Hypoplastic Left Heart: Management Challenges in Resource-Limited Settings

Heart problems are common in newborn babies, affecting approximately 5-10 in 1000 babies. Some are more serious than others, but most babies born with heart problems do not have other health issues. Of those babies who have a serious heart problem, almost 1 in 4 will have heart surgery in their first year. In the UK, pregnant women are offered a scan at around 20weeks to try and spot any heart problems. In most cases there is not a clear reason for the problem, but sometimes other issues, such as genetic conditions, are discovered. In recent years the care given to these babies after they are born has improved their chances of surviving. However, it is recognised that babies born with heart problems have a risk of delays in their learning and development. This may be due to their medical condition, or as a result of surgery and complications after birth. In babies with heart problems, there is a need for more research on ultrasound and magnetic resonance imaging (MRI) to understand how the brain develops and why these babies are more likely to have delays in learning and development. This paper discusses the way ultrasound and MRI are used in assessing the baby's brain. Ultrasound is often used to spot any problems, looking at how the baby's brain develops in pregnancy. Advances in ultrasound technologies have made this easier. MRI is well-established and safe in pregnancy, and if problems in the brain have been seen on ultrasound, MRI may be used to look at these problems in more detail. While it is not always clear what unusual MRI findings can mean for the baby in the long term, increased understanding may mean parents can be given more information about possible outcomes for the baby and may help to improve the counselling they are offered before their baby's birth.

The outcome of patients with hypoplastic left heart (HLH) is determined by many factors, particularly by the first-step palliative procedure in newborns undergoing the Norwood procedure, its Sano modification, or, rarely, through challenging biventricular repairs. Duct stenting combined with bilateral pulmonary artery banding (PAB) is a new method employed as an alternative first-step approach in a number of centers worldwide. We describe this interventional-surgical "hybrid approach" as an additional strategy for the treatment of newborns with HLH syndrome and HLH complex. Between 1998 and April, 2006, 58 newborns underwent ductal stenting and bilateral PAB. These patients underwent surgical bilateral PAB initially, followed by percutaneous duct stenting; the only exception to this were patients in whom duct stenting was performed as a rescue procedure. Various balloon-expandable and self-expandable stents with different widths and lengths were used during the 8-year period of this study. Balloon dilatation of the atrial septum was performed when indicated. This included 5 patients in whom the atrial septum was stented. Aortic arch reconstruction (AAR) combined with a bidirectional cavopulmonary connection (BCPC) was performed at a median age of 4.8 months (range, 2.6-7.5), and total cavopulmonary connection (TCPC) was performed at a median age of 3.1 years (range, 2.5-4). Nine patients were listed for heart transplantation (HTX) and transplanted with AAR when a donor heart was available. Depending on growth of left ventricular structures, biventricular repair (BVR) was performed at a median age of 7.1 months (range, 3.5-10). Overall, 8 of 58 patients (13.8%) treated by the transcatheter-surgical hybrid approach died during the study period. The mortality rate for duct stenting was 1.7% (l/58), and it was 1.7% for bilateral PAB as well. Twenty-seven patients received an AAR/BCPC; 2 of them died (7.4%). Additionally, 1 of 2 patients with AAR/BCPC died while on the waiting list for HTX, resulting in a total mortality rate of 11% with an actuarial survival rate of 89%. One patient is still awaiting AAR + BCPC. Three patients died while on the waiting list for HTX despite successful bilateral PAB and duct stenting. The 30-day mortality rate for TCPC (n = 11), HTX (n = 8), and 18 patients with BVR was 0. The actuarial survival rate for patients with BVR is 93%. Postnatal transcatheter-surgical hybrid palliation expands the surgical options for newborns with HLH. Using hybrid palliation, Norwood stage I operation can be avoided in the neonatal period, the waiting period for children scheduled for cardiac transplantation can be extended, and observation for left ventricular growth suitable for biventricular repair as well.

