Current status of pediatric cardiac surgery

Transcription

1 Current status of pediatric cardiac surgery Sabine H. Daebritz Dept. of Cardio-vascular Surgery Heart Center Duisburg, Germany Normal circulation 1

2 Complex cardiac lesions Stenoses Shunts Malconnections Hypoplasias mixed circulation Palliation - Correction Palliation: Addition of a mistake to to stabilize a given mistake - Banding of the pulmonary artery pulmonary blood flow - Aorto-pulmonary shunt (Blalock, central) pulmonary blood flow 2

3 Balancing of pulmonary blood flow PA-Banding Classical and modified d Blalock- Taussig shunt Blalock-Taussig shunt Stenosis of PA in up to 33% 3

4 Disadvantages of palliation No correction of pathologic circulation Cyanosis Myocardial hypertrophy Volume load of ventricles Morbidity of palliative procedure Increase of overall strain Palliation - Correction Correction: Creation of a `normal Circulation either with normal anatomy (etexample: TGA) or with pathologic anatomy (example: univentrikular heart) If possible: normal anatomy (example: TGA: arteriel switch - Senning/Mustard) 4

5 Complex cardiac malformations Correction as early as possible Avoidance of: Cyanosis Myocardial hypertrophy Volume overload of the ventricles Morbidity of palliative procedure Reduction of overall strain Examples of early correction of cardiac malformations Truncus arteriosus: AVSD: Fallot: AVSD und Fallot: Univentrikular heart: >2 kg 3. /4. month < 6 months > 3. /4. month early Glenn 5

6 Limitations of early correction Technical limitations (CPB, cannulas, prosthetic material, homografts..) High pulmonary resistance of the newborn (early Glenn / Fontan not possible) Example: anatomical / functional correction Transposition of the great arteries (TGA) Hypoplastic left heart syndrome (HLHS) Cardiac transplantation 6

7 Transposition of the great arteries TGA Physiologic circulation Circulation in TGA PDA ASD VSD Transposition of the great arteries 7

8 Arterial switch I Arterial switch II 8

9 Arterial switch III Arterial switch IV 9

10 Results of arterial switch 100 Survival n = 312, Mortality since %, n=261 survival [%] time [months] Daebritz Ann Thorac Surg 2000 Follow-up 3.6+/-2.7 (3-14.7) years 97% good / excellent 95% without cardiac medication Freedom from reop. 5/10 years 96% / 94% Hypoplastic left heart syndrom (HLHS) 10

11 HLHS Transplantation Norwood I (stage I) Mortality 19,1% Glenn (stage II) Fontan (stage III) HLHS 11

12 Norwood - Stage I - Ligation of Ductus arteriosus - Transsection of the pulmonary trunk - Closure of the PA distally distal (Patch?) - Inzision of the Aorta beyong aortic isthmus Norwood - Stage I Tailoring of the homograft patch 12

13 Norwood - Stage I - Rekonstruction of the Aorta by inssertion of homograft patch into - proximal descending Aorta - Aortic arch - ascending Aorta - pulmonary trunk Norwood - Stage I - Completion of the anastomosis 13

14 Norwood - Stage I - Insertion of modified BT - shunt - alternative I: initial completion of proximal anastomosis (for continuous cerebral perfusion) -alternative ti II: RV-PA Shunt (Sano) (no decrease of diastolic pressure) Glenn anastomosis 14

15 Fontan anastomosis Lateral tunnel Mortality of stage I - III Norwood I % Non - HLHS 19 % % only % bidir. Glenn (n=106) 3.8 % Fontan (n=69) 2.8 % Daebritz JTCVS

16 Fontan-Operation: Separation of pulmonary and systemic circulation in univentricular hearts Fontan, Thorax 1971 `Fontan paradox (de Leval) Good hemodynamics of a normal circulation: low (inf.) V. cava pressure < 10mmHg pulmonary artery pressure > 15 mmhg `The paradox of the Fontan circulation is that it imposes a caval hypertension and a pulmonary hypotension * * de Leval, Ped Cardiology

17 Late mortality of the Fontan Palliation Survival after lateral tunnel Fontan at 5 and 10 years: 3% and 91% * Survival including extracardiac TCPC (59%) at 5, 10 and 15 years: 90%, 86% and 82% # * Stamm JTCVS 2001, # Hosein EJCTS 2007 Actuarial survival according to the date of operation Survival [%] p = (n=109) (n=85) Time [years] 17

18 Congenital Heart Surgeons Society* Results of transplantation in HLHS: Survival after 1, 2 and 3 years: 85%, 64% and 62% *Jacobs, JTCVS 1998;116: Age distribution HTx >18 years Munich Grosshadern number of 15 patients < > 1Jahr 1 year Jahre years 5-12Jahre 6-11 years 12-18Jahre years Age Altersverteilung groups 18

19 Survival after HTx < 18 years of age 1,0,8,6 6-year survival 80% Kum. Übe erleben,4,2 0, FOLLOW UP in Jahren Quality of life: excellent / good 19

20 Summary HTX Good results in childhood Advantage: anatomical `correction Diasadvantage: lifelong medical treatment Results become better Problem: organ shortage, particularly for neonates Complex cardiac malformations Correction: As early as possible As complete as possible As anatomical as possible Cardiac transplantation as alternative to non-anatomical correction 20

21 Nature does nothing without t use, and more is useless, if less is enough, because nature loves the simplicity and does not like luxury Isaac Newton 21