B O S S CHD prevalence rate at birth 6.16 per live births

Transcription

1 Coarctation of the Aorta (COA)

2 B O S S CHD prevalence rate at birth 6.16 per live births

3 COA coarctation of the Aorta LV

4 Coarctation of the Aorta with PDA without PDA Prominent posterior infolding/shelf Simple Complex: + VSD, AS, MS, CTA sin.

5 Coarctation of the Aorta Diagnosis may be difficult If PDA opened no discrepant pulses no murmur heart is normal COA is hidden by PDA PGE

6 LV dysfunction Diminished stroke volume of LV Increased LVEDP, LAp Pulmonary venous congestion - PAH Dimin. i Myocardial perfusion - metabolic acidosis i Compensatory mech.: LVEDV/Frank-Starling m, HR, MYO contr

7 Coarctation of the Aorta Neonatal Adult Symptoms Heart failure Hypertensis UE Murmur - + Discrepant pulses + / - (PDA) + Differential cyan. + / - (PDA, VSD) - RTG cardiomegaly Rib notching ECG dominant RV Hypertr. LV Imaging ECHO ACG, MRI

8 Critical COA, newborn

9 Critical COA

10 Critical COA, newborn

11 Pharmacological promote of PDA patency Prostaglandin E1 (Aloprostan) in continual i.v. infusion Inicial dose 0,025 µg/kg/min., maintaining 0, µg/kg/min. CAVE: apnoic pause, hyperpyrexia

12 Critical newborn, PDA - prostaglandin

13 Angiocardiografy g Neonatal COA + hypoplastic aortic arch + patent PDA

14 Treatment/COA Resection/COA + anastomosis end-to-end Subclavian flap aortoplasty - Waldhausen s technique Resection/COA + reversal Waldhausen aortoplasty. t Aortic arch aortoplasty using a homograft Extraanatomical by-pass Pulmonary banding at 1/3 operated newborns (complex coarctations ti with a large left-to-right t shunt

15 COA resection and anastomosis end-to-end

16 Waldhausen s aortoplasty of the aortic isthmus

17 COA resection and reversal Waldhausen s aortoplasty

18 COA resection and aortic arch aortoplasty Amato s technique

19 Asymmetric 4CH view, foetus /COA 26th W. RA LA RV LV

20 Stenosis of aortic isthm COA/ foetus 26.th W AO * LA COA DAO

21 Coarctation of the Aorta Neonatal Adult Symptoms Heart failure Hypertensis UE Murmur - + Discrepant pulses + / - (PDA) + Differential cyan. + / - (PDA, VSD) - RTG cardiomegaly Rib notching ECG dominant RV Hypertr. LV Imaging ECHO ACG, MRI

22 Diastolic runoff patern - DAO

23 LCAR AOA COA RPA DAO

24 Diastolic runoff patern - COA 4.2 m/s (70 torr)

25 Severe adult coarctation of the aorta Rib notching

26 Extraanatomical by-pass

27 Balloon Angioplasty/ RECOA

28 1999 APL RECOA: aneurysm 2004

29 30 o RAO, 20 o CAUD. Stenting ti RECOA

30 Interrupted aortic arch (IAA)

31 Embryonic arch diagram

32 Interrupted aortic arch A1 B1 C1 B2 B1 RAA

33 Interrupted aortic arch

34 Interrupted aortic arch

35 Interrupted aortic arch p Fetal dg.

36 Interrupted aortic arch Fetal dg.

37 Interrupted aortic arch B1 RCAR BCT LCAR LCAR RSA AO PA * DAO A B C

38 Interrupted aortic arch A1 LCAR AOA LSA PA * DAO

39 *

40 Interrupted aortic arch Symptoms and treatment = neonatal COA Diagnosis heart failure ECG: dominant RV RTG: kardiomegaly, pulmonary vascularity Differential cyanosis (not in a large VSD) Treatment anastomosis DAO and AOA VSD closure

41 Persistent Truncus Arteriosuss (PTA)

42 PTA - persistent truncus arteriosus

43 Persistent truncus arteriosus

44 Persistent truncus arteriosus

45 Persistent truncus arteriosus

46 Persistent truncus arteriosus

47 Persistent truncus arteriosus

48 PTA - persistent truncus arteriosus

49 Persistent truncus arteriosus

50 Persistent truncus arteriosus Clinical features cyanosis heart failure Accentuated periph.pulses ( diastole!) click, murmur, continuous AS, AI ECG: biventricular hypertrophy, dominant RV RTG: cardiomegaly, pulmonary congestion Treatment t VSD closure, truncal valve - LV Conduit RV pulmonary arteries

51 Anomalous pulmonary venous connection (Total: TAPVC) (Parcial: PAPVC)

52 Total anomalous pulmonary venous connection Intracardiac infracardiac (infradiafragm.) supracardiac

53 TAPVC - totální anomální návrat plicních žil

54 Supracardiac TAPVC (LVV)

55 Supracardiac TAPVC (LVV)

56 Supracardiac TAPVC (LVV)