1. C ASE REPORT FOR V. GRADE STUDENTS Cardiology Dr. Kemény Viktória

2. M.D. – 6 MONTH OLD BOY Was born on 40th week of gestational age – pvn Normal pregnancy Normal delivery Normal perinatal period Birth Weight: 3700 gr

3. Taken home from the Hospital on the third day General practitioner checked him on the fifth day of his life – healthy newborn Mother had enough breast milk, the baby was eating well, his weight was increasing

4. 3 weeks later mother was complaining about feeding difficulties (!!!) – got a prescription for formula (not enough BM?!) On the 4th week : recheck at the GP same weight as a week ago tachydyspnea hepatomegaly holosystolic heart murmur

5. C ARDIOLOGICAL EXAMINATION Status: clinical signs of congestive heart failure (CHF) (tachydsypnea, hepatomegaly) 4/6 holosystolic heart murmur palpable femoral pulses

6. Echocardiography: - 8mm communication between the two ventricules on the membranous part of the intraventricular septum (flow velocity of the left-right shunt: 4,5 m/s) - Normal atrial septum - Normal left ventricular function, but the aorta/left atrium ratio was 12/17 mm!!! - Normal flow in aorta and pulmonary trunk - Competent AV- and semilunar valves

7. Echocardiography

8. Diagnosis Membranous VSD Congestive heart failure VSD is the most common congenital heart disease and accounts for 15% to 20% of all such defects!

9. VSD The ventricular septum can be devided into a small membranous portion and a large muscular portion. The muscular septum has three component: the inlet, the trabecular and the outlet (infundubular or conal) septum. Prevalence: Perimembranous defects: 70% Outlet (infundibular or conal) defects: 5-7 % Inlet (or AV canal) defects: 5-8 % Trabecular (muscular) defects: 5-20 %

11. Q UESTIONS What happened to our baby during the first weeks?! Why did not hear the doctors the heart murmur on the first week? What was the first sign of the heart failure ?

12. P OSTNATAL CHANGES IN THE PULMONARY VASCULAR RESISTANCE

13. C HANGES IN C IRCULATION AFTER B IRTH The PVR is as high as the SVR near or at term Lung exspansion results reduction of PVR, increase in pulmonary blood flow and fall in PA pressure Even in the presence of large VSD in a newborn, the PVR remains elevated, and therefore a large shunt does not occur, until the infant reaches 6 to 8 weeks of age when the PVR decreases, the shunt increases and CHF may develope

14. T HERAPY : Loop diuretic (+ Potassium) ACE inhibitors (afterload reducing med.) /Some centers use digoxin too – our patient didn’t get/ After a week of therapy the baby’s weight increased, breathing became normal, the hepatomegaly decreased.

15. M ANAGEMENT Infants with large VSD, CHF: if growth failure cannot be improved by medical therapy, the VSD should be operated on within the first 6 months of life Surgery should be delayed for infants who respond to medical therapy If the PA pressure is more than 50% of systemic pressure, surgical closure should be done by the end of the first year Older infants with large VSD and elevated PVR should be operated on as soon as possible Infants who have small VSD without CHF or pulmonary hypertension are usually not candidate for surgery

16. T AKE HOME MESSAGE Even serious CHD-s can be „silent” in the first days/weeks of life Causes, of this are the changes of circulation after birth First clinical sign of the cardiological problems can be the feeding difficulty, the growth retardation CHF signs in infants: tachydyspnea + hepatomegaly (right- and left sided failure) The management of the CHD depends on different conditions/abnormalities/changes

17. T HANK Y OU FOR Y OUR ATTENTION !