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loud that it becomes palpable , such as in Eisenmenger syndrome . 34 An ECG is helpful as it shows RV hypertrophy .
The heart rate is slower in later childhood and adolescence , so it may then be possible to separate the two components of the second heart sounds , where a louder second component ( pulmonary closure ) suggests pulmonary hypertension .
Pulmonary primary hypertension is rare and , despite the current available management , still has a poor longterm prognosis . There is often a delay of six months or longer before the diagnosis is considered , particularly in a child with increasing breathlessness on exertion . Identifying a louder pulmonary closure suggests pulmonary hypertension , further aided by an ECG showing RV hypertrophy . Confirmation of the diagnosis and starting appropriate treatment may improve the short- and long-term outlook . 35
Pulmonary hypertension may occasionally be the only sign pres-
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Alicia Fagerving ( WMSE ), Laboratoires Servier / CC BY-SA : bit . ly / 3wXlMxq |
Box 2 . Features that should prompt referral in infancy and childhood
• Tachypnoea .
• Differential pulses .
• Cyanosis .
• Poor weight gain .
• If the infant or child is dysmorphic or has syndromic features suggestive of a genetic disorder .
• Genetic disorders , even in the absence of a murmur , may warrant referral because a cardiac abnormality — either a congenital anomaly or a cardiomyopathy — may be present . — For example , children with a hypermobility connective tissue disorder may develop mitral valve prolapse and mitral incompetence , a dilated aortic root and ascending aorta and require long-term surveillance .
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ent despite a serious congenital heart |
Source : Rashed ER et al 2022 33 |
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abnormality , such as cor triatriatum , |
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supravalvular mitral stenosis , a parachute |
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mitral valve or a smallish LV . |
unrecognised cardiac abnormality , is |
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There are generally no murmurs . 34 The rise in the pulmonary venous |
rarely found . A careful history may uncover a |
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pressures leads to a rise in the pulmo- |
history of asthma ; the parents some- |
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nary arterial pressures as evidenced |
times refer to this as bronchitis or |
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by a louder pulmonary closure or |
bronchiolitis , which has now evolved |
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summated second heart sound in the pulmonary area . Diagnosis and appropriate treatment of surgically |
into exercise-induced bronchospasm . 38 The development of a cough and wheeze , especially on stopping |
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correctable lesions may result in good |
the exercise , supports the diagnosis . |
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long-term outcomes . |
Provocative pulmonary function studies |
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may be helpful or , alternatively , a |
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PALPITATIONS |
therapeutic trial with a bronchodilator |
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This symptom , especially common |
before sustained activity may aid the |
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in later childhood and adolescence , |
diagnosis . |
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is rarely associated with an arrhythmia . 12 The issues related to the diagnosis of SVT in the newborn and in early |
Figure 5 . Coarctation of the aorta . |
CHEST PAIN Left-sided chest pain is common and |
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infancy have been discusses earlier . 29 |
usually attributed to the heart . How- |
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The presence of pre-excitation on |
ever , such pain is often experienced |
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the interval ECG indicates an ante- |
while at rest and rarely with exer- |
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grade conducting pathway . A normal |
cise , with the child able to run with- |
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ECG does not exclude an accessory |
out developing chest pain . Ischaemic |
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pathway with retrograde conduc- |
chest pain is rare in childhood . |
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tion . A careful history may suggest |
Consider ischaemic pain in any |
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ectopic beats rather than palpitations ; |
child / adolescent who has had Kawa- |
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patients complain of a strong beat |
saki disease and has persistent cor- |
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that relates to the post-ectopic beat , which has a greater stroke volume because of greater filling of the ven- |
onary aneurysms , especially a giant aneurysm . 9 If in doubt , especially in older children or adolescents , a stress |
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tricles . A 24-hour Holter will deter- |
exercise test can be helpful . |
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mine whether the ectopics are atrial |
Be mindful that non-specific chest |
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or ventricular in origin , as well as the |
pain may be a manifestation of myo- |
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overall ectopic load . Treatment is sel- |
carditis / pericarditis as was seen in the |
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dom required and is only necessary if |
recent COVID-19 pandemic following |
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there are runs of ectopics , especially arising from the ventricle . |
11 , 39 the Pfizer and Moderna vaccines . Occasionally , adolescents may present |
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The sudden onset and sudden |
with a giant pericardial effusion asso- |
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decay of a rapid heart rate suggest a |
ciated with non-specific chest pain . 40 |
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probable diagnosis of an arrhythmia , |
Rarely , there is an anomalous ori- |
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such as SVT . This is almost the only |
gin of the coronary arteries , especially |
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tachyarrhythmia seen in childhood and |
the left coronary artery arising from |
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adolescence , particularly if the heart is normal .
In contrast , a sudden onset followed by a gradual decay , or a gradual onset and decay , is unlikely to be caused by
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Figure 6 . Neonatal post ductal pulse oximetry .
the order of 150-160bpm ; this may
|
frequently disappear ; they rarely lead |
cardiac electrophysiologist is war- |
the right coronary sinus ; this may lead to ischaemic or non-specific chest pain . The anomaly is usually readily detected on echocardiogram . 41 If in doubt , a CT angiogram will clarify the |
an arrhythmia . Consider other causes |
be slightly irregular compared with a |
to EAT . |
ranted as the diagnosis is complex , the |
diagnosis . Localised coronary artery |
for the probable sinus tachycardia . |
regular rapid heart rate of the order |
An exercise stress test may be indi- |
treatment problematic and the out- |
stenosis remains a nightmare — even |
Adolescents are usually able to dis- |
of 180bpm or more seen with SVT . |
cated if there are multifocal ventricu- |
come guarded . 37 |
for the cardiologist . |
tinguish between the rapid heart rate |
The diagnosis is made even more dif- |
lar ectopics to exclude the possibility |
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experienced with exercise and the |
ficult as the ectopic atrial focus often |
of catecholaminergic polymor- |
BREATHLESSNESS WITH ACTIVITY |
CARDIOMYOPATHY |
rapid heart rate that may arise with the |
looks like a normal P wave . A 24-hour |
phic ventricular tachycardia . This is |
Children and adolescents with breath- |
A family history of cardiomyopathy |
onset of an arrhythmia . Documenting |
Holter is helpful , particularly if there |
important as catecholaminergic pol- |
lessness while playing sport are often |
prompts referral of children for an |
an episode of the tachycardia is essential to make a diagnosis , initiate appropriate intervention and transition from |
is a high average rate that fails to settle overnight . Note that a prolonged delay in the diagnosis of EAT may |
ymorphic ventricular tachycardia may result in sudden death in otherwise-healthy children . 36 |
referred for exclusion of a cardiac cause . Further investigation may be warranted if their shortness of breath is worse |
echocardiogram to ensure they do not have a similar problem . 42 If a genetic cause is found in the index patient , and |
oral medication to an electrophysiol- |
result in a dilated cardiomyopathy |
Sudden collapse , especially if |
than that experienced by their peers |
this is not detected in the child , ongo- |
ogy study and ablation . |
from the incessant tachycardia . Atrial |
occurring after activity , raises the pos- |
with the same degree of physical activ- |
ing surveillance and echocardiograp- |
Ectopic atrial tachycardia ( EAT ) |
ectopics are common prenatally and |
sibility of a channelopathy , such as |
ity . However , a cardiac cause , such as |
ghy are not needed . That is particularly |
may produce a raised heart rate in |
into the newborn period and then |