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Objectives Growth hormone deficiency (GHD) in adults is associated with an increased risk of cardiovascular morbidity and mortality. Although children with GHD are also believed to have a similar cardiovascular disease (CVD) risk beginning at an early age, the available data in children is scarce. We aimed to determine the various CVD risk parameters in children with isolated GHD (IGHD). Methods A cross-sectional case-control study was conducted at a tertiary care centre in North India comparing various auxological, biochemical, and echocardiographic parameters between 20 IGHD children aged 5–15 years and their age and sex-matched healthy controls. Results The mean age of children with IGHD and controls was similar (10.5 ± 2.6 yr vs. 9.9 ± 2.7 yr, p=0.48). Children with IGHD had significantly higher waist-hip-ratio (p=0.01), total cholesterol (p=0.02), non-high-density lipoprotein–cholesterol (p=0.02), serum homocysteine (p Conclusions Children with IGHD showed abnormalities in several biochemical and cardiac parameters that may be associated with an increased CVD risk in later life. More extensive studies, including younger children with IGHD, are needed to determine the lower ages at which the CVD risk is detectable.

Objective Several studies have demonstrated a shift in the spectrum of infective endocarditis (IE) in the developed world. We aimed to investigate whether demographic and microbiologic characteristics of IE have changed in India. Design A retrospective analysis of patients with in north India between 2010 and 2020. Methods The clinical and laboratory profiles of 199 IE admitted to an academic hospital patients who met the modified Duke criteria for definite IE were analysed. Results The mean age was 34 years, and 84% were males. The main predisposing conditions were injection drug use (IDU) (n = 71, 35.7%), congenital heart disease (n = 46, 21.6%), rheumatic heart disease (n = 25, 12.5%), and prosthetic device (n = 19, 9.5%). 17.1% of patients developed IE without identified predispositions. Among 64.3% culture-positive cases, the most prevalent causative pathogens were Staphylococcus aureus (46.1%), viridans streptococci (7.0%), enterococci (6.0%), coagulase-negative staphylococci (5.5%), gram negative bacilli (5.5%), polymicrobial (5.5%), and Candida (1.0%). The tricuspid (30.3%), mitral (25.6%), and aortic (21.6%) valves were the most common sites of infection, and 60.3% had large vegetations (>10 mm). Systemic embolization occurred in 55.3% of patients at presentation. Cardiac surgery was required for 13.1%. In-hospital mortality was 17.1% and was associated with prosthetic devices (p-value, 0.001), baseline leucocytosis (p-value, 0.036) or acute kidney injury (p-value, 0.001), and a microbial etiology of gram negative bacilli or enterococci (p-value, 0.005). Conclusion IDU is now the most important predisposition for IE in India, and S. aureus has become the leading cause of native valve endocarditis with or without IDU.

There are no established clinical or laboratory markers of preload adequacy and fluid responsiveness in management of neonatal shock. Functional echocardiographic preload markers are evaluated in children and adults, but there is no data in neonatal septic shock. We evaluated five functional echocardiographic preload markers during intravenous volume resuscitation in neonatal septic shock.(1) To compare baseline functional echocardiographic preload markers between neonates with septic shock and their "matched" healthy controls. (2) To compare echocardiographic preload markers before and after intravenous volume resuscitation.In this cohort study, we enrolled neonates with septic shock (cases) and recorded five preload markers - inferior vena cava collapsibility index (IVC-CI), left ventricular end-diastolic (LVEDV)end-systolic volume (LVESV) and their indices (LVEDVI, LVESVI) - before initiation of intravenous fluid resuscitation (baseline evaluation). An equal number of "matched hemodynamically stable" controls were recruited, who underwent functional echocardiographic assessment once. In neonates with shock, we recorded these markers again after volume resuscitation.We analyzed 46 neonates (23 cases and 23 controls). Neonates with shock had significantly elevated baseline IVC-CI as compared to controls [53% (21, 100) vs. 20% (15, 24) respectively,Neonates with septic shock had significantly elevated IVC-CI at baseline as compared to hemodynamically stable neonates. None of the preload markers changed significantly after volume resuscitation as compared to the baseline values including IVC-CI, which was almost significant.

Background and aim of the study The morphological heterogeneity of anomalous pulmonary venous drainage in mixed type total anomalous pulmonary venous connection (TAPVC) has important implications in preoperative diagnosis and surgical repair resulting in high mortality in these patients. Methods A retrospective review of 14 patients with mixed type TAPVC undergoing biventricular repair between January 2012 and December 2019 was conducted. A descriptive analysis was done, highlighting the anatomic variation, diagnostic and surgical approach, and surgical outcomes in these patients. Results The most common anatomic pattern was "3 by 1" (79%) followed by "2 by 2" (21%). The correct diagnosis by transthoracic echocardiography was made in 10 (71%) of the 14 patients. In contrast, preoperative computed tomographic (CT) angiography was performed in 10 patients and correct diagnosis was obtained in 8 (80%) of them. Pulmonary venous obstruction was seen in one patient before surgery. The in-hospital mortality was 14% (2/14). Four patients had pulmonary hypertensive crisis in the postoperative period. The average follow-up was 54 ± 27 months (range: 17-98 months) after surgical repair, and all surviving patients were asymptomatic. There was no late death. No clinically apparent sequelae were seen in six patients in whom isolated left superior pulmonary vein drainage was left uncorrected. Conclusion An accurate diagnosis of anatomic pattern in mixed type TAPVC can be difficult to establish in all the patients before surgery. Detailed intraoperative assessment, individualized surgical approach, and aggressive perioperative management may reduce surgical mortality. Operative survivors have good midterm outcome.

