Congenital heart diseases — strategy for treatment Ivan

Congenital heart diseases - strategy for treatment Ivan Milev Special hospital for surgery, Filip Vtori, Skopje Macedonia Banja Luka, October 2008

Congenital heart diseases (CHD) § Definition § Epidemiology (0, 7 - 1, 7%) *Europian journal Epidemiology, 2007 § § Individual correction Precise diagnosis Оptimal treatment Мinimal invasive diagnostic method § Neonatal mortaliy rate 1971 2001 general neonatal mortality 1971 2001 CHD neonatal mortality

*Common CHD Types of CHD % of all Ventricular septal defect Patent ductus arteriosus Atrial septal defect Pulmonary valve stenosis Aortic coarctation Tetrady of Fallot Congenital aortic stenosis Transposition of the great vessels Atrioventricular canal *National data for health statistic in Republic of Macedonia, 2005 year 30. 0 9. 0 7. 5 7. 2 6. 2 5. 6 5. 2 4. 8 3. 9

CHD – the trends The facts § Noninvasive techniques (3 D echo, 64 -VCT) § Changes in pediatrics cath lab § Time, experience, training, OR in stand by

CHD- diagnostic procedures Frequent echo mistake § ASD venosus type § Aortic coarctation § Muscular VSD § PDA § T/PAPVD – venous confluens Diagnostic heart cath § § SV Т. Fallot with pulmonary atresia Pulmonary atresia without VSD Multiply VSD’s

CHD- surgical treatment Podelba: - palijativen - reparativen Specifi~nosti: -Mal bodi indeks anestezija ekstrakorporalna cirkulacija kardioplegija postoperativen monitoring

Tipovi na hirurgija (31. 12. 2007) N = 4533

Preдoperativна riзик stratifiкација N=138 pac • • • Возраст : 39. 5 11 god. Пол : 59 (42%) ma{ki /79 (58%) `enski NYHA class : III/IV Euroscore 26. 5% KAB 9 (6, 5%) PAH 45 (33%)pac so cijanoza 3 (2%) Reoperacii 2 (1%) Bubre`na insuficiencija 10 (7. 2%) Respiratorna insuficiencija 76 (55. 1%) E`екцона фракcија :

Kongenitalni zaboluvawa N= 138 pac. ASD tip II so trik rekon VSD so trikusp. rek. VSD so pro{ir. na RVOT 107 pac 48 pac 11 pac 6 pac 2 pac Can. atrioventr. 2 pac Pulm. stenosa 1 pac Ductus arteriosus 4 pac Coarctatio 3 pac Eissenmanager Sy 2 pac Mortalitet 2. 2% (3 pac) Dilativna cardiom. 2 pac Follow up 1 - 84 meseci Hypertrof. Cardiom. 1 pac.

Hirur{ka korekcija na VSD n =11 pac Hirur{ka korekcija na ASD n =107 pac ASD tip II so trik rekon Desna atrioplastika Plastika na pulmonalnite veni KAB So direktna sutura So perikarden pa~ VSD so trikusp. rek. VSD so pro{ir. na RVOT 48 pac 2 pac 9 pac 87 pac 20 pac 6 pac 2 pac

Hirur{ki pristap- minilateralna torakotomija n= 8 pac. so ASD Prednost: -mal cikatriks -rana mobilizacija -pomala bolna osetlivost

Sy Eissenmanager – case I va operacija: 07. 12. 2001 report 01/2005 dekompenzacija poradi nov Eho naod: sekundarna PAH (PA syst/med 110/65) MReg. +3, TReg +4, VSD, Pulm art. 42 mm Hirurgija: PA banding, Mitr. i tricuspidal rekonstrukcija. PA- 29 mm, PAsyst/med 68/43 II ra operacija: 05. 11. 2004 Eho naod: mitral reg. +4 (prolaps i dilatacija na anulusot), Trikuspidna reg. +3 Predop. PA syst/med 110/60, LAP 30 Hirurgija: MV zamena, Tricusp. rek, VSD zatvorawe so pa~ Postop. PAsyst/med 60/23 totalen AV blok 18. 02/2005 impantiran stalen pace maker 18. 08. 2007 PCT VSD zatvorawe Th: ACE inhibitori, spironolakton, diuretici, Sinkum Follow up 7 godini

