Nishant Shah

The Effect of Recombinant Human Erythropoietin on the Development of Retinopathy of Prematurity

In addition to its hematopoietic effects, erythropoietin causes an increased release of endothelin-1 and the stimulation of angiogenesis and thereby it may have possible role in development of retinopathy of prematurity (ROP). Our objective was to determine if an association exists between recombinant human erythropoietin (rhEPO) treatment and the development of ROP. Our case-control study involved 85 very low birthweight infants with birthweights <1500 g born during 2003 and 2004. All the infants were divided into two groups on the basis of whether they got rhEPO or not. The rhEPO was given at the dose of 200 to 250 units/kg/dose three times a week for 10 doses. Further duration of rhEPO therapy was decided on the basis of the clinical response. Ophthalmological examinations were done at the age of 5 to 6 weeks and were repeated 1 to 4 weeks after the first examination according to the severity of the ROP disease during their in-hospital stay. Of 85 infants, 56 (66%) received rhEPO and 29 (34%) did not. In the rhEPO-treated group, 12 infants (21%) had ROP; in the non-rhEPO group, 11 infants (38%) developed ROP. This difference is not statistically significant (odds ratio = 2.63; P = 0.10). There was no correlation between the use of rhEPO and the stage of ROP (random sample = -0.01; P = 0.89). There was no significant difference in the incidence of plus, prethreshold, or threshold disease and the treatment required for ROP between the rhEPO-treated and the nontreated group. The study showed there is no significant difference in the incidence and severity of ROP between the rhEPO-treated and nontreated group.

Improvement of Cardiac Auscultation Skills in Pediatric Residents With Training

Cardiac murmurs, most of which are harmless, are present in more than 50% of children. Good auscultation skills are required to prevent unnecessary referrals. The auscultation skills of a group of 21 pediatric residents were assessed. Based on their identification of key features such as S1, S2, timing, grade, location, quality of the murmur, and any extra sounds, residents were asked to make a clinical diagnosis. The overall diagnostic accuracy for all residents was 30% and improved with years of training. The average score for correctly identified key features was 46% with no significant improvement with year of training. Residents who had completed a cardiology rotation scored better than the others (41% vs 18% for correct diagnosis). Clinical auscultation skills of pediatric residents in our institution showed significant improvement with training, especially in the ability to recognize a harmless heart murmur.

NT-proBNP Levels Improve the Ability of Predicting a Hemodynamically Significant Patent Ductus Arteriosus in Very Low-Birth-Weight Infants.

Purpose: A hemodynamically significant Patent ductus arteriosus (HsPDA) in premature infants is known to be associated with significant morbidity. Recently brain natriuretic peptides and superior mesenteric artery (SMA)-resistive indices have been used to effectively diagnose HsPDA. Objective: To assess the sensitivity and specificity of N-terminal proBNP (NT-proBNP) in predicting an HsPDA diagnosed by clinical and echocardiographic criteria including pulsatility index (PI) of SMA. Materials and Methods: All preterm neonates <1500 g were evaluated with echocardiograms and NT-proBNP levels on the 3rd to 5th day of life and then every week until the echo showed either a closed PDA or non-HsPDA. Results: Sixty-nine babies with mean gestational age of 27 weeks were included in the study. NT-proBNP levels were significantly higher in the HsPDA group (n=22) with a mean±SEM of 24420±3190 compared to 3072±332 in the non-HsPDA group (n=47) (P<0.001). NT-pro BNP level of 5900 pg/ml had 96% sensitivity and 90% specificity of predicting HsPDA. Conclusions: With frequently changing hemodynamics in low-birth weight infants, including NT-proBNP and PI of SMA improve the ability of assessing the effects of a HsPDA and will help timing of intervention.

