The mortality rates of pulmonary hypertension complicating pregnancy remains high despite advances in treatment options. The normal physiological changes in pregnancy and labor combined with progressive increase in pulmonary vascular resistance may lead to cardiac decompensation in peri- and postpartum periods and is associated with high peripartum mortality. Therefore, prevention of pregnancy is still recommended in diagnosed cases of pulmonary hypertension. We report our experience in managing five cases of pulmonary hypertension undergoing elective cesarean delivery. All five patients had good maternal and neonatal outcomes and were discharged uneventfully on pulmonary vasodilators. Multidisciplinary team approach was associated with successful maternal and neonatal outcome in our cases. The obstetric anesthetists were integral members of the team and were introduced to the patients at the earliest. Early detection, treatment of pulmonary hypertension, risk stratification, and individualized perioperative plan are very important to ensure successful outcome.

Background: Early detection of congenital heart disease (CHD) is critical, particularly in low- and middle-income countries where access to pediatric cardiology services is limited. While auscultation remains a first-line screening tool, its accuracy is operator dependent. Advances in digital stethoscopes and artificial intelligence (AI) may enhance diagnostic performance. Objective: To evaluate the accuracy of an AI-based algorithm integrated with a digital stethoscope for detecting CHD in children, using echocardiography as the reference standard. Methods: In this prospective study, heart sounds were recorded from 721 subjects (382 with CHD, 339 controls) using the digital stethoscope at four standard auscultatory sites. Of these, 301 recordings were used for algorithm training and 420 for blinded forward testing. Recordings were denoised, transformed into mel-spectrograms, and analyzed using a convolutional neural network (CovNN) to classify CHD probability. A threshold probability of > 0.5 across > 2 sites was used to predict CHD. Performance was compared against echocardiography. Results: In forward testing, the AI algorithm correctly identified 222 of 233 CHD cases and 171 of 187 controls, yielding a sensitivity of 95.2% and specificity of 91.4%. The algorithm remained effective in noisy environments and in uncooperative children, where conventional auscultation is challenging. Conclusion: An AI algorithm integrated with a digital stethoscope demonstrated high sensitivity and specificity for CHD detection in children. By leveraging mel-spectrogram features, it overcomes key limitations of isolated time-domain or frequency domain analysis and shows promise as a scalable, non-invasive screening tool for early CHD detection, particularly in resource-limited settings.

Pulmonary arterial hypertension (PAH) in India is a substantial yet under-recognized contributor to cardiovascular morbidity and mortality. Its progressive course, nonspecific early manifestations, and heterogeneous aetiologies often lead to delayed diagnosis and fragmented care. The unique epidemiological profile of India, characterized by a higher prevalence of PAH due to factors such as uncorrected congenital heart disease, environmental exposure, and chronic infections, underscores the need for a management approach tailored to Indian settings. These realities intersect with systemic challenges, including late presentation, resource constraints, a lack of local guidelines, and the costs of diagnostic workups and therapies, which hinder the delivery of standardized care. Variabilty in clinical practice in PAH management underscores the need for guidelines that account for local context and resources. Despite robust international recommendations, including those from the European Society of Cardiology and the European Respiratory Society, implementation of standardized risk stratification and treatment algorithms remains inconsistent across Indian centers. This variability reflects disparities in infrastructure, expertise, and affordability rather than differences in disease biology. This consensus document reviews current guidelines and advances in the management of PAH, consolidating evidence with potential for adoption in India and other low- and middle-income countries (LMICs). This document also highlights the need to establish centers of excellence, develop specialized care protocols, and increase awareness to improve patient outcomes.

Pulmonary hypertension (PH) in children and adolescents is a multifactorial condition affecting the pulmonary vasculature that can lead to life-threatening complications, especially during acute decompensations. There is sudden/progressive right heart failure (RHF) with circulatory insufficiency and multiorgan hypoxemia during PH crises. Early diagnosis, individualized approach, and hemodynamic assessment are vital for effective management. Echocardiography, followed by radiodiagnostic imaging and cardiac catheterisation, are important diagnostic tools. A multifaceted approach prioritises lowering pulmonary vascular resistance, improving right ventricular function, and optimising cardiac output to control PH crises in the intensive care setting effectively. This review outlines the pathophysiology, clinical presentation, diagnosis, and management of acute PH in the pediatric intensive care unit (ICU). Therapeutic options range from cardinal ICU measures and targeted pharmacotherapy to advanced interventions such as extracorporeal life support and transplantation. This review highlights current evidence, enumerates recent advances in management, identifies gaps in knowledge, and underscores the need for further research to improve outcomes in pediatric acute PH.

