Breathlessness gets releived on putting child to shoulders
Decreased intake during feeds
Swelling over body
Repeated respiratory tract infections
Inability to gain weight.
Feeding difficulties
Frequency
Volume of each feed
Time spent on each breast
Gets diaphoretic or not
Will awaken for next feed after brief time.
Infective endocarditis:
High fever with chills
Bleeding manifestations (petechial hemorrhages/hematuria/hemoptysis)
Convulsions/unconsciousness/altered sensorium
Tender pads of fingers (Osler’s nodes)
Dental procedures/surgery.
Cerebral abscess (in children > 2 years with cyanotic heart disease):
Fever/vomiting
Focal seizures
Hemiparesis/Focal neurological deficits.
Cerebral infarct (in children < 2 years with cyanotic heart disease):
Vomiting
Focal seizures
Hemiparesis/focal neurological deficits.
Impact on child:
Growth and development
Schooling (academic performance, sports restriction, teachers and peers attitude, school days lost).
Horseness of voice (Large PDA)
Pink frothy sputum.
History of Risk Factors
Maternal risk factors:
Fever with rash in mother during pregnancy (Intrauterine infection)
Irradiation/alcohol intake
Drug intake (Phenytoin, steroids, lithium, carbamazepine, sodium valproate)
Diabetes mellitus (increased incidence of TGA and hypertrophic cardiomyopathy)
Hypertension in pregnancy
Fetal echocardiography done or not (essential in presence of suggestive history)
Affected siblings.
Fetal risk factors:
Prematurity (increased incidence of VSD/
PDA)
Large baby (Transposition of great arteries)
Birth asphyxia and respiratory distress (persistent pulmonary hypertension and myocardial dysfunction)
Cyanosis/prolonged jaundice.
PAST HISTORY
Previous hospitalization
Previous surgery.
FAMILY HISTORY
Diabetes in family members (TGA)
Congenital heart disease in family (endocardial fibroelastosis)
Parental consanguinity (autosomal recessive conditions, e.g. Friedreich ataxia).
GENERAL PHYSICAL EXAMINATION a. Position of child—decubitus
b. Vitals:
1. Pulse (in all 4 limbs)
Rate
Rhythm
Volume
Condition of the arterial wall
Comparison between two radial pulses
Radio-femoral delay
Any special character
Other peripheral pulses 2. Respiration
3. BP (in all 4 limbs in older child).
4. Temperature
c . Anthropometry: Assess growth of child d. Head to toe examination: Pallor/cyanosis/
clubbing/hepatojugular reflux/pedal edema e. Examination of neck veins:
Engorged or not
If engorged—pressure, pulsation, hepatojugular reflux
f. Signs of infective endocarditis:
Osler’s nodes (pin head size tender papule seen in the pulp of fingers)
Petechiae
Janeway’s lesions (nontender maculopa-pular lesion in palm)
Roth spots (seen on ophthalmoscopy)
Splinter hemorrhages (linear longitudinal.
hemorrhage under the nail) g. Signs to assess nutritional status:
Look for signs of malnutrition
Eye changes (vitamin A deficiency)
Bossing of skull/beading of ribs (vitamin D deficiency)
h. Dysmorphic facies:
Microcephaly
Cataract (rubella)
i. Other congenital anomalies
Vertebral, anal, renal, coloboma, atresia chonae, mental retardation, genital and ear anomalies
j. Scars of previous surgeries or other procedures.
SYSTEMIC EXAMINATION
CARDIOVASCULAR SYSTEM
Inspection
Precordium
Deformity or bulging seen
Apical impulse; diffuse pulsation of precordium
Engorged superficial veins.
Any pulsation present in aortic, pulmonary, parasternal areas, epigastrium, suprasternal area, carotid pulsation, inferior angle of scapula (Suzman’s sign in coarctation of aorta).
Inspection of the BACK
Scoliosis
Gibbus
Drooping of the shoulder
Winging of scapula.
