Prostaglandin E1 Infusion Research Articles

Pulmonary Overcirculation Requiring Surgical and Pulmonary Flow Restrictor Device Intervention in Critical Coarctation of the Aorta-A Case Series.

The use of prostaglandin infusion to maintain patency of the ductus arteriosus in patients with critical coarctation of the aorta (CoA) to support systemic circulation is the standard of care. However, pulmonary overcirculation resulting from a patent ductus arteriosus in patients with critical CoA is not well described in the literature. We report two cases of critical CoA that required invasive measures to control pulmonary blood flow before surgical repair of the CoA. Both patients had signs of decreased oxygen delivery, hyperlactatemia, and systemic to pulmonary flow via the ductus arteriosus. One patient required surgical pulmonary artery banding and the second patient underwent pulmonary flow restrictor device placement for the control of pulmonary blood flow. A rapid improvement in oxygen delivery and normalization of lactate levels were observed after control of pulmonary overcirculation. Both patients underwent successful surgical repair of the coarctation A and were discharged home.

The Need for Preoperative Prostaglandin E1 for d-Transposition After Balloon Atrial Septostomy

BackgroundThe necessity of prostaglandin E1 (PGE) usage before arterial switch operation (ASO) in infants with d-transposition of the great arteries (dTGA) after balloon atrial septostomy (BAS) remains controversial. MethodsThis study is a single-centre, retrospective review of infants with dTGA who underwent ASO from January 2014 to December 2021. Parameters analysed included post-BAS oxygen saturation, time from BAS to PGE discontinuation, necessity of reinitiation, interval before PGE restart, and lowest saturation before PGE reintroduction. ResultsAmong the 35 cases of dTGA who underwent ASO, 31 (88%) required BAS, with 23 (65%) requiring PGE infusion. Of those 23 infants, 14 (60%) necessitated PGE reinitiation after discontinuation. A significant difference in post-BAS oxygen saturation was observed between the groups requiring PGE reinitiation (79.2% ± 4.7%) and those not needing reinitiation (89.0% ± 2.0%) (P < 0.001). The relative risk for the reinitiation group with BAS oxygen saturation levels ≤80% was 2.5 (95% confidence interval: 1.3-4.6). No disparity was observed in postoperative outcomes or PGE adverse effects such as fever, apnoea, bradycardia, and congestive heart failure requiring diuretic between the groups. ConclusionsGiven no significant differences in PGE adverse effects and a 2.5 times higher risk of reinitiation with post-BAS saturation below 80%, maintaining PGE until saturation reaches 80% for a few days before discontinuation may help reduce the risk of rebound hypoxaemia.

Propranolol Therapy in Tetralogy of Fallot: Treating the Echocardiogram or Treating the Patient.

Hypercyanotic spells are one of the defining clinical features of Tetralogy of Fallot (TOF). Limited data exist on peak Doppler right ventricular outflow tract (RVOT) gradient as a risk factor for the development of hypercyanotic spells, frequency of prophylactic use of propranolol based on peak RVOT gradient, and its impact on preventing the occurrence of hypercyanotic spells. We aimed to quantify peak RVOT gradients as measured on transthoracic echocardiography in infants with unrepaired TOF and assess for correlation with clinical symptoms of hypercyanotic spells. We also assessed the frequency of pre-operative use of propranolol, indication for medication initiation, and occurrence of hypercyanotic spells with or without propranolol use. Retrospective analysis was performed on patients at our institution who were born between February 1, 2011 and May 31, 2023. Patients were excluded if they were maintained on prostaglandin infusion or underwent palliative shunt placement or balloon valvuloplasty prior to complete surgical repair. Demographics, occurrence of hypercyanotic spells, propranolol use, peripheral oxygen saturation, age at surgical repair, and peak RVOT gradient at the time of propranolol initiation were collected from the electronic medical record. If no propranolol use was recorded, the single highest maximum RVOT gradient prior to surgery was collected. 203 patients were identified, of which 92 patients were included in analysis. Thirty-six (39%) patients received propranolol and 19% of patients developed hypercyanotic spells prior to surgery. Patients with higher peak RVOT gradients were more likely to be started on propranolol even in the absence of overt symptoms, and they also demonstrated more systemic desaturation. Additionally, peak RVOT gradient was found to be a poor predictor for the development of hypercyanotic spells. Wide clinical variation exists in the prophylactic use of propranolol for prevention of hypercyanotic spells. Peak RVOT gradient is not a reliable tool for prophylactic propranolol initiation to prevent hypercyanotic spells.

