9 (18%) for primary prevention. An endocardial system was implanted in 39 (76%) patients and an epicardial systems in 12 (24%) patients. A total of 20 (39%) patients received appropriate shocks for ventricular fibrillation(VF). 5 patients received inappropriate shocks, 4 due to sinus tachycardia and 1 due to rapidly conducted atrial fibrillation. Those who received an inappropriate shock had a significantly shorter ICD-programmed VF detection cycle length compared to those who did not receive an inappropriate shock (320 ms versus 270 ms, p = 0.062). This single center study demonstrates a high rate of appropriate ICD shocks (39%) and a low rate of inappropriate ICD shocks. Accurate programming of ICD devices in the pediatric population is paramount to avoid inappropriate ICD shocks.

Randomised study found that improved nutritional intake was associated with better sleep patterns in prepubertal children who were both short and lean.

AIMS: Nutrition and sleep are prerequisites for linear growth and we addressed the under-researched role of sleep in this equation. METHODS: This was a prospective randomised, double-blinded, placebo-controlled study of nutritional supplements in 164 healthy lean, short, prepubertal children with 83 in the supplement group and 81 in the placebo group. From November 2010 to November 2013, we focussed on children aged three to nine years referred for specialist growth assessments to the Schneider Children's Medical Center, Israel. Progress was assessed using anthropometric measurements, sleep questionnaires and three-day food diaries at baseline and after the six-month intervention. RESULTS: Children in the supplement group who took at least 50% of the recommended dose had shorter sleep latency than those who did not (p = 0.046). Children who fell asleep in less than 15 minutes had significantly improved standard deviation scores for weight (0.25 ± 0.34 versus 0.07 ± 0.36, p = 0.044) and height (0.09 ± 0.13 versus 0.03 ± 0.13, p = 0.057) than those who took longer to fall asleep. Positive correlations were found between mean sleep duration and caloric and macronutrient intake per kilogram. CONCLUSION: Adequate nutritional intake was associated with better sleep patterns and may enhance linear growth.

Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment.

Neonatal hemochromatosis (NH) is an acute liver disease associated with both hepatic and extrahepatic iron deposition and is a leading cause of neonatal liver transplantation. The concept that NH is an alloimmune disease has led to the emergence of a new treatment approach utilizing exchange transfusion and intravenous immunoglobulin therapy. We present a two-day old neonate with progressive liver dysfunction who was diagnosed with NH. Magnetic resonance imaging confirmed tissue iron overload. Treatment with intravenous immunoglobulins and exchange transfusion led to rapid improvement in liver function. Follow-up physical examination at the age of 8 months showed normal development and near normal liver function. A repeat abdominal magnetic resonance scan at 8 months showed no signs of iron deposition in the liver, pancreas, or adrenal glands. The present report provides further support for the use of exchange transfusion and immunoglobulin therapy in NH and is the first to document resolution of typical iron deposition by magnetic resonance imaging.

Osteomyelitis of the mandible in a group of 33 pediatric patients with congenital insensitivity to pain with anhidrosis.

OBJECTIVE: To determine the frequency of mandibular osteomyelitis (OM) in patients with congenital insensitivity to pain with anhidrosis (CIPA) and to relate its appearance to possible risk factors. METHODS: The records of 33 patients were reviewed for data concerning events of jaw OM, oral trauma, maxillofacial interventions, or OM of long bones. RESULTS: Eighteen percent of the patients had mandibular OM. Of the six patients, preceding oral laceration was documented in one and tooth extraction in two. Seventy percent of the patients had OM of the limbs, but only 15% overlapped, having both jaw and limb OM. Half of the patients with mandibular OM had also OM of the limbs during the following year. There seems to be a correlation between high frequency of limb OM (at least 5 events per patient) and appearance of mandibular OM. CONCLUSION: The incidence of mandibular OM is very high among patients with CIPA and can result in pathologic fracture and the need for open reduction and internal fixation. The reason for this phenomenon is presently not clear. Preventive and therapeutic strategy for CIPA patients should be undertaken to minimize this severe complication.