Park’s Pediatric Cardiology for Practitioners

487 Pages·2020·12.385 MB·English

by  Myung Park, Mehrdad Salamat

Seventh Edition
PARK’S
PEDIATRIC
CARDIOLOGY
FOR PRACTITIONERS
Myung K. Park, MD, FAAP, FACC
Professor Emeritus (Pediatrics)
Former Director of Pediatric Cardiology
Former Director of Preventive Cardiology and Weight Management Clinic
University of Texas Health Science Center
San Antonio, Texas
Mehrdad Salamat, MD, FAAP, FACC
Clinical Associate Professor of Pediatrics
Texas A & M University Health Science Center
Bryan, Texas
Attending Cardiologist
Driscoll Children’s Hospital
Corpus Christi, Texas
Elsevier
1600 John F. Kennedy Blvd.
Ste 1800
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PARK’S PEDIATRIC CARDIOLOGY FOR PRACTITIONERS, SEVENTH EDITION ISBN: 978-0-323-68107-0
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Printed in the United States of America
Last digit is the print number: 9 8 7 6 5 4 3 2 1
To my wife Issun,
our sons (Douglas, Christopher, and Warren),
daughters-in-law (Bomin and Soyeon), and
grandchildren (Natalie, Audrey, and Madeleine).
Myung K. Park, MD
In memory of my father,
whose love for reading was contagious,
whose love for education will remain eternal.
Mehrdad Salamat, MD
P R E FAC E
Since the publication of the sixth edition of Park’s Pediatric new chapter on pediatric preventive cardiology was added
Cardiology for Practitioners in 2014, important advances have to discuss metabolic syndrome, cardiovascular risk factors,
been made not only in the diagnosis but also in the medical dyslipidemia screening, obesity, and smoking to emphasize
and surgical management of children with congenital and the need for practitioners’ efforts in the prevention of heart
acquired heart diseases. These advances make it necessary to disease during childhood. Eligibility recommendations for
update this book. Extensive updating and revisions have been athletic participation have been updated based on the new
made throughout the book at the level that is appropriate for 14-point evaluation, new classification of sports, and rec-
cardiology fellows, primary care physicians, residents, medi- ommendations for specific heart conditions based on a
cal students, and other health care providers. This compre- recent American Heart Association and American College of
hensive book will also serve as a quick review for practicing Cardiology Joint Scientific Statement.
cardiologists. Any health care provider who is interested in Sections dealing with BP and systemic hypertension have
learning about pediatric cardiology topics will also find this been expanded because the normal BP standards based on
book very informative. Despite extensive revision, the book the age and height percentile published by the National
maintains its original goal of providing practitioners with High Blood Pressure Education Program (HBPEP) are not
fundamental and practical information for the management only scientifically and logically unsound but also impractical
of children with cardiac problems. Thus, the general layout for busy practitioners to follow. This is an important issue
of the book has been preserved to serve as a small reference because the consequences of the diagnosis and management
book, avoiding excessive theoretical and sometimes contro- of hypertension based on scientifically unsound BP stan-
versial discussions or detailed surgical descriptions com- dards are immense. As such, this important topic is discussed
monly found in subspecialty textbooks. in depth, and borderline blood pressure levels that require
Although every topic and chapter has been updated, physicians’ attention are presented with data from the San
certain topics were given more revision than others. Antonio Children’s Blood Pressure Study. The data by the
Moderate revisions were made to the following topics: non- HBPEP are presented in the Appendix only for the sake of
invasive imaging techniques, device management of certain completeness. In addition, often neglected basic knowledge
heart conditions, management of selected congenital heart of BP measurement in clinical practice has been reviewed in
defects, infective endocarditis, acute rheumatic fever, car- some detail. The new 2017 classification of BP levels for chil-
diomyopathies, cardiac arrhythmias, congestive heart fail- dren and adults are presented along with the revised recom-
ure, pulmonary hypertension, ambulatory blood pressure mendations.