Objectives Pulmonary hypertension is a common association in children with nonrestrictive ventricular septal defect. It increases perioperative mortality and morbidity. Oral sildenafil is an effective pulmonary vasodilator. In this study, we assessed effects of perioperative oral sildenafil therapy on pulmonary artery pressure and early surgical outcomes. Methods This was a single centre, prospective randomized control study. Thirty children with nonrestrictive ventricular septal defects with pulmonary hypertension were divided into two groups. In the sildenafil group (n = 15, mean age 23.3 months), oral sildenafil was administered two weeks before surgery. In the control group (n = 15, mean age 36 months), preoperative sildenafil was not given. Sildenafil was continued postoperatively in both groups, provided the postoperative pulmonary artery pressure was over 50% of systemic pressure. Results There was no perioperative mortality, pulmonary hypertensive crisis and there were no intolerable side effects related to sildenafil in either group. Mean pulmonary artery pressure showed a reduction in both groups. Sildenafil group showed statistically significant improvement in duration of cardiopulmonary bypass (100.27 ± 21.09 min vs. 125.40 ± 26.83 min, p = .008), mechanical ventilation requirement (22.79 ± 17.13 h vs. 30.53 ± 13.05 h; p = .04), epinephrine requirement (22% patients vs. 48% patients; p = .03) and hospital stay (6.13 ± 1.40 days vs. 7.53 ± 1.92 days; p = .05). Conclusion Oral Sildenafil therapy is an inexpensive and well-tolerated method for reducing pulmonary hypertension secondary to non-restrictive ventricular septal defect. It has noteworthy advantages regarding early surgical outcomes like reduced cardiopulmonary bypass time, improved mechanical ventilation time, lower inotrope requirement and shorter hospital stay if used preoperatively in select patient population.

Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.

Congenital heart defects continue to remain a major cause of serious morbidity and mortality, especially in the developing world. This study was planned to get some insight into the role of modifiable nutritional elements in structural CHD etiology. This cross-sectional, observational study was conducted at a hospital in Northern India from January 2017 to December 2017. Infants 6 mo with structural CHD as cases and those without structural heart disease were enrolled as controls. Blood samples were collected from mother-child pairs and tested for serum folate, vitamin B12, and homocysteine. It was found that 46.7% infant-mother pairs, out of 45 in the cases group had folate deficiency, whereas in the control group, only 20% had folate deficiency, which was statistically significant (p = 0.013). Baby (0-6 mo) with congenital heart defects (CHD) and their mothers are more likely to have low folate levels compared to those not having CHD.

Background Structural congenital heart defects (CHD) take a huge toll of congenital defects in children in India. Limited information is available regarding modifiable risk factors for its causation. This study was planned with an aim to determine the prevalence of congenital rubella infection in Indian infants with structural CHD’s. Methodology This cross-sectional, observational study was conducted at a tertiary care hospital in Northern India over 1 year period (1 July 2016 to 30 June 2017). Infants Results A total of 80 infants (M : F = 56 : 24), having mean age 69.4 (±56.5) days; were enrolled. In these infants, prevalence of congenital rubella infection (either infant’s IgM rubella positive or infant’s IgG rubella titers higher than mother’s) was 8.75% (7/80). A total of 12.5% of studied mothers were seronegative for rubella IgG antibodies. Statistically significant association was found between the occurrence of congenital rubella and cataract (p = 0.0039), splenomegaly (p = 0.007) and microcephaly (p = 0.0084) in infants having structural CHD. Conclusions Congenital rubella syndrome still remains an important modifiable cause for structural CHD in India. Sincere efforts for rubella elimination via further strengthening current vaccination strategy would help in decreasing burden of structural CHD in India.

Pulmonary arterial hypertension (PAH) - a complex and progressive disease that carries significant morbidity and mortality despite optimal medical treatment. Combination therapy for PAH can be more effective than monotherapy. The present randomized trial compared the safety and efficacy of sildenafil ambrisentan combination therapy with sildenafil monotherapy. Twenty-two patients of Eisenmenger syndrome and five patients of idiopathic PAH were randomized to two arms. There was a significant improvement in NYHA functional class and mean pulmonary artery pressure, while an insignificant improving trend was observed for 6-min walk distance and oxygen saturation, following the 12 weeks of combination therapy. An upfront combination therapy was found to be safe and effective in the management of PAH patients., Graphical abstract Image 1