Skin for any sinus, ulcer, venous prominence, scar mark.
Palpation
Aortic area:
Pulsation
Palpable heart sound (A2)
Thrill.
Pulmonary area:
Pulsation
Palpable hear sound (P2)
Thrill.
Mitral area
Apex beat for site and character
Palpable heart sound
Thrill.
Tricuspid area
Left parasternal heave (its presence signifies right ventricular hypertrophy)
Palpable heart sound
Thrill.
Direction of venous blood flow (in engorged superficial veins)
Thrill in carotid arteries (carotid shudder)
Pulsations:
Epigastric
Suprasternal pulsation (signifies aortic stenosis, PDA and rarely pulmonary stenosis)
Pulsation over the back
Liver: Pulsatile due to gross tricuspid regur-gitation
Palpable pericardial rub
Trachea position
Pedal edema: Rarely seen, sacral and shin oedema should be looked for.
Percussion
Outer borders of heart
Sequence of auscultation
Upper right sternal border (Aortic area)
Upper left sternal border (Pulmonary area)
Lower left sternal border
Apex
Apex—left lateral decubitus position
Lower left sternal border—sitting, leaning forward, held expiration Auscultation
Aortic Area (2nd right intercostal space)
Compare S1 to S2: S1 should be softer. If the same, think Mitral Stenosis
identify ejection murmur
identify ejection click if present.
Pulmonary Area (2nd left intercostal space)
listen for split S2 (A2/P2)
identify the intensities of A2 and P2
time split S2 with respiration
normally widens with inspiration, closes with expiration
wide split S2-RBBB, RV volume overload, PS, RV failure
wide fixed split = ASD
paradoxical split = LBBB, severe AS, severe LV dysfunction, pacemaker.
Tricuspid Area (lower left sternal border)
Listen for intensity of S1
Soft: LV dysfunction, first degree heart block, severe AR/MR
Loud: Mitral stenosis.
Identify quality, timing and intensity of systolic murmurs.
Ejection quality versus regurgitant quality
Pansystolic versus early or mid to late systolic murmur.
Apex
Listen for S3 and S4
Identify diastolic rumble
Determine radiation of murmur, e.g. mitral regurgitation murmur to axilla
Additional sounds like pericardial rub.
RESPIRATORY SYSTEM
Crepitations/rhonchi ABDOMEN
Hepatomegaly/splenomegaly/Ascites
Continous murmur—onset after S1, peaks at S2 and disappears in late diastole. Best heard at 2nd left intercostal space and also below clavicle.
ASD (Ostium Primum and Endocardial Cushion Defect)
Asymmetric or exercise intolerance, easy fatigability and recurrent pulmonary infections especially in large left to right shunt and mitral regurgitation.
Hyperdynamic precordium
Wide split S2 and ejection systolic murmur are characteristic
Holosystolic murmur—apical harsh radiating to left axilla.
ASD (Ostium Secundum)
Generally asymptomatic. If symptomatic it leads to failure to thrive in young while in older children presents as exercise intolerance
Mild left precordial bulge, palpable parasternal lift
Wide split S2 and ejection systolic murmur are characteristic
Mild diastolic murmur (Increased flow across tricuspid valve) at left lower sternal border (Qp: Qs 2:1).
TETRALOGY OF FALLOT
Cyanosis not present at birth, occurs later in 1st year. Most prominent in mucus membrane of lips, mouth, finger nails and toenail. With long standing cyanosis—dusky blue skin surface, gray sclera, engorged blood vessels and marked clubbing.
Dyspnea on exertion. Infant plays for short time then sit or lie down. Older children walk a block or so before stopping to rest.
CNS
Look for focal deficits.
DIAGNOSIS
Congenital, cyanotic/acyanotic heart disease with L → R or R → L shunt; with/without sinus rhythm;
with/without CCF; with/without pulmonary hypertension; with/without Infective endocarditis;
with/without failure to thrive; most probable diagnosis being.
