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Critical congenital heart disease: What should the neonatologist know?

Introduction The incidence of congenital heart disease (CHD) is estimated at approximately 8 per 1000 live births, with up to 25–30% of CHD deemed critical congenital heart disease (CCHD). CCHD includes lesions that necessitate early interventions to avoid significant morbidity and mortality and require multidisciplinary subspecialty care from pediatric cardiologists, cardiothoracic surgeons, neonatologists, anesthesiologists, and pediatric cardiac intensivists. Detection of CCHD by prenatal ultrasound (fetal anomaly…

Hemodynamic management in special circumstances

Key points 1. Neonates with hypertrophic cardiomyopathy due to diabetes who have cardiovascular instability benefit from a strategy that avoids positive inotropy and high peak end-expiratory pressure and prioritizes left heart volume loading and increased left ventricular afterload. 2. Neonates with pre-ductal arteriovenous malformations may have flow-mediated pulmonary hypertension and pseudocoarctation physiology if left-to-right ductal shunt (either due to natural decline in systemic vascular resistance or…

Hemodynamic management in resource-challenged countries

Key points Low- and middle-income countries contribute to a major portion of the global neonatal disease burden, accounting for >90% of neonatal mortality. Perinatal asphyxia, gram-negative sepsis, and prematurity are major contributors to the neonatal disease burden in LMIC countries. Understanding the pathophysiology of the underlying disease process is crucial to appropriate hemodynamic management. Hemodynamic management should include an integrated evaluation of the clinical/laboratory parameters, with…

Pathophysiologically based management of pulmonary hypertension of the newborn

Key points Acute pulmonary hypertension (aPH), classically referred to as persistent pulmonary hypertension of the newborn (PPHN) when occurring during the perinatal transition period, is characterized by failure of the normal postnatal decline in pulmonary vascular resistance (PVR). Elevated pulmonary arterial pressure (PAP) may also occur via alternative mechanisms, including left ventricular diastolic heart failure and excessive pulmonary blood flow. Hypoxemia, with or without accompanying systemic…

Chronic pulmonary hypertension

Key points Chronic pulmonary hypertension (cPH), a severe form of pulmonary vascular disease, is characterized by a sustained and progressive elevation of pulmonary vascular resistance, pulmonary artery pressures, and resultant exposure of the right ventricle to high afterload. cPH most commonly affects extreme preterm infants with bronchopulmonary dysplasia (BPD) but has also been identified in preterm infants without overt lung disease. Comprehensive assessment should aim to…

Pathophysiology and assessment of acute pulmonary hypertension of the newborn

Introduction Acute pulmonary hypertension (aPH) in the neonatal period is common in sick newborn infants being treated in the neonatal intensive care unit (NICU). The causes and underlying pathophysiology are multifactorial. Acute pulmonary hypertension in the newborn is secondary to impaired or delayed relaxation of the pulmonary vasculature associated with a diverse group of cardiopulmonary pathologies such as meconium aspiration syndrome (MAS), congenital diaphragmatic hernia (CDH),…

Hemodynamically based management of circulatory compromise in the newborn

Key points There is little outcome-based evidence to guide circulatory support in the newborn. In clinical situations of high risk for circulatory compromise, there are a range of hemodynamics. A “one size fits all” approach to neonatal circulatory support is neither logical nor likely to be successful. It is logical to define the individual hemodynamic in a baby and apply therapy on the basis of those…

Glucocorticoids and adrenal function in neonates with hypotension

Key points 1. Corticosteroids are increasingly used in the acutely ill newborn population to increase blood pressure and reduce inotrope exposure. 2. There are clinical and disease states in infants in which glucocorticoids have been studied and found to have potential benefit. Populations who may respond to corticosteroids include ill term and preterm infants with hypotension and infants with pulmonary hypertension, meconium aspiration syndrome, hypoxic-ischemic encephalopathy,…

Hemodynamics of the neonate following perinatal hypoxic-ischemia and the effects of therapeutic hypothermia

Introduction Globally, neonatal encephalopathy precipitated by perinatal hypoxia-ischemia remains a common cause of brain injury. The incidence varies from 1 to 3 per 1000 to up to 25 per 1000 in developed and developing countries, respectively. Therapeutic hypothermia has been demonstrated to improve both survival and neurological morbidity ; however, there remains a significant burden of mortality and long-term neurological sequelae among survivors. Birth asphyxia, defined…

Assessment and management of septic shock and hypovolemia

Key points Sepsis can progress rapidly from mild clinical signs to full-blown septic shock with high morbidity and mortality. The clinical recognition of the onset of sepsis and the onset of neonatal septic shock can be challenging. In conjunction with the use of comprehensive cardiorespiratory monitoring, early and serial assessment with ultrasound aids in the recognition of the type of septic shock and in the following…