Background Government of India is committed to eliminate measles and control rubella/congenital rubella syndrome (CRS) by 2020. In 2016, CRS surveillance was established in five sentinel sites. We analyzed surveillance data to describe the epidemiology of CRS in India. Methodology/Principal findings We used case definitions adapted from the WHO-recommended standards for CRS surveillance. Suspected patients underwent complete clinical examination including cardiovascular system, ophthalmic examination and assessment for hearing impairment. Sera were tested for presence of IgM and IgG antibodies against rubella. Of the 645 suspected CRS patients enrolled during two years, 137 (21.2%) were classified as laboratory confirmed CRS and 8 (1.2%) as congenital rubella infection. The median age of laboratory confirmed CRS infants was 3 months. Common clinical features among laboratory confirmed CRS patients included structural heart defects in 108 (78.8%), one or more eye signs (cataract, glaucoma, pigmentary retinopathy) in 82 (59.9%) and hearing impairment in 51. (38.6%) Thirty-three (24.1%) laboratory confirmed CRS patients died over a period of 2 years. Surveillance met the quality indicators in terms of adequacy of investigation, adequacy of sample collection for serological diagnosis as well as virological confirmation. Conclusions/Significance About one fifth suspected CRS patients were laboratory confirmed, indicating significance of rubella as a persistent public health problem in India. Continued surveillance will generate data to monitor the progress made by the rubella control program in the country., Author summary Rubella infection during the first trimester of pregnancy can affect the fetus, resulting in spontaneous abortion, stillbirth or an infant born with a combination of birth defects known as congenital rubella syndrome (CRS). Government of India is committed to eliminate measles and control rubella/CRS and has completed nationwide immunization campaigns using measles-rubella vaccine targeting children aged 9 months to 14 years. A case-based surveillance for CRS is one of the strategies for achieving elimination. The Indian Council of Medical Research and the Ministry of Health and Family Welfare initiated surveillance for CRS in five sentinel sites to estimate the disease burden. During 2016–18, the surveillance sites enrolled 645 suspected CRS patients, 137 (21.2%) were classified as laboratory confirmed CRS and 8 (1.2%) as congenital rubella infection. Common clinical features among laboratory confirmed CRS patients included structural heart defects (78.8%), one or more eye signs (cataract, glaucoma, pigmentary retinopathy (59.9%)) and hearing impairment (38.6%). Thirty-three (24.1%) laboratory confirmed CRS patients died over a period of 2 years. The surveillance data indicated significance of rubella as persistent public health problem in India.

The pathogenesis of myocardial infarction (MI) involves environmental and genetic risk factors, with the latter putatively playing significant roles in younger patients. Genetic variability in coagulation factors comprises one such group. The coagulation factor 13 subunit A (F13A1) Val34Leu polymorphism (rs5985) has yielded variable findings in literature, with no prior South Asian data.We studied the frequency of this polymorphism using the amplification-created restriction-enzyme site (ACRES) polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) in 101 MI patients aged below 40 years and 103 controls along with plasma fibrinogen and serum homocysteine levels.The distribution of Val/Val, Val/Leu and Leu/Leu genotypes was similar among cases (72.3%, 26.7% and 1.0%) and controls (78.6%, 19.4% and 1.9%, respectively). Val and Leu allele frequencies were 85.6% and 14.4% among patients and 88.3% and 11.7% among controls, respectively (p = .416). Mean plasma fibrinogen was higher in patients vis-à-vis controls (3.1 versus 3.7 g/l; p .001) but homocysteine was elevated in both patients (52%) and controls (67%) (p = .225). Multivariate analysis revealed hypertension (p .001, OR 6.16) and smoking (p .001, OR 5.48) to impart strongest risk followed by positive family history, plasma fibrinogen levels and male gender.Despite its small sample size, this first South Asian study suggests neither protective nor deleterious effects of the F13A1 Val34Leu polymorphism on the risk of MI in young persons. The Leu allele frequency is intermediate to that reported from the West and the Far East. Traditional risk factors contribute greatly to risk even in younger MI patients in South Asia.

Objective Transcatheter closure is the first-choice strategy for the management of appropriate patients with patent ductus arteriosus (PDA). The management of large PDAs is challenging due to the limited available sizes of approved devices and the inherent risks of surgical ligation, especially in adults with calcified PDAs. This study aimed to assess the outcomes of the off-label use of large occluders at a tertiary center. Methods This retrospective review included patients who underwent transcatheter PDA closure with large occluders (≥16 mm) over 16 years. The baseline patient data, procedural details, angiograms, and immediate outcomes were recorded and patients were followed up at 3, 6, 12 months after the intervention and annually thereafter. Results Of the 685 patients who underwent transcatheter PDA closure, 36 patients (mean age 16.6 ± 12.5 years) needed occluders ≥ 16 mm in size. Cocoon duct occluder, Cera duct occluder, Amplatzer atrial septal occluder (ASO), and Cera muscular ventricular septal defect occluders were used for PDA closure. There was no device embolization, one patient in whom ASO was used had residual shunt with intravascular hemolysis requiring surgery, and one patient had mild left pulmonary artery narrowing after the intervention, which was managed conservatively. No patient had residual shunt and one patient had persistent pulmonary hypertension at an intermediate duration of follow-up. Conclusion Transcatheter PDA closure with the use of large devices, which are available in Asia and Europe, is an effective and safe method, especially in adolescents and adults. However, a close follow-up of these patients is mandatory.