DIFFERENTIAL DIAGNOSIS
VENTRICULAR SEPTAL DEFECT
Age of presentation: 6-10 weeks
History of dyspnea, feeding difficulty, poor growth, profuse perspiration, recurrent pulmonary infection
CVS—hyperkinetic precordium, palpable parasternal lift, systolic thrill
S1S2 masked by murmur at left lower sternal border, At 2nd left intercostal space S2 widely split with accentuated P2
Pansystolic murmur best heard at left lower sternal border.
PATENT DUCTUS ARTERIOSUS
Age of presentation: 6-10 weeks
Retardation of growth with large shunt; older children—effort intolerance, palpitation, frequent chest infections.
Wide pulse pressure
CVS—apex prominent and heaving, thrill maximum at 2nd left intercostal space
S1 accentuated, S2 difficult to appreciate due to murmur at 2nd intercostal space. S2 in small shunt is normally split while in large shunt it is narrowly split.
Child assumes squatting position for relieving dyspnea and is usually able to resume activity in few minutes.
Cyanotic spell: Onset is spontaneous and unpredictable, most frequent in morning on awakening/excessive crying. Child becomes hyperapneic, restless, cyanosis increases followed by gasping respiration. It could be short episode of generalized weakness and sleep or severe episode of unconsciousness, convulsion, hemiparesis.
INVESTIGATIONS
To Prove Heart Disease
Chest X-ray: Look for:
Heart size—cardiothoracic ratio up to 55% is normal.
Pulmonary vascular markings—increased or decreased.
Any evidence of respiratory infection (infiltrates/consolidation).
Presence of thymus shadow.
ECG: Look for:
Sinus rhythm
Heart rate
Axis
Chamber hypertrophy.
Echocardiography
Pressure in chamber of heart
Position and size of defects
Vegetations (infective endocarditis).
Color Doppler: Estimation of pressure in heart chambers
Cardiac catheterization.
To Rule Out Complications
Hemogram with ESR—infective endocarditis and respiratory tract infections.
Blood culture—for infective endocarditis.
CT Scan—if Abscess/Infarct in CNS.
To Look For Risk Factors
TORCH titers (Intrauterine infection).
Investigations to Monitor Cyanotic Child
Arterial blood gases
Complete blood count
Clotting studies—PT, PTTK, fibrogen levels
Blood glucose and serum calcium levels
Serum iron level
Uric acid level in older cyanotic children since they are prone for hyperuricemia.
TREATMENT
Medical Management
Treatment of chest infections
Prevention and treatment of anemia
Prevention and treatment of infective endocarditis
Control of congestive cardiac failure—salt and fluid restricted diet, diuretics, digitalis, ACE inhibitors.
Management of cyanotic spells—knee chest position, humidified oxygen, morphine, correction of acidosis, propranolol, vasopressors like methoxamine.
Surgical Management
Depending upon the cause.
GUIDELINES OF WORKING GROUP ON MANAGEMENT OF CONGENITAL HEART DISEASES IN INDIA
Atrial Septal Defect (ASD)
Spontaneous closure: Rare if defect >8 mm at birth.
Rare after age 2 years.
Patent foramen ovale: Echocardiographic detection of a asymptomatic patent foramen ovale is a normal finding in newborns.
Indication for closure: ASD associated with right ventricular volume overload.
Ideal age of closure:
i. In asymptomatic child: 2-4 years
ii. Symptomatic ASD in infancy (congestive heart failure, severe pulmonary artery hypertension):
Early closure is recommended.
iii. If presenting beyond ideal age: Elective closure irrespective of age.
Ventricular Septal Defect (VSD)
Timing of Closure
Large VSD with uncontrolled congestive heart failure: As soon as possible.
Large VSD with severe pulmonary artery hypertension: 3-6 months.
