1. DOI: 10.1542/peds.2003-0898-L
2004;114;409-417Pediatrics
Robert L. Geggel
Hospital
Conditions Leading to Pediatric Cardiology Consultation in a Tertiary Academic
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2. Conditions Leading to Pediatric Cardiology Consultation in a
Tertiary Academic Hospital
Robert L. Geggel, MD
ABSTRACT. Objective. To determine the basis for
cardiac consultations for pediatric patients in an aca-
demic hospital setting.
Methods. The activities of the cardiology consulta-
tion service were tabulated for 12 months, from July 2001
to June 2002. Patients were identified from 4 sources, ie,
a monthly log of patient encounters maintained by the
consultation service, encounter forms submitted to the
billing office, consultation notes maintained in a central
file, and a departmental list of echocardiography studies.
Patients who required clearance for noncardiac surgical
procedures were generally evaluated in the cardiology
clinic and not by the consultation service. Patient data
were obtained from consultation and echocardiography
reports and from hospital computer-based records for
discharge summaries for inpatient admissions, emer-
gency department encounter summaries, and laboratory
reports. For each patient, consultations were tabulated as
separate encounters if they occurred on different days in
the emergency department, during separate admissions,
or for different clinical concerns during a single admis-
sion.
Results. A total of 2071 consultations were performed
for 1724 patients. The age at the time of consultation was
6.6 ؎ 9.3 years (median: 1.2 years; range: 1 day to 60.6
years). A total of 1507 patients (87.4%) had a single con-
sultation; 217 patients (12.6%) had multiple encounters,
ranging from 2 to 9, accounting for 564 consultations
(27.2%). Clinical concerns included murmurs (18.5%), car-
diac function (12.7%), arrhythmias (12.7%), intercurrent
illnesses among cardiac patients (11.3%), cyanosis (6.3%),
syndromes (5.7%), chest pain (5.2%), syncope/dizziness
(4.5%), subacute endocarditis (4.4%), follow-up evalua-
tions of fetal diagnoses (4.3%), Kawasaki disease (3.4%),
cor pulmonale (3%), recent cardiac surgery or catheteriza-
tion (1.6%), cerebrovascular accidents (1.2%), and miscel-
laneous conditions. Four diagnoses accounted for 91% of
murmur evaluations, ie, patent ductus arteriosus, ventric-
ular septal defects, innocent murmurs, and pulmonary
branch murmur of infancy. The most common murmur
diagnosis in the neonatal intensive care unit was patent
ductus arteriosus (68%), in the well-child nursery was
ventricular septal defect (64%), and on the medical ward
was innocent murmur (62%). The most common basis for
evaluation of function was oncologic disease. Among
patients evaluated for function, there were 3 new diag-
noses of structural congenital heart disease, all involving
neonates with aortic arch obstruction. Approximately
two-thirds of arrhythmias were supraventricular in ori-
gin. The most common arrhythmias requiring treatment
were supraventricular tachycardia and atrial flutter/fi-
brillation, the latter occurring mainly among older pa-
tients with structural heart disease. Diagnoses made with
fetal echocardiography accounted for 14.3% of newborn
consultations and included 83% of patients with cyanotic
cardiac disease. Three syndromes accounted for 57% of
consultations for this indication, ie, VACTERL associa-
tion (vertebral anomalies, anal atresia, congenital heart
disease, tracheoesophageal fistula, renal abnormality,
and limb anomalies), trisomy 21, and infant of diabetic
mother. Chest pain and syncope/dizziness were fre-
quently evaluated in the emergency department and, in
this setting, accounted for 13 and 10% of all evaluations
and 19 and 25% of evaluations for new patients, respec-
tively. For patients evaluated for chest pain, the most
common basis was musculoskeletal/costochondritic
(42%) or idiopathic (22%). There was a cardiac or pericar-
dial basis in 11% of cases; these patients either had
known heart disease associated with this complication or
systemic symptoms, abnormal cardiac auscultatory find-
ings, and electrocardiographic features of pericarditis.
Syncope/dizziness most commonly had a vasovagal
(50.5%) or orthostatic (24.7%) basis. There was a cardiac
basis in 5.4% of cases; these patients were more likely to
have symptoms associated with exercise. Although endo-
carditis was a frequent clinical concern (91 patients), only
3 cases were identified, involving 2 patients with struc-
tural heart disease and 1 neonate with an indwelling
intracardiac catheter. Two other patients had central ve-
nous lines, intravascular thrombus, and fungemia. Ka-
wasaki disease was the most common acquired condition
leading to consultation. Cor pulmonale was most com-
monly screened among patients with congenital dia-
phragmatic hernia, chronic lung disease of prematurity,
pneumonitis, reactive airway disease, or cystic fibrosis.
Patients with recent cardiac surgery or cardiac catheter-
ization typically had postpericardiotomy syndrome or
complications associated with vascular access. Approxi-
mately 20% of cases of cerebrovascular accidents had a
cardiac basis.
Conclusions. Although a variety of conditions were
assessed, some were encountered more frequently. Fu-
ture educational curricula developed for cardiac training
of pediatric residents should appropriately emphasize
conditions necessitating consultation. Pediatrics 2004;
114:e409–e417. URL: www.pediatrics.org/cgi/doi/10.1542/
peds.2003-0898-L; congenital heart disease, health educa-
tion, patient care.
