1
Case Report :CONGESTIVE HEART FAILURE
Presenter : Thirukumaran Thiagarajan (100100274): Kanagavalli
Vijayakumar (100100403)Supervisor: dr. Lily Irsa, Sp. A (K)
INTRODUCTION
Congenital heart disease occurs in 0.50.8% of live births. The
incidence is higher in stillborns (34%), abortuses (1025%), and
premature infants (about 2% excluding patent ductus arteriosus
[PDA]). Congenital cardiac defects have a wide spectrum of severity
in infants: about 23 in 1,000 newborn infants will be symptomatic
with heart disease in the 1st year of life. The diagnosis is
established by 1 week of age in 4050% of patients with congenital
heart disease and by 1 month of age in 5060% of patients.1 The
initial evaluation for suspected congenital heart disease involves
a systematic approach with three major components. First,
congenital cardiac defects can be divided into two major groups
based on the presence or absence of cyanosis, which can be
determined by physical examination aided by pulse oximetry. Second,
these two groups can be further subdivided according to whether the
chest radiograph shows evidence of increased, normal, or decreased
pulmonary vascular markings. Finally, the electrocardiogram can be
used to determine whether right, left, or biventricular hypertrophy
exists. The character of the heart sounds and the presence and
character of any murmurs further narrow the differential diagnosis.
The final diagnosis is then confirmed by echocardiography or
cardiac catheterization, or by both.2 Congestive heart failure is
the clinical condition in which the heart fails to meet the
circulatory and metabolic needs of the body. Almost all infants who
will develop congestive heart failure from congenital heart disease
do so by age 6 months.3 The clinical syndrome of congestive heart
failure as seen in infants and children represents the inability of
the heart and circulation to meet the metabolic demands of the body
despite various compensatory hemodynamic and neurohumoral
mechanisms. This clinical picture may emerge when the myocardium is
subjected to excessive loading conditions (volume and/or pressure),
primary alterations in contractile function, marked changes in
chronotropic state (tachy- or bradydysrhythmias), or various
combinations of these factors. As a result, signs develop of
pulmonary and systemic venous congestion, impaired systemic
perfusion, and findings that indicate such adaptive mechanisms as
changes in heart rate, vasoconstrictor tone, renal function, and
ventricular hypertrophy. Over time, these changes may become
maladaptive.4
DEFINITIONCongestive heart failure (CHF) is a clinical syndrome
in which the heart is unable to pump enough blood to the body to
meet its needs, to dispose of venous return adequately, or a
combination of the two. 5
ETIOLOGY5Common causes of Congestive heart failure (CHF) are
volume or pressure overload, or both, caused by congenital heart
disease, acquired heart disease and myocardial diseases.
Tachyarrhythmias and heart block can also cause heart failure at
any age. By far the most common causes of CHF in infancy are
congenital heart diseases (CHDs). Beyond infancy, myocardial
dysfunctions of various etiologies are important causes of CHF.
Among the rare causes of CHF are metabolic and endocrine disorders,
anemia, pulmonary diseases, collagen vascular diseases, systemic or
pulmonary hypertension, neuromuscular disorders, and drugs such as
anthracyclines.
a) Congenital Heart Disease (CHD)Volume overload lesions such as
ventricular septal defect (VSD), patent ductus arteriosus (PDA),
and endocardial cushion defect (ECD) are the most common causes of
CHF in the first 6 months of life. In infancy, the time of the
onset of CHF varies predictably with the type of defect. Table 1
list commons defects according to the age at which CHF develops.
When looking at the table, the following should also be noted. 1.
Children with tetralogy of Fallot (TOF) do not develop CHF unless
they have received a large aortatopulmonary artery (PA) shunt
procedure, for example, too large a Gore-Tex interposition shunt
(modified Blalock-Taussig shunt).
2. Atrial septal defect (ASD) rarely causes CHF in the pediatric
age group, although it causes CHF in adulthood.
3. Large left-to-right shunt lesions, such as VSD and PDA, do
not cause CHF before 6 to 8 weeks of age because the pulmonary
vascular resistance does not fall low enough to cause a large
left-to-right shunt until this age. The onset of CHF resulting from
these left-to-right shunt lesions may be earlier in premature
infants (within the first month) because of an earlier fall in the
pulmonary vascular resistance in these infants.
Table 1: Causes of Congestive Heart Failure Resulting from
Congenital Heart DiseaseAge of OnsetCause
At birthHLHS
Volume overload lesions:
Severe tricuspid or pulmonary insufficiency
Large systemic arteriovenous fistula
First weekTGA
PDA in small premature infants
HLHS (with more favorable anatomy)
TAPVR, particularly those with pulmonary venous obstruction
Others:
Systemic arteriovenous fistula
Critical AS or PS
1st 4th weekCOA with associated anomalies
Critical AS
Large left-to-right shunt lesions (VSD, PDA) in premature
infants
All other lesions previously listed
4th 6th weekSome left-to-right shunt lesions such as ECD
6th week4th monthLarge VSD
Large PDA
Others such as anomalous left coronary artery from the PA
AS, aortic stenosis; COA, coarctation of the aorta; ECD,
endocardial cushion defect; HLHS, hypoplastic left heart syndrome;
PA, pulmonary artery; PDA, patent ductus arteriosus; PS, pulmonary
stenosis; TAPVR, total anomalous pulmonary venous return; TGA,
transposition of the great arteries; VSD, ventricular septal
defect.
