Impact of persistent pulmonary hypertension and oxygenation on brain injury in neonates with neonatal encephalopathy treated with therapeutic hypothermia.

OBJECTIVE: To investigate the effects of persistent pulmonary hypertension (PPHN) and oxygenation on outcome of neonates with neonatal encephalopathy (NE) treated with therapeutic hypothermia (TH). STUDY DESIGN: We compared the outcome of neonates with NE treated with TH with or without PPHN. RESULTS: 384 neonates with NE were treated with TH; 24% had PPHN. The fraction of inspired oxygen was higher in the first 4 days of life (p < 0.001) in neonates with PPHN. They had a significantly lower arterial partial pressure of oxygen in the first 4 days of life (p = 0.005) and higher on days 3-4 of life (p < 0.001). They were more often intubated (p < 0.001) and more often had concomitant hypotension (p < 0.001). They had higher mortality (p = 0.009) and more often developed brain injury (p = 0.02). CONCLUSION: PPHN occurred frequently in neonates with NE treated with TH and was associated with a higher incidence of adverse outcome.

Anomalous systemic venous connection to left atrium in a heart with usual atrial arrangement.

Anomalous systemic venous connection to left atrium is rare anomaly. Previously published cases described this anatomy in patients with left isomerism. Depending on the size of the atrial septal defect, patients usually present with varying degrees of cyanosis and right heart hypoplasia. Here, we report a case of anomalous systemic venous connection to left atrium in a newborn with the usual atrial arrangement.

Blalock-Taussig Shunt versus Ductal Stenting as Palliation for Duct-Dependent Pulmonary Circulation.

Objectives: There is limited data published from outside North America and Europe comparing the outcomes of a modified Blalock-Taussig shunt (MBTS) and ductal stenting as the first palliative procedure for infants with duct-dependent pulmonary circulation. This study reports the National Heart Center's, in Muscat, Oman, experience in comparing the outcomes of these 2 interventions. Methods: This retrospective study included all infants with duct-dependent pulmonary circulation who received either a MBTS or ductal stenting from 2016-2019. The primary outcomes were death or re-interventions. Secondary outcomes included death, subsequent re-interventions, survival to subsequent surgical intervention, survival to hospital discharge, post-procedural mechanical ventilation and duration of intensive care unit stay. Results: A total of 71 patients were included in the study, 33 (46%) of whom received ductal stenting. The prevalence of the primary outcome (death or re-intervention) in the patent ductus arteriosus (PDA) stent group was 54.5% versus 31.6% in the MBTS group but this was not statistically significant (P = 0.06). There was no difference between the 2 groups in terms of time to next surgical intervention (P = 0.233). The PDA stent group had shorter post-procedural, mechanical ventilation and intensive care unit stay durations (P <0.05). Syndromic patients were at higher risk of mortality compared to non-syndromic patients. Conclusion: MBTS and ductal stenting are both acceptable modalities as a palliative intervention for infants with duct-dependant pulmonary circulation. Syndromic patients are at higher risk of mortality. This can be considered an important factor for patient selection.

Evaluation of pulmonary blood flow in bilateral bidirectional Glenn shunts: value of 4-D flow cardiac magnetic resonance in the evaluation of pulmonary artery confluence stenosis.

Bilateral bidirectional Glenn shunts are associated with the risk of developing pulmonary artery bifurcation stenosis, resulting in variable pulmonary blood flow to either lung. This could negatively impact the subsequent stages of the single ventricle palliation pathway. This report highlights the value of 4D flow sequence from the cardiac magnetic resonance imaging in demonstrating the pulmonary blood flow characteristics following a bilateral bidirectional Glenn procedure. Mapping the blood flow pattern and its quantification to each lung provide objective insights into the possible predisposing factors for the development of pulmonary bifurcation stenosis.

Angiotensin-converting enzyme inhibition and pre-superior cavopulmonary connection haemodynamics in infants with single-ventricle physiology.

INTRODUCTION: Preliminary animal and human data suggest that angiotensin-converting enzyme inhibition has a role in pulmonary vascular remodelling. We sought to assess the effect of ACEi versus placebo on pulmonary artery pressure and transpulmonary gradient amongst infants undergoing single-ventricle palliation. MATERIALS AND METHODS: Using the publicly available Pediatric Heart Network Infant Single-Ventricle trial dataset, we compared mean PA pressure at pre-superior cavopulmonary connection catheterisation (primary outcome), transpulmonary gradient, pulmonary-to-systemic flow ratio, and post-SCPC oxygen saturation (secondary outcomes) in infants receiving enalapril versus placebo. RESULTS: A total of 179 infants underwent pre-SCPC catheterisation, of which 85 (47%) received enalapril. There was no difference between the enalapril and placebo group in the primary and the secondary outcomes. Mean PA pressure in the enalapril group was 13.1 ± 2.9 compared to 13.7 ± 3.4 mmHg in the placebo group. The transpulmonary gradient was 6.7 ± 2.5 versus 6.9 ± 3.2 mmHg in the enalapril and placebo groups, respectively. The pulmonary-to-systemic flow ratio was 1.1 ± 0.5 in the enalapril group versus 1.0 ± 0.5 in the placebo group and the post-SCPC saturation was 83.1 ± 5.0% in the enalapril group versus 82.2 ± 5.3% in the placebo group. In the pre-specified subgroup analyses comparing enalapril and placebo according to ventricular morphology and shunt type, there was no difference in the primary and secondary outcomes. CONCLUSION: ACEi did not impact mean pulmonary artery pressure or transpulmonary gradient amongst infants with single-ventricle physiology prior to SCPC palliation.