Pre`ivuvawe 97. 8% Rano pre`ivuvawe (30 dena) 97. 8%( Mortalitet 2. 2% - 3 pac. ) N= 138 pac. Po~inati pacienti: 1. A. J. 22 god. Dg: Canalis atrioventricularis. Visoka PAH Хирургија- kompletna korekcija При~ина за смрт - тешко одвојување од машината за екстракорпорална циркулација. Висока доза на катехоламини. Мултиорганска слабост. Егзитус 5 -ти послеоперативен ден 2. K. И. 11 год. ( familna anamneza - majka po~inata od dilatativna kardiomiopatija) Дг Примарна дилатативна кардиомиопатија Хирургија- Митрална и трикуспидна реконструкција, zadna LV plastika При~ина за смрт- мултиорганска слабост, мезентеријална исхемија. Егзитус 25 -ти послеоперативен ден 3. M. K. 14 god. Дг Canalis atrioventricularis. PAH. Sy Down Поslеоперативно отпуштена дома. Повторно прием после 2 месеци со клиника на тешко воспалување на белите дробови. При~ина за смрт тешка респиратрна инфекција со срцево попуштање. Касно pre`ivuvawe (84 месеци) 1. K. D. 23 god. 98. 5% (Mortalitet 1. 5%2 pac. ) Dg: HOCM ; Hirurgija: dezobstrukcija na LVOT Egzitus posle 9 meseci- ventrikularna fibrilacija 2. S. S. 25 god. Dg: Primarna dilatativna kardiomiopatija; Hirurgija mitralna i trikuspidna rekonstrukcija so zadna LV plastika Egzitus posle 10 meseci - LV pseudoanevrizma

Interventional procedures in Filip Vtori (2003 -2008) § 84 pt with CHD, 35 male / 52 female, age 3 months - 46 years *TTE was performed in all, TEE in 3, additional diagnostic heart catheterization in 14 pts

Amplatz septal ocluder, n= 36 Common CHD (7%) usually can be treated with interventional technique Surgical treatment is still standard, mortality rate < 1%, and residual shunt in 78% (long term follow up) Result between interventional vs surgical treatment are equal

ASD type sec - results and follow up § Average 3 mm underestimation of the size (TTE v. s angio) § 10 -30 mm ASD devices § In hospital stay 18± 4 hours § TEE during procedure in 3 pts § Without general anesthesia in all § ASA 10 mg/kg. TT/day during the next 6 months § 3 months endocarditis prophylaxis

Pulmonary valve stenosis n=23* § 7 % of all CHD § Тreatment of choice, PG >50 mm. Hg § BVP < RVSP and transpulmonary PG § Rare complications (PR) § Treatment in cyanotic CHD (paliative) § No efficiency in infundibulary PS, without valve stenosis § * 2 pts with TF

BVP of the PS - results and follow up § Average reduction of SPG 34 ± 9 mm. Hg § Seven (7) were treated with beta blockers after procedure § One young women was surgically treated due to severe RVOT hypertrophy § PR < 1 grade in all § Long life endocarditis prophylaxis

PDA - coils and devices; n=17 § Nine (9 ) % of all CHD § Varies types of devices and catheters § Coils, simply and effective (d < 2. 5 mm) § Small residual shunts with spontaneous resolving § Rеsidual shunts (5%-10%)

PDA- results and follow up § 17/18 pts. were completely closed, without residual shunt after one day § Eleven (11) Amplatzer ocluders and 6 coils were implanted § In hospital stay 28 ± 4 hours § One boy referred for surgical treatment due to large PDA § Endocarditis prophylaxis 3 months

VSD- Amplatz perimembraneous ocluder § Five (5) pts with perimembraneous VSD § Age 5 -45 years § Child with Trisomy 21 and VSD § A-V loop § ECG without AV block in all § Amplatz perimembraneous ocluder 8 -14 mm

Aortic coarctation, n=5 § Balloon angioplasty, 4 pts § Stenting of aorta in one § reduction of PG of 30 ± 5 mm. Hg

Closure of paravalvular mitral leak-case report § female, 55 years, mitral disease, PAH, DM II, obesity, COPD, hypothireosis §October, 2005 MVR (mech) §May, 2007 valve thrombosis, pulmonary edema (urgent reoperation) §Postoperative paravalvular leak 3 degree § August 2007, closure of the leak with implantation of muscular VSD occluder, 8 mm

Summary § All pts with ASD, VSD were successfully treated, as well as 17/18 pts with PDA § The average reduction of PG in pts with PS and aortic coarctation was > 40%. § All pts were discharged from hospital next day, with mean in hospital stay of 28 ± 8 hours. § None early complications (embolisation, severe arrhythmia, bleeding, infection) § High grade of security, safety and efficiency § Minimal physical and mental stress for the pts/ superior vs open heart surgery

Summary, trends § New approaches and visions in different CHD § Develop of new devices and techniques § Importance of TTE, TEE (3 D), 64 CT in diagnostics and follow up § Hybrid - minimal invasive method; new perspective § Early neonatal urgent procedures, and early (in utero) transport of the newborns in referent pediatric center