b) Acquired Heart Disease Acquired heart disease of various
causes can lead to CHF. With acquired heart disease, the age at
onset of CHF is not as predictable as with CHD, but the following
generalities apply: 1. Endocardial fibroelastosis, a rare primary
myocardial disease, causes CHF in infancy; 90% of cases occur in
the first 8 months of life. 2. Viral myocarditis tends to be more
common in small children older than 1 year. It occurs occasionally
in the newborn period, with a fulminating clinical course with poor
prognosis. 3. Myocarditis associated with Kawasaki disease is seen
in children 1 to 4 years of age. 4. Acute rheumatic carditis is an
occasional cause of CHF that occurs primarily in school-age
children. 5. Rheumatic valvular heart diseases, usually volume
overload lesions such as mitral regurgitation (MR) or aortic
regurgitation (AR), cause CHF in older children and adults. These
diseases are uncommon in industrialized countries. 6. Dilated
cardiomyopathy may cause CHF at any age during childhood and
adolescence. The cause of the majority of dilated cardiomyopathy is
idiopathic, but it may be caused by infectious, endocrine, or
metabolic disorders or autoimmune diseases or may follow
antineoplastic treatment (e.g., anthracycline). 7.Doxorubicin
cardiomyopathy may manifest months to years after the completion of
chemotherapy for malignancies in children. 8.Cardiomyopathies
associated with muscular dystrophy and Friedreich's ataxia may
cause CHF in older children and adolescents. 9.Patients who
received surgery for some types of CHD (such as a Fontan operation,
surgery for TOF, transposition of the great arteries, and other
cyanotic defects) may remain in or develop CHF.
c) Miscellaneous Causes Miscellaneous causes of CHF include the
following: 1.Metabolic abnormalities (severe hypoxia and acidosis,
as well as hypoglycemia and hypocalcemia) can cause CHF in
newborns. 2. Endocrinopathy such as hyperthyroidism can cause CHF.
3. Supraventricular tachycardia (SVT) causes CHF in early infancy.
4. Complete heart block associated with structural heart defects
causes CHF in the newborn period or early infancy. 5. Severe anemia
may be a cause of CHF at any age. Hydrops fetalis may be a cause of
CHF in the newborn period and severe sicklemia at a later age.
6.Bronchopulmonary dysplasia seen in premature infants causes
predominantly right-sided heart failure in the first few months of
life. 7. Primary carnitine deficiency (plasma membrane carnitine
transport defect) causes progressive cardiomyopathy with or without
skeletal muscle weakness that begins at 2 to 4 years of age. 8.
Acute cor pulmonale caused by acute airway obstruction (such as
seen with large tonsils) can cause CHF at any age but most commonly
during early childhood. 9. Acute systemic hypertension, as seen in
acute postinfectious glomerulonephritis, causes CHF in school-age
children. Fluid retention with poor renal function is important as
the cause of hypertension in this condition.
PATHOPHYSIOLOGY4According to the Frank-Starling law, as the
ventricular end-diastolic volume (or preload) increases, the
healthy heart increases cardiac output until a maximum is reached
and cardiac output can no longer be augmented (see Figure. 1 ).
When the left ventricular (LV) end-diastolic pressure reaches a
certain point, pulmonary congestion develops with pulmonary
congestive symptoms (tachypnea and dyspnea). Congestive symptoms
occur even with normally functioning myocardium if the
end-diastolic pressure is greatly increased, such as with infusion
of a large amount of fluid or blood. An increase in the stroke
volume is also achieved in the failing heart when the preload is
increased, but the failing heart does not achieve the same level of
maximal cardiac output as the normal heart, and congestive symptoms
(dyspnea and hepatomegaly results). Figure 1. Effects of
anticongestive medications on the Frank-Starling relationship for
ventricular function. In persons with a normal heart, cardiac
output increases as a function of ventricular filling pressure
(upper curve). In patients with heart failure, the normal
relationship between cardiac output (or stroke volume) and filling
pressure (preload) is shifted lower and to the right such that a
low-output state and congestive symptoms may coincide. Congestive
symptoms (dyspnea, tachypnea) may appear even in a normal heart if
the filling pressure reaches a certain point. At one extreme, the
addition of a pure inotropic agent, such as digoxin, primarily
increases the stroke volume with minimal impact on filling pressure
(so that the patient may still have congestive symptoms).
Conversely, the addition of a diuretic primarily decreases the
filling pressure (with improved congestive symptoms) but without
improving cardiac output. Clinically, it is common to use multiple
classes of agents (usually a combination of inotropic agents,
diuretics, and vasodilators) to produce both increased cardiac
output and decreased filling pressure.The increased stroke volume
results in increased wall tension, which in turn increases oxygen
consumption. Increase in the wall tension is also seen in dilated
ventricular cavity, according to the Laplace law.Wall stress =
pressure ; radius/2 wall thicknessThe Laplace law, although an
oversimplification, emphasizes two points: 1. The bigger the left
ventricle and the greater the radius, the greater the wall
stress.