Small sized VSD with normal pulmonary artery pressure, left to right shunt >1.5:1: Closure by 2-4 years.
Small outlet/perimembranous VSD with any degree of aortic regurgitation: Surgery whenever aortic regurgitation is detected.
Small VSD with more than one episode of infective endocarditis: Early VSD closure recommended.
Patent Ductus Arteriosus (PDA)
Spontaneous closure: Small PDAs in full term baby may close up to 3 months of age, large PDAs are unlikely to close.
Timing of Closure
Large/ moderate PDA, with congestive heart failure, pulmonary artery hypertension: Early closure (by 3-6 months).
Moderate PDA, no congestive heart failure:
6 months to 1 year. If failure to thrive, closure can be accomplished earlier.
Small PDA: At 12-18 months .
Silent PDA: Closure not recommended.
Indomethacin/ ibuprofen not to be used in term babies.
PDA in a Preterm Baby
Intervene if baby in heart failure (small PDAs may close spontaneously).
Indomethacin or Ibuprofen (if no contrain-dication).
Surgical ligation if above drugs fail or are contraindicated.
Prophylactic indomethacin or ibuprofen therapy:
Not recommended.
Obstructive Lesions
Coarctation of Aorta (CoA) Timing of intervention:
With left ventricular dysfunction / congestive heart failure or severe upper limb hypertension (for age): Immediate intervention.
Normal left ventricular function, no congestive heart failure and mild upper limb hypertension:
Intervention beyond 3-6 months of age.
No hypertension, no heart failure, normal ventricular function: Intervention at 1-2 years of age.
Cyanotic Congenital Heart Disease
Tetralogy of Fallot (TOF)
Medical therapy: Maintain Hb >14 g/dL (by using oral iron or blood transfusion). Beta blockers to be given in highest tolerated doses (usual dose 1-4 mg/kg/day in 2 to 3 divided doses).
Timing of surgery: All patients need surgical repair.
Stable, minimally cyanosed: Total correction at 1-2 years of age or earlier according to the institutional policy.
Transposition of Great Arteries (TGA)
Timing of Surgery
TGA with Intact interventricular septum
If <3-4 weeks of age: Arterial switch operation immediately.
If >3-4 weeks of age at presentation:
Assess left ventricle by ECHO. If the left ventricle is decompressed:Senning/
Mustard at 3-6 months, or rapid two stage arterial switch. If the left ventricle is still prepared, very early arterial switch operation is indicated.
TGA with ventricular septal defect: Arterial switch operation, by 3 months of age.
Total Anomalous Pulmonary Venous Connection (TAPVC)
Types of TAPVC
Type I: Anomalous connection at supraca-rdiac level (to innominate vein or right superior vena cava).
Type II: Anomalous connection at cardiac level (to coronary sinus or right atrium).
Type III: Anomalous connection at infradia-phragmatic level (to portal vein or inferior vena cava).
Type IV: Anomalous connection at two or more of the above levels.
Each type can be obstructive (obstruction at one of the anatomic sites in the anomalous pulmonary
venous channel) or non-obstructive. Type III is almost always obstructive.
Timing of Surgery
Obstructive type: Emergency surgery.
Non obstructive type: As soon as possible (beyond neonatal period if baby is clinically stable) .
Those presenting after 2 years of age: Elective surgery whenever diagnosed, as long as pulmonary vascular resistance is in operable range.
Guidelines for Infective Endocarditis Prophylaxis
1. Maintain good oral hygiene and a regular dental check up.
2. Unrepaired cyanotic heart diseases are high-risk conditions for infective endocarditis, therefore prophylaxis is mandatory.
3. Atrial septal defect (secundum type) and valvular pulmonic stenosis are low-risk conditions for infective endocarditis and prophylaxis is not recommended.
4. Other acyanotic congenital heart diseases including a bicuspid aortic valve are moderate risk and prophylaxis is recommended.
