Balu Vaidyanathan

What Determines Nutritional Recovery in Malnourished Children After Correction of Congenital Heart Defects

BACKGROUND: Malnutrition is common in children with congenital heart disease (CHD), especially in developing countries. OBJECTIVE: To examine the impact of corrective intervention on the nutritional status of children with CHD and identify factors associated with suboptimal recovery. METHODS: Consecutive patients with CHD in a tertiary center in South India were evaluated for nutritional status before and 2 years after corrective intervention. Anthropometry was performed at presentation and every 6 months for 2 years, and z scores were compared. Malnutrition was defined as a weight-for-age, height-for-age, and weight/height z score <−2. Determinants of malnutrition were entered into a multivariate logistic regression analysis model. RESULTS: Of 476 patients undergoing corrective intervention (surgical: 344; catheter-based: 132) z scores of less than −2 for weight for age, height for age, and weight/height were recorded in 59%, 26.3%, and 55.9% of patients, respectively, at presentation. On follow-up (425 patients [92.5% of survivors; 20.63 ± 13.1 months of age]), z scores for weight for age and weight/height improved significantly from the baseline (weight: −1.42 ± 1.03 vs −2.19 ± 1.16; P < .001; weight/height: −1.15 ± 1.25 vs −2.09 ± 1.3; P < .001). Height-for-age z scores were not significantly different. Malnutrition persisted in 116 (27.3%) patients on follow-up and was associated with a birth weight of ≤2.5 kg, nutritional status at presentation, and height of parents and not with type of cardiac lesion, dietary intake, or socioeconomic factors. CONCLUSIONS: This study from South India demonstrates severe malnutrition in over half of the patients with CHD and is not always reversed by corrective surgery or intervention. Persistent malnutrition after corrective intervention is predicted by nutritional status at presentation, birth weight, and parental anthropometry.

Transesophageal echocardiography for device closure of atrial septal defects: case selection, planning, and procedural guidance.

transcatheter closure is an effective alternative to surgery in most patients with atrial septal defects (ASDs) of the secundum type ([1,2][1]). Factors that decide suitability for transcatheter closure include size of the defect and presence of adequate tissue rims around the defect ([3][2]).

Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review

Patients with systemic onset juvenile idiopathic arthritis (SoJIA) are rarely known to develop coronary artery dilatation. The American heart association (AHA) statement on evaluation of suspected Kawasaki disease (KD) would lead some SoJIA patients (particularly in the early stages of the disease) to be inaccurately classified as KD. In addition to the institution of inappropriate therapy with IVIG, misdiagnosis as KD can delay definitive treatment for these SoJIA patients who probably have a worse predicted outcome. We present a 6-year-old male patient with SoJIA who was initially classified as incomplete KD. The child developed life-threatening macrophage activation syndrome (MAS). Previous literature regarding coronary dilatation in SoJIA is also reviewed.

A Systematic Three-Dimensional Echocardiographic Approach to Assist Surgical Planning in Double Outlet Right Ventricle

Double outlet right ventricle (RV) describes a range of pathology where both great arteries arise, either entirely or in greater part from the morphologic RV. Accurate prediction of the optimal surgical approach is challenging. Ideally, assuming that the ventricles are balanced with respect to their size, a biventricular repair is normally attempted. The feasibility of the different surgical options for repair depends on morphologic features including the location and size of the ventricular septal defect, and the relationship of the great arteries. Anderson et al. recommend the term interventricular communication, in preference to ventricular septal defect, when describing the defect between the 2 ventricles in double outlet RV. The outlet muscular septum that forms the upper boundary of the true ventricular septal defect in double outlet RV is often sited in a position above the right ventricular cavity. Any closure of the true ventricular septal defect beginning at the crest of the septum would involve crossing into the right ventricular space. The remaining defect between the 2 ventricles is therefore best described as the interventricular communication. The term interventricular communication has been used in the text for the purpose of consistency. The position of this interventricular communication in relation to the great vessels is variable. These are described as being subaortic, subpulmonary, or noncommitted, where the interventricular communication lies remote to either great vessel’s origin. In rare cases, there is fibrous continuity between the 2 arterial valves and the interventricular communication that lies beneath these is described as being doubly committed. Two-dimensional echocardiography has traditionally been used to plan surgical intervention, utilizing multiple separate sonographic views and “sweeps.” Three-dimensional (3D) echocardiography has the advantage of incorporating a depth of field to the sonographic images so that the atrioventricular valves, ventricular septal defect, and great arteries can be visualized within a single sonographic projection, obtained by cropping of a 3D echocardiographic volume. This has the potential to enhance understanding of the relationship of the anatomic structures needed for surgical planning. We describe a systematic 3D echocardiographic approach, which we have adopted to assist decision making for the surgical management of this group of lesions. This approach addresses the anatomic concerns with respect to biventricular repair and whether a RV to pulmonary artery conduit will be required. Biventricular repair options include closure of the interventricular communication with or without an arterial switch procedure. Complex interventricular baffles can necessitate enlargement of the interventricular communication and there may be a need to relieve subpulmonary or subaortic stenosis. Straddling atrioventricular valves may preclude septation and single ventricle palliation is sometimes pursued. The echocardiographic approach is described below using clinical examples. Address for correspondence and reprint requests: John M. Simpson, M.D., F.R.C.P., F.E.S.C., 6th Floor Sky Offices, Evelina Childrens Hospital, Westminster Bridge Road, London SE1 7EH, UK. Fax: +44-207-188-4556; E-mail: john.simpson@gstt.nhs.uk

