Positive outcome in a patient with severe hypoxic-ischaemic encephalopathy.

A male infant was born at 40 and 4/7 weeks of gestation via caesarean section for non-reassuring foetal heart tracing. The infant was non-responsive in the delivery room. with no heart rate detected until 40 min of life. The infant's physical examination and laboratory findings were consistent with severe hypoxic-ischaemic encephalopathy. Given the presumption of a very poor neurological prognosis, redirection to comfort care was recommended to the family. However, the family opted for intensive care. The infant underwent therapeutic hypothermia and management of multiorgan dysfunction. The infant survived with no findings of ischaemic injury on MRI and was discharged with no respiratory support and taking all feeds by mouth, with normal development at a year and a half of age. This case report demonstrates the imperative to understand family goals and to acknowledge the need for ongoing humility in providing prognostication for families.

Early Transient Hypoglycemia and Test Performance in At-Risk Newborns.

OBJECTIVE: This study aimed to evaluate if early (within the first 3 hours after birth) transient neonatal hypoglycemia (TNH) is associated with poor academic performance in infants at-risk for hypoglycemia. STUDY DESIGN: This was a retrospective cohort study of at risk-infants (late preterm infants, small and large for gestational age infants, and infants of diabetic mothers [IDMs]) who were born in 1998 and 1999 at the University of Arkansas for Medical Sciences and had ≥1 recorded glucose concentration. The outcome measure was proficiency on 4th grade literacy and mathematics achievement tests. Three glucose concentration cutoffs for defining hypoglycemia (<35, <40, and <45 mg/dL) were investigated. Logistic regression models were developed to examine the association between early TNH and achievement test proficiency based on perinatal factors. RESULTS: Among 726 infants, 472 had one, 233 had two, and 21 had three risk factor(s). Early TNH (glucose concentration <35, <40, and <45 mg/dL) was observed in 6.3, 11.6, and 20.5% of the study cohort, respectively. Irrespective of the cutoff used, the frequency of early TNH (number of patients with early TNH in a risk category divided by the total number of patients in that category) was significantly greater among infants with multiple risk factors. After controlling for perinatal factors, early TNH (cutoffs <35 and <40 mg/dL) was significantly associated with decreased probability of proficiency in literacy but not mathematics. Despite that early TNH was more common in IDMs and infants with three risk factors, the category or number of risk factors did not impact academic proficiency. CONCLUSION: Early TNH (<35 and <40 mg/dL) was associated with lower adjusted probability of proficiency on 4th grade literacy achievement tests in at-risk infants. The impact of early TNH on academic performance was similar irrespective of category or number of risk factors. KEY POINTS: · Transient hypoglycemia was associated with lower proficiency on 4th grade tests in at-risk infants.. · The category of risk factors among at-risk infants did not impact 4th grade academic proficiency.. · The number of risk factors among at-risk infants did not impact 4th grade academic proficiency..

Characteristic Morphologies of the Bicuspid Aortic Valve in Patients with Genetic Syndromes.

BACKGROUND: In patients with bicuspid aortic valve (BAV), complications including progressive aortic stenosis and aortic dilatation develop over time. The morphology of cusp fusion is one of the determinants of the type and severity of these complications. We present the association of morphology of cusp fusion in BAV patients with distinctive genetic syndromes. METHODS: The Mayo Clinic echocardiography database was retrospectively reviewed to identify patients (age ≤ 22 years) diagnosed with BAV from 1990 to 2016. Cusp fusion morphology was determined from the echocardiographic studies, while coexisting cardiac defects and genetic syndromes were determined from chart review. RESULTS: A total of 1,037 patients with BAV were identified: 550 (53%) had an isolated BAV, 299 (29%) had BAV and a coexisting congenital heart defect, and 188 (18%) had BAV and a coexisting genetic syndrome or disorder. There were no differences in distribution of morphology across the three groups. However, right-noncoronary (RN) cusp fusion was the predominant morphology associated with Down syndrome (P = .002) and right-left (RL) cusp fusion was the predominant morphology associated with Turner syndrome (P = .02), DiGeorge syndrome (P = .02), and Shone syndrome (P = .0007), when compared with valve morphology in patients with isolated BAV. Isolated BAV patients with RN cusp fusion had larger ascending aorta diameter (P = .001) and higher number of patients with ≥ moderate aortic regurgitation (P = .02), while those with RL cusp fusion had larger sinus of Valsalva diameter (P = .0006). CONCLUSIONS: Morphological subtypes of BAV are associated with different genetic syndromes, suggesting distinct perturbations of developmental pathways in aortic valve malformation.

Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects.

