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Annals of Pediatric Cardiology logoLink to Annals of Pediatric Cardiology
. 2013 Jan-Jun;6(1):105–108.

Selected abstracts presented at the Annual Conference of the Paediatric Cardiac Society of India 2012

PMCID: PMC3634237
Ann Pediatr Cardiol. 2013 Jan-Jun;6(1):105–108.

Development of the AIIMS Integrated CPB–ECMO

S Chauhan, B Vijayakanthi, AK Bisoi, Y Chauhan

All India Institute of Medical Sciences, New Delhi, India

Background: Extracorporeal membrane oxygenation (ECMO) is occasionally required in the postoperative period to support a precariously functioning heart. Delay in implementing ECMO due to the time required in setting up the ECMO circuit may make the difference between survival and death in the cardiac postoperative pediatric population, hence we have devised and developed in our institute, the Integrated cardiopulmonary bypass (CPB)-ECMO circuit, which has proved beneficial in improving the survival of patients. Methods: Integrated CPB-ECMO was primarily used in patients with the transposition of great arteries (TGA) with intact ventricular septum (IVS), of age more than six weeks, and echocardiographic features suggestive of left ventricle regression, (that is, septal motion with right ventricle and crescent or D-shaped left ventricle,) operated for Primary Arterial Switch Operation. Design: The two parallel circuits of ECMO and CPB are integrated at the level of the membrane oxygenator (MO) and the heat exchanger (HE). During surgery (on CPB), the ‘hard shell’ non-collapsible cardiotomy reservoir is used; and after surgery (on ECMO), the collapsible soft reservoir ‘bladder’ of the ECMO is used. The entire assembly is performed prior to the initiation of surgery. (2) Circuit Assembly: During Surgery (on CPB): Blood → Superior vena cava (SVC) and Inferior vena cava (IVC) cannulae → Y-piece → ‘hard shell’ reservoir → roller pump → heat exchanger (HE) → membrane oxygenator (MO) → aortic cannula → patient. After Surgery (on ECMO): Blood → SVC and IVC cannulae → single right atrial cannula → ‘bladder’ reservoir → roller pump → heat exchanger (HE) → membrane oxygenator (MO) → aortic cannula → patient. Results: The integrated ECMO-CPB circuit was used in 63 children, with diagnosed cases of TGA with IVS, with regressed left ventricle, and were at high risk for the requirement of postoperative ECMO support, after the arterial switch operation. The survival in this group of patients was 76% (48 out of 63 patients), and 24% (15 out of 63 patients) died. The causes of death were sepsis, renal failure, and intracranial hemorrhage. The cost, with conventional circuit and ECMO circuit later, in the Intensive Care Unit (ICU) was approximately $1530, whereas, with an integrated CPB-ECMO circuit it was $990. Conclusions: The Integrated Circuit ECMO is a useful tool in patients with high predictability of postoperative mechanical support, and thereby, improves the survival rates. Not only does it save time, but also reduces the hesitancy to initiate ECMO, and hence, cuts down the overall cost of surgery.

Ann Pediatr Cardiol. 2013 Jan-Jun;6(1):105–108.

PROPRANOLOL IN INFANTS WITH VENTRICULAR SEPTAL DEFECT WITH HEART FAILURE (VSD-PHF STUDY)

RS Ahuja, S Ramakrishnan, SS Kothari, K Bhatt, SK Gupta, R Juneja, A Saxena, VK Bahl

All India Institute of Medical Sciences, New Delhi, India

Background: Infants with ventricular septal defect (VSD) may develop congestive heart failure in spite of conventional medical therapy. We investigated the effects of additional beta-blockade in such infants. Methods: In this single-center, open-label trial, infants with VSD and heart failure were randomized to ‘propranolol’ and ‘no propranolol’ in addition to the conventional treatment. A total of 80 patients were enrolled; 40 in each group. The median follow-up was seven months (range = 1-32 months). The primary endpoint was a composite endpoint of death, hospitalization, and referral for surgery. Results: Fourteen (35%) patients in the conventional arm and 10 (25%) patients in the beta-blocker arm had reached the primary endpoint (P = ns). Worsening of heart failure occurred more commonly in the conventional treatment arm compared to the propranolol treated arm (27.5 vs. 5% respectively; P = 0.015). Two patients in the conventional treatment arm and in one patient in the propranolol arm died. No episodes of bradycardia or bronchospasm were reported with propranolol treatment. Conclusions: The addition of propranolol was well-tolerated by infants with VSD and heart failure. The addition of a beta-blocker over and above the conventional treatment led to symptomatic improvements and reduced the worsening of heart failure. However, there was no difference in the death, hospitalization, or need for surgery.