Dedicated pediatric cardiac intensive care unit in a developing country: Does it improve the outcome?

INTRODUCTION AND AIM Focussed cardiac intensive care is known to produce better outcomes. We have evaluated the benefits of a dedicated Pediatric Cardiac Intensive Care Unit (PCICU) in the early postoperative outcomes of patients undergoing surgery for congenital heart disease. METHODS Prospectively collected data of 634 consecutive patients who underwent congenital heart surgery from September 2008 to September 2009 were analyzed. Midway through this period a dedicated PCICU was started. The patients who were treated in this new PCICU formed the study group (Group B, n = 318). The patients who were treated in a common postoperative cardiac surgery ICU formed the control group (Group A, n = 316). Early postoperative outcomes between the two groups were compared. RESULTS The two groups were comparable with respect to demographic data and intraoperative variables. The duration of mechanical ventilation in the dedicated pediatric cardiac ICU group (32.22 ± 52.02 hours) was lower when compared with the combined adult and pediatric surgery ICU group (42.92 ± 74.24 hours, P= 0.04). There was a shorter duration of ICU stay in the dedicated pediatric cardiac ICU group (2.69 ± 2.9 days vs. 3.43 ± 3.80 days, P = 0.001). The study group also showed a shorter duration of inotropic support and duration of invasive lines. The incidence of blood stream infections was also lower in the dedicated pediatric ICU group (5.03 vs. 9.18%, P = 0.04). A subgroup analysis of neonates and infants <1 year showed that the advantages of a dedicated pediatric intensive care unit were more pronounced in this group of patients. CONCLUSIONS Establishment of a dedicated pediatric cardiac intensive care unit has shown better outcomes in terms of earlier extubation, de-intensification, and discharge from the ICU. Blood stream infections were also reduced.

EMERGENCY BALLOON DILATION OR STENTING OF CRITICAL COARCTATION OF AORTA IN NEWBORNS AND INFANTS: AN EFFECTIVE INTERIM PALLIATION

Background: Management of native uncomplicated coarctation in neonates remains controversial with current evidence favoring surgery. The logistics of organizing surgical repair at short notice in sick infants with critical coarctation can be challenging. Methods and Results: We reviewed data of 10 infants (mean age of 2.9 ±1.6 weeks) who underwent catheter intervention for severe coarctation and left ventricular (LV) dysfunction between July 2003 and August 2007. Additional cardiac lesions were present in 7. Mean systolic gradient declined from 51±12 mm Hg to 8.7±6.7 mm Hg after dilation. The coarctation segment was stented in five patients. Procedural success was achieved in all patients with no mortality. Complications included brief cardiopulmonary arrest (n =1), sepsis (n = 1) and temporary pulse loss (n = 2). LV dysfunction improved in all patients. Average ICU stay was 5±3.4 days and hospital stay was 6.5±3.4 days. On follow-up (14.1±10.5 months), all developed restenosis after median period of 12 weeks (range four to 28 weeks). Three (two with stents) underwent elective coarctation repair, two underwent ventricular septal defect (VSD) closure and coarctation repair and one underwent pulmonary artery (PA) banding. Two patients who developed restenosis on follow-up were advised surgery, but did not report. Two (one with stent) underwent redilatation and are being followed with no significant residual gradients. Conclusion: Balloon dilation ± stenting is an effective interim palliation for infants and newborns with critical coarctation and LV dysfunction. Restenosis is inevitable and requires to be addressed.

Conotruncal anomalies in the fetus: Referral patterns and pregnancy outcomes in a dedicated fetal cardiology unit in South India.