2. At any given radius (LV size), the greater the pressure
developed in the LV, the greater the wall stress.
In heart failure, cardiac hypertrophy (with increased wall
thickness) develops to balance the increased pressure and keep the
wall stress unchanged, and reduction of heart size decreases wall
stress and improves cardiac output or LV function.Among many
compensatory responses to the failing heart is the activation of
two important neurohormonal mechanisms: the sympathetic nervous
system and the renin-angiotensin-aldosterone system. Although these
responses are an attempt to preserve cardiovascular homeostasis and
thus are beneficial initially, chronic stimulation of these systems
may be deleterious in the natural history of myocardial
dysfunction. 1. One major compensatory mechanism for increasing
cardiac output is an increase in sympathetic tone, secondary to
increased adrenal secretion of circulating epinephrine and
increased neural release of norepinephrine. The initial beneficial
effects of adrenergic stimulation include increased heart rate and
myocardial contractility with resulting increase in cardiac output.
However, chronic adrenergic stimulation eventually leads to adverse
myocardial effects, including increased afterload, hypermetabolism,
arrhythmogenesis, and direct myocardial toxicity.
a) Catecholamines are toxic to cardiac muscle, perhaps by
producing calcium overload or by inhibiting the synthesis of
contractile proteins. b) High catecholamine levels decrease the
density of -adrenergic receptors on the surface of the myocardial
cell, which may be the major cause of functional loss of the
catecholamine-mediated positive inotropic response. In clinical
settings, the reduction of adrenergic stimulation by the use of
-adrenergic blockers has resulted in clinical improvement in
patients with dilated cardiomyopathy, in whom increased levels of
catecholamines have been shown to be present.2. The reduced blood
flow to the kidneys in patients with CHF causes a marked increase
in renin output, and this in turn causes the formation of
angiotensin II. Angiotensin II leads to further increase in
reabsorption of both water and salt from the renal tubules.
Angiotensin II may cause a trophic response in vascular smooth
muscle (with vasoconstriction) and myocardial hypertrophy.
Angiotensin II also promotes myocardial fibrosis. Thus, although
the hypertrophic response is adaptive by attempting to restore wall
stress to normal, angiotensin II plays a maladaptive role in CHF by
initiating fibrosis and altering ventricular compliance. Thus,
reasons for using -adrenergic blockers and angiotensin-converting
enzyme (ACE) inhibitors in the treatment of CHF are to block the
maladaptive role of the adrenergic and
renin-angiotensin-aldosterone systems.
DIAGNOSIS History 1. Poor feeding of recent onset, tachypnea
that worsens during feeding, poor weight gain, and cold sweat on
the forehead suggest CHF in infants. 2. Older children may complain
of shortness of breath, especially with activities, early
fatigability, puffy eyelids, or swollen feet.
Physical ExaminationPhysical findings of CHF may be classified
as follows, depending on their pathophysiologic mechanisms. The
more common findings are in italics. 1 The following are found as
compensatory responses to impaired cardiac function: a.
Tachycardia, gallop rhythm, and weak and thready pulses are common.
b.Cardiomegaly is almost always present. Chest x-ray films are more
reliable than physical examination in demonstrating cardiomegaly.
c.There are signs of increased sympathetic discharges (e.g., growth
failure, perspiration, cold and wet skin).
2. Pulmonary venous congestion (from left-sided failure) results
in the following manifestations: a. Tachypnea is common and is an
early manifestation of CHF in infants. b. Dyspnea on exertion
(equivalent to poor feeding in small infants) is common in
children. c. Orthopnea may be seen in older children. d. Wheezing
and pulmonary crackles are occasionally audible. 3. Systemic venous
congestion (related to right-sided failure) results in the
following: a. Hepatomegaly is common but it is not always
indicative of CHF. A large liver may be palpable in conditions that
cause hyperinflated lungs (asthma, bronchiolitis, during hypoxic
spells) and in infiltrative liver disease. Conversely, the absence
of hepatomegaly does not rule out CHF; hepatomegaly may be absent
in (early) left-sided failure. b. Puffy eyelids are common in
infants. c. Distended neck veins and ankle edema, which are common
in adults, are not seen in infants. d. Splenomegaly is not
indicative of CHF; it usually indicates infection.
X-Ray StudiesThe presence of cardiomegaly should be demonstrated
by chest x-ray films. The absence of cardiomegaly almost rules out
the diagnosis of CHF. The only exception to this rule arises when
the pulmonary venous return is obstructed; in such cases, the lung
parenchyma shows pulmonary edema or venous congestions.
ElectrocardiographyECGs help determine the type of heart defect
causing heart failure but are not helpful in deciding whether CHF
is present.
EchocardiographyEcho studies may confirm enlargement of
ventricular chambers and impaired LV systolic function (decreased
fractional shortening or ejection fraction) as well as impaired
diastolic function by the use of Doppler techniques. A more
important role of echo may be due to its ability to determine the
cause of CHF. Echo is also helpful in serial evaluation of the
efficacy of therapy.
