Assessment and management of neonates with unrepaired congenital heart disease.

PURPOSE OF REVIEW: To review preoperative assessment and management of neonates with congenital heart disease (CHD). RECENT FINDINGS: The spectrum for neonates with CHD can be wide and complex. An in-depth understanding of their physiology is the first step in assessing their hemodynamics and developing an effective therapeutic strategy. SUMMARY: There is significant heterogeneity in the anatomy and physiology in newborns with CHD. Their complex pathophysiology can be simplified into seven basic subtypes, which include systolic dysfunction, diastolic dysfunction, excessive pulmonary blood flow, obstructed pulmonary blood flow, obstructed systemic blood flow, transposition physiology, and single ventricle physiology. It is important to note these physiologies are not mutually exclusive, and this review summarizes the hemodynamic and therapeutic strategies available for the preoperative neonate with CHD.

Status of Multidisciplinary Collaboration in Neonatal Cardiac Care in the United States.

While outcomes for neonates with congenital heart disease have improved, it is apparent that substantial variability exists among centers with regard to the multidisciplinary approach to care for this medically fragile patient population. We endeavored to understand the landscape of neonatal cardiac care in the United States. A survey was distributed to physicians who provide neonatal cardiac care in the United States regarding (1) collaborative efforts in care of neonates with congenital heart disease (CHD); (2) access to neonatal cardiac training; and (3) barriers to the implementation of protocols for neonatal cardiac care. Responses were collected from 10/2018 to 6/2019. We received responses from 172 of 608 physicians (28% response rate) from 89 centers. When compared to responses received from physicians at low-volume centers (< 300 annual bypass cases), those at high-volume centers reported more involvement from the neurodevelopmental teams (58% vs. 29%; P = 0.012) and a standardized transition to outpatient care (68% vs. 52%; P = 0.038). While a majority of cardiothoracic surgery and anesthesiology respondents reported multidisciplinary involvement, less than half of cardiology and neonatology supported this statement. The most commonly reported obstacles to multidisciplinary engagement were culture (61.6%) and logistics (47.1%). Having a standardized neonatal cardiac curriculum for neonatal fellows was positively associated with the perception that multidisciplinary collaboration was "always" in place (53% vs. 40%; P = 0.09). There is considerable variation among centers in regard to personnel involved in neonatal cardiac care, related education, and perceived multidisciplinary collaboration among team members. The survey findings suggest the need to establish concrete standards for neonatal cardiac surgical programs, with ongoing quality improvement processes.

Adaptations of paediatric cardiology practice during the COVID-19 pandemic.

During the initial surge of the COVID-19 pandemic in the spring and summer of 2020, paediatric heart centres were forced to rapidly alter the way patient care was provided to minimise interruption to patient care as well as exposure to the virus. In this survey-based descriptive study, we characterise changes that occurred within paediatric cardiology practices across the United States and described provider experience and attitudes towards these changes during the pandemic. Common changes that were implemented included decreased numbers of procedures, limiting visitors and shifting towards telemedicine encounters. The information obtained from this survey may be useful in guiding and standardising responses to future public health crises.

Management strategies for the preemie ductus.

PURPOSE OF REVIEW: Patent ductus arteriosus (PDA) remains the most common cardiovascular condition afflicting neonates. Despite 5 decades of scientific inquiry pediatric cardiologists and neonatologists still cannot answer the simple question of which PDAs should be treated. RECENT FINDINGS: Although the volume of the shunt is difficult to calculate, echocardiography, biochemical markers, and clinical exam can provide clues to the magnitude and physiologic consequences of the shunt. Epidemiologic data exists showing a positive relationship between a PDA and numerous morbidities. As a result, for most of the 20th and early 21st century, nearly all PDAs where indiscriminately considered to be hemodynamically significant and attempts to close it where initiated shortly after birth. However, no randomized trials of PDA closure have been able to show significant differences between affected and unaffected groups. In fact, surgical ligation has repeatedly been associated with increased morbidities and worse long-term neurodevelopmental outcomes. As a result, most clinicians favor a strategy of watchful waiting. SUMMARY: In this review, we aim to summarize the scientific literature, along with some of the contemporary biases, that exist with regards to the pathophysiology, genetics, and treatment strategies for the neonatal PDA.

Addressing Disparities in Pediatric Congenital Heart Disease: A Call for Equitable Health Care.

While significant progress has been made in reducing disparities within the US health care system, notable gaps remain. This article explores existing disparities within pediatric congenital heart disease care. Congenital heart disease, the most common birth defect and a leading cause of infant death, has garnered substantial attention, revealing certain disparities within the US health care system. Factors such as race, ethnicity, insurance coverage, socioeconomic status, and geographic location are all commonalities that significantly affect health disparities in pediatric congenital heart disease. This comprehensive review sheds light on disparities from diverse perspectives in pediatric care, demonstrates the inequities and inequalities leading to these disparities, presents effective solutions, and issues a call to action for providers, institutions, and the health care system. Recognizing and addressing these disparities is imperative for ensuring equitable care and enhancing the long-term well-being of children affected by congenital heart disease. Implementing robust, evidence-based frameworks that promote responsible and safe interventions is fundamental to enduring change.

Perioperative Care Models for Neonates With Congenital Heart Disease: Evolving Role of Neonatology Within the Cardiac Intensive Care Unit.