Background The spatial relationship of the coronary sinus–great cardiac vein (CS–GCV) to free posterior portion of the mitral valve annulus (MVA) and left circumflex coronary artery (LCx) has gained importance with the advent of cardiovascular interventional procedures such as percutaneous transvenous mitral annuloplasty (PTMA) and mitral isthmus (MI) ablation. Methods In 50 normal (nondilated cardiomyopathy, or non-DCM) and 20 dilated cardiomyopathy (DCM) cadaveric hearts, the diameter and distance from the MVA of CS–GCV and its spatial relationship to LCx along the free posterior portion of the MVA were studied. Results The diameter of the CS–GCV increased from the beginning to termination in both non-DCM and DCM cases. The CS–GCV was located farthest from the MVA in the vertical plane in the middle of its course and in the horizontal plane at its beginning. The LCx was located above the CS–GCV in direct contact with the epicardial aspect of MI in 12% non-DCM and 15% DCM cases and was wedged between the CS–GCV and MI in 20% non-DCM and 15% DCM cases. Conclusions Knowledge of the separation between the CS–GCV and MVA in the horizontal and vertical planes could help in selecting suitable candidates and the preprocedural prediction of success of PTMA. Awareness of the spatial relationship of LCx with CS–GCV in MI would help to reduce the risk of incomplete MI block due to a heat sink effect or damage to the LCx by direct thermal injury during MI ablation.

BACKGROUND Right ventricular (RV) function alterations are invariably present in all patients after tetralogy of Fallot (TOF) repair. Unlike the developed world where most of the patients with TOF are corrected in infancy, average age of presentation and thus surgery for these patients in the developing world may be higher. We aimed to study the correlation between RV function parameters such as tricuspid annular peak systolic excursion (TAPSE), fractional area change (FAC), and tricuspid annular peak systolic velocity (S') with early outcome variables after intracardiac repair for TOF. MATERIALS AND METHODS Fifty patients with a preoperative diagnosis of tetralogy of Fallot scheduled for corrective surgery were included in this single-center, prospective observational study. A preoperative transthoracic echocardiogram was performed to measure RV function parameters (FAC0, TAPSE0, S'0). Transthoracic echocardiography was repeated postoperatively to measure FAC1, TAPSE1, S'1 (day 1) and FAC2, TAPSE2, and S'2 (day 3). The relationship between preoperative and postoperative RV function parameters with in-hospital mortality, duration of mechanical ventilation, and intensive care unit stay was studied. RESULTS The median age of patients was 6 years (range 1-14 years). Multiple stepwise logistic regression analysis showed RV FAC as best predictor of clinical outcome. Area under the receiver operating characteristic curve for postoperative RV function parameters, that is, FAC, TAPSE, and S' to predict early or delayed recovery was 0.944, 0.875, and 0.655, respectively. CONCLUSIONS Among the RV function parameters studied, RV FAC best predicted the early outcome variables after TOF repair, followed by TAPSE while lateral tricuspid annular velocity S' being the least predictive.

The diagnosis of Kawasaki disease (KD), a common pediatric vasculitis, is based solely on clinical criteria. There is a need for a robust laboratory marker that can help differentiate KD from other acute, febrile, childhood illnesses and also to predict cardiac involvement. We conducted a cross-sectional study of 25 consecutive patients admitted with diagnosis of KD from January 2013 to April 2014 and compared them with age- and sex-matched febrile controls. We studied the serum pro-brain natriuretic peptide (ProBNP) [ProBNP and N-terminal pro-B-type natriuretic peptide (NT-ProBNP) levels], a marker of myocardial dysfunction, in children with KD in acute and convalescent phases of disease. These levels were also estimated in febrile controls for comparison. The ProBNP (ProBNP and NT-ProBNP) levels were much higher in the acute phase of the KD patients compared to levels in the convalescent phase of KD (p = 0.000014). Similarly, the levels in the acute phase were higher when compared to the age- and sex-matched febrile controls (p = 0.000126). The receiver operating curve (ROC) analysis for the ProBNP levels in the acute phase of KD yielded an area under the curve of 0.954 ± 0.034 (p

Objective A substantial portion of the Indian cardiac surgery population experiences rheumatic valve disease that progresses to severe pulmonary artery hypertension (PAH) in a few patients. Right ventricular (RV) function, particularly in the perioperative period, has been studied sparsely. The authors describe serial RV function and clinical variables in the perioperative period in patients with severe PAH secondary to left heart disease. Design Prospective, observational study. Setting University hospital. Participants Patients with PAH. Interventions The study comprised consecutive patients referred for open cardiac surgery from January 2012 to June 2013 who also had an estimated right ventricular systolic pressure≥50 mmHg on referral echocardiogram. Composite echocardiographic assessment of right ventricular size and linear/two-dimensional tissue Doppler systolic function and diastolic function analysis were performed at predetermined intervals. Data from right heart catheterization, inotrope use, fluid requirements, mechanical ventilation logs, and intensive care stay also were acquired. Measurements and Main Results A complete dataset was obtained in 20 of 22 patients enrolled in the study. Serial comparison of most RV echocardiographic function variables were noted to be abnormal at baseline, deteriorating further in the immediate postoperative period and trending to a partial recovery at discharge from the intensive care unit, particularly for longitudinal assessment of the RV. Fractional area change, although abnormal, was noted to be preserved. Pulmonary artery systolic pressures registered significantly declined after intervention. The clinical course was largely uneventful. Conclusion Although linear echocardiographic RV function was grossly abnormal in the perioperative period in this patient subset with PAH, there was apparent disjunction with the clinical course.