Cardiac CatheterizationEndomyocardial biopsy obtained during
cardiac catheterization offers a new approach to specific diagnosis
of the cause of CHF, such as inflammatory disease, infectious
process, or metabolic disorder. When viral myocarditis is
suspected, the polymerase chain reaction provides a means of
isolating the offending viral agent from biopsy specimens. In a
patient with dilated cardiomyopathy, evaluation of biopsy
specimens, including genetic analysis, may provide data permitting
the diagnosis of specific metabolic causes, such as carnitine
deficiency.TREATMENT4The treatment of CHF consists of (1)
elimination of the underlying causes, (2) treatment of the
precipitating or contributing causes (e.g., infection, anemia,
arrhythmias, fever), and (3) control of the heart failure state.
Eliminating the underlying causes is the most desirable approach
whenever possible. Surgical correction of congenital heart defects
is such an approach. Every patient with CHF should receive maximal
medical treatment, but continuing with long-term anticongestive
measures is unwise when the heart defect can be safely repaired
through surgery. The heart failure state is controlled by the use
of multiple drugs, including inotropic agents, diuretics, and
afterload-reducing agents, along with general supportive measures.
TREATMENT OF UNDERLYING CAUSES OR CONTRIBUTING FACTORS 1. When
surgically feasible, treatment of underlying congenital heart
defects and valvular heart disease is the best approach for
complete cure. 2. If hypertension is the underlying cause of CHF,
antihypertensive treatment should be given. 3. If arrhythmias or
advanced heart block is the cause of or a factor contributing to
heart failure, antiarrhythmic agents or cardiac pacemaker therapy
is indicated. 4. If hyperthyroidism is the cause of heart failure,
this condition should be treated. 5. Fever should be controlled
with antipyretics. 6. When there is a concomitant infection, it
should be treated with appropriate antibiotics. 7. For anemia,
packed cell transfusion is given to raise the hematocrit to 35% or
higher. GENERAL MEASURES 1. A cardiac chair or infant seat is used
to keep infants in a semiupright position to relieve respiratory
distress. 2. Oxygen (40% to 50%) with humidity is administered to
infants with respiratory distress if pulse oximetry indicates
compromise of blood oxygenation. 3. Adequate calories and fluid
should be provided to permit appropriate weight gain. Infants in
CHF need significantly higher caloric intakes than recommended for
average children. The required caloric intake may be as high as 150
to 160 kcal/kg/day for infants in CHF. Compounding this problem is
that these infants typically cannot take in needed calories even
for normal growth owing to tachypnea, increased work of breathing,
diminished strength of sucking, and difficulty with coordination of
sucking and swallowing. a. Increasing caloric density of feeding
may be required, and it may be accomplished with fortification of
feeding. b. Frequent small feedings are better tolerated than large
feedings in infants. c. If oral feedings are not well tolerated,
intermittent or continuous nasogastric (NG) feeding is indicated.
To promote normal development of oral-motor function, infants may
be allowed to take calorie-dense oral feeds throughout the day and
then be given continuous NG feeds overnight. d. Salt restriction in
the form of a low-salt formula and severe fluid restriction are not
indicated in infants. Use of diuretics has replaced these measures.
e. Parents should be taught proper feeding techniques.
4. In older children, salt restriction (8
Mild vasoconstriction: >10
Vasoconstriction: 1520
Digitalis Glycosides Digoxin is the most commonly used digitalis
preparation in pediatric patients. Inotropic agents increase the
cardiac output (or contractile state of the myocardium), resulting
in an upward and leftward shift of the ventricular function curve
relating cardiac output to filling volume of pressure (see Fig. 1
). When inotropic agents are used with a vasodilator or a diuretic,
a much greater improvement is seen both in the contractile state
and in congestive symptoms than when a single class of agent is
used
Table 5 --Oral Digoxin Dosage for Congestive Heart
FailureAgeTotal Digitalizing Dose (g/kg)Maintenance Dose[*]
(g/kg/day)
Prematures205
Newborns308
2 yr3040810
The maintenance dose is 25% of the total digitalizing dose in
two divided doses. The IV dose is 75% of the oral dose.
The pediatric dosage of digoxin is much larger than the adult
dosage on the basis of body weight. Pharmacokinetic studies
indicate that infants and children require a larger dose of digoxin
than adults to attain comparable serum levels, primarily because of
a larger volume of distribution and, less important, more rapid
renal clearance, including tubular secretion. The volume of
distribution of digoxin is 7.5 L/kg in neonates, 16 L/kg in infants
and children, and 4 L/kg in adults. Afterload-Reducing
AgentsVasoconstriction that occurs as a compensatory response to
reduced cardiac output seen in CHF may be deleterious to the
failing ventricle. Vasoconstriction is produced by a rise in
sympathetic tone and circulating catecholamines and an increase in
the activity of the renin-angiotensin system. Reducing afterload
tends to augment the stroke volume without a great change in the
contractile state of the heart and therefore without increasing
myocardial oxygen consumption (see Fig. 1). When a vasodilator is
used with an inotropic agent, the degree of improvement in the
inotropic state as well as in congestive symptoms is much greater
than when a vasodilator alone is used. Combined use of an inotropic
agent, a vasodilator, and a diuretic produces most improvement in
both inotropic state and congestive symptoms (see Fig.
