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Heart Disease

Congenital Heart Diseases in Children and Hopes....!!

By VijayBalajiPublished 7 months ago 7 min read

Highest number of morbidity and motality occuring in children is due to cardiovascular system disease. And mostly commonly due to structural abnormalites and defects

Very few percentage is due to rheumatic fever caused by streptococcal infection and M protein from the pathogen will cross react with the heart muscles causing severe infection and affecting the blood system

Despite substantial decline in the incidence of rheumatic fever, rheumatic heart disease continues to be prevalent in India. Systemic hypertension is increasingly recognized in childhood and may predispose to cardiovascular morbidity in adulthood

Congenital heart disease (CHD) encompasses a broad and diverse range of conditions that manifest from prenatal period to late adulthood. In common usage, CHD refers to structural heart defects that are present at birth. History, physical examination, chest X-ray, ECG and echocardiography help in identifying the presence of CHD, except perhaps in the early newborn period where the diagnosis can be challenging.

Congenital Heart Diseases can be classified as Cyanotic and Acyanotic, means when the deoxygenated blood delivers to the skin causing cyanosis and even with supply of oxygenated blood the heart function is abnormal. And also to remember the defect can cause blood to flow from right to left and visa versa

Cyanotic heart diseases includes : Reduced Pulmonary blood flow > Intact interventricular septum: Pulmonary atresia with intact ventricular septum, critical pulmonic stenosis with right to left shunt at atrial level, Ebstein anomaly; isolated right ventricular hypoplasia

Unrestrictive ventricular communication: All conditions listed under VSD with pulmonic stenosis

Increased pulmonary blood flow : Pre-tricuspid: Total anomalous pulmonary venous communication, common atrium

Post-tricuspid: All single ventricle physiology lesions without pulmonic stenosis, persistent truncus arteriosus, transposition of great vessels

Acyanotic heart diseases includes: Left To Right shunts : Pre-tricuspid: Partial anomalous pulmonary venous drainage, atrial septal defect

Ventricular: Ventricular septa! defects (VSD)

Great artery: Aorto-pulmonary window, patent ductus; ruptured sinus of Valsalva

Both pre- and post-tricuspid: Atrioventricular septa! defect, left ventricle to right atrial communications

While it is often easy to recognize the presence of CHD in older children, manifestations of heart disease can often be subtle in newborns and young infants. Conditions that do not primarily involve the cardiovascular system can result in clinical manifestations that overlap with those resulting from CHD in the newborn.

Nonetheless, careful clinical evaluation is often rewarding and allows identification of CHD in most infants and many newborns. The following clinical features should alert the paediatrician regarding the presence of CHD.

Cyanosis Parents seldom report cyanosis unless itis relatively severe (saturation <80%). It is often easier for them to notice episodic cyanosis (when the child cries or exerts).

Difficult feeding and poor growth. The parent of an infant with CHD may complain that the child has difficulty with feeds. This is usually a feature of an infant with congestive heart failure resulting from CHD

The history may be of slow feeding, small volumes consumed during each feed, tiring easily following feeds and requirement of periods of rest during feeds. Excessive sweating involving forehead or occiput is commonly associated. Not infrequently, no history of feeding difficulty may be obtained, but examination of the growth charts will reveal that the child's growth rate is not appropriate for age.

A recent decline in growth rate (falling off the growth curve) or weight that is inappropriate for age may result from a large left to right shunt. Characteristically, growth retardation affects weight more that height.

Difficult breathing. Tachypnea (respiratory rates more than 60/min in infants 50/min in older infants; >40/min after 1 yr) is a characteristic manifestation of heart failure in newborns. For infants, subcostal or intercostal retractions together with flaring of nostrils are frequently associated with tachypnea.

Frequent respiratory infections. The association of respiratory infections that are frequent, severe and difficult to treat with large left to right shunts is not a specific feature.

Specific syndromes. The presence of chromosomal anomalies or other syndromes that are associated with CHD should alert the clinician to the presence of specific cardiac defects. Trisomy 21 is the commonest anomaly associated with heart disease; others include trisomy 13 and 18, Turner and Noonan syndromes, and velocardiofacial and Di George syndromes

Major Criteria for Clinical Diagnosis of Congenital Heart Disease:

Systolic Murmur Grade III or More , Diastolic Murmur , Central Cyanosis , Congestive Cardiac Failure

Minor Criteria:

Systolic Murmur Less than Grade III , Abnormal Second Sound , Abnormal Electrocardiogram

Complication of CHD:

Pulmonary arterial hypertension (PAH) :Lesions that have the greatest likelihood of developing PAH include cyanotic heart disease with increased pulmonary blood flow. Here irreversible changes in pulmonary vasculature develops rapidly often during infancy. It is particularly important to correct or appropriately palliate these lesions early (ideally within the first few months of life).

Larger acyanotic post-tricuspid shunts are also prone to early development of PAH and should be ideally corrected early, preferably within the first year. In pre-tricuspid shunts, P AH develops slowly and unpredictably.