A
lthough a variety of cardiac conditions and
symptoms occur in the pediatric age range,
the relative frequency of issues addressed in
cardiac consultations in a hospital setting has not
From the Department of Cardiology, Children’s Hospital, and the Depart-
ment of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Accepted for publication Apr 26, 2004.
doi:10.1542/peds.2003-0898-L
Address correspondence to Robert L. Geggel, MD, Children’s Hospital,
300 Longwood Ave, Boston, MA 02115. E-mail: robert.geggel@cardio.
chboston.org
PEDIATRICS (ISSN 0031 4005). Copyright © 2004 by the American Acad-
emy of Pediatrics.
www.pediatrics.org/cgi/doi/10.1542/peds.2003-0898-L PEDIATRICS Vol. 114 No. 4 October 2004 e409
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3. been determined. Education of medical students and
residents in pediatric cardiology is challenging both
because structural congenital heart disease is uncom-
mon, affecting Ͻ1% of children, and because condi-
tions potentially having a cardiac basis frequently
have other causes.1–5 To establish the appropriate
emphasis for clinical instruction in pediatric resi-
dency training programs, I determined the basis for
cardiac consultations in a tertiary academic pediatric
hospital.
METHODS
Study Population
The activities of the cardiology consultation service were tab-
ulated from July 1, 2001, to June 30, 2002. Patients were evaluated
in the emergency department, neonatal and pediatric intensive
care units, inpatient wards, and occasional outpatient settings at
Children’s Hospital, Boston, and in the neonatal intensive care
units and nurseries at Brigham and Women’s Hospital and Beth
Israel-Deaconess Hospital. There is a general cardiology ward and
a cardiac intensive care unit at Children’s Hospital, Boston, which
are not staffed by the consultation service. Patients were identified
from 4 sources, ie, a monthly log of patient encounters maintained
by the consultation service, encounter forms submitted to the
billing office, consultation notes maintained in a central file, and a
departmental list of echocardiographic studies. Although cardiol-
ogy consultation is generally required for echocardiography, our
departmental policy has permitted independent ordering of echo-
cardiographic tests by the oncology service, to evaluate cardiac
function associated with chemotherapy protocols, and by the neo-
natology service, to screen for patent ductus arteriosus among
premature infants.
Patients who required clearance for noncardiac surgical proce-
dures were generally evaluated in the cardiology clinic and not by
the consultation service. The study was approved by the Chil-
dren’s Hospital Committee on Clinical Investigation.
Data Tabulation
Patient data were obtained from consultation and echocardiog-
raphy reports and Children’s Hospital computer-based records for
discharge summaries for inpatient admissions, emergency depart-
ment encounter summaries, and laboratory reports. Tabulated
data included patient age, date, site, and basis of consultation,
whether the patient was new or was known to the cardiology
service, performance of echocardiography, electrocardiography,
or chest radiography, and final diagnosis. For each patient, con-
sultations were tabulated as separate encounters if they occurred
on different days in the emergency department, during separate
admissions, or for a different clinical concern during a single
admission. Patient ages were tabulated as age at the first encoun-
ter for a given diagnosis. Chest pain was classified with previously
reported criteria.2
Statistical Analyses
Patient ages are expressed as mean Ϯ SD. Analyses of the
occurrence of heart disease among patients with supraventricular
tachycardia or atrial fibrillation/atrial flutter and among patients
with syncope/dizziness associated with exertion were performed
with Fisher’s exact test. Comparisons of the age at first encounter
between patients with supraventricular tachycardia and those
with atrial fibrillation/atrial flutter and between patients with
supraventricular tachycardia with or without congenital heart
disease were made with the Wilcoxon rank sum test.
RESULTS
Number of Consultations
During the study period, 2071 consultations were
performed for 1724 patients. The age at the time of
consultation was 6.6 Ϯ 9.3 years (median: 1.2 years;
range: 1 day to 60.6 years). A single consultation was
performed for 1507 patients. Two hundred seventeen
patients (12.6%) had multiple encounters, ranging
from 2 to 9, accounting for 564 consultations (27.2%).
The monthly total of consultations ranged from 154
to 201 (average: 173). Patients were evaluated most
often in the neonatal intensive care unit, inpatient
wards, or the emergency department, for known car-
diac patients (Fig 1).
Reasons for Consultations
Murmurs
Evaluation of a murmur represented 18.5% of en-
counters (Tables 1 and 2). The median age was 6 days
(range: 1 day to 18.5 years). Echocardiography was
used for all patients except for ϳ50% of patients with
pulmonary branch murmurs of infancy or other in-
nocent murmurs and for 10% of patients with ven-
tricular septal defects, each of which was judged to
be small. For patients evaluated for a murmur, the
most common diagnosis or clinical concern in the
neonatal intensive care unit was patent ductus arte-
riosus (68%), in the well-child nursery was ventricu-
lar septal defect (64%), and on the medical ward was
innocent murmur (62%).
Fig 1. Numbers of consultations performed on a monthly basis at different sites. Numbers in parentheses indicate the total numbers of
patients evaluated. “Other” sites included the preoperative clinic, post-anesthesia care unit, radiology department, and infusion center.
ER indicates emergency department; NICU, neonatal intensive care unit; PICU, pediatric intensive care unit.