Staged biventricular repair in a premature neonate with critical aortic stenosis, severe mitral regurgitation, and fetal hydrops: a case report

BackgroundThe surgical management of critical aortic stenosis, mitral regurgitation, and left ventricular dysfunction is a significant clinical challenge. Whether left ventricular function will recover to support systemic circulation after the relief of aortic stenosis is a concern. In this setting, surgical or balloon aortic valvotomy combined with bilateral pulmonary artery banding and atrial septectomy may allow time for left ventricular adaptation, while the systemic circulation is supported by the right ventricle through the ductus arteriosus. We describe the case of a premature neonate with critical aortic stenosis, severe mitral regurgitation, and fetal hydrops who successfully underwent staged biventricular repair after bilateral pulmonary artery banding, atrial septectomy, balloon aortic valvuloplasty, and stent implantation for ductus arteriosus.Case presentationA 29-year-old female was referred to our hospital at 25 weeks of gestation with fetal echocardiography findings of critical aortic stenosis, severely impaired left ventricular function, severe mitral regurgitation, and restrictive foramen ovale. At 33 weeks of gestational age, the baby was born via cesarean delivery. Prostaglandin E1 infusion was immediately initiated, and the neonate underwent emergecy bilateral pulmonary artery banding and atrial septectomy. On the second day, a balloon aortic valvuloplasty was performed. The neonate underwent stent implantation to open the ductus arteriosus and multiple-balloon aortic valvuloplasty. At 4 months of age, he underwent biventricular repair consisting of surgical aortic valvuloplasty, atrial septal defect closure, bilateral pulmonary artery debanding, and ductus arteriosus ligation. At 1 year of age, he underwent the Ross –Konno procedure. Six years after the operation, the patient’s general condition was stable, and the patient is doing well.ConclusionsStaged biventricular repair was successfully achieved in a premature neonate with fetal hydrops and critical aortic stenosis associated with severe mitral valve regurgitation and left ventricular dysfunction.

Prostaglandin E1-induced urticaria with harlequin change in a neonate with transposition of great vessels: A case report

Prostaglandin E1 (PGE1) is an emergency drug used in neonates with critical congenital heart diseases (CHDs) to maintain the ductal patency. There are many adverse effects of this drug explained – cutaneous side effects being one of them, of which limited literature is available. In this case report, we describe a term neonate with critical CHD, started on PGE1 infusion to maintain ductal patency. Baby developed erythematous migratory annular rashes to harlequin colour change in a dose-dependent manner. The severity of rashes decreased, and there was a complete resolution with tapering down of drug doses to a minimum level to maintain a ductal patency. No antihistamines or steroids were administered for the treatment. The PGE1 should be tapered to a minimum dose to maintain the required saturation in case of severe cutaneous reaction and should not be stopped abruptly as it is a lifesaving drug in critical CHD.

Association between balloon atrial septostomy and prostaglandin E1 therapy until repair of transposition of the great arteries in neonates.

In patients with transposition of the great arteries, the continuation of prostaglandin E1 is more frequent in patients with intact ventricular septum in comparison to patients with ventricular septal defect. Ballon atrial septostomy did not eliminate the need for prostaglandin E1 infusion until the time of surgery in both subgroups of patients.