1).Afterload-reducing agents now occupy a prominent role in the
treatment of infants with CHF secondary to large left-to-right
shunt lesions (e.g., VSD, AV canal, PDA). Infants with large
left-to-right shunts have been shown to benefit from captopril and
hydralazine. Beneficial effects of afterload-reducing agents are
also seen in dilated cardio-myopathy,
doxorubicin(Adriamycin)-induced cardiomyopathy, myocardial
ischemia, postoperative cardiac status, severe MR or AR, and
systemic hypertension. These agents are usually used in conjunction
with digitalis glycosides and diuretics for a maximal
benefit.Afterload-reducing agents may be divided into three groups
based on the site of action: arteriolar vasodilators, venodilators,
and mixed vasodilators. Dosages of these agents are presented in
Table 6 . 1. Arteriolar vasodilators (hydralazine) augment cardiac
output by acting primarily on the arteriolar bed, with resulting
reduction of the afterload. Hydralazine is often administered with
propranolol because it activates the baroreceptor reflex, with
resulting tachycardia.
2. Venodilators (nitroglycerin, isosorbide dinitrate) act
primarily by dilating systemic veins and redistributing blood from
the pulmonary to the systemic circuit (with a resulting decrease in
pulmonary symptoms). Venodilators are most beneficial in patients
with pulmonary congestion but may have adverse effects when preload
has been restored to normal by diuretics or sodium restriction.
3. Mixed vasodilators include ACE inhibitors (captopril,
enalapril), nitroprusside, and prazosin. These agents act on both
arteriolar and venous beds. ACE inhibitors are popular in children
with chronic severe CHF, whereas sodium nitroprusside is used
primarily in acute situations such as following cardiac surgery
under cardiopulmonary bypass, especially in patients who had
pulmonary hypertension and those with postoperative rises in PA
pressure. When nitroprusside is used, blood pressure must be
monitored continuously. ACE inhibitors reduce systemic vascular
resistance by inhibiting angiotensin II generation and augmenting
production of bradykinin.
Table 6 --Dosages of VasodilatorsDrugRoute and
DosageComments
Hydralazine (Apresoline)IV: 0.10.2 mg/kg/dose, every 46 hr
(maximum 2 mg/kg every 6 hr)May cause tachycardia; may be used with
propranolol
Oral: 0.753 mg/kg/day, in 24 doses (maximum 200 mg/day)May cause
gastrointestinal symptoms, neutropenia, and lupus-like syndrome
NitroglycerinIV: 0.52 g/kg/min (maximum 6 g/kg/min)Start with
small dose and titrate based on effects
Captopril (Capoten)Oral: Newborn: 0.10.4 mg/kg/dose, 14 times a
dayMay cause hypotension, dizziness, neutropenia, and
proteinuria
Infant: 0.56 mg/kg/day, 14 times a dayDose should be reduced in
patients with impaired renal function
Child: 12.5 mg/dose, 12 times a day
Enalapril (Vasotec)Oral: 0.1 mg/kg, once or twice dailyPatient
may develop hypotension, dizziness, or syncope
Nitroprusside (Nipride)IV: 0.58 g/kg/minMay cause thiocyanate or
cyanide toxicity (e.g., fatigue, nausea, disorientation), hepatic
dysfunction, or light sensitivity
Prazosin (Minipress)Oral: first dose, 5 g/kg; increase to 25150
g/kg/day in 4 dosesHas fewer side effects than hydralazine;
orthostatic hypotension or tachyphylaxis may develop
OTHER DRUGS -Adrenergic Blockers. As with their beneficial
effects reported in adult patients with dilated cardiomyopathy,
-adrenergic blockers have been shown to be beneficial in some
pediatric patients with chronic CHF who were symptomatic despite
being treated with standard anticongestive drugs (digoxin,
diuretics, ACE inhibitors). Recent evidence suggests that the
adrenergic overstimulation often seen in patients with chronic CHF
may have detrimental effects on the hemodynamics of heart failure
by inducing myocyte injury and necrosis rather than being a
compensatory mechanism, as traditionally thought. -Adrenergic
blockers should not be given to those with decompensated heart
failure. They should be deferred until reestablishment of good
fluid balance and stable blood pressure and should be started with
a small dose and gradually increased. Carvedilol, a nonselective
-adrenergic blocker with additional 1-antagonist activities, when
added to standard medical therapy for CHF, has been shown to be
beneficial in children with dilated cardiomyopathy ( Bruns et al,
2001 ). The patients included in the study were those with
idiopathic dilated cardiomyopathy, chemotherapy-induced
cardiomyopathy, postmyocarditis myopathy, and muscular dystrophy
and those who had chronic heart failure following surgeries for
congenital heart defects (such as a Fontan or Senning operation).
The initial dose was 0.09 mg/kg twice daily and the dose was
increased gradually to 0.36 and 0.75 mg/kg as tolerated, up to the
maximum adult dose of 50 mg/day. Side effects of the drug include
dizziness, hypotension, and headache (also see Dilated
Cardiomyopathy).