Infants and Children with Tachycardia: Natural History and Drug Administration

Tachyarrhythmias can occur at any age from the developing fetus through adulthood. However, in deference to adult-onset ischemic cardiac issues, abnormal heart rhythms occurring in the young are often due to developmental alterations of the cardiac conduction tissue, genetically-inherited changes of myocardial cellular ion membrane properties and both pre- and post-surgical repair of associated structural congenital heart anatomical defects. And different from adults, abnormal rhythms occurring in the young can spontaneously disappear with progressive patient growth. Both supra- and ventricular tachyarrhythmias occur in the young although atrial rhythm abnormalities far exceed those of the ventricle. In both, pharmacologic therapies to alter tissue conduction and refractoriness remain the mainstay for initial intervention in the infant and young child, reserving more invasive and potentially harmful ablation therapies for drug-refractory cases. The purpose of the review is to present common and uncommon tachyarrhythmias which can occur in the fetus and throughout infancy. Emphasis will be placed on their electrocardiographic identification, recognition of any associated structural congenital heart defects and recommended pharmacologic management. Drug therapies will be divided according to mechanism of action and discussions of which particular agent is potentially best-suited to treat which specific tachyarrhythmia. A listing of current pharmacologic agents used in the young with appropriate dosages is included.

B-type natriuretic peptide: biomarker of persistent pulmonary hypertension of the newborn?

OBJECTIVE We assessed the utility of plasma B-type natriuretic peptide (BNP) in infants with persistent pulmonary hypertension of the newborn (PPHN) in the prediction of inhaled nitric oxide (iNO) requirement. METHODS This prospective study involved neonates (gestational age ≥ 34 weeks) with PPHN and confirmatory echocardiographic findings. Plasma BNP was assayed once within 12 hours of meeting criteria for iNO requirements and twice every 24 to 48 hours thereafter. RESULTS Infants requiring iNO (n = 14) had higher first BNP levels compared with others (n = 5) (455.5 ± 350.6 vs. 30.1 ± 25.3 ng/dL, p < 0.003). The sensitivity, specificity, positive and negative predictive values, and 95% confidence intervals (CI) for plasma BNP greater than 30 ng/dL to predict iNO requirement were 100 (85-100), 80 (37-80), 94 (80-94), and 100 (46-100)%, respectively. Corresponding values at a cut-off plasma BNP concentration greater than 85 ng/dL were 79 (62-79), 100 (53-100), 100 (79-100), and 63 (33-63)%, respectively. CONCLUSION BNP had excellent sensitivity and negative predictive value for iNO requirement and above 30 ng/dl maybe a useful prognostic biomarker in PPHN.

INFECTIVE ENDOCARDITIS PROPHYLAXIS – CURRENT PRACTICE AMONGST PEDIATRIC CARDIOLOGISTS: ARE WE FOLLOWING 2007 GUIDELINES?

The indications for antibiotics prophylaxis for prevention of Infective endocarditis (IE) have been revised in 2007. A web-based, anonymous survey was conducted in 2013 to evaluate the current practice for IE prophylaxis amongst the pediatric cardiologists. Total 302 responses were received. The

Teenage heart murmurs.

The first-time appearance of a murmur in an adolescent can create a substantial amount of anxiety in the parents and the teenager. The appropriate evaluation and diagnosis is very important in decision-making regarding sports participation in this population. Accurate identification of the innocent murmurs can obviate the need for echocardiography. Identification of a pathologic murmur may reduce morbidity and, possibly, mortality in critical lesions such as hypertrophic cardiomyopathy. This article discusses the physiology and characteristics of different murmurs, and outlines an approach to cardiac murmurs in adolescents.

Refractory Idiopathic Ventricular Tachycardia in a Newborn Treated Successfully With Phenytoin: Old Therapies Are Still Effective in the Current Era