Background: Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH). Objective: The objective of the study was to see the feasibility of transcatheter closure of CPSS and their outcome. Materials and Methods: We analyzed the data of 24 patients of CPSS who underwent transcatheter closure from five institutions (March 2013 to April 2019). Baseline evaluation included echocardiography with bubble contrast study, ultrasound examination of the abdomen, computed tomography angiogram, and cardiac catheterization with test balloon occlusion of the CPSS. The evaluation showed cyanosis due to PAVM in 12, PH in 8, and respiratory distress in 2. Two had both cyanosis and PH. Criteria for eligibility for complete catheter closure of CPSS included demonstration of intrahepatic portal vein (PV) radicals together with a PV pressure of ≤18 mmHg on occlusion. Results: The median age and weight were 8 years (0.5–21) and 19.5 kg (4.2–73), respectively. Transcatheter closure was performed in 21 patients (22 procedures) using a variety of occlusive devices and stent-graft exclusion was done in one patient. Closure was not done in 3 in view of high portal venous pressures and hypoplastic PVs. During the follow-up (median: 42 months and range: 61 days–4.8 years), saturation normalized in 14 patients with PAVM. PH declined in all eight patients who underwent the procedure. Respiratory distress improved in two patients. Conclusions: Early and short-term follow-up results of catheter closure of CPSS appear promising. However, further, follow-up is needed to demonstrate long-term effectiveness.

Background Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality. Risk stratification and initiation of dual or triple combination therapy has a better clinical response, especially in high-risk patients. Unfortunately, prostacyclin analogues are not marketed in India; hence, the use of these medications is limited. We report the benefits and difficulties of using iloprost inhalation in patients with advanced PAH in India. Methods In this prospective observational study, we included patients with group 1 PAH. Inhaled iloprost was initiated as an add-on therapy for patients who had clinical, echocardiographic or laboratory deterioration on dual oral medications. Patients with clinical instability were excluded. All patients underwent thorough clinical evaluation, detailed echocardiogram and laboratory investigations. Patients were started on inhaled iloprost 2.5 μg six times daily and closely followed up. The dose was escalated if necessary. On follow-up, clinical echocardiographic and laboratory evaluation was done on all patients. Results Fourteen patients (11 women) with a median age of 32 years (2-66 years) with group 1 PAH were started on inhaled iloprost as an add-on therapy. Improvement in clinical parameters, WHO functional class, echocardiographic-derived right ventricular function, and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels were observed in 10 of 14 patients. A median increase of 31% (4.2, 106%) in the distance travelled during 6-minute walk test, a median increase of 45% (-20, 120%) in right ventricular fractional area change, a median increase of 27% (-16.7, 60%) in tricuspid annular peak systolic excursion and a median decrease of 36.7% (-69.6, 17.2%) in NT-pro-BNP levels were observed after initiation of medication. Three patients had progression of symptoms and were then referred for lung/heart-lung transplant. One patient developed progression of symptoms after an excellent initial response and transitioned to subcutaneous treprostinil. Improvement in clinical, echocardiographic and laboratory features allowed us to successfully perform surgical Potts shunt in 2 patients. The medications were well tolerated with minimal and transient side-effects. There were no deaths. Conclusion Inhaled iloprost can be used with acceptable benefits and minimal side-effects in patients with PAH.