Ann Pediatr Cardiol. 2013 Jan-Jun;6(1):105–108.

COMPREHENDING AND IDENTIFYING THE ETIOLOGY OF CONGENITAL SEPTAL DEFECTS USING CONVENTIONAL GENETIC ANALYSES

SB Syed, NR Koneti, S Kola, S Dadala, J Akka, HP Mundluru

Institute of Genetics and Hospital for Genetic Disease, Osmania University, Care Hospital, Banjara Hills, and National Institute of Nutrition, Hyderabad, India

Background: Congenital septal defects (CSD) are highly heterogeneous malformations with genetically complex traits, involving both the genetic and environmental factors. The present study is aimed to understand the possible etiological factors that contribute to an increase in the severity of disease. Cytogenetic analysis using fluorescence in situ hybridization (FISH) and comet assay were performed to identify the microdeletions in chromosome 22 and to measure the oxidative DNA damage in the lymphocytes, respectively. Single nucleotide polymorphisms (SNPs) of Methylenetetrahydrofolate reductase (MTHFR) (677C >T) and reduced folate carrier (RFC) (A80G) were analyzed to identify the role of folic acid. Screening of the GATA 4 gene was studied using the polymerase chain reaction-based, single-stranded, conformational polymorphism (PCR-SSCP) for mutations that are responsible for the abnormal cardiac development during embryogenesis. Methods: One hundred children with non-syndromic CSD, were studied with age-matched controls. Blood samples were collected from the Department of Pediatric Cardiology, Care Hospital, Hyderabad, with prior clearance from the Institutional Ethics Committee and written consent from the parents. The lymphocytes were isolated from fresh blood for comet assay as well as for the identification of 22 q11.2 microdeletions, using specific FISH probes on G-Banded glass slides. The red blood cells (RBCs) were used for the estimation of folic acid. An inflammatory marker like the C-reactive protein (CRP) was analyzed using the enzyme-linked immunosorbent assay (ELISA) for the correlation of oxidative DNA damage. Genomic DNA was isolated for PCR and restriction fragment length polymorphism analysis of SNPs 677C> T and A80G genes. The genomic deoxyribonucleic acid (DNA) was also used to screen the GATA4 gene mutations by PCR-SSCP. Results: The mean values of RBC folate are less in patients (228.74 ± 158.9 ng/ml) compared to controls (455.65 ± 03.8 ng/ml). A higher level of CRP was shown in patients (2.1 ± 1.43 ng/ml) compared to the controls (0.93 ± 0.45 ng/ml), demonstrating the severity of oxidative DNA damage. Microdeletions of 22q11.2 and significant DNA damage were observed in CSD patients. The SNPs of MTHFR 677C> T and RFC 80A> G was observed in 32.5 and 29%, respectively. Screening of the GATA4 gene using PCR-SSCP analysis did not show any mutations. Conclusions: Low folate levels and genetic polymorphism have shown increased oxidative DNA damage in children with congenital septal defects.

Ann Pediatr Cardiol. 2013 Jan-Jun;6(1):105–108.