A multidisciplinary team is needed to optimally care for infants with congenital heart disease (CHD). Different compositions of teams trained in cardiology, critical care, cardiothoracic surgery, anesthesia, and neonatology have been identified as being primarily responsible for perioperative care of this high-risk population in dedicated cardiac intensive care units (CICUs). Although the specific role of cardiac intensivists has become more well defined over the past two decades, the responsibilities of neonatologists remain highly variable in the CICU with neonatologists providing care along with a unique spectrum of primary, shared, or consultative care. The neonatologist can function as the primary physician and assume all or share responsibility with the cardiac intensivists for the management of infants with CHD. A neonatologist can provide care as a secondary consultant physician in a supportive role for the primary CICU team. Additionally, neonates with CHD can be mixed with older children in a CICU, cohorted in a dedicated space within the CICU or placed in a stand-alone infant CICU without older children. Although variations exist between centers on which model of care is deployed and the location within a CICU, characterization of current practice patterns represents the initial step required to determine optimal best practices to improve the quality of care for neonates with cardiac disease. In this manuscript, we present four models utilized in the United States in which the neonatologist provides neonatal-cardiac-focused care in a dedicated CICU. We also outline the different permutations of location where neonates can be cared for in dedicated pediatric/infant CICUs.

Optimizing Neurodevelopmental Outcomes in Neonates With Congenital Heart Disease.

Neurodevelopmental impairment is a common and important long-term morbidity among infants with congenital heart disease (CHD). More than half of those with complex CHD will demonstrate some form of neurodevelopmental, neurocognitive, and/or psychosocial dysfunction requiring specialized care and impacting long-term quality of life. Preventing brain injury and treating long-term neurologic sequelae in this high-risk clinical population is imperative for improving neurodevelopmental and psychosocial outcomes. Thus, cardiac neurodevelopmental care is now at the forefront of clinical and research efforts. Initial research primarily focused on neurocritical care and operative strategies to mitigate brain injury. As the field has evolved, investigations have shifted to understanding the prenatal, genetic, and environmental contributions to impaired neurodevelopment. This article summarizes the recent literature detailing the brain abnormalities affecting neurodevelopment in children with CHD, the impact of genetics on neurodevelopmental outcomes, and the best practices for neonatal neurocritical care, focusing on developmental care and parental support as new areas of importance. A framework is also provided for the infrastructure and resources needed to support CHD families across the continuum of care settings.

Preoperative Management of Neonates With Congenital Heart Disease.

Clinicians caring for neonates with congenital heart disease encounter challenges in clinical care as these infants await surgery or are evaluated for further potential interventions. The newborn with heart disease can present with significant pathophysiologic heterogeneity and therefore requires a personalized therapeutic management plan. However, this complex field of neonatal-cardiac hemodynamics can be simplified. We explore some of these clinical quandaries and include specific sections reviewing the anatomic challenges in these patients. We propose this to serve as a primer focusing on the hemodynamics and therapeutic strategies for the preoperative neonate with systolic dysfunction, diastolic dysfunction, excessive pulmonary blood flow, obstructed pulmonary blood flow, obstructed systemic blood flow, transposition physiology, and single ventricle physiology.

Improved survival after variceal bleeding in patients with cirrhosis over the past two decades.

Over the past two decades, new treatment modalities have been introduced for the management of variceal bleeding. The aim of this retrospective study in a single center was to assess whether these treatments have improved the prognosis for cirrhotic patients with variceal bleeding. We reviewed the clinical records of all patients with cirrhosis admitted to our Liver Intensive Care Unit due to variceal bleeding during the years 1980, 1985, 1990, 1995, and 2000. Whereas balloon tamponade was still the first-line treatment in 1980, patients treated in 2000 received a vasoactive agent, an endoscopic treatment, and an antibiotic prophylaxis in, respectively, 90%, 100%, and 94% of cases. The in-hospital mortality rate steadily decreased over the study period: 42.6%, 29.9%, 25%, 16.2%, and 14.5% in 1980, 1985, 1990, 1995, and 2000, respectively (P < .05). Mortality decreased from 9% in 1980 to 0% in 2000 in Child-Turcotte-Pugh class A patients, from 46% to 0% in class B patients, and from 70% to 32% in class C patients. This improved survival was associated with a decrease of rebleeding (from 47% in 1980 to 13% in 2000) and bacterial infection rates (from 38% to 14%). On multivariable analysis, endoscopic therapy and antibiotic prophylaxis were independent predictors of survival. In conclusion, in-hospital mortality of patients with cirrhosis and variceal bleeding decreased threefold over the past two decades, in concurrence with an early and combined use of pharmacological and endoscopic therapies and short-term antibiotic prophylaxis.

Clinicopathological forms and prognostic index in Budd-Chiari syndrome.

BACKGROUND: A recent study in patients with Budd-Chiari syndrome showed the value of a prognostic index including age, Pugh score, ascites and serum creatinine. Surgical portosystemic shunt did not appear to improve survival. AIMS: To validate these findings in an independent sample; to evaluate a classification into three forms according to the presence of features of acute injury, chronic lesions, or both of them (types I, II or III, respectively); and to assess whether taking into account this classification would alter our previous conclusions. METHODS: Multivariate Cox model survival analysis, first on 69 new patients; second, on these 69 and 54 previous patients, all diagnosed since 1985. RESULTS: Previous prognostic index had a significant prognostic value (P<0.0001) which was further improved by taking into account type III form (P<0.001). Type III form was associated with the poorest outcome. No significant impact of surgical shunting on survival was disclosed. CONCLUSIONS: The prognosis of Budd-Chiari syndrome can be based on age, Pugh score, ascites, serum creatinine and the presence of features indicating acute injury superimposed on chronic lesions (type III form). The idea that surgical shunting has no significant impact on survival is reinforced by these findings.