Objective To develop surface landmarks for blind axillary vein puncture for pacemaker lead implantation. Methods and results Patients for routine coronary angiography were counseled for participating in our study. 20 patients who gave consent were taken up for axillary venogram after proper positioning at the time of coronary angiogram. The venograms of these 20 patients, were reviewed and the landmarks were used to develop a blind axillary puncture technique. Success rate of 100% was achieved with surface landmark guided axillary vein puncture. The implantation time while using surface landmark guided axillary puncture was not significantly longer than when venography based approach was used. Another interesting observation made from the study was that increasing BMI had a positive correlation with the time taken for venous access, the fluoroscopic time and the volume of contrast used, all the associations being statistically significant. Thus, the surface landmark guided technique is more safe and expeditious in non obese patients and probably in pediatric patients as well. Moreover, the new surface landmark guided approach is a significant safety step in terms of reducing the unwanted and avoidable radiation exposure to the hands. Conclusion The results of this study demonstrate that placement of endocardial permanent pacemaker and ICD leads via the developed surface landmarks is effective and safe and is devoid of the harmful effects of radiation and contrast exposure.

Background & objectives: Adriamycin though considered as an effective anticancer drug, leads to irreversible cardiomyopathy (CMP) and congestive heart failure (CHF). The aim of this study was to determine the protective effect of carvedilol in adriamycin (ADR)-induced cardiomyopathy (CMP) in cancer patients. Methods: Patients with lymphoreticular malignancy in whom ADR therapy was planned were randomized into two groups: carvedilol and control. Twenty seven patients each were enrolled in carvedilol and control groups. In the carvedilol group, 12.5 mg once daily oral carvedilol was given during six months. The patients were evaluated by echocardiography before and after chemotherapy. Left ventricular ejection fraction (EF) and systolic and diastolic diameters were calculated. Results: At six months of follow up, six patients in the carvedilol group and five in the control group had died. The mean EF (63.19 vs. 63.88%) and fraction shortening (FS) (34 vs. 34.6) of the carvedilol group were similar at follow up, but in the control group, the mean EF (67.27 vs. 60.82%, P =0.003) and FS (38.48 vs. 34.6, P

INTRODUCTION Left ventricular ejection fraction (LVEF) is the single most important predictor of prognosis in patients with coronary artery disease (CAD) and left ventricular (LV) dysfunction. Equilibrium radionuclide ventriculography (ERNV) is considered the most reliable technique for assessing LVEF. Most of these patients undergo two dimensional (2D) echocardiography and myocardial viability study using gated myocardial perfusion imaging (MPI) or gated F-fluorodeoxyglucose (F-FDG) PET. However, the accuracy of LVEF assessed by these methods is not clear. This study has been designed to assess the correlation and agreement between the LVEF measured by 2D echocardiography, gated blood pool single photon emission computed tomography (SPECT), Tc tetrofosmin gated SPECT, and F-FDG gated PET with ERNV in CAD patients with severe LV dysfunction. PATIENTS AND METHODS Patients with CAD and severe LV dysfunction [ejection fraction (EF)

Variant origin of left circumflex coronary artery (LCx) from right aortic sinus is a well-recognized coronary variation, usually without any clinical consequences. However, the variant origin and trajectory of the artery may have major implications during percutaneous coronary intervention, coronary artery surgery, aortic and mitral valve replacement procedures. We observed a variant LCx in a heart specimen belonging to 45-year-female with no history of hypertension, diabetes mellitus and coronary artery disease. The artery arose along with the right coronary artery from a common ostium in right aortic sinus and depicted a retroaortic course. The vessel was located at the level of aortic annulus and 6.6 mm above mitral valve annulus. The degree of luminal stenosis in variant LCx was higher than that in right coronary artery (RCA) and left anterior descending artery (LAD). Appropriate anatomical knowledge of the location and course of variant LCx is important for successful coronary interventions and valve replacement procedures.

