Showing papers by "Mark P. Johnson published in 2018"
TL;DR: After cardiac surgery in neonates, the presence of an impaired MFE was associated with lower survival at 36 months of age for the entire cohort and for the subgroup with hypoplastic left heart syndrome.
Abstract: OBJECTIVES Pregnancies with congenital heart disease in the foetus have an increased prevalence of pre-eclampsia, small for gestational age and preterm birth, which are evidence of an impaired maternal-foetal environment (MFE). METHODS The impact of an impaired MFE, defined as pre-eclampsia, small for gestational age or preterm birth, on outcomes after cardiac surgery was evaluated in neonates (n = 135) enrolled in a study evaluating exposure to environmental toxicants and neuro-developmental outcomes. RESULTS The most common diagnoses were transposition of the great arteries (n = 47) and hypoplastic left heart syndrome (n = 43). Impaired MFE was present in 28 of 135 (21%) subjects, with small for gestational age present in 17 (61%) patients. The presence of an impaired MFE was similar for all diagnoses, except transposition of the great arteries (P < 0.006). Postoperative length of stay was shorter for subjects without an impaired MFE (14 vs 38 days, P < 0.001). Hospital mortality was not significantly different with or without impaired MFE (11.7% vs 2.8%, P = 0.104). However, for the entire cohort, survival at 36 months was greater for those without an impaired MFE (96% vs 68%, P = 0.001). For patients with hypoplastic left heart syndrome, survival was also greater for those without an impaired MFE (90% vs 43%, P = 0.007). CONCLUSIONS An impaired MFE is common in pregnancies in which the foetus has congenital heart disease. After cardiac surgery in neonates, the presence of an impaired MFE was associated with lower survival at 36 months of age for the entire cohort and for the subgroup with hypoplastic left heart syndrome.
44 citations
TL;DR: Prenatal diagnosis of 22q11.2 deletion syndrome can be used to counsel expectant parents regarding pregnancy outcome and guide neonatal management and microarray is warranted in all cases of structural abnormalities diagnosed prenatally.
Abstract: 22q deletion syndrome (22q112DS) is most often correlated prenatally with congenital heart disease and or cleft palate The extracardiac fetal phenotype associated with 22q112DS is not well described We sought to review both the fetal cardiac and extracardiac findings associated with a cohort of cases ascertained prenatally, confirmed or suspected to have 22q112DS, born and cared for in one center A retrospective chart review was performed on a total of 42 cases with confirmed 22q112DS to obtain prenatal findings, perinatal outcomes and diagnostic confirmation The diagnosis was confirmed prenatally in 67% (28/42) and postnatally in 33% (14/42) The majority (81%) were associated with the standard LCR22A-LCR22D deletion 95% (40/42) of fetuses were prenatally diagnosed with congenital heart disease Extracardiac findings were noted in 90% (38/42) of cases Additional findings involved the central nervous system (38%), gastrointestinal (14%), genitourinary (166%), pulmonary (7%), skeletal (19%), facial dysmorphism (21%), small/hypoplastic thymus (26%), and polyhydramnios (30%) One patient was diagnosed prenatally with a bilateral cleft lip and cleft palate No fetus was diagnosed with intrauterine growth restriction The average gestational age at delivery was 38 weeks and average birth weight was 3,105 grams Sixty-two percentage were delivered vaginally and there were no fetal demises A diagnosis of 22q112 deletion syndrome should be considered in all cases of prenatally diagnosed congenital heart disease, particularly when it is not isolated Microarray is warranted in all cases of structural abnormalities diagnosed prenatally Prenatal diagnosis of 22q112 syndrome can be used to counsel expectant parents regarding pregnancy outcome and guide neonatal management
35 citations
TL;DR: Gastroschisis can be complicated by a wide variety of prenatal and postnatal events, and the most severe outcomes occur in patients with bowel necrosis at birth, intestinal atresias, or vanishing gastroschISis.
24 citations
TL;DR: Late preterm delivery did not affect outcomes compared to term delivery in uncomplicated GTC, and Patients who underwent primary reduction had better outcomes than patients who underwent silo placement.
Abstract: To evaluate if gestational age (GA), mode of delivery and abdominal wall closure method influence outcomes in uncomplicated gastroschisis (GTC) Retrospective review of NICU admissions for gastroschisis, August 2008–July 2016 Primary outcomes were: time to start enteral feeds (on-EF), time to discontinue parenteral nutrition (off-PN), and length of stay (LOS) A total of 200 patients with GTC were admitted to our NICU Patients initially operated elsewhere (n = 13) were excluded Patients with medical/surgical complications (n = 62) were analyzed separately The study included 125 cases of uncomplicated GTC There were no statistically significant differences in the outcomes of patients born late preterm (34 0/7–36 6/7; n = 70) and term (n = 40): on-EF 19 (5–54) versus 17 (7–34) days (p = 029), off-PN 32 (12–101) versus 30 (16–52) days (p = 046) and LOS 40 (18–137) versus 37 (21–67) days (p = 029), respectively Patients born before 34 weeks GA (n = 15) had significantly longer on-EF, off-PN and LOS times compared to late preterm patients: 26 (12–50) days (p = 001), 41 (20–105) days (p = 004) and 62 (34–150) days (p < 001), respectively There were no significant differences in outcomes between patients delivered by C-section (n = 62) and patients delivered vaginally (n = 63): on-EF 20 (5–50) versus 19 (7–54) days (p = 072), off-PN 32 (12–78) versus 33 (15–105) days (p = 083), LOS 42 (18–150) versus 41 (18–139) days (p = 068), respectively There were significant differences in outcomes between patients who underwent primary reduction (n = 37) and patients who had a silo (88): on-EF 15 (5–37) versus 22 (6–54) days (p < 001), off-PN 28 (12–52) versus 34 (15–105) days (p = 004), LOS 36 (18–72) versus 44 (21–150) days (p = 004), respectively In our experience, late preterm delivery did not affect outcomes compared to term delivery in uncomplicated GTC Outcomes were also not influenced by the mode of delivery Patients who underwent primary reduction had better outcomes than patients who underwent silo placement
20 citations
TL;DR: The findings in this study suggest that L- and R-CDH appear to behave differently, and that factors that make L- CDH fatal and O/E LHR, high-volume herniated liver may not apply to the fetus with R-cdH.
Abstract: Objectives: Whereas left-sided congenital diaphragmatic hernias (L-CDH) have been extensively studied and their prognostic parameters delineated, right-sided hern
19 citations
TL;DR: Using a range of T cell concentrations within the donor inoculum, this work was able to characterize the phenotype of IUHCT-induced GVHD and establish a clear threshold for its induction between 3% and 5% graft CD3+ cell content.
5 citations