Aberrant Subclavian Artery in Interrupted Aortic Arch with Severe Aortic Outlet Obstruction: Cerebral Blood Flow as a Possible Determinant of Embryonic Cardiovascular Development?

Aberrant subclavian artery (ASCA) is frequently observed in interrupted aortic arch (IAA) with aortic/subaortic obstruction. Developmental significance of ASCA in IAA in utero remains elusive. Newborns with prenatally diagnosed isolated IAA under continuous prostaglandin E1 infusion were studied. Cross-sectional areas of aortic valve opening (AVOCSA) and patent ductus arteriosus (PDACSA) were represented by echocardiographic measurement of (diameter)2 indexed by body surface area (m2). Types of IAA and presence of ASCA were examined in relation to sizes of AVOCSA and PDACSA. Twenty-four newborns with IAA (six type A and 18 type B) were reviewed. Male dominance was seen in type B (male 72%). Twenty-three patients had left aortic arch. No type A patients had ASCA, but 50% of type B had ASCA; AVOCSA was significantly smaller in type B than in type A (p = 0.003). In type B, PDACSA was significantly larger in those with ASCA than without (p = 0.003), but AVOCSA exhibited no significant size difference between these two subgroups. Chromosome 22q11 deletion was only seen in type B (56%) and showed no significant correlation with the presence of ASCA. In type B IAA, the presence of ASCA was associated with larger PDACSA, suggesting an adaptive enlargement of the ductus arteriosus and ASCA in response to reduced antegrade flow across small AVOCSA, which may be augmenting cerebral blood flow. Preservation of cerebral blood flow may be another important determinant affecting embryonic cardiovascular development.

Ductus Arteriosus Stenting as a Method of Palliative Treatment of Truncus Arteriosus Type A3: Literature Review and Clinical Case

Introduction. Van Praagh A3 variant of truncus arteriosus (TA A3) is a condition when the one pulmonary artery branch origins from the TA, the other is filled through the patent ductus arteriosus (PDA).&#x0D; PDA constriction in the early neonatal period can cause absence of blood supply to the one pulmonary artery branch. To prevent this, prolonged infusion of prostaglandin E1, PDA stenting or systemic-to-pulmonary shunt are used. These methods allow to postpone total repair (TR) and reduce mortality risks associated with neonatal period.&#x0D; The aim. To analyze research papers dedicated to endovascular approach of TA A3 staged treatment and to present our own experience.&#x0D; Materials and methods. We conducted a systematic literature search and analyzed various options for the TA A3 staged treatment. After the review, we used endovascular approach as the first stage of treatment in our clinical case.&#x0D; We studied a newborn weighing 4 kg with TA A3 (left pulmonary artery branch origins from the non-coronary sinus of the truncal valve, right pulmonary artery branch filling through the PDA). Nakata index was 83 mm2/m2. McGoon ratio was 1. We decided to perform PDA stenting with the aim of postponing surgical correction until the patient’s optimal age and weight.&#x0D; Discussion. In total, the number of publications about TA A3 staged treatment is limited. Most institutions prefer TA A3 staged treatment in newborns, because it is associated with a lower risk of complications than early TR. In our clinical case, the first step was PDA stenting. The staged approach allowed us to perform TR with a good result at the age of 3 months. Before TR, Nakata index and McGoon ratio were increased to 248 mm2/m2 and 1.9, respectively.&#x0D; Conclusions. The current trend of TA A3 repair has few different options of staged treatment. Staged approach helps to get out of the newborn period and achieves the optimal condition for TR. Our patient successfully received PDA stenting as the first stage of treatment, which created conditions for the pulmonary artery branches growth. The second stage was TR with good long-term results. After analyzing the publications, we noted that the use of endovascular PDA stenting is a good and safe alternative to surgical palliative treatment.