Cardiovascular compromise in the preterm infant during the first postnatal day

Key points The very-low-birth-weight infant is hemodynamically vulnerable due to a unique set of risk factors, which include an immature myocardium with poor response to volume load and afterload, immature autonomic vasoregulation, and thus ineffective cardiovascular compensatory mechanisms, a propensity to develop specific or non-specific inflammatory responses, the imposition of positive airway pressure, systemic-to-pulmonary shunts, and variability in the degree of placental restoration of blood volume…

Pathophysiology-based management of the hemodynamically significant patent ductus arteriosus in the very preterm neonate

Key points The simplistic “treat all or treat none” approach to management of a patent ductus arteriosus (PDA) has been increasingly challenged in recent years as the variation in clinical and hemodynamic presentation has been realized. Spontaneous closure rates, poor efficacy of medical treatment, and a combination of selection bias, high open-label treatment rates, and inappropriate outcome measures in clinical trials are among the major factors…

Interventional management of the patent ductus arteriosus

Key points Percutaneous (or transcatheter) PDA closure is an emerging technique that has gained popularity in the most recent years and is being performed in progressively smaller and younger patients. Advancements in the technique, the launch of devices that are more appropriate for the preterm ductal morphology, and increased experience have expanded the use of percutaneous closure with high rates of technical success and few major…

Pharmacological management of patent ductus arteriosus in the very preterm neonate

Key points The pharmacological management of PDA in the very preterm neonate remains a controversial topic, as the risks and benefits of the pharmacotherapeutic options remain unclear. NSAIDs (indomethacin and ibuprofen) and acetaminophen are the most common and effective pharmacological agents used for PDA closure. No major differences in the efficacy between the three agents have been established in comparative effectiveness trials. However, choice of therapy…

Diagnosis, evaluation, and monitoring of patent ductus arteriosus in the very preterm infant

Key points Although presence of a patent ductus arteriosus (PDA) can easily be confirmed with echocardiography, diagnosis of a hemodynamically significant PDA is more challenging and not standardized. Evaluation of hemodynamic and clinical significance of a PDA should include assessment of the size of the PDA, magnitude of shunt volume, the ability of the heart to accommodate and compensate for the shunt, and the impact of…

Clinical applications of near-infrared spectroscopy in neonates

Key points The status of cerebral oxygenation is not always represented appropriately by systemic arterial oxygenation, especially when there are changes in cerebral blood flow or cardiac output. Oxygenation monitoring of the brain by near-infrared spectroscopy (NIRS) is therefore an important additive measure in neonatal intensive care. Monitoring cerebral oxygenation by NIRS, in addition to arterial saturation monitoring by pulse oximetry, blood pressure, and brain function…

Comprehensive, real-time hemodynamic monitoring and data acquisition: An essential component of the development of individualized neonatal intensive care

Key points Accurate assessment of the hemodynamic status in critically ill neonates requires blood pressure measurements to be interpreted in the context of indirect (clinical signs) and direct (measurements and assessments) indicators of systemic circulation (cardiac output) and regional organ blood flow. Further judiciary validation of emerging technological approaches to evaluate systemic circulation and regional blood flow in a continuous and noninvasive manner is necessary. Comprehensive…

Cardiac magnetic resonance imaging in the assessment of systemic and organ blood flow and the function of the developing heart

Key points Cardiac magnetic resonance (CMR) Imaging techniques provide noninvasive assessments of the newborn circulation with high accuracy and repeatability. Cine CMR produces three-dimensional assessments of chamber volumes and myocardial mass. Phase contrast CMR can quantify blood flow in any major vessel. CMR techniques have provided normative ranges for neonatal left and right ventricular development and quantification of PDA shunt volume and have guided optimization of…

Assessment of cardiac output in neonates: Techniques using the fick principle, indicator dilution technology, doppler ultrasound, electrical biosensing technology, and arterial pulse contour analysis

Key points Cardiac output monitoring in preterm and term neonates is feasible but remains challenging despite the availability of different technologies. The best systems to monitor cardiac output in the clinical setting in neonatal intensive care at present are transthoracic echocardiography, transpulmonary indicator dilution, electrical biosensing technology, and arterial pulse contour analysis. Noninvasive cardiac output monitoring is inversely related to accuracy; hence there will always be…

Advanced cardiac imaging in the newborn: Tissue doppler imaging and speckle tracking echocardiography

Key points Tissue Doppler imaging (TDI) is a modality that employs the Doppler effect to assess muscle wall characteristics throughout the cardiac cycles including velocity, displacement, deformation, and event timings. Two-dimensional speckle tracking echocardiography (2DSTE) is a non-Doppler technique that applies computer software analysis of images generated by conventional ultrasound techniques to assess parameters of myocardial motion (displacement, velocity) and deformation (strain, strain rate) in all…