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.

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.

Training pathways and careers for neonatologists interested in cardiovascular care.

Neonatologists and neonatal-perinatal trainees continue to be invested in the cardiovascular care of the newborn, many focusing their careers in this area of expertise. Multiple formalized structured and non-structured training pathways have evolved for neonatologists caring for infants with congenital heart disease and other cardiovascular pathologies. Furthermore, the evolution of neonatal hemodynamic science over the past decade has also spawned a formal training pathway in hemodynamics consultation to enhance standard of care and guide the management of infants at risk for cardiovascular compromise. Neonatologists have also chosen to expand upon on their neonatology training with clinical and research exposure to enhance their roles in neonatal cardiovascular care, including fetal care consultation, delivery room management, and perioperative cardiac intensive care consultation. To provide insight and career guidance to interested neonatal trainees and early career physicians, this perspective article highlights several different pathways in the care of neonates with cardiovascular disease.

Normative Echocardiographic Values for Right and Left Ventricular Function in Extremely Premature Neonates.

OBJECTIVE: To identify the measures of biventricular function and surrogates of pulmonary vascular resistance that can be expected in relatively stable extremely premature neonates, and evaluate maturational changes in myocardial performance in this patient population. STUDY DESIGN: This was a prospective observational study. Clinically stable extremely preterm newborns were divided into 3 cohorts based on gestational age: cohort 1 (240/7-256/7 weeks), cohort 2 (260/7-276/7 weeks), and cohort 3 (280/7-296/7 weeks). Serial echocardiograms were obtained on day of life 3-5, 7-10, and just prior to discharge. RESULTS: In total, 46 subjects met the criteria aimed at capturing only the most clinically healthy and stable newborns less than 296/7 weeks of gestational age. Myocardial performance was reliably assessed by echocardiography with high inter-reader correlation. Normative values were identified for right ventricular function, left ventricular function, and surrogates of pulmonary vascular resistance. CONCLUSIONS: Biventricular systolic performance is significantly different in the clinically stable extremely premature neonate when compared with healthy full-term newborns. All participants had evidence of poor biventricular compliance at birth which improved with maturation. Extreme prematurity does not appear to adversely affect myocardial functional maturation at the time of term corrected age and/or discharge.

Management of Congenital Diaphragmatic Hernia Treated With Extracorporeal Life Support: Interim Guidelines Consensus Statement From the Extracorporeal Life Support Organization.

The management of infants with congenital diaphragmatic hernia (CDH) receiving extracorporeal life support (ECLS) is complex. Significant variability in both practice and prevalence of ECLS use exists among centers, given the lack of evidence to guide management decisions. The purpose of this report is to review existing evidence and develop management recommendations for CDH patients treated with ECLS. This article was developed by the Extracorporeal Life Support Organization CDH interest group in cooperation with members of the CDH Study Group and the Children's Hospitals Neonatal Consortium.

Left ventricular cardiac output is a reliable predictor of extracorporeal life support in neonates with congenital diaphragmatic hernia.

OBJECTIVE: Congenital diaphragmatic hernia (CDH) is a treatable condition but despite optimal management, some patients require extracorporeal life support (ECLS). This study evaluates the association of left ventricular (LV) cardiac output and the need for ECLS. STUDY DESIGN: Single center, retrospective chart review from 1/1/2012 through 7/1/2018. Twenty-seven patients met criteria for evaluation with six patients (22%) requiring ECLS. Echocardiography was used to assess ventricular function. RESULTS: There was a statistically significant decrease in LV cardiac index of 1.33 L /min/m2 vs 1.80 L /min/m2 (p = 0.04) for patients requiring ECLS and non-ECLS, respectively. There was no difference between the 2 groups with regards to vasoactive medication use, surfactant use, iNO use, severity of pulmonary hypertension, or right ventricular (RV) function. CONCLUSION: In newborns with CDH, decreased LV cardiac output is more strongly associated with need for ECLS than severity of pulmonary hypertension or RV dysfunction.

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.

Effect of pump type on outcomes in neonates with congenital diaphragmatic hernia requiring ECMO.

PURPOSE: With the exception of neonatal respiratory failure, most centers are now using centrifugal over roller-type pumps for the delivery of extracorporeal membrane oxygenation (ECMO). Evidence supporting the use of centrifugal pumps specifically in infants with congenital diaphragmatic hernia (CDH) remains lacking. We hypothesized that the use of centrifugal pumps in infants with CDH would not affect mortality or rates of severe neurologic injury (SNI). METHODS: Infants with CDH were identified within the ELSO registry (2000-2016). Patients were then divided into those undergoing ECMO with rollertype pumps or centrifugal pumps. Patients were matched based on propensity score (PS) for the ECMO pump type based on pre-ECMO covariates. This was done for all infants and separately for each ECMO mode, venovenous (VV) and venoarterial (VA) ECMO. RESULTS: We identified 4,367 infants who were treated with either roller or centrifugal pumps from 2000-2016. There was no difference in mortality or SNI between the two pump types in any of the groups (all infants, VA-ECMO infants, VV-ECMO infants). However, there was at least a six-fold increase in the odds of hemolysis for centrifugal pumps in all groups: all infants (odds ratio [OR] 6.99, p<0.001), VA-ECMO infants (OR 8.11, p<0.001 and VV-ECMO infants (OR 9.66, p<0.001). CONCLUSION: For neonates with CDH requiring ECMO, there is no survival advantage or difference in severe neurologic injury between those receiving roller or centrifugal pump ECMO. However, there is a significant increase in red blood cell hemolysis associated with centrifugal ECMO support.

Kawasaki disease: four case reports of cardiopathy with an institutional and literature review.

Kawasaki disease (KD) is a systemic vasculitis condition with a relatively unknown etiology. First described in 1967 by Tomisaku Kawasaki in Japan, KD has come to be widely diagnosed in every region of the world. The disease has a high prevalence in children ages 6 months to 5 years, particularly in those of Japanese descent. Patients often present with a high fever, rash, lymphadenopathy, and conjunctival injections, but there is no diagnostic test for KD. This paper presents data from our Kawasaki registry including 99 patients with emphasis on Kawasaki cardiopathy. Three patients died from complications of KD, and 1 patient underwent heart transplant for massive aneurysmal dilatation. The 4 explanted hearts showed a spectrum of pathological findings (acute thrombosis, vasculitis, and myocarditis), and 1 patient showed the long-term sequelae of vasculitis in the form of massive aneurysmal dilatation. Among the survivors, 30% showed aneurysmal dilatation. This paper reviews the most recent information regarding Kawasaki cardiopathy and underlying molecular mechanisms.