Papers by Nageswara Rao Koneti

Annals of Pediatric Cardiology
Background : Outcome data of children with heart disease who acquired COVID-19 infection are limi... more Background : Outcome data of children with heart disease who acquired COVID-19 infection are limited. Aims : We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. Settings and Design : This is a retrospective, multicentric, observational study. Materials and Methods : The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. Results : From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3–96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9–41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1–814.7, P = 0.046). Conclusions : Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.

NeoReviews, 2015
A term male infant born to a second gravida mother is referred to our hospital for new-onset resp... more A term male infant born to a second gravida mother is referred to our hospital for new-onset respiratory distress. He was born by normal vaginal delivery and had Apgar scores of 8, 9, and 9 at 1, 5, and 10 minutes, respectively. The newborn begins breastfeeding immediately after birth and is shifted to the mother’s side. After 18 hours of delivery, he is noted to have fast breathing. A pediatric consultation is sought; a provisional diagnosis of congenital heart disease is made, and the patient is referred to our hospital. The antenatal period is uncomplicated. The results of targeted imaging for fetal anomalies performed at 20 weeks of gestation are reportedly normal. At admission, the newborn weighs 2.74 kg and has a temperature of 96.8°F (36oC), a heart rate of 175 beats per minute, a respiratory rate of 70 breaths per minute, and a blood pressure in the right upper limb of 58/26 mm Hg. The capillary filling time is prolonged to 4 seconds with weak and feeble pulses, and there is moderate to severe intercostal and subcostal recessions with an audible grunt. The baseline saturation is 85% in room air, which improves to greater than 95% on hood oxygen. On systemic examination, he has an …
Asian Cardiovascular and Thoracic Annals, 2008
A 2-year-old boy with cyanosis was found to have normal situs and looping with anomalous drainage... more A 2-year-old boy with cyanosis was found to have normal situs and looping with anomalous drainage of a right-sided superior vena cava to the left atrium, and intact interatrial septum in association with anomalous drainage of the left pulmonary veins to the right superior vena cava. He underwent successful surgical repair of this rare congenital malformation.
Annals of Pediatric Cardiology, 2013
We report the successful transcatheter closure of right pulmonary artery fistula to left atrium i... more We report the successful transcatheter closure of right pulmonary artery fistula to left atrium in a six-year-old boy, who had presented with cyanosis and shortness of breath. The two-dimensional echocardiogram with bubble contrast study demonstrated the communication between right pulmonary artery and left atrium. Computerized tomography confirmed the diagnosis and delineated the anatomy. The fistula was closed successfully by a transcatheter trans-septal approach using an 18/20 duct occluder.
A 3-day-old neonate presented with features suggestive of coarctation of aorta. Echocardiography ... more A 3-day-old neonate presented with features suggestive of coarctation of aorta. Echocardiography showed a large organized thrombus in the transverse arch causing obstruction to theaortic arch and carotids with partial recanalization. Patient underwent surgical thromboendarterectomy with arch reconstruction. The evaluation did not reveal any hematological abnormalities and suspected to be due to fetal thromboembolism. Patient improved well, and no neurological deficits were observed during follow-up.
We report the successful re-screwing of the embolized duct occluder (DO) in three children for re... more We report the successful re-screwing of the embolized duct occluder (DO) in three children for retrieval and to attempt redeployment. The DO was embolized into descending aorta immediately after the deployment in one child and within 24 h after the procedure in two further patients. The DO was re-screwed back by the DO delivery cable, using “sheath in sheath” in all three cases; however, successful retrieval could be done only in two. Repositioning in the patent ductus arteriosus (PDA) was done using the same device in those two children and surgical removal was needed in third child with perimembranous ventricular septal defect.

Tetralogy of Fallot (TOF) is a structural congenital heart defect with functional significance, o... more Tetralogy of Fallot (TOF) is a structural congenital heart defect with functional significance, occurring 1 in 3,300 live births. Several studies have reported mutations in essential transcription factors responsible for the development of TOF. NKX2.5 is a homeobox containing transcription factor, expresses at various stages of heart development. The rationale of this study was to estimate the frequency of NKX2.5 Arg25Cys mutation in TOF children from India. Using PCR-RFLP the frequency was estimated in TOF and controls. Clinical characteristics and maternal data from 84 unrelated TOF patients was collected and evaluated in comparison with 117 healthy individuals. TOF patients were tested for 22q11.2 micro deletion for the inclusion of sporadic cases. In 6% of TOF cases (n=84) a positive family history of congenital heart defects was revealed and 85.7% of children had various clinical symptoms. Significant reproductive history such as abortions, intrauterine and perinatal deaths wer...
A female neonate was born to a primigravid mother by emergency lower segment cesarean delivery at... more A female neonate was born to a primigravid mother by emergency lower segment cesarean delivery at 33 weeks’ gestation and noted to have generalized edema, bradycardia, ascites, and hepatosplenomegaly. In view of respiratory embarrassment, the ascitic abdomen was tapped within the first few minutes after delivery. The infant’s appearance and the fluid obtained are shown in Fig 1. Figure 1. A hydropic newborn with abdominal distension and fluid from the ascitic tap (done in view of respiratory distress). The infant was admitted to the neonatal intensive care unit and given fluids and respiratory support. On examination, the newborn was edematous, with a heart rate of 50 beats per minute, respiratory rate of 70 breaths …