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4. Function
Cardiac function evaluation accounted for 12.7%
of consultations (Table 1). The patient age was 8.7 Ϯ
8.5 years (median: 6.4 years). Common conditions
prompting evaluation of cardiac function included
oncologic disease (40.5%), congenital heart disease
(13.6%), sepsis (10.6%), postoperative general surgi-
cal procedures (7.2%), neuromuscular disease (6.8%),
arteriovenous malformations (3.8%), and hemato-
logic disease (sickle cell disease or ␤-thalassemia)
TABLE 1. Sites and Bases for Consultations
Basis for consultation No. of Cases
ED, Known ED, New NICU Well
Nursery
PICU Medical
Ward
Other Total
Murmur 17 254 73 3 34 3 384
Function 35 4 20 53 150 2 264
Arrhythmia 106 43 20 8 15 62 8 262
Intercurrent illness 210 2 21 233
Cyanosis (postnatal diagnosis) 13 12 47 5 8 1 86
Syndrome 97 2 4 15 118
Chest pain 57 35 13 2 107
Syncope/dizziness 25 45 22 1 93
Subacute bacterial endocarditis 5 2 9 17 58 91
Fetal diagnosis follow-up
Arrhythmia 6 1 7
Cyanotic heart disease 45 45
Acyanotic heart disease 25 25
Normal postnatal status 8 5 13
Kawasaki disease 1 3 1 65 70
PAH/cor pulmonale 11 37 14 62
Recent cardiac surgery 25 25
Recent catheterization 8 8
CVA/TIA 8 3 6 7 24
Other conditions
Surgical clearance 2 15 11 28
Cardiomegaly 8 4 2 6 20
Abnormal ECG,-nonarrhythmic 4 2 3 8 1 18
Pericardial effusion 4 3 10 17
ALTE 2 1 12 15
Thrombus 3 1 8 12
Systemic hypertension 1 1 1 6 1 10
Vascular ring 1 1 1 6 9
Rheumatic fever 1 6 7
Miscellaneous 9* 4† 2‡ 1§ 2¶ 18
ALTE indicates apparent life-threatening episode; CVA/TIA, cerebrovascular accident/transient ischemic attack; ED, emergency depart-
ment; NICU, neonatal intensive care unit; PAH, pulmonary artery hypertension; PICU, pediatric intensive care unit; ECG, electrocar-
diogram.
* Five central venous line malfunctions (for prostacyclin or antibiotic administration), 2 hemoptysis in patients with pulmonary vascular
obstructive disease, hypercalcemia, and 1 femoral artery pseudoaneurysm (catheterization performed 1 year previously).
† Two thoracopagus conjoined twins, 1 sibling with congenital heart disease, and 1 rule out coarctation.
‡ Two siblings with congenital heart disease.
§ Rule out pulmonary embolism.
¶ Tachypnea.
TABLE 2. Consultations for Evaluation of a Murmur
Diagnosis No. of Cases
ED, New NICU Well
Nursery
PICU Medical
Ward
Other Total Echocardiography
Aortic stenosis 1 1 2 4 4
Atrial myxoma 1 1 1
Atrial septal defect, secundum 1 6 1 1 9 9
Atrioventricular canal, transitional 1 1 1
Innocent or flow murmur 2 19 5 21 1 48 26
Mitral regurgitation 1 1 1
Mitral stenosis 1 1 1
Patent ductus arteriosus 135 5 1 1 1 143 143
Rule out patent ductus arteriosus, not present 37 37 37
Peripheral pulmonary stenosis 4 27 5 1 8 1 46 24
Pulmonary stenosis 3 1 4 4
Truncus arteriosus 1 1 1
Ventricular septal defect 5 21 47 1 2 76 68
Ventricular septal defect/coarctation 1 1 1
Resolved before consultation 7 4 11 0
Abbreviations as in Table 1.
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5. (3.4%). There were 3 new diagnoses of structural
congenital heart disease among neonates who were
examined initially in the emergency department or
pediatric intensive care unit; 2 had severe coarctation
and the other an interrupted aortic arch.
Arrhythmias
Evaluation of an arrhythmia accounted for 12.7%
of consultations (Tables 1 and 3). Atrial arrhythmias
represented 63% of consultations for the indication of
arrhythmia. The most common arrhythmias requir-
ing treatment were supraventricular tachycardia,
atrial flutter, and atrial fibrillation. Compared with
patients with atrial flutter/atrial fibrillation, patients
with supraventricular tachycardia were younger
(7.9 Ϯ 8.5 years; median: 5.4 years; vs 26.1 Ϯ 12.1
years; median: 22.7 years; P Ͻ .001) and had a lower
incidence of congenital heart disease (8% vs 78%, P Ͻ
.001). Patients with supraventricular tachycardia
with congenital heart disease presented at older ages
than did those without structural defects (median
age: 15.1 years vs 3.1 years; P ϭ .05).
Of the 17 patients evaluated for possible pro-
longed QT interval, 1 neonate had congenital prolon-
gation of the QT interval. The other patients had
prolongation of the interval resulting from medica-
tion effects (n ϭ 4; clomipramine, nortriptyline, or
pentamidine) or metabolic defects (n ϭ 6; hypocal-
cemia in 5 cases and hypokalemia in 1 case) or had a
normal interval (n ϭ 6). An associated disease was
present for 1 patient each with first-degree atrioven-
tricular block (anorexia nervosa), transient second-
degree atrioventricular block (Lyme disease), and
neonatal complete heart block (maternal systemic
lupus erythematosus).
