Development of a neonatal cardiac curriculum for neonatal-perinatal medicine fellowship training.

BACKGROUND: The topic of neonatal cardiovascular care in neonatal-perinatal medicine (NPM) fellowship training has continued to transform due to increased complexity of patients, development of specialized units, continued Accreditation Council for Graduate Medical Education requirements, and clinical practice variation across centers that care for neonates with congenital heart disease (CHD). METHODS: We developed a neonatal cardiac curriculum comprised of eight interactive sessions with novel active learning concepts specific to our NPM fellows. A self-assessment survey in comfort in managing infants with CHD and perceived competency in neonatal cardiology topics was performed by all neonatology fellows at baseline and after completion of the curriculum. The American Board of Pediatrics Subspecialty In-training Exam (SITE) scores for fellows were compared to that of the national average. RESULTS: The average comfort score (0-100) of the first-year fellows increased from 33 to 76, and that of the second and third-year fellows increased from 72 to 86, and 75 to 86, respectively. The first-year fellows improved their competency score by 44 points (3 standard deviations), the second-year fellows improved their score by about 26 points (one standard deviation), and there was an overall 9-point increase in the competency score of all fellows (one standard deviation). The average local SITE score was lower than the national average before the initiation of this curriculum, became nearly equal to the national average score at the end of the first year the curriculum was implemented, and has progressively become higher since then. CONCLUSION: Due to the variable clinical exposure and differing practice models of managing CHD a neonatal cardiac curriculum may be beneficial to NPM trainees.

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.

The ductus arteriosus in neonates with critical congenital heart disease.

The ductus arteriosus (DA) has a paradoxical biological role in neonates with congenital heart disease (CHD) and can present with significant management challenges. Critical congenital cardiac lesions rely on the patency of the DA to provide either systemic or pulmonary blood flow. A patent DA (PDA) that remains open can also have adverse consequences depending on the degree of systemic to-pulmonary shunting and volume of ductal steal. As such, the presence of a PDA may pose a challenge in the medical management and timing of surgical repair. In this perspective article, we provide an understanding of the role of the DA in the circulatory system in neonates with CHD and discuss traditional and emerging approaches to support the pulmonary and systemic circulations with manipulation of the DA.

The role of furosemide and fluid management for a hemodynamically significant patent ductus arteriosus in premature infants.

A patent ductus arteriosus (PDA) in infants born premature can present significant management challenges for neonatal providers. Quantifying a hemodynamically significant PDA (hsPDA) represents the first hurdle, however, identifying the best evidence-based approach amongst conservative, pharmacologic, and/or interventional management options has proven to be even more complicated. Within the conservative arm, furosemide to reduce pulmonary edema and improve lung function has spawned several discussions given the concerns for its upregulation of prostaglandin E2 in the kidneys and conflicting outcomes data. There remains no consensus regarding furosemide use in hsPDAs. In this perspective article, we summarize the approach to defining a hsPDA, review the current practice of furosemide use in the management of hsPDA, and suggest an approach to fluid management and diuresis to address the question: is the routine use of furosemide in hsPDA merited in current practice?

The association between pulmonary vascular disease and respiratory improvement in infants with type I severe bronchopulmonary dysplasia.

OBJECTIVE: To describe the association between echocardiographic measures of pulmonary vascular disease and time to respiratory improvement among infants with Type I severe bronchopulmonary dysplasia (sBPD). STUDY DESIGN: We measured the pulmonary artery acceleration time indexed to the right ventricular ejection time (PAAT/RVET) and right ventricular free wall longitudinal strain (RVFWLS) at 34-41 weeks' postmenstrual age. Cox-proportional hazards models were used to estimate the relationship between the PAAT/RVET, RVFWLS, and the outcome: days from 36 weeks' postmenstrual age to room-air or discharge with oxygen (≤0.5 L/min). RESULT: For 102 infants, the mean PAAT/RVET and RVFWLS were 0.27 ± 0.06 and -22.63 ± 4.23%. An abnormal measurement was associated with an increased time to achieve the outcome (PAAT/RVET: 51v24, p < 0.0001; RVFWLS; 62v38, p = 0.0006). A normal PAAT/RVET was independently associated with a shorter time to outcome (aHR = 2.04, 1.11-3.76, p = 0.02). CONCLUSION: The PAAT/RVET may aid in anticipating timing of discharge in patients with type I severe BPD.