Outcome of patients with hypoplastic left heart (HLH) is mainly influenced by the successful first-step palliation according to the Norwood procedure. An alternative approach is heart transplantation (HTX). The feasibility of ductal stenting in newborns with duct-dependent systemic blood flow and bilateral pulmonary artery banding has been reported. But it remains to be elucidated whether this approach allows a new strategy for patients with HLH. In patients with various forms of HLH (n=11) and prostaglandin E-1 administration, ductal stenting was performed with balloon expandable Jo stents or Saxx stents. Bilateral pulmonary artery banding was surgically accomplished 1 to 3 days after the transcatheter procedure. Unrestricted blood flow through the interatrial septum was secured by balloon dilatation atrial septotomy, as required. Interventional procedures were performed with no mortality. Stent and ductal patency were achieved for up to 331 days. Two patients underwent HTX, and 8 patients had a palliative 1-stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5 to 6 months. There were 2 deaths. One patient with preoperative right heart failure died after the reconstructive surgery, and 1 patient died 4 months after ductal stenting and bilateral banding awaiting HTX. The present study is the first clinical trial showing that stenting the duct followed by bilateral pulmonary artery banding in newborns with HLH allows the combination of neoaortic reconstruction, which is part of first-stage palliation of HLH, with the establishment of a bidirectional cavopulmonary connection. Additionally, it allows the chance for HTX after extended waiting periods.

To characterize, using magnetic resonance imaging (MRI), the distribution of blood flow and oxygen transport in human fetuses with subtypes of congenital heart disease (CHD) that present with neonatal cyanosis. Blood flow was measured in the major vessels of 152 late-gestation human fetuses with CHD and 40 gestational-age-matched normal fetuses, using cine phase-contrast MRI. Oxygen saturation (SaO2 ) was measured in the major vessels of 57 fetuses with CHD and 40 controls. Compared with controls, we found lower combined ventricular output in fetuses with single-ventricle physiology, with the lowest being observed in fetuses with severe forms of Ebstein's anomaly. Obstructive lesions of the left or right heart were associated with increased flow across the contralateral side. Pulmonary blood flow was reduced in fetuses with Ebstein's anomaly, while those with Ebstein's anomaly and tricuspid atresia had reduced umbilical flow. Flow in the superior vena cava was elevated in fetuses with transposition of the great arteries, normal in fetuses with hypoplastic left heart, tetralogy of Fallot or tricuspid atresia and reduced in fetuses with Ebstein's anomaly. Umbilical vein SaO2 was reduced in fetuses with hypoplastic left heart or tetralogy of Fallot. Ascending aorta and superior vena cava SaO2 were reduced in nearly all CHD subtypes. Fetuses with cyanotic CHD exhibit profound changes in the distribution of blood flow and oxygen transport, which result in changes in cerebral, pulmonary and placental blood flow and oxygenation. These alterations of fetal circulatory physiology may influence the neonatal course and help account for abnormalities of prenatal growth and development that have been described in newborns with cyanotic CHD. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Thirty-Year Survival after Cardiac Surgery for Patients with Turner Syndrome

Sirenomelia is a rare developmental malformation and is incompatible to life. The incidence of sirenomelia, as recorded in the literature, is estimated to be approximately between 1.5 and 4.2 per 1,00,000 births. Around 15% of sirenomelia cases are associated with twin pregnancy, most often in monozygotic cases with an incidence of 7%. In monozygotic twins, the risk of sirenomelia is nearly 100–150 times higher as compared to dizygotic twins or singleton pregnancies. Until now, only two cases of sirenomelia associated with hypoplastic left heart have been reported in the literature. Here, we present a monozygotic twin pregnancy, where one fetus was diagnosed with sirenomelia associated with hypoplastic left heart syndrome and the co-twin was absolutely healthy.

We present an infant with hypoplastic left heart with persistent fever despite two courses of antibiotics and repeatedly negative blood cultures. He eventually underwent surgical extraction of two stents. The stent cultures became positive; he was treated with 4 weeks of antibiotics and the fever resolved.

Diabetes mellitus (DM) is the most common endocrine disorder in children and type 1 DM is the most common; however increasing incidence of type 2 DM is being reported, especially in western societies. Occurrence of psychiatric disorders has been associated with type 2 DM but is rare in those with type 1 DM; therefore the case of an 11-year-old girl who was treated for diabetic ketoacidosis, but suddenly developed acute psychotic symptoms shortly after regaining consciousness is reported. Atypical antipsychotic medications were avoided in this case for concerns of their risk of causing abnormal glucose metabolism.

Intramedullary tuberculoma (IMT) of the conus medullaris is an extremely rare tumour that constitutes a diagnostic and management challenge in a resource-limited setting. We report a case of conus medullaris, IMT in a young immunocompetent, patient with no prior clinical features of pulmonary or extra-pulmonary tuberculosis. The patient presented with six months history of progressive and persistent mid back pain and slight weakness of both lower limbs of 3 months duration. Physical examination revealed a well-nourished man with power of 3/5 and hyperreflexia on both lower limbs. Chest radiograph and other investigations for tuberculosis were negative. Magnetic resonance imaging (MRI) of the lumbosacral spine showed fusiform expansion of the conus medullaris, with a well circumscribed, ring enhancing, intramedullary mass straddle between T12 and L1. Patient had gross total resection with no intraoperative monitoring assistance and no post-operative worsening of neurological function. Histology showed granulomatous lesion with central caseation in keeping with a tuberculoma. Patient was commenced on post-operative anti-tuberculous therapy with physiotherapy, with full motor recovery at six months post-surgical intervention and anti-tuberculous therapy. Intramedullary tuberculoma can be considered as one of the differential diagnoses of intradural, intramedullary tumour of the conus, even in immunocompetent individual with no clinical features of tuberculosis.

