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Heartline IRCCS S. Martino Genova Cardiology meeting 14-15 novembre 2014.

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Presentazione sul tema: "Heartline IRCCS S. Martino Genova Cardiology meeting 14-15 novembre 2014."— Transcript della presentazione:

1 Heartline IRCCS S. Martino Genova Cardiology meeting novembre 2014

2 STENOSI STENOSI INSUFFICIENZA INSUFFICIENZA FORME MISTE FORME MISTE Prevalente stenosi Prevalente insufficienza

3 Secondo le stime più recenti, questa affezione colpisce il 4,6% della popolazione oltre i 75 anni, un quinto dei quali deve essere operato nel giro di 2-3 anni di anni, altrimenti non può sopravvivere. Nonostante la gravità, questa per gli esperti è una malattia sottovalutata e spesso non trattata in maniera appropriata Da una ricerca dell’istituto britannico Opinion Matters su un campione rappresentativo della popolazione italiana over 60 è emerso che solo il 7,7% delle persone è preoccupata per le valvole cardiache. Sono i tumori (24,6%) e il morbo di Alzheimer (20%) le malattie che spaventano di più. The Huffington Post

4  Congenite  Valvola uni/bicuspide  Acquisite  Displasia senile fibro-calcifica  Malattia reumatica

5  Congenite  Valvola uni/bicuspide  patologia di per sé?  Acquisite  Displasia senile fibro-calcifica malattia aterosclerotica?  Malattia reumatica  simile patogenesi?  Altre rare patologie  Patologie acquisite su base congenita? Fibrosi e calcificazione su valvole tricuspidi/bicuspidi asimmetriche?

6  Abbiamo in mente un aspetto “ideale” di una valvola cardiaca  Più ci si allontana da questo ideale più aumenta il rischio di patologia acquisita  Varianti anatomiche  Senilità sono perfetti substrati per vere patologie

7 Anomalo numero delle cuspidi Unicuspide Bicuspide Quadricuspide Atresia Anomala proporzione fra le cuspidi Anomala struttura Cuspidi displastiche Quadricuspid valve AO valve atresia

8  Accentuazione delle lunule  Fenestrazioni Vegetazioni maranticheescrescenze di Lambl formazioni trombotiche con evoluzione fibrotica

9 Ho S Y Eur J Echocardiogr 2009;10:i3-i10 (A)Bicuspid and dysplastic valve with both coronary arteries arising from the anterior facing sinus. (B) Bicuspid valve with the leaflets and sinuses arranged in left–right fashion. A raphe is present in the right leaflet (C) A valve with three thickened leaflets. One of the leaflets is considerably larger than the other two. (D) This valve with three leaflets has been cut longitudinally to show the thick and gelatinous looking leaflets. (E) There are abundant calcific nodules in this severely stenotic valve from an elderly patient. (F)This unicommisural and unifoliate valve has a tiny eccentric orifice.

10 Lo stato della valvola riguarda il cardiochirurgo?  Opzione chirurgica Solo la valvola Valvola e radice aortica Solo radice aortica  Morfologia della valvola Numbero delle cuspidi Fibrosi/calcificazione  Patologie associate Coronarie Bulbo/Aorta ascendente

11 Sinutubular junction Immediately above the three aortic sinuses Ascending aorta From the bulbus up to the first bifurcation of the aortic arch Aortic bulbus The enlarged part where the cusps are situated The enlarged part where the cusps are situated Diameter 1.5 superior in respect to proximal ascending aorta Diameter 1.5 superior in respect to proximal ascending aorta

12  1 –2 % della popolazione  maschio/femmina 3:1  Familiarità  Cuspidi spesso asimmetriche  Ostio di aspetto semilunare  Cuspidi: “antero-posteriore”60-70% 2 coronarie nel seno anteriore “lato a lato”30-40% 1 coronaria per seno Presenza variabile di un rafe o falsa commissura 36-59%  Distribuzione coronarica a dominanza sinistra 4 volte piu comune  origine sovracommissurale dell’ostio cor. sin. 2 volte più comune

13  Aortic valve disease is one of the most common congenital cardiac defects, occurring in 5% of all children with heart disease.  Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, affecting 1-2% of the population, with strong male predominance.  Individuals may have a normally functioning BAV, and may be unaware of its presence and the potential risk of impending complications.  They may typically remain asymptomatic until the third or fourth decade of life, when the valve becomes dysfunctional.  BAV is associated with both valve disease and aortic disease, thereby leading to increased morbidity and mortality, including  other cardiovascular malformations coronary anomalies, coartaction etc.  aortic valve disorders  aortic wall abnormalities  endocarditis Bicuspid aortic valve phenotype and aortic disease: a magnetic resonance study David W Fitz *, James C Carr and Edwin Wu J Cardiovasc MR 2011

14  Four valve morphologies on 217 cases:  type 1, fusion of the right and left cusps (n=152);  type 2, fusion of the right and non-coronary cusps (n=48);  type 3, fusion of the left and non-coronary cusps (n=9);  and unicuspid, two fusions (n=8). further characterized by the number of sinuses, two or three, and the presence or absence of a raphe. Conclusion  Type 1 BAV is the most frequent phenotype.  Two sinus valves are more common among type 2 and type 3 phenotypes.  Type 2 is associated with  moderate to severe aortic stenosis  a larger mid-ascending aortic diameter. Bicuspid aortic valve phenotype and aortic disease: a magnetic resonance study David W Fitz *, James C Carr and Edwin Wu J Cardiovasc MR 2011 Type 1 Type 2

15  Anatomo-Clinical demonstration of progressive aortic dilatation  Abnormalities in components of the extracellular matrix or aberrant vascular matrix remodeling might contribute to abnormal valvulogenesis and a structurally weakened aortic root.  Other studies supports the concept of an independent aortic remodeling process  throughout childhood among patients with a bicuspid aortic valve  may precede frank aneurysm formation.

