Immediate effects and outcome of in-utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis.

OBJECTIVE: To assess the immediate effects of fetal pulmonary valvuloplasty on right ventricular (RV) size and function as well as in-utero RV growth and postnatal outcome. METHODS: Patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS) who underwent fetal pulmonary valvuloplasty at our center between October 2000 and July 2017 were included. Echocardiographic data obtained before and after the procedure were analyzed retrospectively (median interval after intervention, 1 (range, 1-3) days) for ventricular and valvular dimensions and ratios, RV filling time (duration of tricuspid valve (TV) inflow/cardiac cycle length), TV velocity time integral (TV-VTI) × heart rate (HR) and tricuspid regurgitation (TR) velocity. Longitudinal data were collected from only those fetuses followed up in our center. Outcome was assessed using the scoring system as described by Roman et al. for non-biventricular outcome. RESULTS: Thirty-five pulmonary valvuloplasties were performed in our institution on 23 fetuses with PAIVS (n = 15) or CPS (n = 8). Median gestational age at intervention was 28 + 4 (range, 23 + 6 to 32 + 1) weeks. No fetal death occurred. Immediately after successful intervention, RV/left ventricular length (RV/LV) ratio (P ≤ 0.0001), TV/mitral valve annular diameter (TV/MV) ratio (P ≤ 0.001), RV filling time (P ≤ 0.00001) and TV-VTI × HR (P ≤ 0.001) increased significantly and TR velocity (P ≤ 0.001) decreased significantly. In fetuses followed longitudinally to delivery (n = 5), RV/LV and TV/MV ratios improved further or remained constant until birth. Fetuses with unsuccessful intervention (n = 2) became univentricular, all others had either a biventricular (n = 15), one-and-a-half ventricular (n = 3) or still undetermined (n = 3) outcome. Five of nine fetuses with a predicted non-biventricular outcome, in which the procedure was successful, became biventricular, while two of nine had an undetermined circulation. CONCLUSION: In selected fetuses with PAIVS or CPS, in-utero pulmonary valvuloplasty led immediately to larger RV caused by reduced afterload and increased filling, thus improving the likelihood of biventricular outcome even in fetuses with a predicted non-biventricular circulation. © 2018 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

[Medical prescriptions to premature and newborn infants in an Austrian neonatal intensive care unit]. / Verschreibepraxis von Arzneimitteln bei Früh- und Neugeborenen auf einer österreichischen neonatologischen Intensivstation.

BACKGROUND: In December 2006, the European Commission issued the Regulation 1901/2006 with the aim to improve the development of medicines for children, because currently many of the pediatric drugs are used outside their authorization. The aim of the present study was to establish a status quo of the medical prescriptions to patients admitted to a neonatal intensive care unit. METHOD: The records of all patients admitted during a three-month period were analyzed retrospectively. Study endpoints were all prescribed medications, including dosage, route and form of administration. Medications were categorized in three groups: licenced, unlicenced and off-label medications. RESULTS: A total of 81 patients with a total of 84 admissions (3 re-admissions) had a median length of stay of 6 days (range: 1-101 days) in the intensive care unit. Of the 84 admissions, 20 were attributable to heart surgery conditions and 11 to extremely low birth weight (less than 1 000 g). A total of 748 prescriptions of 82 different medications were registered with an average of 6 (range: 1-38) different drugs per stay. 364 prescriptions (49%) were licenced, 250 (34%) were off-label, and 134 (18%) were unlicenced. During 77% of all 84 admissions, we used at least one off-label or unlicenced drug. CONCLUSION: The data of our study demonstrate that the availability of licenced drugs for neonates is greatly limited. The results are consistent with studies in other European countries. This study underlines once more the need of adequate testing of medicines in children to assess their safety and efficacy. Whether the new European regulation will achieve this goal remains to be seen.

Early and mid-term outcome of the arterial switch operation in 114 consecutive patients : A single centre experience.

BACKGROUND: The arterial switch operation (ASO) has become the treatment of choice in patients with simple or complex transposition of the great arteries (TGA). The purpose of this study was to assess early and mid-term outcome after ASO in a single centre. PATIENTS AND METHODS: Between 1995 and December 2005, 114 consecutive patients underwent an ASO at our institution, performed by one single surgeon. Patients charts, surgical reports and echocardiograms were retrospectively reviewed. Patients were analyzed in 3 different groups: Group I consisted of 77 neonates with TGA and intact ventricular septum, group II of 13 patients with TGA and ventricular septal defect which had to be closed surgically and group III of 24 patients with various forms of TGA in a complex setting. The patient's median weight was 3.23 kg (1.65-8.30). Twenty-five neonates were born preterm, 18 were diagnosed prenatally. Median follow-up time was 20.7 months (0.3-128.6). RESULTS: The thirty day mortality was 1.75% (2/114), late mortality 0.88% (1/112) accounting for an overall mortality of 2.63%. There was only one early coronary event and so far no late events. Ten of 111 survivors required reoperation, all but 1 from group III. Prevalence of supravalvular pulmonary stenosis was 4.7%. Freedom from reoperation at 5 years of follow-up time was 87.5%. One patient required permanent pacing, no other late arrhythmias occurred. In our series the only risk factor for increased mortality and morbidity was a body weight of less than 2500 g at the time of operation. No better outcome could be demonstrated in the prenatally diagnosed patients. CONCLUSION: The ASO can be performed safely and with low mortality and morbidity even in patients with complex TGA. Follow-up of these patients is required to detect residual problems like supravalvular pulmonary stenosis, coronary problems, arrhythmias and aortic valve dysfunction.

Increased prevalence of latex-sensitization among children with chronic renal failure.

BACKGROUND: Type-I-allergy to natural rubber latex (NRL) has been shown to be more prevalent among certain groups of patients. Children suffering from chronic renal failure (CRF) could be a suspected risk group because of their intense exposure to latex through catheters, gloves and anesthetic equipment during frequent hospitalizations from early life on. We investigated the prevalence of latex-sensitization among this group of patients and sought to identify risk factors. METHODS: Ninety-three patients (mean age 10.5 years) suffering from CRF were assessed by questionnaire-based history (details on renal disease, number and kind of surgical procedures, family and personal history of atopic diseases, allergic reactions to NRL, and the use of pacifiers) and by measurement of total and latex-specific serum immunoglobulin (Ig)E. RESULTS: Ten of 93 (10.8%) patients showed elevated latex-specific IgE-levels. One of 10 patients reported clinical symptoms to latex-allergen, but no allergic reactions to NRL during medical care were reported. Sensitized patients were significantly more likely to be atopic, reflected by a positive history of other allergies as well as elevated total serum IgE-levels, and had a significantly higher number of urogenital surgeries (P = 0.02 in all cases, Fisher's exact and Wilcoxon test, respectively). CONCLUSION: This study demonstrates that children with CRF are at increased risk of latex-hypersensitivity. Significant associations with atopy and repeated surgeries were observed. Larger studies are required to elucidate whether these children are also at increased risk of anaphylaxis and therefore deserve preventive measures.