(BP) monitoring, and syncope in children. Major revisions I am very pleased to report that my long-time friend, Dr.
were made to the following topics: pulse oximetry, Kawasaki Mehrdad Salamat, has accepted my invitation to become a
disease, long and short QT syndromes, pediatric preventive coauthor of this book and to become the primary author
cardiology, and athletes’ sports participation. The neona- of the future editions of the book. Dr. Salamat is an ardent
tal pulse oximetry screening was extensively revised with teacher of pediatric cardiology, beloved by medical students,
inclusion of scientific background of the technique along residents, pediatricians, nursing staff, and his peers. His par-
with recommended algorithm by the American Academy of ticipation as the coauthor has made this revision more objec-
Pediatrics. Kawasaki disease was extensively updated, includ- tive and balanced. I am certain that Dr. Salamat will continue
ing evaluation of suspected incomplete Kawasaki disease with to maintain the original goal of this book in the future. I wish
a recommended algorithm. Cardiac arrhythmias, especially him the best in carrying out the paramount responsibility.
supraventricular tachycardia, long QT syndrome, and short Myung K. Park, MD
QT syndrome, received significant amount of revision. A San Antonio, Texas
vi
F R E Q U E N T LY U S E D A B B R E V I AT I O N S
AR aortic regurgitation MS mitral stenosis
AS aortic stenosis MVP mitral valve prolapse
ASA atrial septal aneurysm PA pulmonary artery or arterial
ASD atrial septal defect PAC premature atrial contraction
AV atrioventricular PAPVR partial anomalous pulmonary venous
BDG bidirectional Glenn operation return
BP blood pressure PBF pulmonary blood flow
BT Blalock-Taussig PDA patent ductus arteriosus
BVH biventricular hypertrophy PFO patent foramen ovale
CAD coronary artery disease PPHN persistent pulmonary hypertension of
CHD congenital heart disease (or defect) newborn
CHF congestive heart failure PR pulmonary regurgitation
COA coarctation of the aorta PS pulmonary stenosis
CV cardiovascular PVC premature ventricular contraction
DCM dilated cardiomyopathy PVOD pulmonary vascular obstructive disease
DORV double-outlet right ventricle PVR pulmonary vascular resistance
D-TGA D-transposition of the great arteries RA right atrium
ECD endocardial cushion defect RAD right axis deviation
echo echocardiography or RAH right atrial hypertrophy
echocardiographic RBBB right bundle branch block
EF ejection fraction RPA right pulmonary artery
FS fractional shortening RV right ventricle or ventricular
HCM hypertrophic cardiomyopathy RVH right ventricular hypertrophy
HOCM hypertrophic obstructive RVOT right ventricular outflow tract
cardiomyopathy S1 first heart sound
HLHS hypoplastic left heart syndrome S2 second heart sound
ICD implantable cardioverter–defibrillator S3 third heart sound
IRBBB incomplete right bundle branch block S4 fourth heart sound
IVC inferior vena cava SBE subacute bacterial endocarditis
LA left atrium SEM systolic ejection murmur
LAD left axis deviation SVC superior vena cava
LAH left atrial hypertrophy SVR systemic vascular resistance
LBBB left bundle branch block SVT supraventricular tachycardia
LPA left pulmonary artery TAPVR total anomalous pulmonary venous
L-TGA L-transposition of the great arteries return
LV left ventricle or ventricular TGA transposition of the great arteries
LVH left ventricular hypertrophy TOF tetralogy of Fallot
LVOT left ventricular outflow tract TR tricuspid regurgitation
MAPCAs multiple aortopulmonary collateral TS tricuspid stenosis
arteries VSD ventricular septal defect
MPA main pulmonary artery VT ventricular tachycardia
MR mitral regurgitation WPW Wolff-Parkinson-White
vii
1
History Taking
OUTLINE
Gestational and Natal History, 2 Chest Pain, 4
Infections, 2 Syncope, 4
Medications, Alcohol, and Smoking, 2 Palpitation, 4
Maternal Conditions, 2 Joint Symptoms, 4
Birth Weight, 3 Neurologic Symptoms, 5
Postnatal History, 3 Medications, 5
Weight Gain, Development, and Feeding Pattern, 3 Family History, 5
Cyanosis, “Cyanotic Spells,” and Squatting, 3 Hereditary Disease, 5
Tachypnea, Dyspnea, and Puffy Eyelids, 3 Congenital Heart Disease, 5
Frequency of Respiratory Infections, 3 Rheumatic Fever, 5
Exercise Intolerance, 3 Hypertension and Atherosclerosis, 5
Heart Murmur, 4
As in the evaluation of any other system, history taking is atrial septal defect (ASD), and transposition of the
a basic step in cardiac evaluation. Maternal history during great arteries (TGA).