Background: Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia wit... more Background: Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies. Objective: To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation. Methods: This is a single.center, nonrandomized, retrospective study conducted during June 2008 to September 2015. Twenty-four patients with PA IVS were selected for the procedure during the study period. The median age and weight of the study population were 8. days and 2.65 kg, respectively. Four patients were excluded after right ventricular angiogram as they showed right ventricular-dependent coronary circulation. The pulmonary valve perforation was attempted using various types of CTO wires based on the tip load with v...
Embryologically, the common arterial trunk (CAT) is due to the failure of septation by the major ... more Embryologically, the common arterial trunk (CAT) is due to the failure of septation by the major outflow cushions. This invariably leads to the presence of an associated large juxta-arterial ventricular septal defect. Overriding of the ventricular septum leads to its biventricular origin in more than 2/3rd of cases. Uncommonly, CAT may be associated with an intact ventricular septum (IVS) and univentricular origin. We describe an infant with CAT, intact IVS, hypoplastic right ventricle, and Ebstein's anomaly of the tricuspid valve who presented to us and palliated successfully. The embryological mechanisms are discussed in this report.

Background : COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited... more Background : COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. Aims : The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. Settings and Design : This is a retrospective, multicentric, observational study. Methods : We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. Results : The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared...

Background : Vascular rings (VRs) present with varied symptoms and may result in significant morb... more Background : Vascular rings (VRs) present with varied symptoms and may result in significant morbidity before an accurate diagnosis is made. Prenatal diagnosis may be useful to plan surgery after birth. Objectives : The purpose of the study was to see the feasibility of accurate diagnosis of VR during antenatal ultrasound examination and describe their outcome. Methods : This is a retrospective observational study between January 2014 and December 2019. Vascular rings were diagnosed on the basis of three vessel tracheal view and neck vessels arrangements on fetal echocardiogram. Postnatal evaluation by transthoracic echocardiography and computerized tomography angiogram was performed. Surgical repair was done as per standard indications. Results : A total of 35 cases of fetal VRs (median gestational age: 24 weeks [range: 19–35]) were diagnosed during the study period. There were four dichorionic diamniotic twin gestation pregnancies. The right aortic arch (RAA) with anomalous left s...
Routine anterograde closure of ventricular septal defects (VSDs) is well established. The techniq... more Routine anterograde closure of ventricular septal defects (VSDs) is well established. The technique however is complex, and requires the creation of an arteriovenous loop. We describe a new technique for transcatheter closure using a retrograde single catheter approach. 77 symptomatic children (42
IHJ Cardiovascular Case Reports (CVCR)

Annals of Pediatric Cardiology
Background : Balloon-expandable pulmonary valves are usually not suitable for dilated native outf... more Background : Balloon-expandable pulmonary valves are usually not suitable for dilated native outflow tracts. Methods : Indian Venus P-valve registry was retrospectively analyzed for efficacy, complications, and midterm outcomes. Straight valve was used in prestented conduits in patients with right ventricular pressure above two-thirds systemic pressure and/or right ventricular dysfunction. Flared valve 1–4 mm larger than balloon waist was used in native outflow in symptomatic patients, large ventricular volumes, and ventricular dysfunction. Objectives : A self-expanding porcine pericardial Venus P-valve is available in straight and flared designs.. Results : Twenty-nine patients were included. Straight valve was successful in all seven conduits, reducing gradients significantly, including one patient with left pulmonary artery (LPA) stent. Flared valve was successfully implanted in 20 out of 22 native outflow tracts. Sharp edges of the older design contributed to two failures. Complications included two migrations with one needing surgery, endocarditis in one, insignificant wire-frame fractures in three, and groin vascular complication in one patient. There were no deaths or valve-related reinterventions at a mean follow-up of 47.8 ± 24.5 months (1–85 months). Modifications of technique succeeded in three patients with narrow LPA. There was significant improvement in symptoms, right ventricular volume, and pulmonary regurgitant fraction. Conclusion : Straight and flared Venus P-valves are safe and effective in appropriate outflow tracts. Straight valve is an alternative to balloon-expandable valves in stenosed conduits. Flared valve is suitable for large outflows up to 34 mm, including patients with LPA stenosis. Recent design modifications may correct previous technical failures. Studies should focus on durability and late complications.
Cardiology in the Young
An 8-day-old neonate was presented with severe respiratory distress and diagnosed as primary pulm... more An 8-day-old neonate was presented with severe respiratory distress and diagnosed as primary pulmonary hypertension of the newborn on functional echocardiogram. Evaluation showed bounding pulse, enlarged umbilical cord, and bruit over the periumbilical region. Transthoracic echocardiography and CT angiogram showed a large fistulous communication between the umbilical vein and artery suggestive of congenital umbilical arteriovenous malformation leading to high-output cardiac failure and pulmonary artery hypertension. The patient was stabilised with medications and ventilation. Transcatheter closure of communication was done using coils, vascular plug, and KONAR-MFTM device. The patient improved from heart failure soon after the procedure and thriving normally at 6 months of follow-up.
Catheterization and Cardiovascular Interventions
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Papers by Nageswara Rao Koneti