Rheumatic heart disease (RHD) remains a major cause of cardiac related mortality and morbidity in the developing countries due to poor diagnosis and lack of proper therapeutics. The definite reason of heart valve injury during RHD is poorly understood. Valvular endothelial cells play an important role in pathogenesis of different cardiovascular diseases. Besides, the regulation of vitamin D (calciferol) and VEGF (vascular endothelial growth factor) results in the functional changes in endothelial cells. However, the crosstalk between vitamin D and VEGF in the pathogenesis of RHD is not yet unfurled. Evidences in the concerned fields are documented by searching through Google Scholar and Pubmed. Literature based survey has revealed that vascular endothelium, especially endothelial cells play important roles in valvular remodelling during cardiovascular diseases. Endothelial cell dysfunction leads to heart valve remodelling, which furthermore initiates the pathogenesis of valvular heart disease. Vitamin D has the potential to maintain the concentration of VEGF in the circulation and induce the function of endothelial cells. Hence, we hypothesize that vitamin D and VEGF homeostasis can alter the function of endothelial cells, which may subsequently trigger the valvular remodelling or even damage of heart valves during the progression of RHD pathogenesis. Our hypothesis shed light on the evidence based knowledge translation of plausible cellular phenomena due to vitamin D/VEGF homeostasis during valvular vandalism in RHD.

Of various nuclear medicine techniques, F-18/flourodeoxyglucose (FDG) positron emission tomography (PET) is considered as the best modality for the assessment of viable myocardium (VM). In this study, we compared the diagnostic accuracy of nitrate augmented Tc-99m tetrofosmin gated G-single-photon emission computed tomography (SPECT) with FDG PET.54 consecutive cases of angiographically proven CAD with severe LV dysfunction were enrolled in the study. The patients underwent Tc-99m tetrofosmin G-SPECT and FDG PET as per the standard protocols and were compared.SPECT data analysis indicated functional abnormalities in 661/918 myocardial segments. F-18 FDG PET revealed VM in 496/661 segments. The diagnostic accuracy of baseline NAC, postnitrate NAC, baseline AC, and postnitrate AC Tc-99m tetrofosmin SPECT was 84%, 87%, 90%, and 94%, respectively. κ values for NAC baseline, NAC postnitrate, AC baseline, and AC postnitrate Tc-99m tetrofosmin G-SPECT were 0.65, 0.70, 0.77, and 0.85, respectively. Attenuation correction revealed viability additionally in 46 segments which were non-viable on NAC postnitrate study (P .001). Nitrate augmentation showed viability additionally in 25 segments which were non-viable on AC baseline scan (P = .004). On patient-based analysis FDG PET changes the management only in 13% (7/54) of patients.Nitrate augmented AC Tc-99m tetrofosmin G-SPECT shows excellent (κ = .85) agreement with FDG PET. FDG PET changes management only in 13% of the patients. Tc-99m tetrofosmin G-SPECT being more widely available and cheaper imaging modality can be reliably used to detect VM where FDG PET is not available.

A congenital left ventricular diverticulum is a rare cardiac malformation. It is a developmental anomaly that occurs during embryogenesis. Presentations vary from asymptomatic patients to sudden death. To date, the treatment described has been surgical correction. The authors report the first transcatheter closure of an isolated congenital left ventricular diverticulum in a 12-year-old symptomatic girl.

Anomalous origin of the right coronary artery (AORCA) is a rare congenital anomaly with an incidence of 0.92% during routine cardiac catheterization. Its presence raises an important concern to the anaesthesiologist because it can lead on to myocardial ischaemia manifesting as either angina pectoris or myocardial infarction, or sudden death in young patients with minimal exertion, even in the absence of atherosclerosis. Patients with AORCA may be intolerant to stress and the high cardiac output condition owing to volume loading. Such a therapeutic manoeuvre may be desirable during renal transplantation to enable better perfusion of the renal graft immediately after grafting the kidney, in order to improve its function. Hence, haemodynamic goals in renal transplant recipient with AORCA can be contradictory during surgery, thereby rendering anaesthetic management challenging. We report a case of acute myocardial ischemia precipitated by fluid loading conditions in a patient with AORCA during renal transplant that was successfully treated with emergent intra-aortic balloon pump therapy intraoperatively. Judicious intraoperative fluid replacement is recommended, and volume overload must be avoided in AORCA patients undergoing surgery.

This was a single-centre study to evaluate the usefulness of tumour necrosis factor-α (TNF-α) blocker, infliximab (IFX), for treatment of Kawasaki disease (KD) in children in Northern Indian. The study was carried out in the Paediatric Allergy-Immunology Unit, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh. The study period was January 2007 to March 2015. Review of records of 23 children with KD who had received IFX was carried out. Median age at presentation was 2 years (range 2 months to 12 years). Indications for using IFX were intravenous immunoglobulin (IVIg) resistance (12/23 patients); severe KD especially when coronary artery abnormalities (CAAs) had developed in spite of IVIg (9/23 patients); retinal vasculitis in association with KD (1 patient) and economic reasons (1 patient). Twenty one (21/23) patients had received IVIg (2 g/kg) as first line therapy. A dose of IFX was 5-7 mg/kg given intravenously. Screening tests for tuberculosis (chest xray, Tuberculin test, QuantiFERON-TB Gold test) were not carried out prior to IFX infusion in any patient. Duration of follow-up was 0-20 months in 13 patients; 21-40 months in 5 patients and40 months in 6 patients. Mean follow-up was 28.78±25.49 months, range 1-84 months. Eleven of 12 patients (11/12) who had IVIg resistance showed prompt resolution with IFX. Nineteen patients (19/23) in the cohort had CAAs. Of these, 12 showed improvement over mean follow-up of 28.78±25.49 months (range 1-84 months) and 4 showed normalisation. No adverse reactions were noted during infusion of IFX. On follow-up, none of these patients has developed tuberculosis or any other significant infection over a cummulative follow-up of 662 months. IFX can be considered as a useful adjunct in treatment of children with KD.