Hypertension masquerading as Pediatric Cardiomyopathy: an exercise in cognitive biases.

Heuristics and cognitive biases constantly influence clinical decision-making and often facilitate judgements under uncertainty. They can frequently, however, lead to diagnostic errors and adverse outcomes, particularly when considering rare disease processes that have common, masquerading presentations. Herein, we present two such cases of newborn infants with hypertensive renal disorders that were initially thought to have cardiomyopathy.

Initial experience with a novel ePTFE-covered balloon expandable stent in patients with near-atretic or severe aortic coarctation and small femoral arterial access.

OBJECTIVES: We report our experience of using the Bentley BeGraft Aortic stent in patients with severe or near-atretic aortic coarctation and small femoral arterial access. BACKGROUND: Use of covered stent is recommended in some settings such as aortic coarctation with associated aneurysm, Turner syndrome, and coarctation with aortic atresia. However, currently available covered stents need larger sheaths that may limit their use in children and patients with smaller arterial access. Newer stents may overcome this limitation. METHODS: Single-centre retrospective study of patients with severe or near-atretic aortic coarctation and small femoral arterial access. RESULTS: Between July and October, 2019, five patients (median age 15 years) with near-atretic or severe coarctation were treated with a Bentley BeGraft Aortic stent. Long sheaths between 9 and 11 Fr were used to implant stents, which were dilated up to 12-16 mm. None of the patients had residual coarctation (gradient >20 mm of mercury) after stenting. None of the patients developed acute vascular injuries or local access related complications at the end of the procedure or during follow-up (range 6-10 months). CONCLUSIONS: Bentley BeGraft aortic stents are important to consider in patients with severe coarctation and provided acute procedural success in patients with small femoral arterial access and widen the applicability in this patient population.

Protein-Losing Enteropathy Following Fontan Palliation.

Protein-losing enteropathy (PLE) is a chronic condition involving multiple organ systems that may develop any time following Fontan completion. The pathogenesis of PLE is complex and multifactorial. Chronic venous hypertension, low cardiac output, and abnormal lymphatics may all play a role in the pathogenesis of PLE. Common signs and symptoms include chronic diarrhea, abdominal pain, and ascites. Diagnosis is based on the presence of signs and symptoms in addition to hypoalbuminemia and elevated stool alpha 1 antitrypsin. Early identification and a comprehensive approach to evaluation and treatment are important, as they may affect survival. The initial evaluation should include cardiac catheterization for hemodynamic assessment. Although an evidence base for treatment is lacking, various medical, interventional, and surgical approaches have been described with variable degrees of success. Commonly used therapies include nutritional support, diuretics, subcutaneous unfractionated heparin, budesonide, and sildenafil. Limited data exist for Fontan conversion or takedown. Assessment for heart transplantation should be considered. PLE mortality is high-approximately 50%-but may be mitigated by aggressive investigation and management. The evolving understanding of the role of lymphatics in the pathophysiology of PLE and the emerging role of interventional lymphatic procedures may further improve outcomes in this patient population.

Hypotension and Brain Injury in Asphyxiated Newborns Treated with Hypothermia.

OBJECTIVE: This study aimed to assess the incidence of hypotension in asphyxiated newborns treated with hypothermia, the variability in treatments for hypotension, and the impact of hypotension on the pattern of brain injury. STUDY DESIGN: We conducted a retrospective cohort study of asphyxiated newborns treated with hypothermia. Mean blood pressures, lactate levels, and inotropic support medications were recorded during the hospitalization. Presence and severity of brain injury were scored using the brain magnetic resonance imaging (MRI) obtained after the hypothermia treatment was completed. RESULTS: One hundred and ninety term asphyxiated newborns were treated with hypothermia. Eighty-one percent developed hypotension. Fifty-five percent of the newborns in the hypotensive group developed brain injury compared with 35% of the newborns in the normotensive group (p = 0.04). Twenty-nine percent of the newborns in the hypotensive group developed severe brain injury, compared with only 15% in the normotensive group. Nineteen percent of the newborns presenting with volume- and/or catecholamine-resistant hypotension had near-total injury, compared with 6% in the normotensive group and 8% in the group responding to volume and/or catecholamines. CONCLUSION: Hypotension was common in asphyxiated newborns treated with hypothermia and was associated with an increased risk of (severe) brain injury in these newborns.