Intercurrent Illnesses
Evaluation of patients with known cardiac disease
presenting with an intercurrent noncardiac illness
(mainly viral syndromes) accounted for 11.3% of
consultations (Table 1). The patient age was 5.3 Ϯ 7.4
years (median: 2.0 years).
Cyanosis
Cyanosis formed the basis of consultations for
6.3% of patients (Tables 1 and 4). Three-quarters of
the patients were Ͻ1 week of age. Of 54 patients with
structural cyanotic heart disease, 45 (83%) were di-
agnosed with fetal echocardiography. The mothers
of these neonates had delivery scheduled at a nearby
hospital. Intercurrent illnesses among patients with
palliated cyanotic heart disease produced increasing
cyanosis if there was fever for patients with system-
ic-pulmonary shunts or dehydration for those with
intracardiac right-to-left shunts.
TABLE 3. Consultations for Evaluation of an Arrhythmia
Arrhythmia No. of Cases
ED, Known ED, New NICU Well Nursery PICU Medical Ward Other Total
Atrial
Atrial fibrillation 5 1 2 8*
Atrial flutter 28 1 1 30†
Atrial premature beats 4 2 4 4 1 1 16
Ectopic atrial tachycardia 2 2 1 1 6
Sinus arrhythmia 2 3 1 6
Sinus bradycardia 2 2 1 3 21 1 30
Sinus tachycardia 4 5 1 7 17‡
Supraventricular tachycardia 34 11 4 1 1 3 54§
Ventricular
Ventricular premature beat 3 2 5 2 7 8 2 29
Ventricular tachycardia 3 2 2 7
Ventricular fibrillation 2 2
Junctional
Junctional ectopic tachycardia 1 1 1 1 4
Junctional escape rhythm 2 1 3
Junctional premature beat 1 1 2
Heart block
First-degree heart block 1 1 2
Second-degree heart block 2 1 3
Congenital complete heart block 1 1
Normal 1 1 2 2 1 7
Palpitations, no identified arrhythmia 7 8 1 16
Pacemaker
ICD discharge 6 6
Dual-chamber pacemaker malfunction 3 3
Prolonged OT interval 1 2 2 11 1 17
Values include prenatal and postnatal evaluations. ICD indicates implantable cardioverter-defibrillator; other abbreviations as in Table 1.
* Six different patients (congenital heart disease in 3; 1 patient each with Ebstein’s anomaly, tricuspid atresia status post Fontan, and
cardiomyopathy).
† Seventeen different patients (congenital heart disease in 15: single ventricle, status post Fontan, n ϭ 9; dextro-transposition of the great
arteries, status post atrial switch procedure, n ϭ 2; cardiomyopathy, n ϭ 2; 1 patient each with tetralogy of Fallot and levo-transposition
of the great arteries).
‡ Fifteen different patients.
§ Thirty-eight different patients.
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6. Syndromes
The evaluation of cardiac involvement in syn-
dromes prompted consultation for 5.7% of patients
(Tables 1 and 5). The median age was 2 days. Three
syndromes represented 57% of the consultations for
this indication, ie, VACTERL association (vertebral
anomalies, anal atresia, congenital heart disease, tra-
cheoesophageal fistula, renal abnormality, and limb
anomalies), trisomy 21, and infant of a diabetic
mother.
Chest Pain
Chest pain accounted for 5.2% of consultations,
13% of all emergency department evaluations, and
19% of emergency department consultations for new
patients (Tables 1 and 6). The patient age was 16.1 Ϯ
7.4 years (median: 15.4 years). For this symptom, the
2 most common categories were musculoskeletal/
costochondritic (42%) and idiopathic (22%) condi-
tions. There was a cardiac or pericardial basis in 11%
of cases; these patients either had a known cardiac
history or had pericarditis presenting with systemic
symptoms and abnormal cardiac auscultatory or
electrocardiographic findings (6). Chest pain with
exertion occurred for 2 patients, each with known
heart disease. One patient with palliated cyanotic
heart disease and normocytic erythrocytes experi-
enced resolution of chest pain with reduction of he-
matocrit values from 71% to 63%. A chest radiograph
was obtained in 92 episodes (86%) and contributed to
the diagnosis for the 13 patients with pulmonary
abnormalities and the 5 patients with pericarditis. An
electrocardiogram was obtained in 106 episodes
(99%) and differed from baseline tracings or was
abnormal for 4 patients with pericarditis. Concern
about possible pulmonary embolism was raised for 6
patients, but the results of imaging studies were
normal.