Abstract 13368: Availability of PGE1 in Northern California Hospitals: Before and After Routine CCHD Screening

Introduction: Fetal echocardiogram and critical congenital heart disease (CCHD) screening has improved early detection of CCHD. CCHD screening has been recommended for all infants since 2011 and mandated in California since July 2013. In patients with suspected ductal dependent congenital heart disease, early intervention with prostaglandin E1 (PGE1) is the standard of care to reduce morbidity and mortality. Hypothesis: In 2004, we assessed the availability of PGE1 in Northern California hospitals that care for newborn infants and found that 32% of hospitals with obstetric services did not have the ability to institute PGE1 infusion. As California has adopted CCHD screening for all newborn infants prior to hospital discharge, our aim was to assess the percentage of hospitals that perform CCHD screening as well as reassess PGE1 availability. Our hypothesis was that CCHD screening would increase the availability of PGE1 at hospitals providing obstetric services. Methods: The initial study contacted 117 Northern California hospitals to determine PGE1 availability. We contacted the same hospitals, ending up with 106 hospitals due to closures and mergers. The newborn nursery and/or NICU for each of these hospitals was contacted to determine CCHD screening practices and availability of PGE1. Mileage to the nearest level IV NICU was used as a gross estimate of distance to a tertiary care center. Results: In 2004, 92 of 117 hospitals had obstetric service, and 30 (32.6%) of those did not have PGE1. In this study, 74 of the 106 provided obstetric services. All performed CCHD screening, but 15 (20.3%) did not have ability to provide PGE1 infusion. 8 of the 15 hospitals were more than 30 miles (range 1.5 - 192 miles) from the closest level IV NICU. Conclusions: All hospitals in Northern California providing obstetric service are performing CCHD screening. However, 20.3% of these hospitals still do not have the ability to administer PGE1 infusion. Of these, 53% were in suburban and rural areas of Northern California, with significant transportation distance to a tertiary care center. Delay in initiating PGE1 infusion in patients with ductal dependent CCHD may lead to morbidity or mortality, and increasing PGE1 availability is an opportunity to improve access to care.

Abstract 13226: Young Infants With Symptomatic Tetralogy of Fallot: Superior Survival With Staged Repair

Background: The optimal strategy to treat symptomatic young infants with tetralogy of Fallot (TOF) is unclear. Objective: We performed a balanced comparison of staged repair (SR) vs. primary repair (PR) at two institutions that have exclusively adopted each strategy. Methods: Between 1993 and 2021, 143 consecutive infants under 4 months of age underwent systemic-to-pulmonary shunt at the Royal Children’s Hospital, Melbourne, Australia. These were compared with 122 consecutive infants in the same age group who underwent PR between 2004 and 2018 at the Children’s National Hospital, Washington, D.C. The primary outcome was mortality. Secondary outcomes were component and cumulative hospital stay, durations of perioperative support, hospital complications, and early and late reinterventions. Propensity-score matching was performed for risk adjustment. Mean follow-up was 9.3 years. Results: In the unmatched comparison, the SR cohort was younger, more commonly on prostaglandin infusion and mechanical ventilation, more likely to require urgent procedures, and had lower weight and smaller pulmonary annulus size. Hospital mortality after initial procedure (SR: 2.8% [4/143] vs. PR: 2.5% [3/122]; p = 0.86) and 10-year survival (SR: 95% [95% confidence interval (CI): 90 - 98%] vs. PR: 90% [95% CI: 81 - 95%]; p = 0.65) were similar. Hazard of reinterventions favored the PR group during early (&lt;6 months) follow-up (hazard ratio: 0.22; 95% CI: 0.11 - 0.48; p &lt; 0.001) but was similar at late follow-up. Most secondary outcomes cumulatively favored the PR group. Among 57 propensity-score matched pairs, the SR group had superior survival at 10 years (98% [95% CI: 88 - 99.8%] vs. 85% [95% CI: 69 - 93%]; p = 0.02). Conclusions: In this two-institutional, propensity-score matched comparison of SR vs. PR in symptomatic young infants with TOF, the SR strategy was associated with superior long-term survival and equivalent late reinterventions, at a cost of increased early reinterventions.

