Approach to Congenital Heart diseases

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Pediatrics Cardiology


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Approach to Congenital Heart diseases:

Approach to Congenital Heart diseases Dr Isha Deshmukh Assistant Professor GMC Mumbai

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CHD - commonest of all congenital lesions and the most common type of heart defects among children . Result from the aberrant development of a normal structure in fetus or failure of progress beyond the early stage of embryonic or early fetal development.

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The CHD as defined by Mitchell et al is a gross structural abnormality of the heart or intra thoracic great vessels that are actually significance. The estimated prevalence of CHD is 8 to 10 per 1000 live births, with a higher rate of stillbirth, spontaneous abortion, and prematurity.

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Multifactorial in origin – genetic and environmental factors Known genetic causes of heart disease inherited chromosomal abnormalities such as trisomy 21, 13, 18 newly recognized genetic point mutations, point deletions and other genetic abnormalities as seen in syndromes  CATCH 22, familial ASD with heart block, Alagille syndrome, Noonan syndrome

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Known ante-natal environmental factors include maternal infections ( Rubella), drugs (alcohol, hydantoin, lithium and thalidomide) and maternal illness (diabetes mellitus, phenylketonuria, and systemic lupus erythematosus ). High levels of stress were more likely to have high levels of a hormone called corticotrophin-releasing hormone (CRH) in their blood.

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A careful family history spanning at least three generations . Consanguinity should be investigated, as it is a clue for autosomal recessive inheritance and a recognized factor for increased risk of recurrence. The risk of recurrence increases if close relatives are also affected.

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Mandolin syndromes that are known to have a high degree of variability of expression ( e.g. Noonan syndrome, Kartagener syndrome, hypertelorism-hypospadias syndrome, and Waardenburg syndrome.

Aetiology of CHD:

Aetiology of CHD

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Clinical manifestations of congenital heart defect vary according to the type and severity of the defect. neonatal period  cyanosis, heart failure, failure to thrive, an abnormal clinical sign detected on routine examination. infancy and childhood  cyanosis, digital clubbing, murmur, syncope, squatting episodes, heart failure, arrhythmia, failure to thrive

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Pulse oximetry – hyperoxia test Differences in BP in upper limbs and lower limbs Peripheral pulses Other congenital malformations including respiratory and genito-urinary system

Prevention of CHD:

Prevention of CHD Primary prevention Modification/abolishment of risk factors  a) Vaccines b) health promotion (via exercise) vitamins especially Folic acid to be started before conceiving. Secondary Prevention Recognition of subclinical disease and early treatment of initial clinical manifestations to prevent progression of disease. Teritary Prevention Limiting disability to the least possible and aiding the recovery from complications e.g. rehabilitation efforts .

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Classification of CHD by Pathogenetic Mechanisms 1. Abnormalities of Mesenchymal Tissue Migration (conotruncal defects ) Subarterial ventricular septal defects Aortopulmonary window(AP window) Double-outlet right ventricle(DORV) Tetralogy of Fallot (TOF) D- Transposition of the great vessels (DTGA) Truncusarteriosuscommunis Interrupted aortic arch, type B Pulmonary atresia with ventricular septal defect (VSD)

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2. Altered Cardiac Hemodynamics Coarctation of aorto with intact ventricular septum Hypoplastic left heart syndrome (HLHS) Aortic valvular stenosis (AS) Interrupted aortic arch, type A Atrial septal defect, secundum type (20ASD) Pulmonary atresia without ventricular septal defect Perimembranous ventricular septal defect (pmVSD)

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3. Abnormalities in Programmed Cell Death Muscular ventricular septal defect Ebstein’s anomaly 4. Abnormalities of Extracellular Matrix Endocardial cushion defects (AV canal defects) 5. Targeted Growth Defects Total anomalous pulmonary venous return (TAPVC) Partial anomalous pulmonary venous return Single atrium

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Table 3: Extracardiac malformations most frequently reported among patients with congenital heart diseases Extracardiac malformations Central nervous system Hydrocephalus Corpus callosum agenesis Defects of the neural tube closure Craniofacial Cleft lip/palate Eyes Microphthalmia/anophthalmia Respiratory Diaphragmatic hernia Pulmonary hypoplasia/ agenesis Tracheoesophageal fistula Pulmonary segmentation anomalies

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Digestive Esophageal atresia/stenosis Duodenal atresia/stenosis Omphalocele Anal atresia/stenosis Musculoskeletal Upper limbs deficiency Polydactyly/syndactyly Costovertebral anomalies Dislocation of the hip

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Clubfoot Genitourinary Renal duplication Urethral/renal pelvis duplication Hydronephrosis Renal agenesis/hypoplasia Cystic kidney disease Ectopic kidney

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Vesicoureteral reflux Hypospadias Spleen anomalies Asplenia/polysplenia


Investigations Routine investigations- CBC Serum electrolytes Chest xray ECG 2 D Echo USG Abdo-pelvis Infantogram


Treatment Oxygen therapy Mechanical Ventilation Fluid theray Management of intercurrent illness Monitoring for weight gain Treatment of Congestive symptoms Surgical intervention

Timing of surgery:

Timing of surgery PGE1 should be administered to a cyanotic newborn with duct dependent condition along with another supportive measure. Surgery should be scheduled on a semi-elective basis after careful evaluation is complete. Surgery -> only effective therapy for neonates with obstructed total anomalous pulmonary venous return and should be performed as soon as the diagnosis is established .

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d-transposition of the great arteries with restrictive patent foramen ovale often need emergent balloon atrial septostomy because of profound hypoxemia. After successful septostomy these newborn are usually quite stable and corrective surgery should be delayed until any end-organic damage resolves .

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A newborn who present with congestive heart failure and shock because of left heart obstructive lesions (e.g., interrupted aortic arch) also can be stabilised by the administration of PGE1 and other supportive care.

Types of surgical procedures:

Types of surgical procedures Palliative surgery refers to procedures where complete repair of the heart defect is not possible and blood flow is controlled either with a shunt or an artificial tube implanted in the heart or with only one ventricle of the heart.

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Cardiac catheterization Diagnostic catheterization 2 . Interventional catheterization. Arterial Switch procedure Blalock-Taussig (BT) shunt Norwood procedure

Pediatric Heart transplantation:

Pediatric Heart transplantation End stage pediatric heart disease Need for ongoing intravenous inotropic or mechanical circulatory support . Complex congenital heart disease not amenable to conventional surgical repair or palliation or for which the surgical procedure carried a higher risk of mortality than transplantation

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Progressive deterioration of ventricular function or functional status despite optimal medical care with digitalis, diuretics, and angiotensin-converting enzyme (ACE) inhibitors. • Malignant arrhythmia or survival of cardiac arrest unresponsive to medical treatment, catheter ablation, or an automatic implantable defibrillator. • Progressive pulmonary hypertension that could preclude cardiac transplantation at a later date. • Growth failure secondary to severe congestive heart failure unresponsive to conventional medical treatment. • Unacceptably poor quality of life.

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