Syncope/Dizziness
Evaluation for syncope or dizziness accounted for
4.5% of consultations, 10% of all emergency depart-
ment evaluations, and 25% of emergency department
evaluations for new patients. The patient age was
14.4 Ϯ 5.4 years (median: 14.9 years). The most com-
mon causes were vasovagal (47 episodes, 50.5%) or
orthostatic (23 episodes, 24.7%). For 5 patients, or-
thostasis was related to medication (clonidine,
quetiapine fumarate, or furosemide). Six episodes
(6.5%) had an idiopathic basis and 2 had other causes
(hyperventilation or menorrhagia). Five patients
(5.4%) each demonstrated a cardiac, neurologic, or
psychiatric basis. Cardiac diseases included 2 new
patients with congenital prolonged QT interval and 1
known patient each with primary pulmonary hyper-
tension, severe subaortic stenosis, and bradycardia
secondary to pacemaker dysfunction. Neurologic
conditions included a seizure disorder, breath-hold-
ing spell, and migraine headache. Patients with a
cardiac basis were more likely to have symptoms
associated with exercise (P ϭ .001). Of 13 patients
who experienced syncope (n ϭ 8) or dizziness (n ϭ 1)
during exercise or syncope shortly after completion
of exercise (n ϭ 4), 7 demonstrated a vasovagal basis,
4 had a cardiac condition, and 1 each exhibited a
psychiatric or idiopathic cause.
Subacute Endocarditis
Evaluation for subacute endocarditis accounted
for 4.4% of consultations (Tables 1 and 7). The patient
age was 7.7 Ϯ 7.3 years (median: 5.2 years). Of 91
consultations for this concern, there were 3 cases of
subacute endocarditis (3.3%) (Table 7). Two events
occurred among known cardiac patients, presenting
to the emergency department with either fever and
bacteremia or an indolent course of weight loss and
anorexia. For each patient, the blood cultures yielded
Streptococcus viridans. A premature neonate had an
umbilical venous line, Staphylococcus aureus bactere-
mia, and right-sided intracardiac vegetations. Two
patients had candidemia and intravascular thrombus
associated with a central venous line.
Transthoracic echocardiograms were obtained in
79 consultations (87%) and transesophageal echocar-
TABLE 4. Consultations for Evaluation of Cyanosis
Diagnosis No. of Cases
ED, Known ED, New NICU PICU Medical Ward Other Total
Acrocyanosis 3 6 2 11
Apnea-bradycardia 1 2 1 4
Cyanotic CHD, Follow-up fetal diagnosis 45* 45
Cyanotic CHD, intercurrent illness 9 1 10
Cyanotic CHD, new diagnosis 2† 7‡ 9
Cyanosis, crying only, neonate 2 1 3
Obstructive sleep apnea 1 1
PPHN 26 3 29
Pulmonary disease§ 1 8 2 5 1 17
Rule out tetralogy spell 1 1
Seizure 1 1
CHD indicates congenital heart disease; PPHN, persistent pulmonary hypertension of newborn; other abbreviations as in Table 1.
* Double-outlet right ventricle, 1; hypoplastic left heart syndrome, 17; pulmonary atresia, intact ventricular septum, 5; single ventricle, 4;
tetralogy of Fallot, 11; dextro-transposition of the great arteries, 5; tricuspid atresia, 2.
† Total anomalous pulmonary venous connection, 2 (1 infradiaphragmatic, 1 supracardiac to innominate vein).
‡ Pulmonary atresia, intact ventricular septum, 1; right ventricular endocardial fibroelastosis, 1; tetralogy of Fallot, 1; total anomalous
pulmonary venous connection, 1; dextrotransposition of the great arteries, 3.
§ Pulmonary conditions included pneumonia associated with gastroesophageal reflux disease and aspiration, bronchiolitis, pulmonary
hypoplasia or dysplasia, laryngomalacia, and postoperative bronchospasm.
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7. diograms in 6 (6.7%). Thrombus or vegetation was
noted only for the patients cited above. Echocardiog-
raphy was deferred for 9 patients who had sterile
blood cultures and for 3 others who had sterile cul-
tures after removal of a central venous catheter and
antibiotic therapy for bacteremia.
The most common clinical background was septi-
cemia with an indwelling central venous catheter (36
episodes, 40%). A new murmur was present for 11
patients, 6 of whom had positive blood cultures.
Echocardiograms were obtained for 10 of these pa-
tients. Each murmur was innocent, ie, 6 Still’s mur-
murs, 4 flow murmurs associated with anemia, and 1
innocent pulmonary branch murmur of infancy.
Follow-up Evaluations of Fetal Diagnoses
Ninety neonates had consultations prompted by
fetal echocardiographic diagnoses, representing 4.3%
of consultations and 14.1% of consultations per-
formed in the neonatal intensive care unit and nurs-
eries (Table 1).
Kawasaki Disease
This clinical concern represented 3.4% of all con-
sultations (Tables 1 and 2). The patient age was 4.3 Ϯ
3.3 years (median: 3.7 years). Of the 70 encounters, 51
patients were diagnosed as having Kawasaki dis-
ease. The other 19 patients were diagnosed as having
viral syndrome (15 patients), urinary tract infection
or pyelonephritis (1 patient, 2 episodes), hypersensi-
tivity reaction to dilantin, or necrotizing fasciitis.