Use of prostaglandin and cyclophosphamide for pediatric lupus with digital gangrene: Back to safety before hitting the axe!

Digital gangrene is a rare presenting feature of childhood lupus and only a reported incidence of 1.3%. We describe two cases of pediatric onset systemic lupus erythematosus (SLE), both 16years old, presenting with digital gangrene and the successful salvage of the digits after using intravenous cyclophosphamide for immunosuppression and use of intravenous prostaglandin E1 infusions for limb reperfusion. Both of the patients responded exceptionally to the infusions with resolution of gangrene and near-total preservation of the functionality of toes.

Clinical utility of repeat fetal echocardiography in congenital heart disease.

To investigate the utility of repeat fetal echocardiography (FE) following a diagnosis of structural congenital heart disease (CHD) on the initial FE. We evaluated how often changes in management and counseling occurred based on subsequent FE findings and sought to determine which types of CHD were more likely to have changes in management and/or counseling based on repeat FE. This was a retrospective review of all patients who presented to our center between January 2012 and January 2019 and who had more than one FE performed for structural CHD. We reviewed consultation notes to determine whether management or counseling had changed based on FE findings at follow-up visits. Management variables included a change in location or mode of delivery, plan for atrial septostomy, initiation of prostaglandin infusion, umbilical line placement and planned admission location (nursery vs neonatal intensive care unit). We defined a counseling change as any of the above changes in management as well as any meaningful refinements in the cardiac diagnosis that led to a change in the overall prognosis or future management. Initial diagnoses were grouped into anatomically/hemodynamically relevant subgroups. Fisher's exact test was used to assess the relationship between the initial diagnosis and changes in management. Post-hoc pairwise comparisons were performed using Dunnett's test. Between January 2012 and January 2019, 267 patients underwent 534 follow-up FE assessments performed for structural CHD. Management change based on repeat FE occurred in 41/267 (15.4%) cases. A change in management was associated with the diagnosis made at the initial visit (P < 0.001). The proportion of cases with a management change was highest among those with an initial diagnosis of pulmonary valve abnormality/non-critical pulmonary stenosis (4/11 (36.4%)), followed by balanced atrioventricular canal (AVC) defect (5/17 (29.4%)) and left ventricular outflow tract obstruction/aortic valve abnormality or coarctation/interrupted aortic arch (19/68 (27.9%)). No management change occurred in fetuses diagnosed with isolated ventricular septal defect (VSD), truncus arteriosus, pulmonary vein anomaly or isolated arch sidedness/branching abnormality. Compared to those with a VSD, management was significantly more likely to be changed in fetuses with a balanced AVC defect (P = 0.025) and left heart lesions (P = 0.002). Right heart lesions showed a trend towards an increased incidence of management change (P = 0.05). A counseling change based on repeat FE occurred in 108/267 (40.4%) cases. The proportion of cases with a counseling change was highest among those with an initial diagnosis of pulmonary valve abnormality/non-critical pulmonary stenosis (8/11 (72.7%)) and hypoplastic left heart syndrome/critical aortic stenosis (5/9 (55.6%)). The clinical utility of follow-up FE is associated with the type of CHD diagnosed. Follow-up FE led to changes in management in several types of CHD, most commonly in cases with an initial diagnosis of right and left outflow obstructive lesions and balanced AVC defect. When developing programmatic protocols for the frequency of FE assessments, the type of CHD should be a major determinant, but additional studies are required to reach a consensus on how often serial FE should be performed for each type of CHD. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

Can We Safely Decrease Early-Term Delivery and Cesarean Section Rate in Pregnancies Complicated by Fetal Transposition of Great Arteries?

