Egészségügy | Kardiológia » Dr. Szatmári András - Veleszületett szívhibák a felnőttkorban. Szívkatéter intervencionális és műtéti lehetőségek

Veleszületett szívhibák a felnőttkorban. Szívkatéter intervencionális és műtéti lehetőségek Dr.Szatmári András GOKI, Budapest Development of "Congenital Cardiology" in the 20th century Neill A, Clark EB: 1995 Milestones in the history of Congenital Heart Surgery Gross Crafoord Taussig Blalock Senning Lillehei Jatene Mustard Fontan Yacoub Extraordinary Advances in Medicine - Improved Survival Rate - Year of birth Simple CDH Moderate CDH Complex CDH 1940 1959 90 55 10 1960 1979 95 65 50 1980 1989 95 90 80 Warnes CA: 32th Bethesda Conference, JACC 2001; 37:1170-5 (Courtesy: E. Oechslin, Zürich) The Changing Profile of Congenital Cardiac Disease Adults with CCD Children with CCD „1980“ „2000“ „2020“ Courtesy: G. Webb, 2001 GUCH betegek - GOKI 900 800 700 600 500 400 300 200 100 0 2004 2005 2006 guch beteg 2007 2008 Betegségek - GUCH • ASD ost.sectíp • Coarctatio,DBP • Komplex

intervenció • Fallot tetralogia ASD II. Katéteres zárás Nincs műtét Rövid ápolás Ritka szövődmény Hosszú távú követés nincs Költség: Szelektált betegcsoport Sikertelen beavatkozás Műtét Nagy beavatkozás Hosszabb ápolás Postop. pericarditis, ritmuszavarok Költség: „mindenki alkalmas” Mindig sikeres (?) ASD II - felnőttkor • Indikáció – Szívelégtelenségre utaló tünetek – Emelkedett kisvérköri nyomás – Jelentős shunt – Cryptogen stroke • Vizsgálat – TTE – TE ? • Követés – 1, 3, 6, 12 hónap, majd évente – Aszpirin 100 mg 3 hónapig Problémák • • • • • Korhatár Pulmonalis hypertonia Késői nagyvérköri embolia Ritmuszavar - pitvarfibrilláció (technikai problémák) Az invazív kardiológus szempontjai Anatómia • Véna femoralis átjárhatóság • ASD nagysága • Bal pitvar nagysága • IAS orientációja • Valv.Eustachi, Chiari network Az invazív kardiológus

szempontjai Anatómia TE vs. stretch átmérő 40 35 átmérő mm 30 25 20 15 10 5 0 0 20 TE 40 Stretch 60 Lineáris (TE) 80 100 120 Lineáris (Stretch) TTE átmérő vs. Amplatzer méret 30 átmérő mm 25 20 15 10 5 0 0 10 TTE 20 ASO 30 40 Lineáris (ASO) 50 60 Lineáris (TTE) 70 AmplatzerR Septal Occluder 6 mm Bal disc Connecting waist jobb disc Az invazív kardiológus szempontjai Technika ASO connecting waist átmérő + 6mm + 6mm = bal disc (pl. 15 mm ASO + 12 mm = 27 mm) vs. (< =) Bal pitvar átmérő, IAS hossz (pl. BP átmérő < 27 mm) ASD – peremek - gondok aorta Vv.pulmonales ASD VCS limbus Venae cavae IAS=valv.fossov VCI Katéter iránya mitralis Sinus coronarius aorta VCI Interatrialis communicatiok zárása 2009.0930 összes 724 pfo 119 sikeres 119 ASD 566 Stretch 16 positio 5 Fen. TCPC 39 545 acut opus 1 Sikeres 39 sikeres 544 ASD II zárás 2009.0930 összes 566 felnött 265 Stretch 7

zárás 258 gyerek 301 zárás 286 positio 5 stretch 9 acut opus 1 Coarctatio aortae – CP stent + BiB ballon CP stent (Cheatham-Platinum) BiB ballon nativ CoAo CP stent felhelyezésa BiB ballonra belső ballon inflatio külső ballon inflatio Natív coarctatio aortae primer stent terápiája Botallo vezeték Angiográfiás klasszifikáció (Toronto) • • • • • A type B type C type D type E type conic short, window tubular complex elongated Nyitott Botallo vezeték - záróeszközök PDA mérések A: ao.ampátmérő aorta B: ao.amphossz C: ao.amp közép D: legszűkebb átm. E: pulm.ampátmérő C B D E a.pulmonalis A Adult type PDA Ao ampulla MAP LPA Ao Adult type PDA Ao MAP LPA Adult type PDA Ao MAP LPA Botallo vezeték a felnőttkorban Follow up of adult patients after surgical repair of tetralogy of Fallot. Early repairs- Late problems - Scars from ventriculotomy arrhythmia focuses - Transannular