Metoprolol was added to standard anticongestive medicines in
patients with chronic CHF from dilated cardiomyopathy. Metoprolol
increased LV fractional shortening and ejection fraction and
improved symptoms. The starting dose was 0.1 to 0.2 mg/kg per dose
twice a day and was slowly increased over a period of weeks to 1.1
mg/kg/day (range 0.5 to 2.3 mg/kg/day) ( Shaddy et al, 1999 ). The
improvement in the LV fractional shortening appears slightly better
with carvedilol than with metoprolol.
SURGICAL MANAGEMENT If medical treatment with the previously
mentioned regimens does not improve CHF caused by congenital heart
defects within a few weeks to months, one should consider either
palliative or corrective cardiac surgery for the underlying cardiac
defect when technically feasible. Cardiac transplantation is an
option for a patient with progressively deteriorating
cardiomyopathy despite maximal medical treatment.
CASE REPORT
Name: Jihan Talita Ulfa SiregarAge: 11 months 16 days Sex:
FemaleDate of Admission: December, 21th 2014
Chief Complaint: Shortness of BreathHistory: These was realized
by the Os parents one month ago. Restlessness and shortness of
breath was seen during activities such as when Os is crying and
consuming milk. These complain was encountered by os since birth
but it got worst in this few months. In the past two months, Os has
a history of interrupted consumption of milk and heavy sweating
during consumption of milk. Fever (-), diarrhea (-), cough (+) flam
(-), vomiting (-) and sweating (+) in the last one month. Os have
defecation problem (constipation) in the last two weeks. Os has no
urination problem.
Pregnant HistoryPatient was conceived at second pregnancy at the
age 28, first child (2 yrs old): normal delivery and healthy. There
was no history of fever, hypertension, diabetic mellitus, and
consumption of drugs and herbal medicine as well jaundice during
pregnancy period.
Birth HistorySpontaneous; attended by midwives; BW 3800 gram; BL
50 cm, cyanotic (-)
Immunization HistoryBCG I (no scar), DPT II, Polio III, Measles
I, Hepatitis III
Feeding HistoryFrom birth to 4 months: Breast milk onlyFrom 4
months up to date : Breast milk, biscuit porridge (SUN) and
Formulated Milk
History of Growth and DevelopmentSitting: - months Crawling: -
monthsStanding: - monthsWalking: - months
History of previous illness: The patient has been suffering from
growth stunt where the growth doesnt match the age and was
experiencing shortness of breath when crying and ingesting milk for
almost more than a month. Patient was then brought by the parents
to Tapsel District General Hospital and was diagnosed as noncynotic
CHF. Later the patient was then referred to Dr. Pringadi District
General Hospital where was diagnosed as CHF ec acynotic CHD.
Finally the patient was referred to Haji Adam Malik General
Hospital on the 21th of Dec 2014.
History of previous medications: none
History of Family Disease: unclear
Pysical ExaminationGeneralized statusBody weight: 5kg, Body
length: 62 cmBody weight in 50th percentile according to age: 8.5
kgBody length in 50th percentile according to age: 73 cmBody weight
in 50th percentile according to body length: 6.5 kg
BW/BL: 5/6.5 x 100% = 77 % (Moderate Malnutrition)BW/age: 5/8.5
x 100% = 59 % (Severe Malnutition)BL/age : 62/73 x 100% = 85% (Mild
malnutrition)Weight for lenght: -3 < Z scores < -1
(underweight)
Presens statusConsciousness: Compos mentis , Body temperature:
36,7 oC. HR 140x/i, RR 50x/i, BP 90/70 mmHg, Anemic (-); Icteric
(-); Cyanosis (-); Edema (-). Dyspnea (+).
Localized statusHead : Large crown closed. Black hair, normal.
Right Eye: Pupil diameter 3 mm. Inferior palpebra conjunctiva pale
(-). Icteric sclera (-). Light reflex (+). Left eye: Pupil diameter
3 mm. Inferior palpebra conjunctiva pale (-). Icteric sclera (-).
Light reflex (+).Ear, nose and mouth were within normal limit. Neck
: Lymph node enlargement (-). TVJ R+2 cmH20
Thorax: Symmetrical fusiformis. Chest retraction (+)
epigastrial, intercostals, suprasternal. HR: 140 bpm, regular,
murmur (+) pansistolik grade III/6 LMCS ICR III-IV.RR: 50x/i,
ireguler, rales -/-Abdomen: Soepel, Normoperistaltic. Liver, spleen
and renal unpalpable.. Extremities: Pulse 136x/i, regular, adequate
p/v, cold acral , CRT < 3.
Urogenital: Female; within normal limit.
