DIAGNOSIS AND MANAGEMENT OF THE NEWBORN WITH SUSPECTED CONGENITAL HEART DISEASE
Section snippets
DELIVERY ROOM RESUSCITATION FOR THE NEONATE WITH KNOWN CONGENITAL HEART DISEASE
The opportunity for optimal care of the newborn with critical congenital heart disease begins before birth. More and more infants with heart disease are born with a diagnosis of probable congenital heart disease because of the increasing use of obstetric ultrasound and fetal echocardiography. This trend is likely to continue as the skills of those performing obstetric ultrasound improve and there is a further dissemination of fetal echocardiography. As a result, many neonates will be born with
Clinical Presentations of Congenital Heart Disease in the Neonate
The timing of presentation and the accompanying symptomatology depend on the nature and severity of the anatomic defect; the in utero effects (if any) of the structural lesion; and following birth, the alterations in cardiovascular physiology secondary to the effects of the closure of the ductus arteriosus and the fall in PVR.
In the first few weeks of life, the many heterogeneous forms of heart disease present in a surprisingly limited number of ways. Signs and symptoms include cyanosis,
Echocardiography
The two-dimensional echocardiogram, supplemented with pulse wave Doppler and color Doppler, has become the primary diagnostic modality for anatomic definition in pediatric cardiology. Echocardiography quickly provides information about the structure and function of the heart and great vessels. The echocardiogram in the neonate is not noninvasive; a complete echocardiogram on a newborn suspected of having congenital heart disease may take an hour or more to perform, and may not be well tolerated
Genetics
A careful search for other congenital anomalies is essential, because congenital heart disease is accompanied by at least one extracardiac malformation 25% of the time.31 The spectrum of associated defects in children with congenital heart disease is broad and likely reflects the complexity of the development process during cardiogenesis.
Congenital heart disease and extracardiac malformations occur with known chromosomal abnormalities, single-gene defects, and syndromes and associations of
TIMING AND TYPE OF SURGERY
For the neonate with congenital heart disease who requires surgical intervention, the medical and surgical teams responsible must decide on the timing and the type of surgery. In the neonate who has sustained end-organ damage secondary to hypoxemia-ischemia, medical management should continue, if possible, until there is evidence of recovery of secondary organ function, to minimize surgical morbidity and risk. In addition, the presence of sepsis should be evaluated for and ruled out. Delaying
LESION-SPECIFIC MANAGEMENT
This section outlines the lesion-specific preoperative management for critical congenital heart malformations that are likely to present in the neonatal period. Defects discussed include lesions with ductal-dependent systemic blood flow, lesions with ductal-dependent PBF, TGA, total anomalous pulmonary venous connection with obstruction, and certain varieties of single ventricle.
SUMMARY
In critical congenital heart lesions, the ultimate outcome depends on timely and accurate diagnosis of the structural anomaly, the evaluation and resuscitation of secondary organ damage, effective lesion-specific preoperative management, and the appropriate timing and type of surgery. Crucial to this process is continuous communication among medical, surgical, and nursing disciplines.
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2018, Clinical Pediatric Emergency MedicineCitation Excerpt :When trying to distinguish pulmonary from cardiac causes of hypoxemia, the hyperoxia test may be useful. In the presence of lung pathology, there will typically be a much greater improvement in the arterial oxygen saturation with administration of 100% oxygen (PaO2> 150) than there is in infants with ductal-dependent pulmonary blood flow or transposition physiology (PaO2 typically <50).7 Neonatal shock has a much broader differential diagnosis, and the physical examination findings are often nonspecific.
Low birth weight and other high-risk conditions
2018, Critical Heart Disease in Infants and Children
Address reprint requests to Bradley S. Marino, MD Division of Cardiology Division of Critical Care Medicine The Children's Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, PA 19104