Airways compression by vascular structures is one of the important comorbidities of congenital heart disease with incidence of approximately 1%-2% in children. Airways compression is a consequence of abnormal configuration of the great vessels producing a vascular ring with enlargement of normal structures (pulmonary arteries or cardiac chambers) or because of surgery. A high index of suspicion for vascular airway compression is important in children with recurrent respiratory complaints. Early diagnosis and management are essential, as chronic airway compression causes significant morbidity. As the underlying anatomical patterns tend to be highly complex, presurgical imaging assessment is essential.

We present a case of an abnormal origin of right subclavian artery and right vertebral artery distal to the origin of left subclavian artery in a 2-year-old patient who presented with cyanotic congenital heart disease with single ventricle physiology. The anomalous origin of a right vertebral artery from the proximal descending thoracic aorta is very rare. We have described the cine-angiographic identification of its origin and course, its embryologic development, and its clinical relevance.

Direct ischemia imaging with F18-FDG can potentially overcome many limitations of SPECT-MPS inherent to “cold imaging”. We compared SPECT-MPS with exercise F18-FDG PET in detection of ischemia in patients with suspected CAD. 45 patients with clinical suspicion of CAD without the history of myocardial infarction were prospectively included. All patients underwent Tc-99m tetrofosmin SPECT-MPS and exercise F18-FDG PET imaging within 7 days of SPECT-MPS, and both modalities were compared with coronary angiography for detecting ischemia. 27 patients had an abnormal coronary angiography (at least one coronary artery with stenosis >50%). Exercise F18-FDG performed better than SPECT-MPS in LAD and LCX territory with comparably good performance in RCA territory. Exercise F18-FDG performed better in single-vessel disease and equally good in multi-vessel disease compared to SPECT-MPS. Performance of exercise 18F-FDG study was significantly better than SPECT-MPS (P = .0014) in the analysis of the 81 vascular territories in the 27 patients with abnormal coronary angiography. Performance of exercise F18-FDG was significantly better than SPECT-MPS in detecting ischemia in suspected CAD patients. Exercise F18-FDG imaging is a potentially useful ischemia imaging modality which offers the advantages of direct ischemia imaging in CAD patients.

Pulmonary vein (PV) isolation with catheter ablation in treating atrial fibrillation carries the risk of injury to phrenic nerve (PN). Left PN (LPN) stimulation continues to be one of the common complications of transvenous left ventricular lead placement during cardiac resynchronization therapy (CRT).In 30 formalin-fixed cadavers, spatial relationship of PNs with PV ostia, left atrial appendage (LAA), and cardiac veins was observed. Segmental location of LPN and cardiac vein crossover was also noted. Right and left PNs coursed abutting the ostium of right superior and left superior PVs in five (16.6%) and one (3.33%) cases, respectively. LPN coursed along the lateral surface of LAA in 20 (66.66%) cases and behind LAA in one (3.33%) case. Out of 18 (60%) cases having two cardiac veins draining free wall of left ventricle (LV) and suitable for CRT lead placement, both cardiac veins were crossed by LPN in two (6.66%) cases. LPN-cardiac vein crossover was located in midlateral segment in 10 (33.3%) cases; mid posterolateral segment in five (16.7%) cases; apical lateral segment and apical posterolateral segment in three (10.0%) cases each.PN is highly susceptible to either injury during catheter ablation or stimulation with LV pacing in certain critical locations. Detailed knowledge of spatial relationship of PNs with cardiac structures could help minimize inadvertent complications during these transcatheter electrophysiological procedures.

Transcatheter device closure of atrial septal defect (ASD) in small children less than 15 kg may be associated with increased complications. Complete atrioventricular heart block (CHB) is a rare complication of ASD device closure in such a setting. We report the case of a 2-year-old girl, less than 15 kg, who underwent device closure of ASD with Amplatzer Septal Occluder and subsequently developed CHB 12 h after the procedure which resolved completely with steroid treatment on fifth day. Case report of a similar kind is rarely reported in the literature. Despite adequate postero-inferior margin CHB may still occur in small children as in our case.

We report a case of a 42-year-old man presenting with shortness of breath and palpitation on exertion, who was evaluated to have left sinus of Valsalva aneurysm rupturing into right atrium. This is a very rare congenital cardiac anomaly with variable clinical presentation ranging from asymptomatic detection on imaging to acute coronary syndrome and sudden cardiac death. Rupture is the most dreaded complication and usually manifests as an acute event. Aneurysmal dilation less commonly affects the left sinus and rupture into the right atrium is still rarer and a chronic insidious presentation as in this case is odd.