Introduction: Vernal Keratoconjunctivitis (VKC) is a chronic ocular condition affecting school-age children, particularly in hot, allergen-rich regions. It presents with symptoms such as intense itching, tearing, foreign body sensation, and sticky discharge, causing discomfort and management challenges in resource-limited settings. VKC impacts public health significantly due to its high prevalence and effect on academic performance and school attendance. Method: This cross-sectional study included 1521 school children aged 6 to 16 years in Western Uttar Pradesh. Comprehensive eye examinations were conducted, and VKC was diagnosed based on conjunctival and limbal papillae. A structured questionnaire collected data on socio-demographic factors, medical care access, and VKC's impact on daily life and school attendance. Descriptive statistics and Pearson chi-square tests were used for analysis. Result: Of the participants, 242 (15.9%) had VKC, with a higher prevalence among boys (56.2%). Most cases were mild (66%), with 34% severe. Symptoms included itching, stinging, tearing, photophobia, and discharge. Severe VKC resulted in higher rates of constant eye rubbing and tear production. School absenteeism was significantly higher in severe cases (78%) compared to mild cases (47%) (P<0.001). Severe VKC also led to more frequent medical consultations (52% vs. 22%, P=0.01). Conclusion: VKC significantly affects school attendance and medical care utilization among children in Western Uttar Pradesh. Improved management practices, better access to medical care, and enhanced support systems are essential for affected children and families.

CADR is more common in HIV-infected persons, although the incidence is declining because of the new safer drugs and early initiation of antiretroviral therapy. The presentation and clinical course does not seem to differ from that in the general population. Antiretrovirals, antituberculosis drugs, and drugs for treating opportunistic infections are most commonly implicated. HIV-associated CADR poses major diagnostic and management challenges because of polypharmacy, overlapping drug toxicities, drug interactions, overlap of CADR with other diseases, and limited alternative drugs. We discuss pragmatic management strategies of HIV-associated CADR focusing on resource-limited settings, where HIV infection and associated CADR are most common.

Tetanus remains a major cause of morbidity and mortality, especially in resource-limited settings. This case series outlines the management of four patients with tetanus referred to our critical care unit in New Delhi from July to November 2024. The comprehensive approach included early administration of tetanus immunoglobulin, rigorous control of spasms and autonomic dysfunction, and careful management of complications. Our team achieved a 100% survival rate in a disease known for its high fatality. We discuss the challenges of tetanus management with the need for sedatives, muscle relaxants for a longer period, approximately 2 weeks, and underscores the importance of immunization and timely, evidence-based critical care.

Morphologic study of the ascending aorta and aortic arch in hypoplastic left hearts: Surgical implications

Background Sexually transmitted infections (STIs) and reproductive tract infections (RTIs) pose significant public health challenges globally, particularly in resource-limited settings. This study aimed to investigate the challenges faced by healthcare providers in managing and preventing STIs/RTIs in India. Materials and methods In-depth interviews were conducted with eight healthcare providers, including counselors, gynecologists, and dermatologists, working in government settings. A semi-structured interview guide was used to explore challenges related to patient care and healthcare system resources. Results The study revealed significant gaps in patient knowledge and awareness about STIs/RTIs, with misconceptions affecting treatment-seeking behavior. Social stigma and cultural barriers were identified as major obstacles to open communication and timely care. Gender-specific challenges in healthcare-seeking behavior and partner notification were noted. Healthcare providers reported inconsistent availability of treatment kits and medications, as well as challenges in ensuring patient compliance. The need for improved healthcare infrastructure, including specialized clinics and better interdepartmental coordination, was highlighted. Conclusion Addressing STI/RTI management challenges requires a multifaceted approach, including enhancing public awareness, ensuring consistent medication supply, establishing specialized clinics, and improving interdepartmental coordination. These findings provide valuable insights for developing targeted interventions to improve STI/RTI management and prevention in resource-limited settings.

Hypoplastic left heart with absence of the aortic valve: prenatal diagnosis using two-dimensional and pulsed Doppler echocardiography.