Pulmonary Artery Hypertension/Cor Pulmonale
Concern about pulmonary artery hypertension ac-
counted for 3.0% of consultations. The patient age
was 3.7 Ϯ 6.9 years (median: 92 days; range: 1 day to
TABLE 5. Consultations for Syndromes or Other Congenital Anomalies
Syndrome No. % CHD CHD Lesions
VACTERL 28 43 VSD, 6; ASD, 2; bicuspid AoV, 2; TOF, 1; RAA-aberrant LSCA, 3*
Trisomy 21 22 45 VSD, 3; CAVC, 2; PDA, 2; TOF, 2
Infant of diabetic mother 17 65 VSD, 1; PS, 1; septal hypertrophy, 9 (2 with LVOT obstruction)
Omphalocele 6 17 VSD and PDA, 1
Cleft palate 6 33 VSD, 1; RAA-aberrant LSCA, 1
Twin-twin transfusion 4 25 VSD, 1
Dandy-Walker syndrome 3 67 VSD, 1; bicuspid AoV, 1
Jejunal atresia 3 33 PS
Rule out DiGeorge syndrome† 3 0
Rule out Marfan syndrome‡ 3 0
Rule out tuberous sclerosis§ 3 50 Intracardiac rhabdomyoma, 1
Miscellaneous¶ 20 45 VSD, 4; VSD and ASD, 1; ASD, 2; bicuspid AoV, 1; MR, 1
AoV indicates aortic valve; ASD, atrial septal defect; CAVC, complete atrioventricular canal defect; LSCA, left subclavian artery; LVOT,
left ventricular outflow tract; MR, mitral regurgitation; PDA, patent ductus arteriosus; PS, pulmonary stenosis; RAA, right aortic arch;
TOF, tetralogy of Fallot; VSD, ventricular septal defect.
* Two patients with RAA-aberrant LSCA also had either VSD or ASD.
† Evaluated for hypocalcemia; 1 patient had DiGeorge syndrome.
‡ Evaluated for pneumothorax, joint dislocation, or superior mesenteric artery aneurysm; none diagnosed with Marfan syndrome.
§ Each with seizure disorder; 2 patients diagnosed with tuberous sclerosis.
¶ Chromosome abnormality other than trisomy 21 (3; partial trisomy 9q, 14q duplication, and partial deletion of 18q); choanal atresia or
stenosis 2, Klippel-Trenaunay-Weber 2; 1 patient each with Beal’s syndrome, cloacal extrophy, coloboma, extrahepatic biliary atresia,
gastroschisis, Holt-Oram syndrome, Kartagener’s syndrome, lobar emphysema, Marden-Walker syndrome, nemoline rod myopathy,
Russel-Silver syndrome, Smith-Lemli-Opitz syndrome, and tracheal stenosis.
TABLE 6. Consultations for Evaluation of Chest Pain
Diagnosis No. of Cases
ED, Known ED, New Medical Ward Other Total
Anxiety 4 1 5
Cardiac disease 7* 3† 2‡ 12
Gastroesophageal reflux 1 1
Idiopathic 9 9 4 2 24
Musculoskeletal/costochondritic 27 14 4 45
Polycythemia 1 1
Pulmonary
Pleural effusion/pleuritis 1 2 3
Pneumonia 2 1 2 5
Pneumomediastinum 2 2
Pneumothorax 2 1 3
Skin infection at cvl site 1 1
Toxin, cannabis 2 3 5
cvl indicates central venous line; other abbreviations as in Table 1.
* Known patients with pulmonary vascular obstructive disease, 2; recurrent pericarditis, 2; tetralogy
of Fallot with systemic level right ventricular pressure produced by an obstructed right ventricular-
pulmonary artery conduit, 1; Kawasaki disease with known giant aneurysm of left anterior descend-
ing coronary artery with echocardiographic confirmation of new clot in this artery, 1; subclavicular
pacemaker pocket infection, 1.
† Pericarditis associated with fever and pericardial friction rub in each patient.
‡ Known patients with Takayasu’s aortitis or severe cardiomyopathy secondary to ␤-thalassemia.
e414 CARDIOLOGY CONSULTATION IN AN ACADEMIC HOSPITAL
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8. 25.6 years). The most common condition was con-
genital diaphragmatic hernia, which was found for
34% of patients evaluated for this concern. Other
conditions prompting evaluation of pulmonary ar-
tery pressure included chronic lung disease associ-
ated with premature birth, pneumonitis or reactive
airway disease, cystic fibrosis, pulmonary hypopla-
sia associated with chest wall deformity or cystic
adenomatoid malformation, obstructive sleep apnea,
pulmonary hemosiderosis, neurologic conditions as-
sociated with hypoventilation, and orthotopic lung
transplantation.
Recent Cardiac Surgery or Cardiac Catheterization
Eight of 25 patients evaluated within 2 months
after surgery had postpericardiotomy syndrome.
Other issues addressed included surgical wound
evaluation, pleural effusion, intervening viral illness,
pain control, and bacterial pericarditis.
Evaluations after catheterization, with 1 exception,
involved interventional procedures. One patient had
transient fever after coil occlusion of collateral ves-
sels, whereas the others had vascular access prob-
lems consisting of pain (local hematoma, infected
hematoma, or arterial pseudoaneurysm) or coolness
of an extremity.
Cerebrovascular Accidents/Transient Ischemic Attacks
Evaluation of a cardiac source for loss of neuro-
logic function accounted for 1.2% of consultations
(Table 1). A cardiac basis was identified for 5 of the
24 patients evaluated for this concern, ie, intracardiac
thrombus for 2 patients with a single ventricle and
bidirectional flow across a patent foramen ovale for 3
patients, including a neonate with a thrombus at the
tip of a central venous line and 2 adolescents. An
additional adult patient had pulmonary vascular ob-
structive disease, polycythemia (hematocrit: 62%),
and microcytic anemia (mean corpuscular volume:
69 FL). The other 18 patients had a variety of condi-
tions, ie, protein C, protein S, or antithrombin III
deficiencies, carotid artery dissection, moyamoya
disease, sickle cell disease, encephalitis, coagulopa-
thy associated with intraventricular hemorrhage, se-
vere hyponatremia caused by psychogenic polydip-
sia, or idiopathic causes.