A full-term female had a prenatal diagnosis of a nonspecific tachyarrhythmia in utero detected at 36 weeks of gestational age during a maternal ultrasound examination. She was delivered at 38 weeks by repeat cesarean section without complications. Her heart rate ranged from 140 to 200 bpm. An initial electrocardiogram showed no preexcitation and a normal QTc interval, but the infant had frequent runs of monomorphic VT with a right bundle-branch block pattern at approximately 200 bpm. Overall, she experienced VT more than 95% of the time, as recorded by bedside monitoring, but was otherwise clinically asymptomatic with normal blood pressures and feeding well. Cardiac echocardiography (echo) showed a normal anatomy and normal left ventricular function. Cardiac magnetic resonance imaging (MRI) results also were normal without evidence of tumor or hemartoma. Interestingly, it was noted that the infant always reverted to sinus rhythm each time she started to cry vigorously. Incessant VT was diagnosed and treated initially with an esmolol infusion at 150 mcg/kg/min immediately after birth, which was later increased to 400 mcg/kg/min. The infant continued to experience VT without any significant change. Amiodarone was added the next day with a loading dose of 5 mcg/kg followed by a maintenance infusion up to 10 mcg/kg/min. Her VT persisted with a combination of esmolol and amiodarone at high doses. The infant experienced congestive heart failure after 1 week, with hepatomegaly, respiratory distress, and lower limb edema. A repeat echo then showed decreased left ventricular function. On day 11, amiodarone and esmolol were replaced with phenytoin. Initially, phenytoin was given with an intravenous loading dose of 15 mg/kg over 1 h followed by a maintenance dose of 10 mg/kg/day. The infant’s VT was successfully converted to sinus rhythm with frequent ventricular ectopy in the form of premature ventricular contractions, bigeminy, trigeminy, and couplets at the termination of the phenytoin loading dose. Subsequent echo studies showed improved left ventricular function. A Holter monitor exhibited a predominant sinus rhythm with only 7.8% of noted monomorphic ventricular ectopy. The morphology of the ventricular ectopy was the same as that of the previous VT. The infant was discharged home with sinus rhythm and continued oral phenytoin on day 13 of life. Her clinic follow-up examination at 4 weeks of age showed sinus rhythm with frequent ventricular ectopy and normal left ventricle function. Her repeated Holter monitor showed a reduction in total ventricular ectopy to 3.4% during this time (Fig. 1).

Complete Atrioventricular Canal Defect: Influence of Timing of Repair on Intermediate Outcomes:

Background: The optimal timing of repair of complete atrioventricular canal defect (CAVC) remains uncertain. When early repair is indicated due to clinical conditions, patients may be potentially exposed to excess morbidity or mortality. We reviewed our experience with repair of CAVC to determine the influence of age on outcomes. Methods: The study included 48 patients who underwent repair of CAVC at our institution from 2004 to 2014. To assess the association of age at surgery with outcomes, logistic regression models were fit to binary outcomes and linear regression models were fit for continuous outcomes. Age at surgery was dichotomized into early (≤90 days; n = 18) and late repair (>90 days; n = 30). Chi-square and two-sample t tests were used to compare early to late repair with respect to patient characteristics and outcomes. Results: Patient characteristics were similar except for mean weight at surgery (3.9 vs 4.6 kg) and presence of greater than equal to moderate left atrioventricular valve regurgitation (LAVVR; 1 vs 11). When assessed by continuous scale or dichotomized at 90 days, there was no association of age at repair with outcomes including median bypass and clamp times, need for pacemaker implantation, pulmonary hypertension requiring oxygen and/or medication, median length of stay, incidence of greater than equal to moderate LAVVR at discharge, and rate of reoperation for LAVVR. Freedom from greater than equal to moderate LAVVR was similar between the groups at latest follow-up. Conclusion: Contemporary repair of CAVC is associated with low mortality and favorable outcomes. In the presence of clear signs of congestive heart failure, primary repair can be safely accomplished in patients under three months of age.

Pulmonary valve restitution following transannular patch repair of tetralogy of Fallot

Chronic pulmonary insufficiency following transannular patch repair of tetralogy of Fallot may mandate restoration of a competent pulmonary valve. Pulmonary valve leaflets that are preserved at initial surgery may grow and develop normal morphology and subsequent valve repair may be possible. We reviewed our experience with native pulmonary valve restitution following transannular patch repair (2001-15). The cohort included 9 patients with a median age of 18.7 (range 10.6-31.3) years. Operative technique involved reapproximation of the anterior commissure of the pulmonary valve. Median length of stay was 3 days, and there were no deaths. At median follow-up of 2.0 (0.4-13.5) years, pulmonary insufficiency was graded as ≤mild (n = 4), mild-moderate or moderate (n = 4) and moderate-severe (n = 1); pulmonary stenosis was ≤mild (n = 8) and moderate (n = 1), with median peak gradient of 21 (16-64) mmHg. No patient required reintervention. At reoperation to treat pulmonary insufficiency in repaired TOF, if residual leaflets are found with favourable anatomy, restitution of the native valve should be considered. This valve-preserving technique avoids the certain failure of a bioprosthesis and is associated with favourable early outcomes. The viability of this option may influence surgeons to leave the pulmonary leaflets in situ at the time of initial repair.