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.

Acute Cardiac Care for Neonatal Heart Disease.

This manuscript is one component of a larger series of articles produced by the Neonatal Cardiac Care Collaborative that are published in this supplement of Pediatrics. In this review article, we summarize the contemporary physiologic principles, evaluation, and management of acute care issues for neonates with complex congenital heart disease. A multidisciplinary team of authors was created by the Collaborative's Executive Committee. The authors developed a detailed outline of the manuscript, and small teams of authors were assigned to draft specific sections. The authors reviewed the literature, with a focus on original manuscripts published in the last decade, and drafted preliminary content and recommendations. All authors subsequently reviewed and edited the entire manuscript until a consensus was achieved. Topics addressed include cardiopulmonary interactions, the pathophysiology of and strategies to minimize the development of ventilator-induced low cardiac output syndrome, common postoperative physiologies, perioperative bleeding and coagulation, and common postoperative complications.

Comprehensive Evaluation of Right Heart Performance and Pulmonary Hemodynamics in Neonatal Pulmonary Hypertension : Evaluation of cardiopulmonary performance in neonatal pulmonary hypertension.

PURPOSE OF REVIEW: Pulmonary hypertension is characterized by an elevation of pulmonary artery pressures and prolonged exposure of the right ventricle to high afterload that collectively contribute to morbidity and mortality in both the term and preterm infants. This review summarizes the pathogenesis, etiologies, and hemodynamic profiles of the conditions that result in pulmonary hypertension in neonates. We explore the application of echocardiographic techniques for the assessment of right ventricular performance and pulmonary hemodynamics that enhance and guide the diagnosis and management strategies in neonates. RECENT FINDINGS: Clinical assessments based on the determinants of mean pulmonary artery pressures (pulmonary vascular resistance, pulmonary blood flow, and pulmonary capillary wedge pressure) provide a physiologic approach in determining the acute and chronic etiologies of pulmonary hypertension in neonates. In addition, advances in neonatal echocardiography now afford the capability to obtain quantitative information that often precedes the qualitative information acquired by conventional methods and also provide sensitive markers of right ventricle performance for prognostic information based on the determinants of mean pulmonary artery pressures. Neonatal pulmonary hypertension represents a physiologic spectrum that accounts for the variance in clinical presentation and response to therapies. Physiology-based approaches to etiological identification, coupled with the emerging echocardiographic methods for the assessment of pulmonary hypertension in neonates will likely help to identify cardiovascular compromise earlier, guide therapeutic intervention, monitor therapeutic effectiveness, and improve overall outcome.

Spectrum of breast disease encountered in HIV-positive patients at a community teaching hospital.

INTRODUCTION: HIV infection directly and indirectly affects breast tissue. This study describes the spectrum of HIV-related breast disease encountered at a community teaching hospital. METHODS: A 9 year retrospective review was performed of HIV-positive patients with a breast-related diagnosis seen at our institution. Patient demographics, HIV status, comorbid disease, medications, clinical findings, diagnostic procedure, pathology, treatment and outcome were recorded. RESULTS: A total of 46 individuals were included with a median age of 47 years (range 24-64 years) and male:female ratio of 1:3 (12 men and 34 women). Mean duration of HIV infection was 7 years during which time 46% of patients had an AIDS defining illness. Median CD4 cell count was 437 cells/mm(3) (range 2 to ≥500 cells/mm(3)) at the time of the breast diagnosis. Breast disease identified included benign conditions (59% total: 92% for men, 47% for women), infection (17% total: 8% for men, 21% for women), cancer (22% total: 0% for men, 29% for women), and atypia (2% total: 0% for men, 3% for women). Patients with a breast infection had a lower median CD4 cell count than those with breast cancer or benign conditions. Gynecomastia was detected in seven out of 12 (58%) men. In these men, antiretroviral therapy (ART) of all drug classes was associated with gynecomastia. Breast cancer occurred only in women and included patients with invasive ductal carcinoma (n = 7), ductal carcinoma in situ (n = 2), and liposarcoma diagnosed in one individual. Specific risk factors for breast cancer in this setting were not identified. Five (11%) patients died, only one from breast disease during the study period. CONCLUSION: These data indicate that increased longevity in patients with chronic HIV infection may be associated with the occurrence of breast conditions in both men and women. A broad spectrum of breast disease should be anticipated in HIV-infected persons living longer with effective ART.