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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.