Publications by authors named "Shreepal Jain"

Article Synopsis
  • Hematopoietic stem cell transplantation for primary immunodeficiency disorders has improved significantly since 1968, with long-term survival rates in India (62.5% to 75%) being lower than those in high-income countries (90%).
  • This study analyzed data from a charitable hospital on 21 pediatric patients who underwent transplantation between March 2019 and March 2022, focusing on infections, immune recovery, and survival rates.
  • Results showed a median age of 3 years at transplantation, with high engraftment success (94%) and a 95% overall survival rate at one year, demonstrating effective immune reconstitution and minimal serious complications.
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Peripherally inserted central catheter (PICCs) are popular means of long-term intravenous access in oncology patients. Fracture and embolization are rare but potentially serious complications. Here we present an unusual fracture of the PICC line in a 9-year-old boy with Ewing's sarcoma with embolization to the right ventricle (RV) and right pulmonary artery (RPA) which was retrieved percutaneously by trans-catheter snare assisted retrieval.

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Objective: We describe the presentation, treatment and outcome of children with multisystem inflammatory syndrome with COVID-19 (MIS-C) in Mumbai metropolitan area in India.

Method: This is an observational study conducted at four tertiary hospitals in Mumbai. Parameters including demographics, symptomatology, laboratory markers, medications and outcome were obtained from patient hospital records and analyzed in patients treated for MIS-C (as per WHO criteria) from 1 May, 2020 to 15 July, 2020.

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Objective: To describe a subset of atrial septal defect (ASD) with severe pulmonary hypertension (PHT) that is suitable for closure.

Background: As per American Heart Association/American College of Cardiology guidelines, ASD with elevated pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) >2/3rd systemic is considered as a contraindication for closure.

Methods: Patients with anatomically large ASD measuring >25 mm and a high probability of reversible pulmonary vascular disease were subjected to fenestrated device closure, despite severe PHT and elevated PVR.

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Patients having atrial septal defect (ASD) with moderate and more importantly severe pulmonary arterial hypertension (PAH) pose a clinical dilemma. Closing ASD in those with irreversible PAH and not closing it when the PAH is reversible can cost patients dearly, both in terms of quality of life and longevity. In our experience, there is no single parameter that can help in decision making in this difficult subset of patients and therefore we recommend a multi-dimensional approach, which takes into consideration clinical, radiological, electrocardiographic and hemodynamic variables as a whole.

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Article Synopsis
  • This study investigates the role of right atrial (RA) and right ventricular (RV) dynamics in predicting outcomes for children with pulmonary arterial hypertension (PAH).
  • Researchers analyzed clinical data from 57 children, focusing on echocardiographic measurements to assess RA active emptying fraction (EaF) and RV fractional area change (FAC).
  • Results indicated that specific RA and RV metrics, particularly RA EaF ≥60% and RVFAC <25%, are strong indicators of clinical worsening and mortality, suggesting their potential as important monitoring tools for affected children.
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We present the case of a 2-year-old girl with congenital stenosis of the left inferior pulmonary vein associated with a large perimembranous ventricular septal defect. The child underwent repair of the left inferior pulmonary vein with autologous left atrial appendage as a pedicled tube, followed by closure of the ventricular septal defect. Important technical steps to minimize the restenosis rate are highlighted.

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Article Synopsis
  • Pulmonary hypertension (PH) is caused by high blood pressure in the pulmonary artery and is often marked by distinctive sounds during heartbeats, particularly loud closures of the pulmonic valve.
  • This study aimed to investigate whether vibrations in pulmonary circulation produce sounds akin to those made by vocal cords in speech, specifically looking for unique sound signatures in patients with pulmonary artery hypertension (PAH) across four specific auscultation sites.
  • By using a digital stethoscope and analyzing heart sounds recorded during cardiac catheterization, researchers found that PAH subjects exhibited a notable reduction in sinusoid formant entropy, indicating a unique vowel-like sound pattern that could lead to non-invasive screening methods for PAH detection.
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Objectives: Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity.

Methods: We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an 'Intraoperative Customized Double-Patch Device' technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months-10 years), and mean weight was 5.

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Surgical repair of total anomalous pulmonary venous connection (TAPVC) can be complicated by the development of pulmonary venous stenosis later on. In addition, the vertical vein, if left unligated, can remain patent and lead to hemodynamically significant left to right shunting. We report an infant who required transcatheter correction of both these problems after surgical repair of TAPVC.