pregnancy is often helpful in the diagnosis of congenital b. Anticonvulsants are suspected of causing CHD. Phe-
heart disease (CHD) because certain prenatal events are nytoin (Dilantin) has been associated with pulmonary
known to be teratogenic. Past history, including the imme- stenosis (PS), aortic stenosis (AS), coarctation of the
diate postnatal period, provides more direct information aorta (COA), and PDA. Trimethadione (Tridione) has
relevant to the cardiac evaluation. Family history also helps been associated with TGA, tetralogy of Fallot (TOF),
link a cardiac problem to other medical problems that may and hypoplastic left heart syndrome (HLHS).
be prevalent in the family. Box 1.1 lists important aspects of c. Angiotensin-converting enzyme (ACE) inhibitors (cap-
history taking for children with potential cardiac problems. topril, enalapril, lisinopril) and angiotensin II receptor
antagonists (losartan) taken during the first trimester
GESTATIONAL AND NATAL HISTORY have been reported to cause congenital malformations
of multiple systems, including cardiac defects (e.g.,
Infections, medications, and excessive alcohol intake may ASD, VSD, PDA, and PS).
cause CHD, especially if they occur early in pregnancy. d. Lithium has been associated with Ebstein’s anomaly.
e. Retinoic acid may cause conotruncal anomalies.
Infections
f. Valproic acid may be associated with various heart
1. Maternal rubella infection during the first trimester of defects such as ASD, VSD, aortic stenosis, pulmonary
pregnancy commonly results in multiple anomalies, atresia with intact ventricular septum, and COA.
including cardiac defects. g. Other medications suspected of causing CHD (VSD,
2. Infections by cytomegalovirus, herpesvirus, and coxsack- TOF, TGA) include progesterone and estrogen.
ievirus B are suspected to be teratogenic if they occur in 2. Excessive alcohol intake during pregnancy has been associ-
early pregnancy. Infections by these viruses later in preg- ated with VSD, PDA, ASD, and TOF (fetal alcohol syndrome).
nancy may cause myocarditis. 3. Although cigarette smoking has not been proved to be
3. Human immunodeficiency virus infection has been asso- teratogenic, it does cause intrauterine growth retardation.
ciated with infantile cardiomyopathy.
Maternal Conditions
Medications, Alcohol, and Smoking
1. There is a high incidence of cardiomyopathy in infants
1. Several medications are suspected teratogens. born to mothers with diabetes. In addition, these babies
a. Amphetamines have been associated with ventricular have a higher incidence of structural heart defects (e.g.,
septal defect (VSD), patent ductus arteriosus (PDA), TGA, VSD, PDA, ECD, COA, HLHS).
2
CHAPTER 1 History Taking 3
BOX 1.1 Selected Aspects of History recent onset may be an early sign of CHF in infants, especially
Taking if the poor feeding is the result of fatigue and dyspnea.
Gestational and Natal History Cyanosis, “Cyanotic Spells,” and Squatting
Infections, medications, excessive smoking, or alcohol intake
The presence of cyanosis should be assessed. If the parents
during pregnancy
think that their child has cyanosis, the physician should ask
Birth weight
them about the onset (e.g., at birth, several days after birth),
Postnatal, Past, and Present History severity of cyanosis, permanent or paroxysmal nature, parts
Weight gain, development, and feeding pattern of the body that were cyanotic (e.g., fingers, toes, lips), and
Cyanosis, “cyanotic spells,” and squatting whether the cyanosis becomes worse after feeding. Evanescent
Tachypnea, dyspnea, puffy eyelids acrocyanosis is normal in neonates.
Frequency of respiratory infection A “cyanotic spell” is seen most frequently in infants with
Exercise intolerance
TOF and requires immediate attention, although it has become
Heart murmur
less frequent because most surgical repairs are done in early
Chest pain
infancy. Physicians should ask about the time of its appearance
Syncope
(e.g., in the morning on awakening, after feeding), duration
Palpitation
of the spells, and frequency of the spells. Most important is
Joint symptoms
Neurologic symptoms whether infants were breathing fast and deep during the spell
Medications or were holding their breath. This helps differentiate between
a true cyanotic spell and a breath-holding spell.
Family History The physician should ask whether the child squats when
Hereditary disease
tired or has a favorite body position (e.g., knee–chest posi-
Congenital heart defect
tion) when tired. Squatting strongly suggests cyanotic heart
Rheumatic fever
disease, particularly TOF. Fortunately, squatting is extremely
Sudden unexpected death
rare now with early surgical repair of cyanotic CHDs.
