Left Ventricular Outflow Tract Obstruction

Transcription

1 Left Ventricular Outflow Tract Obstruction Department of Paediatrics

2 Left Ventricular Outflow Tract Obstruction Subvalvular aortic stenosis Aortic Stenosis Supravalvular aortic stenosis Aortic Coarctation

3 Subvalvular aortic stenosis SAS

4

5

6 Subvalvular aortic stenosis Fibrotic/membranotic ring Muscular Postoperative HOCM AVSD

7 Subvalvular aortic stenosis

8 SAS: AVSD Goose neck

9

10 Subvalvar aortic stenosis

11

12 SAS: HOCM

13 Subvalvular aortic stenosis SAS operations in Bromton Hospital , follow-up for 2.5 years (n=50) 1-3 operations (30% re-do) Aortic regurgitation common Age at primary operation 8 years, at secondary op 14 and at third op Need 15 years for re-operations 30% Moderate to severe AI was present in 20% of patients with primary operations and 60% of patients with re-do surgery Barkhordian et al. Interactive Cardiovascular & Thoracic Surgery. 6(1):35-8, 2007 Feb.

14 SAS+AI

15 SAS+AI

16 Supravalvular aortic stenosis SVAS

17 Supravalvular aortic stenosis Hemizygous deletion of several genes on chromosome 7q11.23 Autosomal dominant disorder In full-blown form includes supravalvular aortic stenosis (SVAS) and multiple peripheral pulmonary arterial stenoses Elfin face Mental and statural deficiency Characteristic dental malformation Infantile hypercalcemia

18 Supravalvular aortic stenosis

19 Supravalvular aortic stenosis

20 Supravalvular aortic stenosis

21 Peippo et al. Duodecim 2001;117:505 12

22 Aortic Stenosis

23 Aortic Stenosis Bicuspid aortic valve (in 1-2% of general population) 15-20% in all adults with aortic stenosis More common in congenital population Atherosclerotic/infammatory process of the tricuspid aortic valve Endocarditis

24

25 Tricuspid Aortic Valve

26 Bicuspid Aortic Valve

27 Aortic Valve

28 Aortic Stenosis Bicuspid Valve

29 Aortic Stenosis Bicuspid Valve

30 Aortic Stenosis Bicuspid Valve

31 Aortic Stenosis Clinical examination Echocardiography Morphology and movement of the valve Pressure gradient Valve area LV size Thickness of septum and LVPW LV function Catheterization sometimes needed Coronary angiography in older patients? PH? if LV failure CT angiography?

32 Aortic Stenosis

33 Aortic Stenosis

34 Aortic Stenosis Systolic pressure gradient (mmhg) Valvar flow area (cm 2 ) Kupari et al. 2008

35 Vmax Aortic Stenosis Classification in adults Valvar area Mild Moderate Severe <3 m/s (36 mmhg) 3-4 m/s (36-64 mmhg) Area of LV outflow tract Velocity in LV outflow tract Velocity of the stenotic flow jet >4 m/s (>64 mmhg) Mean grad <25 mmhg mmhg >40 mmhg Flow area >1.5 cm cm 2 <1 cm 2 <0.6 cm 2 /m 2

36 Aortic Stenosis Peak gradient mmhg Mean gradient mmhg AS evaluation and follow-up what Doppler parameters (*15 American ja 9 non-american CHD centres) mild Moderate Severe *36-50? *64-80 *25-40? >*45-64 AoV area > <1 (0.8 cm 2 ) cm 2 /m 2 Khalid O et al Pediatr Cardiol 2006; 27(6): 661-

37 Aortic Stenosis Thoraxcentre, Erasmus Medical Centre Rotterdam Adult congenital AS patients with Aortic aortic velocity stenosis >2.5 m/sec, is a no aortic regurgitation progressive disease! N=84 (age y; mean 27 y) Yap et al. International Journal of Cardiology 122 (2007)

38 Aortic Stenosis

39 Aortic Stenosis

40

41 Aortic Stenosis

42 Aortic Coarctation

43 Aortic Coarctation

44 HAA CoA

45