MID-TERM OUTCOMES OF A HAND MADE TRILEAFLET-VALVED RIGHT VENTRICLE TO PULMONARY ARTERY CONDUIT

Z Makhija, R Sharma

Fortis Hospital, New Delhi, India

Background: We have been using a new technique to make bovine pericardial valved conduits, to overcome the shortage of cryopreserved homografts for the right ventricle (RV) to pulmonary artery (PA) reconstructions. The GORE PRECLUDE pericardial membrane (0.1 mm thickness) is used for making the trileaflet valve. We have reviewed our experience to analyze the postoperative outcomes and their mid-term results. Methods: Between 2007 and 2012, 203 patients underwent primary right ventricle outflow tract reconstruction using bovine pericardial valved conduits in our center; 71.9% (n = 146) of the patients were males. Their age ranged from six days to forty-two years. Diagnoses included Ventricle Septal Defect (VSD), Pulmonary Atresia (PA), Major aortopulmonary collateral arteries (MAPCAs) (n = 61), VSD, PA (n = 52), Truncus Arteriosus (n = 20), Double outlet right ventricle, VSD, Pulmonary stenosis (PS) (n = 18), Tetralogy of Fallot/Absent Pulmonary Valve (n = 12), Corrected transposition, VSD, PS (n = 11), Transposition of great arteries, VSD, PS (n = 7), and Ross procedure (n = 11); 26.6% (n = 54) patients had undergone a prior palliative shunt procedure. The sizes of the conduits implanted ranged from 12 to 24 (median 18). Results: Conduit-related, early complication rate was 0.5% (n = 1) (conduit revision for early pulmonary artery thrombosis). The mean hospital stay was 13.2 ± 31.6 days. Early mortality was 7.4% (n = 15). The mean follow-up period was 61 months. 11.3% (n = 23) of the patients underwent conduit replacement. Of these, acquired distal conduit stenosis was observed in 10.8% (n = 22) and 0.5% (n = 1) developed infective endocarditis. Late mortality was 0.5% (n = 1). A gradient >30 mm Hg was detected in 13.4% (n = 27) of the patients, while 15.3% (n = 31) developed conduit valve regurgitation greater than 2+. Conclusions: The handmade valved conduit seems to offer equivalent results, as compared to other conduits available today at midterm follow-up. Long-term results are awaited. Easy availability and low cost are additional advantages of this conduit.

Ann Pediatr Cardiol. 2013 Jan-Jun;6(1):105–108.

DO PREOPERATIVE HEMODYNAMIC DATA AND REACTIVITY TESTS PREDICT THE POSTOPERATIVE REVERSIBILITY OF PULMONARY ARTERIAL HYPERTENSION IN PATIENTS WITH LARGE VENTRICULAR SEPTAL DEFECT AND BORDERLINE OPERABILITY?

A Sridhar, BS Nithyalakshmi, MVS F Farzana, SC Pillai, R Premsekar, R Subramanyan, R Agarwal, KM Cherian

Frontier Life Line Hospital and Dr. K. M. Cherian Heart Foundation, Chennai, India

Background: The decisions on operability for shunt lesions presenting late with severe pulmonary arterial hypertension (PAH) and borderline operability are often not based on precise cut-off values of hemodynamic data. There is a paucity of studies in which hemodynamic data are tested against late outcomes after surgery. The gold standard method suggested in the previous studies is measurement of pulmonary vascular resistance (PVR) index at least a year after defect closure. The aim of the study is to assess the reliability of the preoperative hemodynamic data and the reactivity test for predicting the postoperative reversibility of Pulmonary Arterial Hypertension (PAH) in patients with isolated large ventricular septal defects (VSD) and borderline operability. Methods: Between 2004 and 2010, 30 patients underwent high-risk surgical VSD closure with no early mortality. Twenty-six patients were on regular follow-up (mean 39.6 ± 16 months). There was one late postoperative death. Fourteen patients on pulmonary vasodilator therapy underwent catheterization after completion of at least a one-year postoperative period. The clinical and hemodynamic data of 15 study patients, including the one who died, were retrospectively analyzed. Asymptomatic patients with PVR index <3 wu.m2 at follow-up catheterization, with or without the need for pulmonary vasodilators, were considered as ‘Responders’. Results: Between three ‘Responders’ and 12 ‘non-responders’, the mean age at surgery (3.2 ± 0.42 vs. 11.55 ± 3.29 years), mean baseline PVR index (3.69 ± 1.2 vs. 10.57 ± 9.1, P= 0.204), average resistance ratio (RR = 0.26 ± 0.05 vs. 0.59 ± 0.25, P = 0.049), and ratio of pulmonary and systemic mean pressures (PAm/SAm ratio) (0.70 ± 0.009 vs. 0.87 ± 0.118, P = 0.003) were significantly lower among the responders. Conclusions: In this study, the preoperative PAm/SAm ratio and resistance ratio appear to be better predictors of the postoperative outcome compared to the other baseline hemodynamic parameters. The preoperative reactivity result had no significant role in predicting the postoperative reversibility of PAH.