Diabetes mellitus, arteriosclerotic heart disease, hyperten-
sion, dyslipidemia, and so on
Tachypnea, Dyspnea, and Puffy Eyelids
Tachypnea, dyspnea, and puffy eyelids are signs of CHF. Left-
sided heart failure produces tachypnea with or without dys-
2. Maternal lupus erythematosus and mixed connective tis- pnea. Tachypnea becomes worse with feeding and eventually
sue disease have been associated with a high incidence of results in poor feeding and poor weight gain. A sleeping respi-
congenital heart block in offspring secondary to placental ratory rate of more than 40 breaths/min is noteworthy. A rate
crossing of maternal anti-Ro and anti-La antibodies. of more than 60 breaths/min is abnormal, even in a newborn.
3. The incidence of CHD increases from about 1% in the gen- Wheezing or persistent cough at night may be an early sign
eral population to as much as 15% if the mother has CHD, of CHF. Puffy eyelids and sacral edema are signs of systemic
even if it is postoperative (see Table A.2 in Appendix A). venous congestion. Ankle edema, which is commonly seen in
adults, is not found in infants.
Birth Weight
Frequency of Respiratory Infections
Birth weight provides important information about the
nature of the cardiac problem. Congenital heart diseases with large left-to-right shunt and
1. If an infant is small for gestational age, it may indicate increased pulmonary blood flow predispose to lower respira-
intrauterine infections or use of chemicals or drugs by the tory tract infections. Frequent upper respiratory tract infec-
mother. Rubella syndrome and fetal alcohol syndrome are tions are not related to CHD, although children with vascular
typical examples. rings may sound as if they have a chronic upper respiratory
2. Infants with high birth weight, often seen in offspring of tract infection.
mothers with diabetes, show a higher incidence of cardiac
Exercise Intolerance
anomalies. Infants with TGA often have birth weights
higher than average; these infants have cyanosis. Decreased exercise tolerance may result from any significant
heart disease, including large left-to-right shunt lesions, cya-
POSTNATAL HISTORY notic defects, valvular stenosis or regurgitation, and arrhyth-
mias. Obese children may be inactive and have decreased
Weight Gain, Development, and Feeding Pattern
exercise tolerance in the absence of heart disease. A good assess-
Weight gain and general development may be delayed in ment of exercise tolerance can be obtained by asking the follow-
infants and children with congestive heart failure (CHF) or ing questions: Does the child keep up with other children? How
severe cyanosis. Weight is affected more significantly than many blocks can the child walk or run? How many flights of
height. If weight is severely affected, physicians should sus- stairs can the child climb without fatigue? Does the weather or
pect a more general dysmorphic condition. Poor feeding of the time of day influence the child’s exercise tolerance?
4 PART 1 Basic Tools in Routine Evaluation of Cardiac Patients
With infants who do not walk or run, an estimate of exer- Gastroesophageal reflux and exercise-induced asthma are
cise tolerance can be gained from the infant’s history of feed- other recognizable causes of noncardiac chest pain in chil-
ing pattern. Parents often report that the child takes naps; dren. Exercise-induced asthma (or bronchospasm) typically
however, many normal children nap regularly. occurs 5 to 10 minutes into vigorous physical activities in a
child with asthma or with inadequately treated asthma. It can
Heart Murmur
occur in a child previously undiagnosed with asthma. A psy-
If a heart murmur is the chief complaint, the physician chogenic cause of chest pain is also possible; parents should
should obtain information about the time of its first appear- be asked whether there has been a recent cardiac death in the
ance and the circumstances of its discovery. A heart murmur family.
heard within a few hours of birth usually indicates a stenotic
Syncope
lesion (AS, PS), atrioventricular (AV) valve regurgitation, or
small left-to-right shunt lesions (VSD, PDA). The murmur of Syncope is a transient loss of consciousness and muscle tone
large left-to-right shunt lesions, such as VSD or PDA, may that result from inadequate cerebral perfusion. Dizziness is
be delayed because of slow regression of pulmonary vascu- the most common prodromal symptom of syncope. These
lar resistance. In the case of stenotic lesion, the onset of the complaints could represent a serious cardiac condition that
murmur is not affected by the pulmonary vascular resistance, may result in sudden death. It may also be due to noncar-
and the murmur is usually heard shortly after birth. A heart diac causes, such as benign vasovagal syncope, dehydration,
murmur that is first noticed on a routine examination of a metabolic abnormalities, or neuropsychiatric disorders.
healthy-looking child is more likely to be innocent, especially Dehydration or inadequate hydration is an important con-
if the same physician has been following the child’s progress. tributing factor.