Laboratory Findings (December, 21st 2014):ParametersValueNormal
Value
Complete Blood Count
Hemoglobin10.30 gr%12,0 14,4 gr%
Hematocrite31.60 %37 41%
Erithrocyte4.06 x 106 /mm34,40 4,48 x 106 /mm3
Leucocyte12.06 x 103 /mm34,5 13.5x 103 /mm3
Platelet385.000 /mm3150.000 450.000 /mm3
MCV77.80 fl81 95 fl
MCH25.40 pg25 29pg
MCHC32.60 gr%29 31 gr%
RDW18.80 %11.6 14.8 %
MPV7.80 fl7,0 10,2 fl
PCT0.30%
PDW7.2%
Hitung Jenis
Neutrofil24,3037-80
Limfosit70,0020-40
Monosit5,202-8
Eosinofil0,201-6
Basofil0,3000-1
Neutrofil absolute2.921,9-5,4
Limfosit absolute8,443,7-10,7
Monosit absolute0.630,3-0,8
Eosinofil absolute0.030,2-0,5
Basofil absolute0,040-0,1
Laboratory Findings (December, 21st 2014):ParametersValueNormal
Value
Carbohydrate Metabolism
Blood Glucose ad random91,00 mg/dL< 200
Blood Gas Analysis
pH7,4377,35 7,45
pCO221,7 mmHg38 42
pO2137,7 mmHg85 100
Bicarbonate (HCO3) 14,3 mmol/L22 26
Total CO215,0 mmol/L19 25
Base Excessive (BE)-8.6 mmol/L(-2) (+2)
O2 Saturation 99.1%95 - 100
Cardiac Marker
Troponin TNegative (g/L)0 0,1
Radiologic Imaging
Chest x-ray interpretation:KV weak. Less Inspiration, no trachea
deviation found(middle trachea), both costophrenicus angel were
sharp, smooth diaphragm, not seen infiltrates in both lung fields,
Egg shaped heart was found. Cardio thoracic Ratio is more than 50
%, apex embedded (apex upwards), bones and soft tissues in good
condition, waist of heart not prominent.Result: CTR of 65%
Cardiomegaly, Heart characteristics: egg on site
Differential Diagnosis: VSD ASD PDA
Working Diagnosis:CHF d/t acynotic CHD
Management: Bed rest O2 Nasal kanul L/i Furosemide 2x5mg
Spironolactone 2x6,25mg Sildenafil 3x1.5mg IV Dobutamin
5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in 1cc perhour Diet F75
125cc/2jam/ggt
Diagnostic Planning: Consul cardiologist Urinalysis
Echocardiography
FOLLOW UP
December , 21st 2014
S Dyspnoe +
OSens: Compos mentis, Temp: 36,7 oC. Anemic (-). Icteric (-).
Edema (-). Cyanosis (-) Dyspnoe (+)Body weight: 5 kg, Body length:
62 cmHeadRight Eye: Pupil diameter 3 mm. Inferior palpebra
conjunctiva pale (-). Icteric sclera (-). Light reflex (+). Left
eye: Pupil diameter 3 mm. Inferior palpebra conjunctiva pale (-).
Icteric sclera (-). Light reflex (+)..
NeckLymph node enlargement (-)
ThoraxSimetris fusiformis. Retraction (+) epigastrial;
intercostals, suprasternal. HR: 150 bpm, reguler; murmur (+)
pansistolik grade III/6 LMCS III-IV.RR: 50 x/i, regular, rales
(-/-)
AbdomenSoepel. normoperistaltic. Liver, spleen and renal
unpalpable.
ExtremitiesPulse 140 x/i, iregular, adequate p/v, cold, CRT <
3.
GenitalFemale; within normal limit.
ACHF ec acynotic CHD d/t dd/ VSD ASD PDA
PManagement: Bed rest O2 Nasal kanul L/i Furosemide 2x5mg
Spironolactone 2x6,25mg Sildenafil 3x1.5mg IV Dobutamin
5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in 1cc perhour Diet F75
125cc/2jam/ggt
Diagnostic Planning: Echocardiography Urinalisis Consul
cardiologist
December , 22nd 2014
S Dyspnoe +
OSens: Compos mentis, Temp: 36,7 oC. Anemic (-). Icteric (-).
Edema (-). Cyanosis (-) Dyspnoe (+)Body weight: 5 kg, Body length:
62 cmHeadRight Eye: Pupil diameter 3 mm. Inferior palpebra
conjunctiva pale (-). Icteric sclera (-). Light reflex (+). Left
eye: Pupil diameter 3 mm. Inferior palpebra conjunctiva pale (-).
Icteric sclera (-). Light reflex (+)..
NeckLymph node enlargement (-)
ThoraxSimetris fusiformis. Retraction (+) epigastrial;
intercostals, suprasternal. HR: 150 bpm, reguler; murmur (+)
pansistolik grade III/6 LMCS III-IV.RR: 50 x/i, regular, rales
(-/-)
AbdomenSoepel. normoperistaltic. Liver, spleen and renal
unpalpable.
ExtremitiesPulse 140 x/i, iregular, adequate p/v, cold, CRT <
3.
GenitalFemale; within normal limit.
ACHF ec acynotic CHD d/t dd/ VSD ASD PDA
PManagement: Bed rest O2 Nasal kanul L/i Furosemide 2x5mg
Spironolactone 2x6,25mg Sildenafil 3x1.5mg IV Dobutamin
5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in 1cc perhour Diet F75
125cc/2jam/ggt
Diagnostic Planning: Echocardiography (today)
Echocardiography
Result: Large ASD, Small PDA, pulmonary stenosis and decreased
systolic function
December , 23rd 2014
S Dyspnoe +
OSens: Compos mentis, Temp: 36,7 oC. Anemic (-). Icteric (-).