Background: Potts shunt has been suggested as an effective palliative therapy for patients with pulmonary artery hypertension (PAH) not associated with congenital heart disease. Materials and methods: This is a prospective single-center study performed to assess outcomes of Potts shunt in patients with PAH who are in functional class III or IV. Results: 52 patients in functional class III/IV with pulmonary arterial hypertension without significant intra or extracardiac shunt on maximal medical therapy were evaluated and counseled for undergoing Potts shunt/patent ductus arteriosus (PDA) stenting. 16/52 patients (13 females) consented for the procedure; 14 patients underwent surgical creation of Potts, and 2 underwent transcatheter stenting of PDA, which physiologically acted like a Potts shunt. Standard medical therapy was continued in patients who did not consent for the procedure. 12/16 patients survived the procedure. Patients who did not survive the procedure were older, with severe right ventricular systolic dysfunction, and functional class IV. Patients who survived the procedure were followed up in the pulmonary hypertension clinic. The Median follow-up was 17 months (1-40 months). 11/13 patients discharged after the operation showed sustained clinical, echocardiographic, and biochemical improvement, which reduced need for pulmonary vasodilator therapy in 10/13 patients. There was one death in the follow-up period 16 months post-surgery due to lower respiratory tract infection. Conclusion: Potts shunt is feasible in patients with PAH without significant intra or extracardiac shunts. It can be done safely with an acceptable success rate. Patient selection, preoperative stabilization, and meticulous postoperative management are essential. It should be performed at the earliest sign of clinical, echocardiographic, or laboratory deterioation for optimal outcomes. Long-term follow-up is required to see a sustained improvement in functional class and the need for a lung transplant in the future. (C) 2021 Cardiological Society of India. Published by Elsevier B.V.

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 to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). Conclusions : The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

4. Lip GY, Windecker S, Huber K, Kirchhof P, Marin F, Ten Berg JM, et al. Management of antithrombotic therapy in atrial fibrillation patients presenting with acute coronary syndrome and/or undergoing percutaneous coronary or valve interventions: A joint consensus document of the European society of cardiology working group on thrombosis, European Heart Rhythm Association (EHRA), European Association of Percutaneous Cardiovascular Interventions (EAPCI) and European Association of Acute Cardiac Care (ACCA) endorsed by the Heart Rhythm Society (HRS) and Asia-Pacific Heart Rhythm Society (APHRS). Eur Heart J 2014;35:3155-79.

Objective To assess outcomes and factors influencing outcomes in neonates requiring cardiac surgery in India. Methods This study reports on review of hospital data from a tertiary care cardiac surgical institute from January-2009 to December-2015. Results A total of 200 neonates were included; of them, 5% of the cases were antenatally diagnosed and most of them had unmonitored transport (111, 55.5%). The overall mortality rate was 13.5%, (n=27) and 178 (89%) underwent complete defect repair. There was a significant association of mortality with shock, the number of inotropes, intra-operative procedure, residual lesion, aortic cross-clamp and deep hypothermic circulatory arrest time (all P<0.05). Logistic regression analysis showed ventilation duration, cardiac-bypass time, shock, and residual cardiac lesion as independent predictors of mortality. Conclusion Cardiac defects were found to have late detection and most transports were unmonitored. Complete surgical repair and shorter cardiac bypass time can potentially improve neonatal cardiac surgical outcomes.

Objective: Abernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH. Methods: This is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH. 10 patients were diagnosed to have an extrahepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature. Results: 10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type 1 and 7 had type 2 EHPCS. 6/7 patient with EHPCS type 2 underwent closure of the shunt. There was no procedure related complication. There was one death 3 months post procedure and one patient who was advised surgical closure was lost to follow up. Closure of the shunt resulted in normalization of the pulmonary artery pressures in 4/5 patients. Conclusion: Congenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.

We describe the presentation, treatment and outcome of children with multisystem inflammatory syndrome with COVID-19 (MIS-C) in Mumbai metropolitan area in India. 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. 23 patients (11 males) with median (range) age of 7.2 (0.8–14) years were included. COVID-19 RT-PCR or antibody was positive in 39.1% and 30.4%, respectively; 34.8% had a positive contact. 65% patients presented in shock; these children had a higher age (P=0.05), and significantly higher incidence of myocarditis with elevated troponin, NT pro BNP and left ventricular dysfunction, along with significant neutrophilia and lymphopenia, as compared to those without shock. Coronary artery dilation was seen in 26% patients overall. Steroids were used most commonly for treatment (96%), usually along with intravenous immunoglobulin (IVIg) (65%). Outcome was good with only one death. Initial data on MIS-C from India is presented. Further studies and longer surveillance of patients with MIS-C are required to improve our diagnostic, treatment and surveillance criteria.