A febrile illness is often associated with the discovery of a A history of exertional syncope may suggest arrhythmias
heart murmur. (particularly ventricular arrhythmias, such as seen in long QT
syndrome, hypertrophic cardiomyopathy, or severe obstruc-
Chest Pain
tive lesions, e.g., severe AS). Syncope provoked by exer-
Chest pain is a common reason for referral and parental anx- cise, that is accompanied by chest pain, or with a history of
iety. If chest pain is the primary complaint, the physician asks unoperated or operated heart disease suggests potential car-
whether the pain is activity related (e.g., Do you have chest diac cause of syncope. Syncope while sitting down suggests
pain only when you are active, or does it come even when you arrhythmias or seizure disorders. Syncope while standing for
watch television?). The physician also asks about the duration a long time suggests vasovagal syncope (often in association
(e.g., seconds, minutes, hours) and nature of the pain (e.g., with dehydration) without an underlying cardiac disease; this
sharp, stabbing, squeezing) and radiation to other parts of the is the most common syncope in children (see Chapter 31 for
body (e.g., neck, left shoulder, left arm). Chest pain of cardiac further discussion). Hypoglycemia is a very rare cause of syn-
origin is not sharp; it manifests as a deep, heavy pressure or cope occurring in the morning. Syncopal duration less than
the feeling of choking or a squeezing sensation, and it is usu- 1 minute suggests vasovagal syncope, hyperventilation, or
ally triggered by exercise. The physician should ask whether syncope caused by another orthostatic mechanism. A longer
deep breathing improves or worsens the pain. Pain of cardiac duration of syncope suggests convulsive disorders, migraine,
origin, except for pericarditis, is not affected by respiration. or cardiac arrhythmias.
Cardiac conditions that may cause chest pain include Family history should include coronary heart disease risk
severe AS (usually associated with activity), pulmonary factors, including history of myocardial infarction in family
hypertension or pulmonary vascular obstructive disease, and members younger than 30 years of age, cardiac arrhythmia,
mitral valve prolapse (MVP). Chest pain in MVP is not nec- CHD, cardiomyopathies, long QT syndrome, seizures, met-
essarily associated with activity, but there may be a history abolic and psychological disorders. A detailed discussion of
of palpitation. There is increasing doubt about the relation- this topic is presented in Chapter 31.
ship between chest pain and MVP in children. Less common
Palpitation
cardiac conditions that can cause chest pain include severe
PS, pericarditis of various causes, and current or history of Palpitation is a subjective feeling of rapid heartbeats. Some
Kawasaki’s disease (in which stenosis or aneurysm of the cor- parents and children report sinus tachycardia as palpitation.
onary artery is common). Paroxysms of tachycardia (e.g., supraventricular tachycardia)
Most children complaining of chest pain do not have a car- or single premature beats commonly cause palpitation (see
diac condition (see Chapter 30); cardiac causes of chest pain Chapter 32). Children with hyperthyroidism or MVP may
are rare in children and adolescents. The three most common first be taken to the physician because of complaints of pal-
noncardiac causes of chest pain in children are costochondri- pitation.
tis, trauma to the chest wall or muscle strain, and respiratory
Joint Symptoms
diseases with cough (e.g., bronchitis, asthma, pneumonia,
pleuritis). The physician should ask whether the patient has When joint pain is the primary complaint, acute rheumatic
experienced recent trauma to the chest or has engaged in arthritis or rheumatoid arthritis is a possibility, although the
activity that may have resulted in pectoralis muscle soreness. incidence of the former has dramatically decreased in recent
CHAPTER 1 History Taking 5
years in the United States. The number of joints involved, lentigines, formerly called LEOPARD syndrome) is often
duration of the symptom, and migratory or stationary nature associated with PS and cardiomyopathy. Selected hereditary
of the pain are important. Arthritis of acute rheumatic fever diseases in which cardiovascular disease is frequently found
typically involves large joints, either simultaneously or in suc- are listed in Table 2.1 along with other nonhereditary syn-
cession, with a characteristic migratory nature. Pain in rheu- dromes.
matic joint is so severe that children refuse to walk. A history
Congenital Heart Disease
of recent sore throat (and throat culture results) or rashes
suggestive of scarlet fever may be helpful. The physician also The incidence of CHD in the general population is about
asks whether the joint was swollen, red, hot, or tender (see 1% or, more precisely, 8 to 12 of 1000 live births. This does
Chapter 20 for further discussion). not include PDA in premature infants. The recurrence risk
of CHD associated with inherited diseases or chromosomal
Neurologic Symptoms
abnormalities is related to the recurrent risk of the syndrome.