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Transposition of the great arteries (TGA) with total anomalous pulmonary venous connection (TAPVC) is a rare association. Very few such cases have been reported. Among them 1 patient underwent anatomic repair.

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The closure of atrial septal defects through right-sided limited posterior thoracotomy has been well established in selected subsets. We present a case of large ostium secundum atrial septal defect, pulmonary valvar stenosis, absent right superior vena cava, and isolated left superior vena cava draining to right atrium via coronary sinus. The child successfully underwent total correction through limited posterior thoracotomy with necessary modifications of intraoperative steps.

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Objectives: To assess feasibility, safety, and efficacy of the use of 40 mm Amplatzer septal occluder (ASO 40) for the closure of large atrial septal defects (ASD).

Background: There is very little data available on closure of large ASDs with ASO 40.

Materials And Methods: Case records of patients who underwent ASD closure with ASO 40 between 2002 and 2014 were retrospectively analyzed.

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We hypothesized that an automated speech- recognition-inspired classification algorithm could differentiate between the heart sounds in subjects with and without pulmonary hypertension (PH) and outperform physicians. Heart sounds, electrocardiograms, and mean pulmonary artery pressures (mPAp) were recorded simultaneously. Heart sound recordings were digitized to train and test speech-recognition-inspired classification algorithms.

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Fontan operation and importance of fenestration in the treatment of unusual and complex forms of double outlet right ventricle (DORV) are well established. Nonetheless, rarely, the creation of fenestration becomes challenging in complex morphologies. We present one such child with situs solitus, dextrocardia, DORV, hypoplastic right ventricle, large ventricular septal defect, severe pulmonic stenosis, extremely small right atrium and left juxtaposed atrial appendages, who underwent Fontan operation.

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We report a successful surgical management of a case presented with a combination of aortopulmonary window (APW) with large ventricular septal defect (VSD) amounting to a single ventricle, with a view to highlight technical considerations during staged single-ventricle palliation.

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Article Synopsis
  • Measurement of oxygen consumption (Vȯ2) is crucial for assessing cardiac index and systemic vascular resistance in children, but it's challenging to obtain accurate readings.
  • A study compared oxygen consumption measurements between respiratory mass spectrometry and a breath-by-breath method in mechanically ventilated children, showing a strong correlation between the two techniques.
  • The results indicated that both methods yielded similar Vȯ2 values and did not significantly affect the calculation of cardiac index, suggesting the breath-by-breath method could be a practical choice in critically ill pediatric patients.
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Article Synopsis
  • Researchers investigated whether heart sounds in children with pulmonary artery hypertension (PAH) would show unique sound patterns due to vibrations from the pulmonary circulation.
  • They recorded and analyzed heart sounds from 27 subjects, separating them into two groups based on mean pulmonary artery pressure (mPAp) values.
  • Findings indicate that a significant reduction in the entropy of heart sound patterns correlates with higher mPAp, suggesting a possible noninvasive way to diagnose PAH in children.
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A 3.5-year-old girl underwent transcatheter closure of patent ductus arteriosus in early infancy during which time her secundum atrial septal defect (ASD) was left alone. When she came for elective closure of ASD, she was found to have bilaterally blocked femoral veins.

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We studied digital stethoscope recordings in children undergoing simultaneous catheterization of the pulmonary artery (PA) to determine whether time-domain analysis of heart sound intensity would aid in the diagnosis of PA hypertension (PAH). Heart sounds were recorded and stored in .wav mono audio format.

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Article Synopsis
  • - The study assessed the risks of cardiac catheterization in children with pulmonary hypertension, revealing a relatively low rate of adverse events (3%) despite increased risks compared to adults.
  • - A total of 75 pediatric patients underwent 97 procedures, with diagnoses primarily including pulmonary arterial hypertension (PAH) related to congenital heart disease; no deaths or serious complications occurred.
  • - Follow-up catheterizations were safe and often led to changes in treatment, highlighting their importance in managing pediatric pulmonary hypertension effectively.
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Article Synopsis
  • Pulmonary artery hypertension (PAH) is hard to detect, but digital stethoscopes might help improve diagnosis by analyzing heart sound frequencies.
  • The study involved 27 children, comparing those with normal pulmonary artery pressure to those with elevated pressure, using advanced sound analysis techniques.
  • Results showed that children with PAH had significantly lower heart sound power in the 21-22 Hz range, suggesting that frequency analysis could enhance PAH diagnosis and inform future auscultation methods.
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