Other Conditions
A variety of other conditions were evaluated (Ta-
ble 1). Eight of 20 patients (40%) who underwent
consultations because of cardiomegaly on chest ra-
diographs had heart disease. The cardiologist and
radiologist judged the cardiac silhouette to be nor-
mal in an equal number, 5 of whom had a large
thymus. For 4 other patients, echocardiograms
showed normal cardiac dimensions. Infants with an
apparent life-threatening event had normal QT inter-
vals and no other identifiable cardiac disorders; 5
patients demonstrated an idiopathic basis, whereas
the others had apnea, periodic breathing, breath-
holding, gastroesophageal reflux, or seizure disor-
der. Systemic hypertension was associated with re-
nal disease for 4 patients, agitation, headache, or
postoperative pain for 5, and medication (fludrocor-
tisone and midodrine) for 1. Evaluation for a vascu-
lar ring was prompted mainly by the symptom of
stridor, which was caused for 2 patients each by
tracheomalacia and laryngomalacia and for 1 patient
each by left main bronchus stenosis, subglottic hem-
angioma, innominate artery compression, and uni-
lateral vocal cord paresis; 1 patient with an incidental
finding of a right aortic arch had a loose vascular
ring with an aberrant left subclavian artery. Of 7
patients evaluated for rheumatic fever, 2 with chorea
and mitral regurgitation were diagnosed with this
condition. Two other patients with movement disor-
ders had either Guillain-Barre´ or antiphospholipid
syndrome. The other 3 patients did not fulfill Jones
TABLE 7. Consultations to Rule Out Subacute Bacterial Endocarditis
No. of Cases
ED, Known ED, New NICU PICU Medical Ward Total
Basis for consultation
Positive BC 1* 1 7* 13† 35 57‡
Fever 5* 1 3 16† 42 24
New murmur 1 1 1 8 11
New S3 1 1
Weight loss, anorexia 1† 1
Fatigue 1 1
Embolic event 1 1 2
Clinical conditions
Repaired/palliated CHD 4* 3 7
Unrepaired CHD 1† 4 5
Oncologic diseases 3 16 19
General surgery within previous 8 wk 4 5 11 20
Indwelling central venous catheter 5* 10† 33 48
Intravenous drug abuser 1 1
Dental abscess 1 1
BC indicates blood culture; other abbreviations as in Tables 1 and 4.
* One patient with each symbol had subacute bacterial endocarditis; see text for details.
† Two patients with possible infected intravascular thrombus; see text for details.
‡ Three organisms accounted for 39 (67%) of the 58 episodes of septicemia (57 presenting with septicemia and 1 presenting with
constitutional symptoms and subsequently found to have bacteremia). Various species of Candida were isolated in 17 cases, Staphylococcus
aureus in 14 episodes, and coagulase-negative Staphylococcus in 8. There were 5 cases of Streptococcus viridans and 2 cases each of
Pseudomonas aeruginosa and group A Streptococcus. Ten other bacteria were isolated only once, for different patients.
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9. criteria, including 2 with fever and an innocent mur-
mur.
DISCUSSION
This review documents for the first time the issues
prompting cardiac consultation in an academic pe-
diatric hospital setting. Although a variety of condi-
tions were assessed, some diagnoses were encoun-
tered more frequently and should be given emphasis
in future curriculum development for cardiac train-
ing for pediatric residents.
Evaluation of a murmur was the most frequent
basis for consultation. Cardiac auscultation skills in
various training programs, including pediatrics,
have been documented to be poor.6–8 Four condi-
tions (patent ductus arteriosus, ventricular septal de-
fect, innocent murmur, and pulmonary branch mur-
mur of infancy) accounted for 91% of diagnoses for
patients with murmurs. Initially developing stetho-
scope skills among pediatric residents for the detec-
tion of these conditions should fulfill clinical needs
frequently. Developing skills for the diagnosis of an
innocent murmur should have additional benefits in
reducing parental morbidity if confirmation by a
cardiologist is subsequently sought, because the abil-
ity of the pediatrician to provide greater reassurance
has been shown to reduce parental anxiety associ-
ated with referral.9
Assessment of function was frequently requested
for a variety of clinical conditions. Newly developed
dysfunction among infants after discharge from the
birth hospital can represent duct-dependent lesions
that elude detection while the ductus is widely
patent.10
The majority of arrhythmias were atrial in origin.