A history of stroke suggests thromboembolism secondary to A history of CHD in close relatives increases the chance
cyanotic CHD with polycythemia or infective endocarditis. of CHD in a child. In general, when one child is affected, the
In the absence of cyanosis, stroke can rarely be caused by par- risk of recurrence in siblings is about 3%, which is a threefold
adoxical embolism of a venous thrombus through an ASD. increase. Having a child with hypoplastic left heart syndrome
Although very rare, primary hypercoagulable states should (HLHS) increases the risk of CHD in subsequent child (to
also be considered which include such conditions as factor approximately 10%) and most centers perform fetal echocar-
V Leiden thrombophilia, antithrombin III deficiency, protein diography. The risk of recurrence is related to the prevalence
C deficiency, protein S deficiency, disorders of fibrinolytic of particular defects. Whereas lesions with a higher prev-
system (e.g., hypoplasminogenemia, abnormal plasminogen, alence (e.g., VSD) tend to have a higher risk of recurrence,
plasminogen activator deficiency), dysfibrinogenemia, factor lesions with a lower prevalence (e.g., tricuspid atresia, per-
XII deficiency, and lupus anticoagulant (Barger, 2000). A host sistent truncus arteriosus) have a lower risk of recurrence.
of other conditions cause secondary hypercoagulable states. Table A.1 in Appendix A lists the recurrence risk figures for
A history of headache may be a manifestation of cerebral various CHDs, which can be used for counseling. The impor-
hypoxia with cyanotic heart disease, severe polycythemia, tance of cytoplasmic inheritance has recently been shown in
or brain abscess in cyanotic children. Although it is claimed some families based on the observation that the recurrence
to occur in adults, hypertension with or without COA rarely risk is substantially higher if the mother is the affected parent
causes headaches in children. Choreic movement strongly (see Table A.2 in Appendix A).
suggests rheumatic fever.
Rheumatic Fever
Medications
Rheumatic fever frequently occurs in more than one family
Physicians should note the name, dosage, timing, and dura- member. There is a higher incidence of the condition among
tion of cardiac and noncardiac medications. Medications may relatives of children with rheumatic fever. Although the
be responsible for the chief complaint of the visit or certain knowledge of genetic factors involved in rheumatic fever is
physical findings. Tachycardia and palpitation may be caused incomplete, it is generally agreed that there is an inherited
by cold medications or antiasthmatic drugs. susceptibility to acquiring rheumatic fever.
A history of tobacco and illicit drug use, which could be
Hypertension and Atherosclerosis
the cause of chief complaints, should be obtained, preferably
through a private interview with the child. Essential hypertension and coronary artery disease show a
strong familial pattern. Therefore, when a physician suspects
FAMILY HISTORY hypertension in a young person, it is important to obtain
family history of hypertension. Atherosclerosis results from a
Hereditary Disease
complex process in which hereditary and environmental fac-
Some hereditary diseases may be associated with certain tors interact. The most important risk factor for atherosclero-
forms of CHD. For example, Marfan’s syndrome is fre- sis is the positive family history with coronary heart disease
quently associated with aortic aneurysm or with aortic or occurring before age 55 years in one’s father or grandfather
mitral insufficiency. Holt-Oram syndrome (ASD and limb and before age 65 years in one’s mother or grandmother.
abnormalities), long-QT syndrome (sudden death caused Clustering of cardiovascular risk factors occur frequently in
by ventricular arrhythmias), and idiopathic sudden death in the same individual (metabolic syndrome), which calls for
the family should be inquired about. PS secondary to a dys- investigation for other risk factors when one risk factor is
plastic pulmonary valve is common in Noonan’s syndrome. found. Detailed discussion of cardiovascular risk factors is
Lentiginous skin lesion (Noonan syndrome with multiple presented in Chapter 33.