Ann Pediatr Cardiol. 2013 Jan-Jun;6(1):105–108.

UNVEILING THE MYSTERY BEHIND LEFT VENTRICULAR REGRESSION IN PATIENTS WITH TRANSPOSITION OF GREAT ARTERIES: A PILOT STUDY

R Kalra, AK Bisoi, P Das, SK Panda

All India Institute of Medical Science, New Delhi, India

Introduction: Arterial switch operation is the treatment of choice for patients with transposition of great arteries (TGA). The option of arterial switch operation is being extended beyond the conventional age limit of six weeks, for patients with TGA and intact ventricular septum. The pathophysiological changes taking place in the regression of left ventricle (LV), as also the factor determining the successful outcome following delayed primary arterial switch operation are not well understood. In this pilot study we have tried to understand the pathological changes during left ventricular regression at the cellular and protein levels and their relation to the postoperative outcomes. Methods: This is single-center, single-surgeon study. Patients with TGA, operated between October 2010 and December 2012, were included in the study. Those patients not giving consent for inclusion in the study were excluded. Intraoperative myocardial biopsies were taken from the left ventricle (LV) and right ventricle (RV) of these patients. A sample from each chamber was divided into two halves. One part was preserved in formalin for histopathological analysis and the other part was cryopreserved at −80°C for protein analysis. Morphometric analysis (number of myocardial syncytial cells, diameter of myocardial cells, area of fibrosis, etc.,) and immunochemical test for apoptosis were carried out on the formalin-preserved sample. The TUNNEL test was done to detect the presence of apoptotic cells. The Masson Trichome stain and Hematoxylin and eosin stain were used to study the area of fibrosis and the morphometric parameters, respectively. Gel electrophoresis was conducted on the cryopreserved sample, to study the differential expression of the myosin heavy chain isoform. Samples from 19 pediatric autopsies of non-cardiac cases were obtained, and tests were carried out along the same lines as for the control. Left ventricular regression was identified by the direction of movement of the interventricular septum. The ‘D’ or ‘banana-shape’ of the left ventricle was suggestive of left ventricular regression, in the short-axis, parasternal view. Results: There were 27 patients with transposition of great arteries included in the study. Sixteen patients had a regressed left ventricle (55-900 days) and 11 patients had preserved left ventricle (28-540 days) at the time of the arterial switch operation. All patients with a regressed ventricle had an intact ventricular septum and no left ventricular outflow tract obstruction. Five of the sixteen (31%) required active extracorporeal support in the postoperative period. Left ventricular regression was associated with a decrease in cell count (P value = 0.001) and increase in the area of fibrosis (P value = 0.02), as compared to TGA with preserved ventricular mass and controls. The phenomenon of apoptosis was more evident in patients with a regressed ventricle as compared to a preserved left ventricle (5.35 + 2.59/200 cells vs. 3 + 1.8/200 cells; P = 0.02). Pathological changes like myocardial fiber dissarray, vacuolization, and fibrosis were evident in the right ventricle of all patients with transposition of great arteries. The Alpha and Beta isoforms of the myosin heavy chain were expressed in the same proportion in patients of TGA, with or without regressed left ventricle. The fibrotic area of 2610.40/micrometre2 was found to have a sensitivity of 80% in predicting the postoperative need for active extracorporeal membrane support. Moreover the degree of fibrosis and cellular loss were not function of age, giving a ray of hope for older patients, with regressed left ventricle. Conclusions: Left ventricular regression in patients with transposition of great arteries is associated with a decrease in cell count and increase in the area of fibrosis. This was not found to be function of age.

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