Edema (-). Cyanosis (-) Dyspnoe (+)Body weight: 5 kg, Body length:
62 cmHeadRight Eye: Pupil diameter 3 mm. Inferior palpebra
conjunctiva pale (-). Icteric sclera (-). Light reflex (+). Left
eye: Pupil diameter 3 mm. Inferior palpebra conjunctiva pale (-).
Icteric sclera (-). Light reflex (+)..
NeckLymph node enlargement (-)
ThoraxSimetris fusiformis. Retraction (+) epigastrial;
intercostals, suprasternal. HR: 180 bpm, reguler; murmur (+)
pansistolik grade III/6 LMCS III-IV.RR: 70 x/i, regular, rales
(-/-)
AbdomenSoepel. normoperistaltic. Liver, spleen and renal
unpalpable.
ExtremitiesPulse 140 x/i, iregular, adequate p/v, cold, CRT <
3.
GenitalFemale; within normal limit.
ACHF ec acynotic CHD d/t ASD PDA PS
PManagement: Bed rest O2 Nasal kanul L/i Furosemide 2x5mg
Spironolactone 2x6,25mg Sildenafil 3x1.5mg IV Dobutamin
5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in 1cc perhour Diet F75
125cc/2jam/ggt
24th December 2014Patient exitus after sudden drop in blood
pressure and worsening dyspneo. Resuscitation failed.
CASE DISCUSSION Theory Case
Common causes of Congestive heart failure (CHF) are volume or
pressure overload, or both, caused by congenital heart disease,
acquired heart disease and myocardial diseases. By far the most
common causes of CHF in infancy are congenital heart diseases
(CHDs).Patient is 11 months old, with the the diagnosis CHF due to
CHD
History1.Poor feeding of recent onset, tachypnea that worsens
during feeding, poor weight gain, and cold sweat on the forehead
suggest CHF in infants. 2. Older children may complain of shortness
of breath, especially with activities, early fatigability, puffy
eyelids, or swollen feet.
Shortness of Breath was realized by the patients parents one
month ago. Restlessness and shortness of breath was seen during
activities such as when patient is crying and consuming milk. These
complain was encountered by patient since birth but it got worst
these past few months. In the past two months patient also has a
history of interrupted consumption of milk and heavy sweating
during consumption of milk.
Physical Examination Tachycardia, gallop rhythm, and weak and
thready pulses are commonSigns of increased sympathetic discharges
(e.g., growth failure, perspiration, cold and wet skin). Tachypnea
is common and is an early manifestation of CHF in infants. Dyspnea
on exertion (equivalent to poor feeding in small infants) is common
in children. HR: 180 bpm, reguler; murmur (+) pansistolik grade
III/6 LMCS III-IV, ExtremitiesPulse 140 x/i, iregular, cold, In the
past two months patient also has a history of interrupted
consumption of milk and heavy sweating during consumption of
milk.
The presence of cardiomegaly should be demonstrated by chest
x-ray films. The absence of cardiomegaly almost rules out the
diagnosis of CHFCTR of 65% - Cardiomegaly, Heart characteristics:
egg on site.
EchocardiographyEcho studies may confirm enlargement of
ventricular chambers and impaired LV systolic function (decreased
fractional shortening or ejection fraction) as well as impaired
diastolic function by the use of Doppler techniques. A more
important role of echo may be due to its ability to determine the
cause of CHF.Results: Large ASD, Small PDA, pulmonary stenosis and
decreased systolic function
TheraphyThree major classes of drugs are commonly used in the
treatment of CHF in children: diuretics, inotropic agents, and
afterload-reducing agents. Diuretics are usually used with
inotropic agents. Rapid-acting inotropic agents (dopamine,
dobutamine) are used in critically or acutely ill infants and
children. Bed rest O2 Nasal kanul L/i Furosemide 2x5mg
Spironolactone 2x6,25mg Sildenafil 3x1.5mg IV Dobutamin
5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in 1cc perhour Diet F75
125cc/2jam/ggt
REFERENCE1. Brennan P , 2007. Chapter 417Epidemiology and
Genetic Basis of Congenital Heart Disease In: Nelson Textbook of
Pediatrics,17th edition, Elsevier Science Philadelphia,
Pennsylvania.2. Lister G,2007. Chapter 418Evaluation of the Infant
or Child with Congenital Heart Disease In: Nelson Textbook of
Pediatrics,17th edition, Elsevier Science Philadelphia,
Pennsylvania.3. William W.H., 2003. Current Pediatric Diagnosis
& Treatment, 16th Edition The McGraw-Hill Companies, Inc,
United States of America.4. Rudolph C.D., 2003. Rudolph's
Pediatrics, Twenty-First Edition, The McGraw-Hill Companies, Inc,
United States of America.5. Park M. K., 2008, Pediatric Cardiology
for Practitioners. 5th edition, Mosby, Inc., an affiliate of
Elsevier Inc, Philadelphia, Pennsylvania.