Secondary Increase of Lactate Levels in Asphyxiated Newborns during Hypothermia Treatment: Reflect of Suboptimal Hemodynamics (A Case Series and Review of the Literature).

Objective To evaluate whether a secondary increase of serum lactate levels in asphyxiated newborns during hypothermia treatment may reflect suboptimal dynamics. Methods-Retrospective case series and review of the literature. We present the clinical course of four asphyxiated newborns treated with hypothermia who presented with hypotension requiring inotropic support, and who displayed a secondary increase of serum lactate levels during hypothermia treatment. Serial serum lactate levels are correlated with blood pressure and inotropic support within the first 96 hours of life. Results Lactate levels initially decreased in the four patients. However, each of them started to present lower blood pressure, and lactate levels started to increase again. Inotropic support was started to raise blood pressure. The introduction of an epinephrine drip consistently worsened the increase of lactate levels in these newborns, whereas dopamine and dobutamine enabled the clearance of lactate in addition to raising the blood pressure. Rewarming was associated with hemodynamics perturbations (a decrease of blood pressure and/or an increase of lactate levels) in the three newborns who survived. Conclusions Lactate levels during the first 4 days of life should be followed as a potential marker for suboptimal hemodynamic status in term asphyxiated newborns treated with hypothermia, for whom the maintenance of homeostasis during hypothermia treatment is of utmost importance to alleviate brain injury.

Adolescents and adults with congenital heart diseases in oman.

OBJECTIVES: The aim of our study was to examine the spectrum, demographics, and mortality rate among adolescents and adults with congenital heart diseases (CHD) in Oman. METHODS: Data was collected retrospectively from the Royal Hospital, Muscat, electronic health records for all patients with a diagnosis of CHD aged 13 years and above. Data was analyzed according to the type of CHD and in-hospital mortality was assessed using Kaplan-Meier survival analysis. RESULTS: A total of 600 patients with CHD were identified, among them 145 (24%) were aged 18 years or below. The median age was 24 years. The majority of patients had a simple form of CHD. Atrial and ventricular septal defects together constituted 62.8% of congenital heart diseases. Most patients were clustered in Muscat (32%) and the Batinah regions (31.1%) of Oman. Patients with tetralogy of Fallot and Fontan had shorter survival time than recorded in the published literature. CONCLUSION: Mostly simple forms of CHD in younger patients was observed. The survival rate was significantly shortened in more complex lesions compared to simple lesions. A national data registry for CHD is needed to address the morbidities and mortality associated with the disease.

Coarctation of the aorta, known yet can be missed.

The clinical presentation of coarctation of aorta (CoA) is well known; however, it is the most common congenital heart disease in the newborn period to be missed, with significant mortality and morbidity associated with missing the diagnosis. We report a 20-day-old newborn boy who presented with congestive heart failure and weak femoral pulses. Chest X-ray (CXR) showed cardiomegaly and pulmonary edema and electrocardiography (ECG) showed extreme right axis deviation and absent left ventricular forces in the left precordial leads. Based on these, clinical suspicion of coarctation of aorta was made and confirmed by echocardiography. After initial stabilization with prostaglandin E2 infusion, child underwent urgent coarctation of aorta repair with uneventful post-operative course. High index of suspicion for coarctation of aorta in a newborn with such presentation is required and urgent referral to Pediatric Cardiology and cardiac surgery center is mandatory to reduce the morbidity and mortality associated with missing the diagnosis.

Pulmonary valve regurgitation following balloon valvuloplasty for pulmonary valve stenosis: Single center experience.

BACKGROUND: Pulmonary valve regurgitation following balloon valvuloplasty for moderate to severe pulmonary valve stenosis is a known late outcome of this procedure. OBJECTIVE: The aim of the study was to characterise the status of pulmonary regurgitation on follow up after pulmonary valve balloon dilatation (PVBD), and to study the determinant of the severity of PR. MATERIALS AND METHODS: We retrospectively reviewed 50 consecutive patients, aged 2 days to 18 years, with isolated pulmonary valve stenosis, who had undergone PVBD in 2004-2009 and were assessed with follow-up Doppler echocardiography. The impact of balloon to annulus ratio, age, and valve anatomy on the late development of moderate and severe pulmonary valve regurgitation following balloon valvuloplasty was analysed. RESULTS: Six patients (12%) had no pulmonary valve regurgitation; 32 (64%) had mild, 9 (18%) had moderate, and 3 (6%) had severe pulmonary valve regurgitation at a mean follow-up of 4 years. Balloon to annulus ratio, age, and valve anatomy were not statistically significant predictors for moderate and severe pulmonary valve regurgitation. CONCLUSIONS: The majority of patients in our population had mild pulmonary valve regurgitation. Moderate to severe pulmonary valve regurgitation was well tolerated at midterm follow-up. Age, balloon to annulus ratio, and valve anatomy were not statistically significant predictors for the late development of moderate and severe valve regurgitation. Large and longer follow-up studies are needed to address this question.