Objective: To describe the referral patterns and pregnancy outcomes of fetuses with conotruncal anomalies (CTA) from a fetal cardiology unit in South India. Methods: Records of 68 women identified to have diagnosis of CTA on fetal echocardiography (mean gestational age 26.8 ± 5.9 weeks; range 17-38 weeks) during the period 2008-2011 were reviewed. Results: The most common indication for referral was suspected congenital heart disease during routine antenatal scan (89.7%). The various CTA diagnosed included Tetralogy of Fallot (TOF, 44.1%), Double outlet right ventricle (DORV, 27.9%), Transposition of great vessels (TGA, 8.8%), TOF with pulmonary atresia (TOF-PA, 8.8%), TOF absent pulmonary valve (TOF-APV, 7.4%) and truncus arteriosus (TA, 2.9%). Extra cardiac anomalies were reported in 4 fetuses (7.1%). Pregnancy outcomes included pregnancies not culminating in live-birth (54.4%), delivery at term (41.2%) with 3 patients (4.4%) being lost to follow-up. Proportion of pregnancies not culminating in live-birth lesion wise include: TOF (53.3%), DORV (52.6%), TGA (50%), TOF -APV (80%), TOF-PA (50%), and TA (50%). Twenty-four babies (35.3%) received post-natal cardiac care with 5 (7.4%) undergoing neonatal surgical procedures. Seven babies (10.3%) died in neonatal period, including 2 who underwent surgery. The accuracy of fetal echo was 96.4% for primary lesion and 67.9% for complete segmental diagnosis. Conclusions: Pre-natal diagnosis of CTA, despite a high diagnostic accuracy, prompted utilization of post-natal tertiary cardiac care in a limited proportion of patients, including those with reparable lesions. Focus in developing countries should shift towards earlier referral, improving awareness about treatment options and a comprehensive evaluation for associated anomalies.

Balloon pulmonary valvotomy as interim palliation for symptomatic young infants with tetralogy of Fallot

Objectives: To describe the case selection, technique and immediate and short-term results of balloon pulmonary valvotomy (BPV) in young infants with tetralogy of Fallot (TOF). Background: Symptomatic young infants with TOF can either undergo corrective surgery or Blalock-Taussig (BT) shunt. Corrective surgery in early infancy is associated with significant morbidity and is not a realistic option in many centers. BT shunt carries the risk of branch pulmonary artery distortion and shunt occlusion. Methods: Infants less than three months with a significant valvar pulmonary stenosis (with or without associated infundibular and annular component) and oxygen saturation ≤80% were offered BPV. The right ventricular outflow tract (RVOT) was crossed with 4F Judkins right coronary catheter and the valve was crossed with 0.014” coronary guide wire. Serial balloon dilatations were done with over the wire coronary balloons (3-4 mm) and Mini Tyshak balloons up to a balloon annulus ratio of 2:1, depending upon the improvement in saturation and formation of annular waist. Results: Seventeen infants less than three months of age with tetralogy of Fallot (median age: 33 days, range: 10-90 days, weight: 3.47 ± 0.87 kg, pulmonary annulus Z score: -5.59 ± 1.04) including eight neonates underwent palliative BPV between May 2004 and March 2007. The mean balloon annulus ratio was 1.4 ± 0.28 and fluoroscopy time was 26.18 ± 20.2 minutes. The mean oxygen saturation increased significantly from 73 ± 7% to 90 ± 3.68% following BPV (p = 0.0001). The only major complication was RVOT perforation and pericardial tamponade in one infant. The mean follow-up period was 23 ± 12 months. Two babies developed significant desaturation requiring surgery in the six months following BPV. There was a significant increase in pulmonary annulus. The z score for the pulmonary annulus improved from -5.59 ±1.04 before BPV to - 4.31 ± 1.9 at the time of last follow-up (p = 0.018). The mean Z score of hilar right pulmonary artery (RPA) increased significantly from -1.19 ± 1.78 before BPV to 0.7 ± 0.91 after BPV (p = 0.001). The mean Z score of hilar left pulmonary artery (LPA) increased significantly from -1.28 ± 1.41 to 0.03 ± 1.29 after BPV (p = 0.005). Eight patients underwent corrective surgery. Conclusions: Balloon pulmonary valvotomy is safe and effective. It significantly improves the growth of pulmonary annulus and branch pulmonary arteries. Thus it can be considered as an interim palliative procedure for symptomatic young infants with TOF and predominant valvar pulmonary stenosis.

Anti-platelet agents in pediatric cardiac practice.

Pediatric patients with a variety of congenital and acquired cardiac conditions receive antithrombotic therapy. Many of the indications are empirical, and have either not been proven in controlled studies or are extrapolated from adult studies. This article reviews the current available literature regarding the use of anti-platelet drugs in the pediatric cardiac population.

Catheter Closure of Pseudoaneurysm of the Main Pulmonary Artery

These images demonstrate the use of an Amplatzer duct occluder (AGA Medical) for treatment of a large pseudoaneurysm of the main pulmonary artery after bilateral bidirectional Glenn operation. The patient is a 3-year-old girl who initially presented at 3 months of age with a diagnosis of single-ventricle severe pulmonary hypertension resulting from increased pulmonary blood flow and coarctation of aorta with normally related great arteries. She underwent pulmonary artery banding with coarctation repair (at 3 months), followed by …