16  Fibrosis  Calcification  Thrombosis  Infections  Hemolisis  DISSECTION  Sudden death?  Fibrosis and Calcification

17 Differences in clinical presentation between patients with tricuspid aortic valves (TAVs) or bicuspid aortic valves (BAVs) and aortic valve disease are evident.  METHODS:  702 patients with aortic valve and/or ascending aortic pathology; 202 also had concomitant coronary artery disease. RESULTS:  A BAV was commonly found in patients with isolated valve disease (BAV 47%, TAV 53%) and frequently associated with ascending aortic dilatation (BAV 80%, TAV 20%).  In patients with coronary artery disease, a TAV was commonly found (TAV 84%, BAV 16%).  The combination of ascending aortic dilatation and coronary artery disease was markedly rare regardless of valve morphology (TAV, 7 out of 38; BAV, 6 out of 127).  The distribution of valve pathology and clinical parameters was similar in patients with TAV and BAV with coronary artery disease (P ≥.12).  Without coronary artery disease, parameters associated with cardiovascular risks were more often seen in patients with TAV than in patients with BAV (P ≤.0001). J Thorac Cardiovasc Surg.J Thorac Cardiovasc Surg Aug 17. pii: S (14) doi: Jackson V Jackson V 1, Eriksson MJ 2, Caidahl K 2, Eriksson P 3, Franco-Cereceda A 4Eriksson MJCaidahl KEriksson PFranco-Cereceda A

18 CONCLUSIONS:  Coronary artery disease is uncommon in surgical patients with BAV, but it is associated with TAV, advanced age, and male gender.  Coronary artery disease and ascending aortic dilatation rarely coexist, regardless of valve phenotype.  Differences in the prevalence of coronary artery disease or ascending aortic dilatation between patients with TAV and BAV are not explained by differences in cardiovascular risks or the distribution of valve pathology. J Thorac Cardiovasc Surg.J Thorac Cardiovasc Surg Aug 17. pii: S (14) doi: Jackson V Jackson V 1, Eriksson MJ 2, Caidahl K 2, Eriksson P 3, Franco-Cereceda A 4.Eriksson MJCaidahl KEriksson PFranco-Cereceda A

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21  The process of valve disease is believed to be initiated with the development of atherosclerosis along the aortic surface of the valve which, subsequent to aortic valvular osteoblast differentiation, calcifies.  Early in the disease process, the thickened aortic valve is said to be sclerotic; that is, although thickening of the valves is present, there is not yet obstruction of the valve orifice.  Later in the disease process, as the thickening of the valve progresses and the valve orifice becomes significantly obstructed, the valve is said to be frankly calcific. Comparsa di elementi infiammatori -Ruolo patogenetico? -Secondaria alla fibrosi?

22  In rheumatic fever, antistreptococcal antibodies produced by B lymphocytes cross- react with host-tissue epitopes, producing inflammation in a number of organ systems, including the heart and its mitral and aortic valves. Any or all 4 valves may be involved.rheumatic fever  Inflammation induces angiogenesis in the normally avascular valve layers; over a period of months or years, thickening of the valve develops as inflamed elastic tissue becomes replaced by irregular masses of collagen fibers.  Recurrent episodes of rheumatic fever produce progressive damage to the host’s valves.

23  renal failure,  familial hypercholesterolemia,  Paget disease,  systemic lupus erythematosus,  ochronosis with alkaptonuria,  Radiation  left ventricular noncompaction systemic lupus erythematosus

24 IgG4-related disease of the aortic valve: a report of two cases and review of the literature. Cardiovasc Pathol.Cardiovasc Pathol Sep 28. pii: S (14) doi: /j.carpath Maleszewski JJ Maleszewski JJ 1, Tazelaar HD 2, Horcher HM 3, Hinkamp TJ 4, Conte JV 5, Porterfield JK 6, Halushka MK 7.Tazelaar HDHorcher HMHinkamp TJConte JVPorterfield JKHalushka MK A case of aortic and mitral valve involvement in granulomatosis with polyangiitis. Cardiovasc Pathol Aug 4. Espitia O1, Droy L2, Pattier S3, Naudin F4, Mugniot A5, Cavailles A4, Hamidou M6, Bruneval P7, Agard C6, Toquet C2. Aortic valve replacement in systemic sclerosis. J Cardiovasc Med (Hagerstown) Mar 12. Ferrari G1, Pratali S, Pucci A, Bortolotti U.

25  Patogenesi simile?  Forme infiammatorie  Fusione delle cuspidi  Calcificazione  Inizia dal margine aortico  Forme diverse all’inizio  Alla fine si somigliano tutte

26  Bicuspidia  Associazione con lesioni aortiche  Post-infiammatorie  Possibili recidive, quadri sistemici  Forme senili  Progressivo aumento


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