5. Repaired congenital heart diseases with prosthetic material need prophylaxis for the first six months after the procedure.
6. Device placement by transcatheter route also requires prophylaxis for the first six months.
7. Prophylaxis is recommended for residual defects after a procedure.
DISCUSSION
LOCATION OF APICAL IMPULSE
Up to 4 years: Left 4th intercostal space lateral to midclavicular line
4-8 years: Left 5th intercostal space lateral to midclavicular line
8-12 years: Left 5th intercostal space medial to midclavicular line
NADAS DICTATUM
Diagnosis of a patient over 2 years of age with cyanotic heart disease as Fallot’s tetrology is 75%
correct.
Table 15.1: Nadas criteria (to determine the presence or absence of congenital heart disease) Major criteria Minor criteria
Systolic murmur Systolic murmur
>Grade III <Grade III
Diastolic murmur Abnormal 2nd heart sound
CCF Hypertension (with absent femorals)
Cyanosis Abnormal chest X-ray
Abnormal ECG
Presence of one major or two minor criteria is essential for indicating the presence of heart disease.
CLASSIFICATION OF CONGENITAL HEART DISEASES
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PATHOLOGY OF ASD
Three types: Secundum, primum and sinus venosus.
Ostium secundum is most common (50-70%).
The defect is present in fossa ovalis, causing shunting from left atrium to right atrium.
Ostium primum seen in 30% of all ASD’s including those as a part of atrioventricular canal defects.
Sinus venosus seen in 10% cases is commonly seen at entry of superior venae cavae into right ventricle and sometimes at entry of inferior venae cavae into right atrium.
Patent foramen ovale is usually of no hemodynamic significance and not considered ASD.
NATURAL HISTORY OF ASD
Spontaneous closure 87%
ASD < 3 mm—100 % closure by 18 months
ASD between 3-8 mm—80% closure by 18 months
ASD >8 mm—rarely closes spontaneously
In untreated ASD—congestive heart failure and Pulmonary artery hypertension develops in adults.
Infective endocarditis prophylaxis not required.
NATURAL HISTORY OF VSD
Spontaneous closure in 30—50% most frequently during 1st 2 years of life.
Small muscular VSD are more likely to close (up to 80%) than membranous.
VSDs (up to 35%) majority of defects that close do so before age of 4 years.
Risks of unoperated VSD—infective endocarditis, arrhythmia, subaortic stenosis and exercise intolerance.
NATURAL HISTORY OF PATENT DUCTUS ARTERIOSUS
Unlike PDA of premature infants, spontaneous closure of PDA of term infants does not occur.
PDA of term infants are due to structural abnormality of ductal smooth muscle.
Risks of PDA—pulmonary vascular obstructive disease, congestive heart failure or recurrent pneumonia.
Spontaneous bacterial endocarditis, more frequent with small PDA.
ACYANOTIC CHD: LEFT TO RIGHT SHUNTS
Atrial septal defect Ventricular septal defect Patent ductus arteriosus Left parasternal impulse Left ventricle type apical impulse Left ventricle type impulse
Wide, fixed S2 Systolic thrill Wide pulse pressure
Pulmonary ejection systolic murmur Pansystolic murmur Systolic or continuous thrill Tricuspid diastolic flow murmur Mitral diastolic flow murmur Mitral diastolic flow murmur rSR pattern in V1 in ECG LV dominance in ECG
ACYANOTIC CHD: OBSTRUCTIVE LESIONS
Pulmonary stenosis Aortic stenosis Coarctation of aorta
Left parasternal leave Narrow pulse pressure Weak or delayed femoral compared to radial
Systolic thrill Systolic thrill High arm blood pressure
Ejection systolic murmur in upper Ejection systolic murmur Prominent carotid, palpable aorta in left sternal border radiating to neck suprasternal notch, palpable collaterals Wide split second sound, delayed Delayed A2 Ejection systolic murmur in
well heard P2 interscapular region