patch- large RVOT free PR; RV volume overload - Partial relief of RVOT obstructionlate residual RVOT obstruction, RV pressure overload Transannular patch Survival late after TOF repair w/wo transannular patch risk for SCD and atrial arrhythmia and ventricular arrhythmia Gatzoulis Lancet 2000 Type of repair: RV-PA conduit Commonly used surgical approach in extreme form of TOF with pulmonary atresia or absent pulmonary valve or, RV-PA conduit/graft anomalous coronary artery crosses the RVOT. RV- PA conduits • RV-PA homografts / bioprosthetic conduits • The life span limited by the development of progressive lumen obstruction. • Freedom from replacement 1. bioprosthetic conduits 1-5 : 68% to 95% at 5 years 0% to 59% at 10 years 2. cryopreserved homografts 4, 6-9: 55% to 94% at 5 years • External compression, calcification, kinking, fibrotic intimal peel formation, and homograft "contraction" are all thought to contribute to conduit deterioration. • Young

patients, because of their size, often require smallerdiameter conduits, which they subsequently outgrow. 1. Jonas at al, Circulation 1985; 2 McGoon at al, Am J Cardiol 1982; 3 Razzouk at al, Circulation 1992; 4 Kirklin at al, Ann Thorac Surg. 1987; 5 Bull at al, J Thorac Cardiovasc Surg 1987; 6 Cleveland at al, Circulation 1992; 7 Sharma at al, Am J Surg. 1993; 8 Hawkins at al, J Thorac Cardiovasc Surg 1992; 9 Bando at al, J Thorac Cardiovasc Surg 1995 The spectrum of late residual problems after TOF repair • Hemodynamic issues • Electrical issues • Associated anomalies The spectrum of late residual problems after TOF repair Hemodynamic issues 1. Pulmonary regurgitation 2. RV outflow tract obstruction associated branch pulmonary artery sten. 3. RV : dilatation, dysfunction, restrictive physiology • • Electrical issues Associated anomalies Residual pulmonary regurgitation (PR) • Consequences: – RV volume overload and dilatation – dysfunction (eventually,

low CO syndrome) – progressive slowing electrical activation (↑ QRS) • Diagnosis of significant PR: – ECHO: PR jet origin in the PA branches; – MRI: PR volume > 75cc/m2, – PR fraction > 20% Residual RV outflow tract obstruction (RVOTO) • Residual RVOTO less frequent, than PR • Residual peripheral stenosis of RPA and LPA are quite frequent. They may be either congenital or due to the previous palliative Blalock – Taussing shunt. • Higher survival rate after TOF repair in those identified with a high RV/LV pressure ratio (>0.7) The spectrum of late residual problems after TOF repair in adults • • 1. 2. 3. • Hemodynamic issues Electrical issues Arrhythmia Conduction abnormalities Sudden death Associated anomalies Arrhythmias and conduction abnormalities after TOF repair • • • • RBBB + LAH in 7-25%; complete AV block in 1-2%; Supraventric. tachyarrhythmia in 10-30%; ventricular arrhythmia in 46-67%; ventricular tachycardia in

10-15%; sustained monomorphic VT in 4-10% Estimates of Sudden Death after TOF surgery Warnes at al. JACC 2008 Anatomical substrate Zeppenfield, Circulation 2007 Risk factors for VT / SCD • • • • • • • • • Repair via ventriculotomy 1 Older age at repair 2-4 Male sex5 Higher NYHA class 2,6,7 QRS > 180ms or rapid increase in QRS-duration6,8 Right ventricular outflow tract aneurysms2 Significant pulmonary regurgitation (PR)2,6,7 Residual RVOT gradient 9 LV systolic dysfunction10 1 Dietl, Circulation 1994; 2 Harrison, JACC 1997; 3 Presbitero, Ann Thorac Surg 1988; 4 Dittrich, Clin Cardiol 1999; 5 Nollert, Ann Thorac Surg 2003; 6 Gatzoulis, Lancet 2000; 7 Therrien, Circ 2001; 8 Gatzoulis Circulation 1995; 9 Chen, Am Heart J 1987; 10Ghai, JACC 2002 The spectrum of late residual problems after TOF repair in adults • • • 1. 2. Hemodynamic issues Electrical issues Associated anomalies LV dysfunction Aortic regurgitation & aortic dilatation