Objective To study the correlation of low-dose 64-row multi-detector computed tomography (MDCT) with echocardiography and cardiac catheterization angiography (CCA) in the assessment of pulmonary arterial anatomy in children with cyanotic congenital heart disease (CCHD). Materials and methods This prospective study included 105 children (74 males, 31 females) with CCHD, in the age group of 2 months to 20 years, who underwent 64-row MDCT examination (low-dose CT protocol), echocardiography and CCA for the assessment of pulmonary arteries, including visualization, presence of confluence, stenosis and collaterals. Statistical analysis was performed using the non-parametric statistical analysis test to evaluate the concordance or discordance between echocardiography, MDCT and CCA. Results 64-row MDCT detected significantly more main and branch pulmonary arteries, patent pulmonary confluences, and more cases of pulmonary artery stenosis. CCA detected more major aorto-pulmonary collaterals than MDCT, whereas echocardiography failed to identify these major aorto-pulmonary collaterals. The effective CT radiation dose to patients less than 2 years of age was in the range of 0.7–2.5 mSv, where as the dose in patients more than 2 years of age ranged from that of 2.1 to 4.2 mSv, which is much less than the radiation dose reported in cardiac catheterization angiography. Conclusion In cases where cardiac MRI cannot be performed, or is not sufficiently informative, low-dose 64-row MDCT correlates well with CCA and can provide adequate information about pulmonary arterial anatomy in children with cyanotic congenital heart disease, and can replace invasive cardiac catheterization angiography with markedly reduced radiation dosage to the patient.

Dilated cardiomyopathy is an important cause of heart failure in children. Often it requires transplantation, but on rare occasions it is curable by micronutrient supplementation. Hypocalcemic nutritional rickets was found to be a cause for dilated cardiomyopathy in a 15-month-old child. The patient responded to calcium and vitamin D supplementation promptly and left ventricular systolic function normalized after 3 months of treatment. Nutritional rickets must the considered as an important curable cause for dilated cardiomyopathy among children especially in regions where nutritional rickets is still common.

Spontaneous coronary artery dissection is a rare cause of acute myocardial infarction. It is more commonly seen in the younger age group particularly in women. We report a case of 14-year-old boy that presented with acute myocardial infarction (MI), with left ventricular (LV) dysfunction and was detected to have left main coronary artery dissection on coronary angiography. Myocardial perfusion imaging did not show any evidence of reversible ischemia. Patient was managed conservatively and was marginally improved. This case highlights one of the rare causes of myocardial infarction and the use of myocardial perfusion imaging in selecting treatment options for management of this rare entity.

Background Transcatheter closure of various congenital and acquired vascular malformations with Amplatzer Vascular plugs I and II has been established. Here we present our experience with device closure. Materials and methods Between October 2006 and August 2012, nine (three males and six females) patients aged between 11 months and 62 years (mean age 19 years) underwent percutaneous device closure with AVP I and II vascular plugs for congenital and acquired arteriovenous malformation and cardiac diverticulum are presented here. Results One case of coronary cameral fistula, four cases of pulmonary arteriovenous fistula, one case of large major aortopulmonary collaterals (in tetralogy of Fallot closed before intracardiac repair), one case of congenital cardiac diverticulum, one case of fistula between external carotid artery and internal jugular vein and one case of iatrogenic carotid jugular fistula were successfully closed with AVP I and II plugs. Overall in nine cases, 16 AVP I and II plugs were deployed to occlude feeding vessels and one cardiac diverticulum. The technical success rate was 100%. No major complications were observed. Conclusion Amplatzer vascular plugs can be used successfully for closure of various congenital and acquired vascular malformations with good result.

The modified Blalock–Taussig shunt is a synthetic shunt between the subclavian and pulmonary artery, used in the treatment of congenital cyanotic heart diseases with pulmonary hypoperfusion. Delayed complications include progressive failure of the shunt, serous fluid leak, and pseudoaneurysm formation. We report two different and rare mediastinal vascular complications following modified BT shunt surgery in this case report. The first one is a seroma, due to serous fluid leakage through the shunt graft, which is a relatively benign complication. The second one is a pseudoaneurysm, arising from the shunt, a frequently fatal complication. Generally, X-ray chest is used for screening in these patients. CT angiography plays a vital role in the diagnosis of both these conditions. Management in pseudoaneurysm should be aggressive, as timely intervention may be life saving, while in seroma the management is most often conservative occasionally requiring surgical intervention.

Rubella infection during pregnancy can result in miscarriage, fetal death, stillbirth, or a constellation of congenital malformations known as congenital rubella syndrome (CRS). The 11 countries in the World Health Organization (WHO) South-East Asia Region are committed to the elimination of measles and control of rubella and CRS by 2020. Until 2016, when the Government of India's Ministry of Health and Family Welfare and the Indian Council of Medical Research initiated surveillance for CRS in five sentinel sites, India did not conduct systematic surveillance for CRS. During the first 8 months of surveillance, 207 patients with suspected CRS were identified. Based on clinical details and serologic investigations, 72 (34.8%) cases were classified as laboratory-confirmed CRS, four (1.9%) as congenital rubella infection, 11 (5.3%) as clinically compatible cases, and 120 (58.0%) were excluded as noncases. The experience gained during the first phase of surveillance will be useful in expanding the surveillance network, and data from the surveillance network will be used to help monitor progress toward control of rubella and CRS in India.