Background: Transposition of the great arteries (TGA) is a common critical neonatal congenital heart defect. After birth, physiological shunts close rapidly, necessitating early treatment with prostaglandin infusion and balloon-atrial septostomy. Timing of delivery is challenging, balancing the risks and advantages of early-term delivery and specialized care. The aim of this study is to assess the safety of a full-term delivery policy in fetuses diagnosed with TGA. Methods: A retrospective chart review was conducted of 17 women with a prenatal diagnosis of fetal TGA at Virgen del Rocío University Hospital between 2015 and 2021. Primary outcomes included: incidence of preterm, early-term, full-term, and late-term delivery, and rate of cesarean section. Secondary outcomes included: Saturday to Sunday admission and birth, and delivery between 0:00 a.m. and 8:00 a.m. Results: Full-term birth was achieved in 94.1%, reaching a low cesarean delivery rate (17.6%). A total of 82.4% of infants were born on weekdays, and only in three of the cases (17.6%) did delivery occur between 0 a.m. and 8 a.m. The median birth weight was 3300 g. Intravenous prostaglandins were administered in all cases, and 94.1% required balloon-atrial septostomy. Conclusions: In our study favoring full-term delivery, we reduce early-term deliveries and the cesarean section rate in prenatally diagnosed TGA.

Hypoplastic Left Heart Syndrome With Low Birth Weight or Prematurity: What Is the Optimal Approach?

BackgroundHypoplastic left heart syndrome with low birth weight or prematurity comprises a high-risk population with no optimal treatment pathway. Using the Pediatric Health Information System, we compared management approaches across the United States. MethodsWe analyzed neonates (≤30 days) with birth weight <2500 grams or gestational age <36 weeks between 2012 and 2021. Four strategies were identified: Norwood procedure, ductus arteriosus stent + pulmonary artery banding, pulmonary artery banding + prostaglandin infusion, or comfort care. Outcomes included hospital survival, discharge disposition, staged palliation completion, and 1-year transplant-free survival. ResultsOf 383 infants identified, 36.4% (n = 134) received comfort care, 43.9% (n = 165) Norwood, 12.4% (n = 49) ductal stent + pulmonary artery bands, and 8.8% (n = 34) pulmonary artery bands + prostaglandins. Neonates receiving comfort care had the lowest gestational age (35 weeks; interquartile range [IQR], 31.5-37 weeks) and birth weight (2.0 kg; IQR, 1.5-2.3 kg); 24.6% (33 of 134) had chromosomal anomalies. Infants undergoing primary Norwood had the highest birth weight (2.4 kg; IQR, 2.2-2.5 kg) and gestational age (37 weeks; IQR, 35-38 weeks). Glenn palliation was performed in 66.1% (109 of 165) compared with ductal stent + pulmonary artery band in 18.4% (9 of 49) and pulmonary artery band + prostaglandins in 35.3% (12 of 34). Only 11.3% (6 of 53) born <2 kg survived to 1 year, all after Norwood. Primary Norwood yielded higher hospital and 1-year transplant-free survival than hybrid strategies. ConclusionsComfort care is routinely provided, particularly for infants with low birth weight, gestational age, or chromosomal anomalies. Primary Norwood offered the lowest hospital and 1-year mortality and highest palliation completion rates; birth weight was the most important factor determining 1-year survival.

ISUOG Practice Guidelines (updated): fetal cardiac screening.

ISUOG Practice Guidelines (updated): fetal cardiac screening.

