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Evaluation of a Newborn With a Murmur

Yuliya Turiy, MD, MPH, Robert J. Yetman, MD, Polly F. Cromwell, MSN, RN, CPNP J Pediatr Health Care. 2013;27(3):226-229.

Introduction
The incidence of murmurs in children has been reported to be as high as 90% (Frommelt, 2004). However, the prevalence of structural heart disease is only 8 to 10 per 1000 live births, with approximately one quarter of these children having critical congenital heart disease (CHD), defined as requiring surgical or catheter intervention in the first year of life (Mahle et al., 2009). Therefore it is the job of the primary care provider to identify the newborns who require investigation of their murmur. This article will focus on the assessment of an otherwise well newborn infant presenting with a murmur.

History
Perinatal history and family history are crucial to the evaluation of a newborn with a murmur. Detailed pregnancy history and medical records should be obtained from the mother, including the following information:

Results of prenatal ultrasounds

A normal prenatal ultrasound does not completely rule out CHD because fewer than 50% of cases of CHD are identified on a routine prenatal ultrasound (Mahle et al., 2009)

Medications Alcohol and other drug exposure Comorbid pregnancy conditions (e.g., hypertension and diabetes mellitus)

Family history should focus on the following information:

Congenital abnormalities Inheritable diseases with known cardiac lesions (e.g., hypertrophic cardiomyopathy) Childhood deaths and siblings or first-degree relatives with structural heart disease

Epidemiologic studies have shown that the relative risks of CHD were 3.2, 1.8, and 1.1 with a family history of CHD in first-, second-, and third-degree relatives, respectively (Oyen et al., 2009)

The following specific questions related to the infant should be asked:

Feeding history (How much? How often? How long?)

Infants with structural heart disease tend to have more difficulty with feedings, have more associated symptoms while feeding, and have poorer weight gain

Any associated symptoms while feeding (e.g., tachypnea, diaphoresis, fussiness, retractions, or cyanosis) Weight gain Activity level and general disposition.

Physical Examination

Vital signs should include heart rate, respiratory rate, blood pressure (BP), and pulse oximetry readings (preductal and postductal). The infant's general disposition and color also should be noted. Inconsolability, diaphoresis, pallor, or central cyanosis should raise the clinician's suspicion for a structural heart disease. Central cyanosis is always abnormal and must be differentiated from acrocyanosis (bluish discoloration of hands and feet), which is a normal and common phenomenon in newborns.
Four Extremity Blood Pressure Measurements

Four extremity BP readings traditionally have been used in the assessment of an asymptomatic murmur in a neonate to eliminate coarctation of the aorta or interrupted aortic arch as diagnostic possibilities. A BP that is 20 mm Hg higher in the arms than in the legs should prompt further investigation. However, Crossland, Funess, Abu-Harb, Sadagopan, and Wren (2004) found a significant variation in the measurement of four-extremity BPs in neonates. These differences can be attributed to equipment differences and difficulty in obtaining an accurate BP in all four extremities in an uncooperative infant. These investigators determined that fourextremity BP measurement is not a reliable screening tool for these conditions. If one of these lesions is suspected based on physical examination, particularly if weak femoral pulses are appreciated, an echocardiogram is warranted regardless of the lower and upper extremity BPs.
Cardiac Examination

Cardiac examination should note the following information:

Location of the point of maximum intensity The presence of precordial bulge, substernal heave, and precordial thrill First and second heart sounds and any additional heart sounds such as S3, S4, and murmurs

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First heart sounds are best heard at the lower left sternal border and usually are single Second heart sounds are heard best along the left upper sternal border; they are audibly split in 80% of newborn infants by 48 hours of age (Duff &McNamara, 1998) and vary with respiration Any abnormality in the second heart sound should raise suspicion for a congenital heart defect Any murmur that is diastolic, louder than a II/VI, or harsh in quality is considered abnormal

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Although physical examination still remains the mainstay of clinical diagnosis, several studies have shown that the effectiveness of auscultation alone is limited and dependent on practitioner's level of experience. One study found that the clinical assessment alone by a pediatric cardiologist had a sensitivity of 80.5% and a specificity of 90.9% for differentiating an innocent murmur from a pathologic one (Griebsch et al., 2007). Ageliki and colleagues (2011) also found that auscultation alone has a limited ability to distinguish a pathologic from an innocent murmur. In addition, they found great variation between the levels of clinical experience (i.e., an attending physician versus a fellow versus a resident). Another study found that fully trained pediatricians could assess the significance of a neonatal heart murmur with a specificity of 95.5% but a sensitivity of only 33%. Interestingly, the same study found no significant difference between the ability of a pediatric cardiologist and a pediatrician to judge a pathologic murmur (Gokman et al., 2009). Any abnormality in the second heart sound should raise suspicion for a congenital heart defect.