The most common arrhythmia was supraventricular
tachycardia, which usually was associated with a
structurally normal heart.11 Atrial fibrillation/atrial
flutter occurred among older patients, who typically
had congenital heart disease; the chronic effects of
pressure or volume overload or surgical scars con-
tribute to the development of these 2 arrhythmias,12
which are commonly seen among patients, as in our
study group, who have undergone atrial baffle repair
of dextrotransposition of the great arteries (Mustard
or Senning procedures), modified Fontan procedures
because of a functional single ventricle, or repair of
tetralogy of Fallot.12,13 Young adults with repaired
congenital heart disease are becoming a larger pa-
tient group, because of improved survival rates, and
often are cared for at pediatric hospitals; therefore,
familiarity with these arrhythmias is important. Ven-
tricular arrhythmias were infrequent and mainly
consisted of ventricular premature beats. Prolonged
QT syndrome usually was associated with a meta-
bolic abnormality or medication-related side effect,14
rather than with congenital prolongation. Heart
block was uncommon and was associated with an-
orexia nervosa,15 Lyme disease,16 or maternal sys-
temic lupus erythematosus.17
Diagnoses made with fetal echocardiography were
a frequent basis for neonatal consultation. Cyanotic
lesions were over-represented among the prenatally
diagnosed patients, in comparison with the occur-
rence in the general population,1 because delivery of
these infants was arranged at a nearby hospital. Such
a policy can decrease morbidity and mortality
rates.18 Patients with palliated cyanotic heart disease
experienced increased cyanosis with intercurrent ill-
ness in the presence of dehydration, because of in-
creased oxygen extraction and lower venous oxygen
saturation, in conditions with right-to-left intracar-
diac shunting or in the presence of fever, because of
decreased shunt perfusion produced by lower sys-
temic vascular resistance, in conditions with system-
ic-pulmonary artery shunting. The oxygen dissocia-
tion curve is steep for arterial oxygen pressure values
of Ͻ50 mm Hg, so that a small decrease in oxygen
tension produces a large decrease in oxygen satura-
tion.19
Chest pain and syncope were frequently evaluated
in the emergency department,3 and each was typi-
cally associated with a benign condition.2,3,20 A seri-
ous organic cause for chest pain was unusual with-
out associated symptoms of illness, positive physical
examination findings related to the cardiac or respi-
ratory systems, or symptoms during exertion.21 The
occurrence of syncope during or shortly after exer-
tion also was commonly observed among patients
with cardiac conditions.3
Syndromes have been reported for 8.5% of patients
with congenital heart disease.22 Recognition of syn-
dromes permits thorough patient evaluation, includ-
ing early detection of heart disease, which may not
produce clinical symptoms as long as pulmonary
vascular resistance is elevated in the early neonatal
period. The 3 syndromes evaluated most frequently
are associated commonly with congenital heart dis-
ease.23–25
Endocarditis was uncommon and occurred among
patients with known congenital heart disease or in-
dwelling venous catheters.26 Risk factors for trans-
thoracic echocardiographic evidence of endocarditis
that were identified in each of 2 pediatric reports
included positive blood cultures, physical findings of
new or changing heart murmur, and congestive
heart failure.27,28 Indications in neonates may be dif-
ferent.26,27 Our experience shows that the feature of a
new murmur should be defined as a new regurgitant
murmur,29 because a hyperdynamic condition asso-
ciated with febrile illness can produce innocent mur-
murs.
Kawasaki disease was the most common acquired
disease leading to consultation. Approximately one-
quarter of patients evaluated for Kawasaki disease
were determined to have other conditions. Consid-
ering the diagnosis of Kawasaki disease for children
with some features of the illness is warranted, be-
cause atypical presentation has been associated with
coronary involvement.30
Various conditions were encountered less fre-
quently. Evaluation of pulmonary hypertension
mainly involved conditions associated with pulmo-
nary disease. Doppler echocardiography can be used
to estimate the level of pulmonary systolic pressure
and to monitor the hemodynamic effects and dosing
of nitric oxide or supplemental oxygen in these con-
ditions.31,32 Consultation after cardiac surgery
e416 CARDIOLOGY CONSULTATION IN AN ACADEMIC HOSPITAL
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10. chiefly involved evaluation for postpericardiotomy
syndrome,33 whereas assessment after catheteriza-
tion mainly involved vascular access concerns
among patients who had undergone interventional
procedures (a risk factor for complications).34 Al-
though neurologic deficits usually had a noncardiac
cause,5 our patient group confirmed risks associated
with cyanotic heart disease,35 Fontan procedure,15
thrombosis related to the use of an indwelling central
venous catheter,36 patent foramen ovale,37 and poly-
cythemia associated with microcytosis.35 Cardiomeg-
aly was not present for 60% of patients evaluated for
this condition.38 Failure to distinguish a large over-
lying thymus gland from true cardiac enlargement
occurred relatively frequently. Systemic hyperten-
sion was associated with renal disease, the most
common cause in pediatrics.39 Stridor, which can be
produced by vascular compression of the airways,40
was associated mainly with airway disease among
our patients.
CONCLUSIONS
Although numerous conditions were evaluated by
the cardiac consultation service, some were encoun-
tered more frequently and should be given emphasis
in curricula developed for pediatric residency train-
ing programs. Future investigations will be needed
to determine the effectiveness of acquiring skills for
the diagnosis of cardiac symptoms and disease dur-
ing pediatric residency.
ACKNOWLEDGMENT
I thank the fellows and attending physicians who helped staff
the cardiology consultation service during the study period.
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11. DOI: 10.1542/peds.2003-0898-L
2004;114;409-417Pediatrics
Robert L. Geggel
Hospital
Conditions Leading to Pediatric Cardiology Consultation in a Tertiary Academic
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