BACKGROUND: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. METHODS: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention. RESULTS: Overall, 1010 patients with BAV were identified, 619 (61%) with isolated BAV and 391 (39%) with BAV and coexisting CHD. The incidence of BAV was highest in patients with coarctation of the aorta (36%) and interrupted aortic arch (36%). In comparison to patients with isolated BAV, patients with BAV and left-sided obstructive lesions more frequently had right-left cusp fusion (P = .0001). BAV in patients with right-sided obstructive lesions was rare, but they more frequently had right-noncoronary or left-noncoronary cusp fusion (P = .01). No significant progression of aortic stenosis or regurgitation was observed in patients with BAV and coexisting CHD; however in patients with isolated BAV the severity of aortic regurgitation increased with age. In patients with isolated BAV, the ascending aorta diameter (z-score) increased with age, peaked around 8-9 years of age, and was larger in comparison to patients with BAV and coexisting CHD. The sinus of Valsalva diameter (z-score) in patients with BAV and ventricular septal defect was larger than isolated BAV patients after 18 years (P < .04). CONCLUSIONS: The morphology of BAV, the pattern and progression of aortic dilatation, and the severity of aortic valve disease vary in pediatric and young adult patients with BAV and coexisting CHD. However, there was no significant BAV disease progression when associated with these CHD.

A meta-analysis of robotic vs. conventional mitral valve surgery.

OBJECTIVES: The present study is the first meta-analysis to compare the surgical outcomes of robotic vs. conventional mitral valve surgery in patients with degenerative mitral valve disease. METHODS: A systematic review of the literature was conducted to identify all relevant studies with comparative data on robotic vs. conventional mitral valve surgery. Predefined primary endpoints included mortality, stroke and reoperation for bleeding. Secondary endpoints included cross-clamp time, cardiopulmonary bypass time, length of hospitalization and duration of intensive care unit (ICU) stay. Echocardiographic outcomes were assessed when possible. RESULTS: Six relevant retrospective studies with comparative data for robotic vs. conventional mitral valve surgery were identified from the existing literature. Meta-analysis demonstrated a superior perioperative survival outcome for patients who underwent robotic surgery. Incidences of stroke and reoperation were not statistically different between the two treatment arms. Patients who underwent robotic surgery required a significantly longer period of cardiopulmonary bypass time and cross-clamp time. However, the lengths of hospitalization and ICU stay were not significantly different. Both surgical techniques appeared to achieve satisfactory echocardiographic outcomes in the majority of patients. CONCLUSIONS: Current evidence on comparative outcomes of robotic vs. conventional mitral surgery is limited, and results of the present meta-analysis should be interpreted with caution due to differing patient characteristics. However, it has been demonstrated that robotic mitral valve surgery can be safely performed by expert surgeons for selected patients. A successful robotic program is dependent on a specially trained team and a sufficient volume of referrals to attain and maintain safety.

A meta-analysis of minimally invasive versus conventional mitral valve repair for patients with degenerative mitral disease.

BACKGROUND: Minimally invasive mitral valve surgery through a mini-thoracotomy approach was developed in the mid-1990s as an alternative to conventional sternotomy, but with reduced trauma and quicker recovery. However, technical demands and a paucity of comparative data have thus far limited the widespread adoption of minimally invasive mitral valve repair (MIMVR). Previous meta-analyses have grouped various surgical techniques and underlying valvular disease aetiologies together for comparison. The present study aimed to compare the clinical outcomes of MIMVR versus conventional mitral valve repair in patients with degenerative mitral valve disease. METHODS: A systematic review of the current literature was performed through nine electronic databases from January 1995 to July 2013 to identify all relevant studies with comparative data on MIMVR versus conventional mitral valve surgery. Measured endpoints included mortality, stroke, renal failure, wound infection, reoperation for bleeding, aortic dissection, myocardial infarction, atrial fibrillation, readmission within 30 days, cross clamp time, cardiopulmonary bypass time and durations of intensive care unit (ICU) stay and overall hospitalization. Echocardiographic outcomes were also assessed when possible. RESULTS: Seven relevant studies were identified according to the predefined study selection criteria, including one randomized controlled trial and six retrospective studies. Meta-analysis of clinical outcomes did not identify any statistically significant differences between MIMVR and conventional mitral valve repair. The duration of ICU stay was significantly shorter for patients who underwent MIMVR, but this did not translate to a shorter hospitalization period. Patients who underwent MIMVR required longer cross clamp time as well as cardiopulmonary bypass time. Both surgical techniques appeared to achieve satisfactory echocardiographic outcomes. Pain-related outcomes was assessed in one study and reported significantly less pain for patients who underwent MIMVR. However, this limited data was not suitable for meta-analysis. CONCLUSIONS: The existing literature has limited data on comparative outcomes after MIMVR versus conventional mitral valve repair for patients with degenerative disease. From the available evidence, there are no significant differences between the two surgical techniques in regards to clinical outcomes. Patients who underwent MIMVR required longer cardiopulmonary bypass and cross clamp times, but the duration of stay in the ICU was significantly shorter than conventional mitral valve repair.