Diagnostic Studies
Pulse Oximetry

The use of pulse oximetry to screen for structural heart disease, in particular for lesions classified as critical congenital cyanotic heart disease, has been studied and is increasingly common for routine assessments of neonates. Current recommendations come from a careful analysis of the published and unpublished data reviewed by a work group comprising the Secretary's Advisory Committee on Heritable Disorders in Newborns and Children, the American Academy of Pediatrics, the American Heart Association, and the American College of Cardiology Foundation (Kemper et al., 2011). These recommendations state that:

Screening should begin after the first 24 hours of life and be completed on the second day of life Readings should be obtained from right upper extremity and either a right or a left lower extremity (preductal and postductal) A screen is considered positive if the oxygen saturation is < 90% in any one extremity, oxygen saturation is < 95% in both extremities on three measures each separated by 1 hour, or a > 3% absolute difference in oxygen saturation between upper and lower extremity readings is noted on three separate measures taken 1 hour apart

Chest Radiographs and Electrocardiograms

In addition to a thorough history and physical examination, chest radiographs and electrocardiograms (ECGs) often are used by practitioners to evaluate an asymptomatic murmur. Several congenital cardiac lesions will have ECG abnormalities:

Right ventricular hypertrophy beyond what is expected in the neonatal period can be indicative of rightsided obstructive lesions such as pulmonary valve stenosis Left ventricular hypertrophy raises concerns for left-sided obstructive lesions such as aortic or subaortic stenosis and coarctation of the aorta Right or left axis deviation can be indicative of an atrial septal defect or atrioventricular canal defect Atrial septal defect also can present as incomplete right bundle branch block (O'Connor, McDaniel, & Brady, 2008)

A study by Danford, Gumbiner, Martin, and Fletcher (2000) showed that an ECG and a chest radiograph can be helpful in diagnosing certain cardiac lesions and do not significantly mislead the examiner. For example, an ECG can aid in diagnosis of an atrial septal defect or pulmonary valve stenosis and a chest radiograph can be helpful in the diagnosis of a moderate to large ventricular septal defect. Danford and colleagues postulate that an ECG and a chest radiograph remain a reasonable option to be included in the full workup of a child with an asymptomatic murmur. However, other studies have found that although some cardiac defects will have ECG and chest radiographic findings, the ability of those findings to change the management of these infants remains controversial. A study by Oeppen, Fairhurst, and Argent (2002) found that a chest radiograph was not only unlikely to help in defining the exact cardiac defect present, but it also did not change the overall management of the patient. In addition, a study looking at the cost-effectiveness of the various modalities of evaluation of a heart murmur found that the most cost-effective evaluation is either immediate referral to a pediatric cardiologist or an echocardiogram performed by an experienced pediatric echocardiographer (Yi, Kimball, Tsevat, Mrus, & Kotagal, 2002). These investigators also found that the addition of a chest radiograph and ECG added little to the initial evaluation and resulted in a selective referral for echocardiography and/or pediatric cardiology that was not cost-effective (Yi et al., 2002).
Echocardiogram

An echocardiogram remains the study of choice to diagnose CHD. It is noninvasive, has a high detection rate for CHD, and can be performed easily at the bedside. Tworetzky and colleagues (1999) found that an echocardiogram has a 95% diagnostic accuracy in both isolated cardiac lesions such as ventricular septal defects and more complex pathology such as tetralogy of Fallot. The use of Doppler techniques and higher resolution probes also has increased the accuracy of the traditional two-dimensional echocardiogram to detect CHD. However, an echocardiogram is not without its own limitations. It is highly operator dependant and is most accurate when performed by a trained pediatric echocardiographer and read by a pediatric cardiologist. In addition, the acoustic windows can vary greatly depending on the size of the patient, and they can be limited in postoperative patients. The quality of the echocardiogram also depends greatly on the cooperation of the patient, which is a difficult task to achieve in a pediatric population.

New imaging techniques such as three-dimensional echocardiography, cardiac magnetic resonance imaging, and computed tomography are emerging to compliment two-dimensional echocardiography. These studies are particularly useful in delineating more complex CHD lesions by creating a full three-dimensional reconstruction of the heart. Cardiac magnetic resonance imaging also is able to determine cardiac function, ventricular volume, and velocity and volume of blood flow without relying on geometrical assumptions (Roest & De Roos, 2012). However, given the higher complexity of these studies, the two-dimensional echocardiogram remains the initial study of choice.

Summary
Currently we have no universal guidelines on further workup of an asymptomatic neonate with a murmur, and the approach to these infants varies greatly from practitioner to practitioner. The initial evaluation of such infants should always include the following elements:

Vital signs Pulse oximetry readings as previously outlined A thorough physical examination

If, on the basis of these data, the examiner feels comfortable with the diagnosis of a benign neonatal murmur, standard follow-up with a general practitioner and close monitoring is within reason. However, if the examiner has any concerns (based either on the data or on his or her comfort level), further evaluation is warranted. In areas where a pediatric cardiology consultation is available, the cardiologist then can make the appropriate recommendations for further studies and follow-up. In areas where a pediatric cardiologist is not readily available, the addition of a chest radiograph or an ECG may aid in the diagnosis of several specific cardiac lesions, and an echocardiogram, if available, may aid in the accurate diagnosis and dictate appropriate followup. For the remotely located clinician, telemedicine is an option. Multiple studies have found that telemedicine in pediatric cardiology is safe, cost-effective, yields accurate diagnoses, and serves as a valuable resource for the primary care clinician (Gomes et al., 2010; Sable et al., 2002). The advent of telemedicine technology has provided these patients and clinicians with access to pediatric cardiologists without the need to travel to a tertiary care center.
References

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J Pediatr Health Care. 2013;27(3):226-229. 2013 Mosby, Inc.