LV dysfunction after TOF repair Mechanism: - Injury to the left coronary artery with an atypical course - Inadequate myocardial protection during previous repair - Chronic LV volume overload due to longstanding palliative arterial shunts and/or restrictive residual VSD - Abnormal LV/RV interaction due to the dilated RV Management strategies after TOF repair Recommendations for Evaluation and Follow-Up of the Repaired Patient • ECG annually (cardiac rhythm and QRS duration.) • Echo annually (RV, LV size, function, PR, TR, RVOT grad) • MRI is now seen as the reference standard for assessment of RV volume and systolic function (every 2 to 3 years) • CT in patients who cannot have an MRI (radiation exposure!) • CP test (exercise capacity and exercise induced arrhythmia) • Other (Holter) Warnes at al, JACC 2008 Recommendations for Evaluation and Follow-Up of the Repaired Patient - Screening for heritable causes of their condition (eg, 22q11 deletion) should be

offered to all patients with TOF - Before pregnancy or if a genetic syndrome is identified, consultation with a geneticist should be arranged for patients with TOF TOF: surgical management • Need for re-intervention after TOF repair - Pulmonary valve repair ± tricuspid valve repair due to severe PR and RV dilatation - Residual VSD - Residual systemic to PA shunt - Residual RVOT obstruction - Branch pulmonary artery stenosis - Aortic valve repair/ replacement due to progressive regurgitation; aortic dilatation management - Arrhythmia management Branch Pulmonary Artery Angioplasty • There are no clear recommendations • May be considered when RV/LV pressure >0.5 or at lower pressure when there is RV dysfunction. • May be considered when there is unbalanced pulmonary blood flow greater than 75%, 25%. • May be considered in otherwise unexplained dyspnea with severe vascular stenosis. Warnes C at al. JACC, 2008 Percutaneous pulmonary valve implantation in humans •

• • • • • 59 patients: 36 TOF, 20 severe PS 58 succesful replacement RV measurements decreased significantly RV and LV function improved by MRI PR reduced significantly Exercise tolerance improved significantly . Khambadkone et al. Circulation 2005 Indicatiok / a beavatkozás célja • Indicatiok – RV - PA conduit dysfunctio (insuff., stenosis, vagy mindkettő) • A beavatkozás célja – conduit stenosis megszüntetése regurgitatio nélkül – Visszaállítani a megfelelő pulmonalis billentyű működést – Meghosszabbítani a conduit élettartamot, késleltetni a sebészi beavatkozást PPVI PPVI A beavatkozás Arrhythmia management Arrhythmia risk reduction after TOF repair . ICD implant Patients with ICD implant for primary and secondary prevention experience high rates of appropriate and effective shocks; however, inappropriate shocks and late lead-related complications are common. - secondary prevention: 70% are monomorphic VT’s

(often well tolerated); no control group studies. - primary prevention, patient selection is still unclear Khairy at al. Circulation 2008 Pregnancy and TOF After repair of TOF the prognosis for a successful pregnancy is good provided there are no important hemodynamic residua and functional capacity is good. - A comprehensive, informed cardiovascular evaluation is recommended before each pregnancy. - Pregnancy is usually well tolerated even in the setting of severe PR, as long as RV function is not more, than mildly depressed and SR is maintained - Patients with TOF have an increased risk of fetal loss. - Non- 22q11 deletion mother: the risk of a fetus having congenital heart disease is approx. 4% to 6% Summary Key postoperative issues are summarized below: Residual pulmonary regurgitation RV dilation and dysfunction from PR, possibly with associated TR Residual RVOTO Branch PA stenosis or hypoplasia Sustained VT Sudden cardiac death AV block, atrial flutter, and/or

atrial fibrillation Progressive AR Syndromal associations