The aim of this study was to determine practice patterns and inter-institutional variability in how acute vasoreactivity testing (AVT) is performed and interpreted in pediatrics throughout the world. A survey was offered to physicians affiliated with the Pediatric & Congenital Heart Disease Taskforce of the Pulmonary Vascular Research Institute (PVRI), the Pediatric Pulmonary Hypertension Network (PPHNET), or the Spanish Registry for Pediatric Pulmonary Hypertension (REHIPED), from February to December 2016. The survey requested data about the site-specific protocol for AVT and subsequent management of pediatric patients with idiopathic pulmonary arterial hypertension (IPAH) or heritable PAH (HPAH). Twenty-eight centers from 13 countries answered the survey. AVT is performed in most centers using inhaled nitric oxide (iNO). Sitbon criteria was used in 39% of the centers, Barst criteria in 43%, and other criteria in 18%. First-line therapy for positive AVT responders in functional class (FC) I/II was calcium channel blocker (CCB) in 89%, but only in 68% as monotherapy. Most centers (71%) re-evaluated AVT-positive patients hemodynamics after 6–12 months; 29% of centers re-evaluated based only on clinical criteria. Most centers (64%) considered a good response as remaining in FC I or II, with near normalization of pulmonary arterial pressure and pulmonary vascular resistance, but a stable FC I/II alone was sufficient criteria in 25% of sites. Protocols and diagnostic criteria for AVT, and therapeutic approaches during follow-up, were highly variable across the world. Reported clinical practice is not fully congruent with current guidelines, suggesting the need for additional studies that better define the prognostic value of AVT for pediatric IPAH patients.

INTRODUCTION:Delayed sternal closure is used in paediatric cardiac surgery as a management strategy for patients with unstable hemodynamics or postoperative bleeding routinely. We hypothesise that planned postponement of sternal closure leads to better outcomes than emergent reopening in the intensive care unit (ICU) in patients exhibiting some hemodynamic indication for the same.METHODS:We retrospectively analysed the outcomes of delayed sternal closure 220/2111 (10.42%) out of which 14 sternums were opened in the ICU after shifting the patients.RESULTS:A total of 220/2111 (10.42%) sternums were left open postoperatively, out of which 14 were opened after shifting to the ICU. Total mortality of the delayed sternal closure was 33/220, i.e. 15%. The patients whose sternums were left open from the theatre had a mortality of 23/206, i.e. 11.16%, whereas those patients whose sternums were opened in the ICU had a mortality of 10/14, i.e. 71.42%.CONCLUSION:In doubtful postoperatively hemodynamic, the choice of leaving the sternum open electively has better outcomes, rather than opening the sternum as a terminal bail out procedure.

Background: Right atrial (RA) function has been studied rarely in childhood pulmonary arterial hypertension (PAH). We sought to determine if RA and right ventricular (RV) area changes measured by echocardiography predicted outcomes. Methods: We reviewed data from children with PAH undergoing cardiac catheterization and echocardiography. RA and RV areas were obtained from the apical 4-chamber view. Clinical worsening indicated initiation of parenteral prostanoid therapy, heart and/or lung transplantation, Potts shunt surgery or death. Results: We studied 57 children (27 females), median age 3 years (range 0.30-17 years), body surface area 0.56 m2 (0.2-1.8), follow up 3 years (0.21-8.35), time to clinical worsening was 1.14 years (0.03-6.14) and mortality was 1.55 years (range 0.88-4.95). We determined from receiver operator curves that RA active emptying fraction (RA EaF) >= 60% predicted clinical worsening (sensitivity 78%, specificity 69%, AUC 0.7) and mortality (sensitivity 100%, specificity 65%, AUC 0.82). RV fractional area change (RVFAC) <25% predicted clinical worsening (sensitivity 72%, specificity 79%, AUC 0.85) and death (sensitivity 67%, specificity 69%, AUC 0.77). The combination of RA EaF >= 60% and RVFAC <33% were best predictors of clinical worsening (sensitivity 72%, specificity 82%, partial AUC 0.65) and mortality (sensitivity 100%, specificity 77%, partial AUC 0.75). Conclusion: In childhood PAH, RA EaF >= 60% and RVFAC <25% were associated with poor outcomes. RA EaF >= 60% and RVFAC <33% were best predictors of clinical worsening and may be useful markers in children with PAH who require closer observation and more intensive therapy. (c) 2018 Elsevier B.V. All rights reserved.