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Content text CONGENITAL HEART DISEASE | CVS EXAMINATION

LONG CASE CONGENITAL HEART DISEASE ● VSD ● TOF VSD TOF Description An abnormal communication between the left and right ventricle that results in left - to - right shunting of blood flow Definition Simultaneous occurrence of cyanotic heart disease ● right-ventricular outflow obstruction (RVOTO) due to pulmonary stenosis ● right-ventricular hypertrophy (RVH) ● ventricular septal defect (VSD) ● overriding of aorta Epidemiology Most common congenital heart disease Epidemiology Most common cyanotic congenital heart disease Association ● Down syndrome ● Edward syndrome ● Patau syndrome ● Fetal alcoholic syndrome ● TORCH Association ● Typical sporadic ● Sometimes associated with genetic syndrome ○ Down syndrome ○ DiGeorge syndrome ● Other cardiac anomalies (40%) Examination findings ● Harsh, pansystolic murmur best heard at LLSE, radiate to all areas ● Systolic thrill ● +/- loud P2 Examination findings ● Harsh systolic murmur best heard over Erb’s point and ULSE Investigation ● Doppler echo - to confirm diagnosis, evaluate size of defect and shunt volume, to exclude other possible associated anomalies ● ECG - RVH ● CXR - Enhanced pulmonary vascular markings, left atrial and ventricular enlargement, enlarged right ventricle and pulmonary artery in late stage Investigation ● SpO2 low ● ECG - Right axis deviation, RVH, P pulmonale ● Echo - detection of features of TOF, quantification of the right ventricular outflow tract pressure gradient ● CXR - absent pulmonary artery segment, decreased pulmonary vascular marking, boat-shaped heart Treatment Small defect Treatment Severe RVOT
● often heal spontaneously, follow up with echo Large defect/ symptomatic ● treat as heart failure ● surgical repair (patch) ○ in child <1y/o with signs of pulmonary hypertension ○ Older child who do not improve with medical therapy Eisenmenger syndrome ● heart / lung transplant ● Require administration of prostaglandin PGE-1 to maintain ductus arteriosus prior to surgery Acute hypoxia ● Oxygen + knee-chest position ● Squatting + IV morphine + fluid + IV beta blocker (to reduce sympathetic nervous system activity and reduce infundibular muscular spasm) + if above measure failed Treat heart failure Surgical intervention ● VSD repair - patch closure of VSD, ensure correct aorta position absolve left ventricle ● Enlargement of RVOT - resection of the obstructive infundibular musculature ● If patient not fit for surgery in 1st year of life, Blalock-Taussig shunt is used as a palliative procedure ○ A shunt that connect pulmonary artery to subclavian artery to increase pulmonary blood flow ○ Not curative, patient needs additional surgery to correct the defect Complications ● Arrhythmia ● Right heart failure ● Eisenmenger syndrome ● Infective endocarditis ● Aortic regurgitation HISTORY TAKING Presenting complaint - Reason for current admission. Diagnosis ● When made (birth, days, weeks or months); where; symptoms at diagnosis (e.g. cyanosis, tachypnoea, poor feeding, failure to thrive, recurrent infection); how (initial investigations: CXR, electrocardiography, echocardiography, angiography). Initial treatment ● Surgical (e.g. palliative, such as balloon septostomy or shunting procedures, or corrective, such as patent ductus ligation).
● Pharmacological (e.g. diuretics, angiotensin-converting enzyme [ACE] inhibitors) and side effects thereof. Past history ● Aetiology (e.g. rheumatic fever, pregnancy complicated by maternal rubella, smoking, alcoholism, or drugs such as lithium, warfarin or phenytoin). ● Indications and number of previous hospital admissions. ● Pattern and timing of change in condition (e.g. when cyanosis or heart failure developed, when and how it was controlled). ● Episodes of infective endocarditis. ● Other complications of disease (e.g. cerebral thrombosis or cerebral abscess with cyanotic heart disease). Treatment ● Past surgery, complications thereof, plans for further operative procedures. ● Past interventional catheter procedures. ● Past ablation therapy, pacemaker or defibrillator placement. ● Medications, past and present, the side effects of these, monitoring levels (e.g. digoxin), treatment plans for future. ● Exercise restrictions (may be inappropriately applied by parents). ● Recommendations for antibiotic prophylaxis for dental procedures; maintenance of dental hygiene. ● Compliance with treatment. ● Any need for identification bracelet. ● Instructions for air travel and high altitude. ● Any recent investigations monitoring treatment (e.g. Holter monitoring; oxygen saturation). ● Any recent changes in treatment regimen, and indications for these. Current state of health ● Symptoms of cardiac failure, such as fatigue and shortness of breath (in comparison with peers), cough, sweating, poor feeding, recurrent chest infections. ● Cyanosis, squatting with ‘Fallot turns’. ● Episodes suggestive of arrhythmias, such as syncope, alteration of consciousness, dizziness, shortness of breath, palpitations in older children (what was the rate; i.e. faster than when usually playing or excited), or a ‘funny feeling’ in the chest, sweating, nausea, vomiting or the parents being unable to count the rapid pulse rate, suggesting a tachyarrhythmia. ● Chest pain (e.g. myocardial ischaemia versus musculoskeletal, pleural or pericardial causes) or headache (e.g. polycythaemia in cyanotic heart disease, severe hypertension, cerebral abscess). ● Recent change in condition. Note the temporal relationship between associated symptoms with any tachycardia or palpitation: they should coincide—functional chest pain can be followed by a reflex sinus tachycardia.
Other association problems ● These may either be related to the main diagnosis (e.g. Down, Marfan) or be more general problems (e.g. poor growth, developmental delay, especially involving gross motor abilities, exercise limitations and psychological effects). Social history ● Disease impact on child; for example, growth, development, schooling (academic performance, sports restrictions, teachers’ attitudes, peers’ attitudes, teasing, amount of school missed and whether schooling is appropriate). ● Disease impact on parents; for example, financial situation, financial burden of disease so far, government allowances being received, marriage stability, restrictions on social life, plans for further children, genetic counselling, or at least an awareness of the risks of recurrence, and availability of fetal echocardiography. ● Disease impact on siblings; for example, sibling rivalry, effect of family financial burden. ● Social supports; for example, social worker, contact with other families of children with similar problems, break from managing child. ● Coping: contingency plans (e.g. plan if child develops severe febrile illness); parents’ degree of understanding regarding cause, prognosis, exercise restriction, and antibiotic prophylaxis for operations and dental procedures. ● Access to local doctor, paediatrician, cardiac outpatients clinic (where, how often), other clinics attended. Family history ● Any early cardiovascular death (e.g. hypertrophic cardiomyopathy [HCM]), unexplained death (e.g. long QT syndrome [LQTS]) or arrhythmias (e.g. Wolff–Parkinson–White [WPW] syndrome); any known cardiovascular diagnoses (e.g. cardiomyopathies); any known syndromes (e.g. Marfan). Immunization ● Any unnecessary delays, local doctor attitudes, parents’ understanding of importance. PHYSICAL EXAMINATION Wash hand Great parents and gain consent to examine Position: supine 45 degree propped up if can obey command, lie flat/ opportunistic examination if unable to obey command Exposure: remove shirt completely General inspection: ● Alert, conscious ● Signs of respiratory distress

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