Paediatric Cardiology. Acyanotic CHD. Prof F F Takawira

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1 Paediatric Cardiology Acyanotic CHD Prof F F Takawira

2 Aetiology Chromosomal Down syndrome, T13, T18 Genetic syndromes (gene defects) Velo-Cardio-facial (22 del) Genetic syndromes (undefined aetiology) Toxins alcohol, warfarin Diabetes, SLE, rubella Most cases without clear explanation recurrence risk incr 3-4 fold

3 Fetal circulation Inferior vena cava Ductus venosus Oxygenated blood from placenta via umbilical vein 50% passes through liver via portal veins 50% crosses ductus venosus and continues up inferior vena cava. IVC also receives blood leaving liver via hepatic veins and returning from lower half of body of fetus Umbilical vein

4 Fetal circulation Foramen ovale Blood from Inferior vena cava: LA Most passes through foramen ovale to left atrium and left ventricle, ascending aorta and coronary circulation RA RV LV Small amount crosses tricuspid valve into right ventricle Tricuspid valve

5 Fetal circulation Superior vena cava PA RA RV Blood returning from head and neck enters right atrium via superior vena cava, is joined by coronary sinus blood and then enters right ventricle and pulmonary artery

6 Fetal circulation Aorta Aorta Ductus arteriosus Ductus arteriosus PA LV RV 2/3 of combined ventricular output from right ventricle 85% of RV output goes via ductus arteriosus into descending aorta and 15% enters lungs via pulmonary arteries Blood in descending aorta travels via internal iliac and then umbilical arteries to placenta

7 Normal Transition Commencement of breathing Decrease in PVR Increase in SVR Closure of the three fetal systemic-pulmonary shunts: Ductus arteriosus Foramen ovale Ductus venosus

8 Age Related Changes Fetal communications ductus arteriosus foramen ovale ductus venosus Pulmonary vascular resistance Growth Importance of 6 week baby check

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11 History Brainstorm: What are the key areas for paediatric history taking relating to heart conditions?

12 History Shortness of breath/tachypnoea Sweating Persistent cyanosis Feeding problems Growth problems Family history of CHD

13 Location of murmurs

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15 Nature of Murmurs pansystolic decrescendo ejection mid diastolic flow truncated continuous clicks, snaps, etc

16 Grades of murmurs Grade 1 scarcely audible Grade 2 Soft, easily audible Grade 3 loud, thrill absent Grade 4 loud, thrill present Grade 5 very loud, audible with stethoscope just off chest Grade 6 audible without a stethoscope

17 Congenital Heart Disease Acyanotic CHD

18 Approach to CHD CHD Cyanotic Acyanotic Increased PBF Decreased PBF Normal PBF Increased PBF TGA Truncus TAPVC HLHS TOF Tricuspid Atres PA, Critical PS Ebstein AS Coarct PS TR, MR Lft-Rght shunt VSD PDA; ASD AVSD Cyanosis, SOB FTT, Sweats Poor feeding, Chest def CCF, Cardiom, Congest Cyanosis No cardiom-usually No CCF Oligaemia Acyanotic, SOB FTT, Sweats Poor feeding, Chest def CCF, Cardiomegaly, Conges

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20 Growth Charts

21 Pectus Carinatum

22 Harrison s sulcus

23 Box diagram of normal heart

24 Acyanotic Left to right shunts VSD PDA ASD AV septal defect (Endocardial Cushion defect)

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27 Ventricular Septal Defect Physiology

28 Box diagram of ASD

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32 Acyanotic Obstructive lesions Pulmonary stenosis Aortic stenosis Coarctation

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36 Evaluation and Management of the Child with Heart Disease Differentiate Normal from Abnormal (dispelling doubts) Decision regarding referral / Investigation Ongoing Care

37 Features of an Innocent Murmur Normal peripheral examination murmur in isolation Typical features systolic ejection musical soft murmurs (grade 2/6 or less) change with body position varies with review / augmented by illness

38 Innocent murmurs Still s Pulmonary flow murmur Carotid bruit Venous hum

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40 Routine Investigation Chest X-Ray position contour pulmonary vascularity ECG right sided forces prominent include V3r / V4r

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42 Advanced Investigation Echocardiography Cardiac catheterisation / Angiography diagnostic haemodynamic intervention

43 Clues to Congenital Heart Disease Does the patient appear normal? Is the patient thriving? Is the patient cyanosed? Are there symptoms of heart failure? Tachypnoea, poor feeding, exercise intolerance etc Are there other signs of heart disease? Clubbing pulses / distribution / blood pressure hepatomegaly pericardial overactivity / thrill Murmur Is there any doubt?

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45 Growth Charts

46 Summary Important and complex area Needs lots of practice Essential to know the basics - ie know what you are looking and listening for Essential to gain experience in CVS examination and looking at and interpreting ECG If in doubt => REFER!!