Left Ventricular Outflow Obstruction in D-Transposition of Great Arteries: A Rare Etiology

A 2-day old neonate [gender: male; weight: 3.2 kg; height: 53cm] presented to the authors institution with an arterial oxygen saturation of 74%. The child was receiving 6 cm H2O continuous positive airway pressure therapy [FIO2: 0.5] and was treated with a prostaglandin E1 infusion [10ng/kg/min]. Transthoracic echocardiography revealed situs solitus, levocardia, normal systemic and pulmonary venous drainage and a 6 mm non-restrictive atrial septal defect with a left to right shunt. The atrioventricular connections were concordant. The aorta was arising from the right ventricle with no right ventricular outflow obstruction [Figure 1]. The pulmonary artery was arising from the left ventricle with confluent good size branch pulmonary arteries with posterior deviation of the conal septum [Figure 2]. A large perimembranous (conoventricular type)1 ventricular septal defect with an inlet [6 mm] and outlet [6 mm] extension and an additional tiny apical defect were demonstrated [Figure 3]. A patent ductus arteriosus [2.7 mm] shunting left to right was also present (not shown). A 2-dimensional echocardiography image in the parasternal long axis view displayed an obstruction to the left ventricular outflow tract [white arrow] and a color Doppler blood flow map demonstrated turbulence in the outflow tract [Figure 4A,B]. In the subcostal view with the probe tilted anteriorly, color flow Doppler mapping showed the ventricular septal defect and also the blood flow acceleration across the left ventricular outflow tract [Figure 5, videolip-1]. At the area of turbulence, the jet width was 0.234 cm[Figure 6A]. A continuous wave Doppler interrogation of the area demonstrated a gradient of ≈ 25 mmHg [Figure 6B]. 2-dimensional transthoracic echocardiography interrogation in the subcostal view identified the etiology of the left ventricular outflow tract obstruction [Figure 7, Videoclip-2]. What is the diagnosis? Figure 2Transthoracic echocardiography image showing the relationship between the pulmonary artery and the left ventricle. View Large Image Figure Viewer Download Hi-res image Figure 3Subcostal 4-chamber transthoracic echocardiography image showing the perimembranous ventricular septal defect. View Large Image Figure Viewer Download Hi-res image Figure 4A&B. Parasternal long axis transthoracic echocardiography 2-dimensional image showing the obstruction in the left ventricular outflow tract ‘white arrow’ [A], and turbulence in the outflow tract by a color Doppler blood flow map ‘white arrow’[B]. View Large Image Figure Viewer Download Hi-res image Figure 5Subcostal transthoracic echocardiography image showing color Doppler flow acceleration over the left ventricular outflow tract and a ventricular septal defect. View Large Image Figure Viewer Download Hi-res image Figure 6A&B. Subcostal transthoracic echocardiography image showing the width of the obstruction [A] and the pressure gradient across the obstruction [B]. View Large Image Figure Viewer Download Hi-res image Figure 7A subcostal transthoracic echocardiography view showing the etiology of the left ventricular outflow tract obstruction in systole. View Large Image Figure Viewer Download Hi-res image

Fluoroscopy-free, echocardiography-guided hybrid stenting of native aortic coarctation in a 920-grams premature infant.

Therapeutic options are limited for the management of extremely low-birth-weight infants with critical aortic coarctation despite high doses of prostaglandin infusion. We report successful hybrid, fluoroscopy-free, echocardiography-guided primary stenting of native aortic coarctation in a 920-grams premature infant.

Neurological, Extracardiac, and Cardiac Manifestations of Ebstein's Anomaly Along With its Genetics, Diagnostic Techniques, Treatment Updates, and the Future Ahead.

