Paediatric Cardiology Acyanotic CHD Prof F F Takawira
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
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
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
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
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
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
Age Related Changes Fetal communications ductus arteriosus foramen ovale ductus venosus Pulmonary vascular resistance Growth Importance of 6 week baby check
History Brainstorm: What are the key areas for paediatric history taking relating to heart conditions?
History Shortness of breath/tachypnoea Sweating Persistent cyanosis Feeding problems Growth problems Family history of CHD
Location of murmurs
Nature of Murmurs pansystolic decrescendo ejection mid diastolic flow truncated continuous clicks, snaps, etc
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
Congenital Heart Disease Acyanotic CHD
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
Growth Charts
Pectus Carinatum
Harrison s sulcus
Box diagram of normal heart
Acyanotic Left to right shunts VSD PDA ASD AV septal defect (Endocardial Cushion defect)
Ventricular Septal Defect Physiology
Box diagram of ASD
Acyanotic Obstructive lesions Pulmonary stenosis Aortic stenosis Coarctation
Evaluation and Management of the Child with Heart Disease Differentiate Normal from Abnormal (dispelling doubts) Decision regarding referral / Investigation Ongoing Care
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
Innocent murmurs Still s Pulmonary flow murmur Carotid bruit Venous hum
Routine Investigation Chest X-Ray position contour pulmonary vascularity ECG right sided forces prominent include V3r / V4r
Advanced Investigation Echocardiography Cardiac catheterisation / Angiography diagnostic haemodynamic intervention
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?
Growth Charts
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!!