Infective endocarditis or endarteritis (IE) : Endocarditis can complicate CHD, especially in patients with significant turbulence created by high-pressure gradients, e.g. restrictive VSD and PDA, tetralogy of Fallot, and left ventricular outflow tract obstruction. Some surgical operations (such as the Blalock-Taussig shunt) are also associated with increased risk of IE or endarteritis. Lesions with little or no turbulent flows, such as ASD are not associated with increased risk. The risk of endocarditis increases after dentition, hence the importance of good dental hygiene in patients with CHD cannot be over emphasized.

Growth and nutrition : This is affected in all forms of CHD and is particularly striking in large left to right shunts. Children with CHD show high prevalence of malnutrition, which improves after correction of the underlying condition. Catch up growth is slow if CHD is corrected late.

Myocardial dysfunction: Chronic volume overload results in ventricular enlargement and ventricular dysfunction that is typically reversed after correction. A small proportion of patients with severe hypoxia also develop severe dysfunction involving both ventricles. Heart failure is mostly the result of hemodynamic consequences of increased pulmonary blood flow, mitral or tricuspid valve regurgitation and severe myocardial hypertrophy. Systolic dysfunction is a relatively less common cause.

Neurologic and neurodevelopmental conse quences Chronic hypoxia, in utero hypoxia and hypo perfusion and open-heart surgery contribute substantially to morbidity. Brain abscess is uniquely associated with cyanotic heart disease (typically beyond the age of 2 yr) where the right to left shunt bypasses the pulmonary filter.

Polycythemia Older children with cyanotic CHD are prone to complications from a chronically elevated red cell turnover. These include symptoms of hyperviscosity, gout, renal failure and gall stones.

Rhythm disorders and sudden death Chronic enlarge ment of heart chambers predispose to tachyarrhythmia. Chronic right atrial enlargement (such as atrial septal defect, Ebstein syndrome, severe tricuspid regurgitation) predisposes Disorders to atrial flutter, which might of Cardiovascular System be persistent - and refractory.

Chronic right ventricular enlargement pre disposes to ventricular tachycardia and may precipitate sudden cardiac arrest. This is a significant long haiterm concern after TOP repair where the pulmonary valve is rendered incompetent. Similarly left ventricular hypertrophy and dysfunction are associated with high risk of ventricular tachycardia.

Cyanotic spells : Patients with the VSD-PS physiology is prone to cyanotic spells. Cyanotic spells are due to an acute decrease in pulmonary blood flow, increased right to left shunt and systemic desaturation due to

(i) Infundibular spasm due to increase in circulating catecholamines, during feeding or crying;

(ii) Activation of mechanic receptors in right ventricle (due to decrease in systemic venous return) or in left ventricle (due to decrease in pulmonary blood flow), leads to peripheral vasodilatation and fall in systemic vascular resistance producing increased right to left shunt and systemic desaturation.

A cyanotic spell is an emergency, which requires prompt recognition and intervention to prevent disabling cerebrovascular insults or death. The spell needs to be taken seriously not just because of the immediate threat but also because it indicates the need for early operation. It is commonly seen below 2 yr (peaks between 2 and 6 months). The onset is spontaneous and unpredictable and occurs more often in early morning, although it can occur at any time in the day.

The infant cries incessantly, is irritable and inconsolable. Tachypnoea is prominent feature; there is deep and rapid breathing without significant subcostal recession. Cyanosis deepens as the spell progresses. Later gasping respiration and apnoea ensues, which leads to limpness and ultimately anoxic seizures. Spells can last from minutes to hours. Auscultation reveals softening or dis appearance of pulmonary ejection murmur.

Prevention of CHD

Education of lay public on the risks associated with consanguinity, drugs and teratogens in the first trimester of pregnancy and widespread immunization against rubella has limited role in preventing CHD. However, most CHD do not have an identifiable etiology and there is no effective strategy for their prevention in the preconceptional period.

Fetal echocardiography is emerging as a modality for early diagnosis of CHD. Conditions that involve major chamber discrepancy (such as hypoplastic left heart syndrome), single ventricles and common AV canal can be identified by routine screening as early as 14-16 weeks gestation. With some refinement, additional conditions such as tetralogy of Fallot, large VSD, transposition of great vessels and persistent truncus arteriosus can be detected.

Once a serious CHD is identified, it is vital to counsel the families about postnatal manifestations, natural history, surgical options and their long-term outlook. Before 20 weeks of gestation, medical termination of pregnancy is an option.

Results of fetal echocardiography enable delivery at a centre with comprehensive paediatric heart program. While echocardiography is recommended for future pregnancies after diagnosis of serious CHD in a child, this practice has low yield because only 2-8% CHD recur.


In the World of intricate life, CHD propose a different challenge and CHD just gives the lads of around 18yrs only and surgical treatment is successful only about 3.15% and together with advancements in medicine and technologies we hope to both prevent and cure the kids.


About the Creator


Meet Mr.Vijay From India, cat enthusiast, Business Devotee Balancing healing with writing, he unwinds to The Weeknd's beats. Join his harmonious journey of medicine and creativity, where every heartbeat and word matter. 🩺📝🐾

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