Ebstein's anomaly is a congenital heart defect characterized by the displacement of the tricuspid valve, and its leaflets to be malformed. Due to the defect involving the tricuspid valve, there is a reverse flow of blood into the right-sided atrium, which may lead to cardiac hypertrophy and edema of the lower extremities. There is a decreased flow of blood out of the right heart due to reduced right ventricular contractility and tricuspid regurgitation. Children afflicted with thisanomaly usually suffer from atrial septal defect and this is usually diagnosed before birth on a routine ultrasound scan. In neonates, cyanosis can be seen due to right-to-left atrial shunting or as a result of severe congestive heart failure. If the infant has pulmonary hypertension, cyanosis is markedly increased as there will be a limitation in pulmonary blood flow. In adults, arrhythmias, cyanosis, and heart failure are seen. The bundle of Kent leads to the formation of an electrical conduction abnormality between the right ventricle and atrium. This leads to a condition commonly known as Wolff- Parkinson-White syndrome in patients. An enlarged spherical heart is usually present on a chest X-ray. ECG changes of Ebstein's anomaly show taller than usual P waves, PR prolongation, and right bundle branch block. There can be certain neurological and extracardiac manifestations too such as hemiplegia, stroke, dysarthria, etc. During fetal life, specifically at 16 and 20 weeks of gestation, the anomaly can be diagnosed via echocardiography. Prostaglandin infusion (PGE1) is given to maintain pulmonary circulation in neonates if cyanosis is seen. In children and adults with congestive cardiac failure due to this anomaly, medical management includes digoxin, beta-blockers, diuretics, and angiotensin converting enzyme (ACE) inhibitors to improve heart failure. Surgical treatment includes valve reconstruction. In this article, we review the pathophysiology, genetics, diagnosis, management, and prognosis of Ebstein's Anomaly along with a comprehensive discussion on its genetics, neurological manifestations, extracardiac features, and current advancements in treatment.

Is Revascularization the Only Mode of Treatment for Diabetic Foot Ulcer?

Introduction and Objective: Diabetic foot infection is a common cause for hospital admissions among diabetics in India. This could be attributed to sociocultural practices, such as walking barefoot, inequitable access to diabetes care, poor education, and poor socioeconomic conditions. The purpose of this study is to highlight the role of diabetic footwear in care of diabetic foot. Materials and Methods: The observational study included 170 patients, presenting with diabetic foot ulcers, studied over a period of 19 months. The patients were categorised in those suffering from neuropathic ulcers and neuroischemic ulcers using biothesiometery and Ankle Brachial Pressure Index. Diabetic foot wears were prescribed based on plantar pressure measurement. The mode of management was noted and need for incidence on follow up was recorded. Results: Our study population predominantly consisted of elderly males with a mean age of 63.2 years and 71.7 % males. Out of 170 patients presenting with diabetic foot ulcer, neuroischemic ulcers were diagnosed in 35 patients, and the rest 135 patients were diagnosed to have neuropathic ulcers. (Graph 1) NIU was observed more common among male patients with 25 males out of 35 patients [71.4 %], similarly NPU were more common in males [72.8 %]. Among the 35 patients who were diagnosed to have neuroischemic ulcers, all required diabetic foot wear, however 10 patients required revascularization procedure, 2 patients required Prostaglandin therapy infusion and 1 patient required stem cell implantation. The group of patients who were diagnosed to have neuropathic ulcers, required only diabetic foot wear and medical therapy which involved antibiotics, antiplatelets, vasodilators and Prostaglandin infusion. Amputation was required in 8 (23%) out of 35 patients suffering from NIU and in 12 (9%) out of 135 patients suffering from NPU. Conclusion: ABI, bioethesiometry and plantar pressure measurement play an important role in planning management and need of diabetic foot wear. Majority of the patients may have neuropathic involvement with recurrence rate and need of amputation significantly reduced by use of diabetic foot wear.

Experience of Intravenous infusion of prostaglandin E1 for cessation of rest pain and further ischemic changes in patients with PVD

Prostaglandins are potent vasoactive agents with wide variety of other actions - vasodilatation, fibrinolysis and inhibition of platelet aggregation. Prostaglandin E1( PGE1) was the agent used since 1973 for cardiovascular diseases, mainly in patients with advanced peripheral vascular disease. PGE1 intra venous infusion has shown to be beneficial in limb threatening ischemia, especially when reconstructive procedures are not feasible. The original research of benefitted 28 cases in last 5 years is presented here.