P24: MANAGEMENT OF RHEUMATIC VALVE DISEASE OF THE AORTIC VALVE USING THE OZAKI PROCEDURE WITH AUTOLOGOUS PERICARDIUM

Abstract Case-Study A 15-year-old boy was referred to our tertiary centre from his local paediatric services with a background of rheumatic fever, severe aortic regurgitation (AR) and mild to moderate mitral regurgitation. He had a history of angina and dyspnoea on exertion, a 2/6 ejection systolic murmur and 2/4 end diastolic murmur. Transthoracic echocardiography showed severe aortic valve insufficiency (with flow reversal seen in the descending aorta and an LV end diastolic volume of 173 ml/m2) and trivial pulmonary valve regurgitation. Autograft failure following the favoured Ross procedure deemed the patient as a candidate for an Ozaki procedure. Autologous pericardium was used to replace the diseased aortic valve. Intraoperative transoesophageal echocardiography showed a deficient left coronary cusp leaflet and a retracted right coronary cusp leaflet. The patient was under cardiopulmonary bypass for 124 minutes and on cross-clamping for 99 minutes with no intraoperative complications. Histological examination of the aortic valve leaflets showed neovascularisation, myxoid changes and disarray of the fibrous stroma. Postoperative recovery was uneventful. The postoperative echocardiogram showed trivial AR, end diastolic volume 217ml, end systolic volume 12 ml and 40% ejection fraction. There was full resolution of the dyspnoea, angina and diastolic murmur on follow-up 4-months postoperatively as supported by healthy valve function on echocardiography. This case highlights that in those of risk of multiple valve pathology, such as in rheumatic valve disease, an Ozaki procedure using autologous pericardium is a viable surgical option for paediatric aortic valve repair with good outcomes. Take-home message In cases of systemic conditions affecting the heart valves where there is multiple valve pathology and risk of autograft failure, such as rheumatic valve disease, the use of autologous pericardium to replace these valves has shown to be a viable option in this paediatric case.

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Management of rheumatic aortic valve disease using the Ozaki procedure with autologous pericardium: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytab170 ◽

2021 ◽

Vol 5 (6) ◽

Author(s):

Ashar Asif ◽

Umberto Benedetto ◽

Victor Ofoe ◽

Massimo Caputo

Keyword(s):

Aortic Valve ◽

Cost Effective ◽

Cardiovascular Death ◽

Left Ventricular ◽

Valve Disease ◽

Mitral Valve Leaflet ◽

Middle Income ◽

Autologous Pericardium ◽

Rheumatic Valve Disease ◽

Operative Recovery

Abstract Background Rheumatic valve disease (RVD) is the most common cause of cardiovascular death in low-middle income nations. Surgical aortic valve (AV) interventions for RVD, especially in children, have proven problematic with graft failure, relapse, and poor compliance with anticoagulation. A novel technique involving neocuspidization of the aortic annulus using autologous pericardium to construct new AV leaflets (the Ozaki procedure) has shown promising outcomes in children with congenital AV disease; however, there are no previous recorded cases using this technique in children with RVD. Case summary We present the case of a 15-year-old male presenting with exertional angina and dyspnoea with a background of previous rheumatic fever. Echocardiography had shown a regurgitant tricuspid AV, left ventricular dilatation with mitral valve leaflet tethering. The patient underwent the Ozaki procedure for his AV regurgitation and was discharged following an uneventful post-operative recovery. The patient had full resolution of symptoms following the procedure and remains well 3 years following his operation. Discussion This case highlights that good outcomes with the Ozaki procedure in RVD are possible 3-years post-operatively and should prompt future studies to evaluate the procedure as a surgical option for paediatric patients in this clinical context. Additionally, the Ozaki procedure may also provide a cost-effective surgical technique requiring minimal additional operative resources and reduced follow-up demand, which would be critical in low-resource clinical settings where RVD is prevalent.

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Valvular heart disease

Oxford Textbook of Medicine ◽

10.1093/med/9780198746690.003.0352 ◽

2020 ◽

pp. 3436-3458

Author(s):

Michael Henein

Keyword(s):

Lupus Erythematosus ◽

Mitral Stenosis ◽

Valve Disease ◽

Pulmonary Vasculature ◽

First Heart Sound ◽

Rheumatic Valve Disease ◽

Congenital Mitral Stenosis ◽

Valve Pathology ◽

Apex Beat ◽

Diastolic Murmur

Rheumatic valve disease remains prevalent in developing countries, but over the last 50 years there has been a decline in the incidence of rheumatic valve disease and an increase in the prevalence of degenerative valve pathology in northern Europe and North America. In all forms of valve disease, the most appropriate initial diagnostic investigation is almost always the echocardiogram. The most common cause is rheumatic valve disease. Other causes include mitral annular calcification, congenital mitral stenosis, infective endocarditis (very rarely), and systemic lupus erythematosus (Liebman–Sachs endocarditis). The important consequences of mitral stenosis are its effect on left atrial pressure, size, and the pulmonary vasculature; it commonly causes atrial fibrillation. Presenting symptoms are typically exertional fatigue and breathlessness; systemic embolism can occur. Characteristic physical signs are irregular pulse, tapping apex beat, loud first heart sound, opening snap, and an apical low-pitched rumbling mid-diastolic murmur.

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Heart valve disease

Oxford Textbook of Medicine ◽

10.1093/med/9780199204854.003.1606_update_002 ◽

2010 ◽

pp. 2736-2757

Author(s):

Michael Henein

Keyword(s):

Developing Countries ◽

North America ◽

Heart Valve ◽

Valve Disease ◽

Heart Valve Disease ◽

Northern Europe ◽

Diagnostic Investigation ◽

Rheumatic Valve Disease ◽

Valve Pathology

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P249 Iatrogenic aortic cusp laceration causing severe acute traumatic aortic regurgitation secondary to complex percutaneous coronary intervention using rotablator;3DTEE-anatomic correlates

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.111 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

V Vadgaonkar ◽

H Tarief ◽

S Shivappa ◽

L Sulaibikh ◽

S Saif ◽

...

Keyword(s):

Heart Failure ◽

Aortic Valve ◽

Aortic Regurgitation ◽

High Speed ◽

Past History ◽

Case Reports ◽

Free Margin ◽

Severe Aortic Regurgitation ◽

Percutaneous Coronary ◽

Coronary Cusp

Abstract Clinical case 59 years old male known case of long standing diabetes and dyslipidemia presented to our institute with Non-STEMI.His past history was significant for multiple admissions with acute coronary syndromes and percutaneous coronary interventions(PCI). Echocardiogram in this presentation showed preserved biventricular systolic function with no valvulopathies. He was taken up for coronary angiogram which showed significant calcified angulated lesion in LAD/LCX(Left anterior descending and circumflex) with diffuse disease in Ramus/RCA. Mode of revascularisation was discussed in detail with the patient for Rotablation-guided PCI to LAD/LCX vs CABG.He preferred and underwent complex PCI to LAD. During the procedure he developed acute heart failure which was managed conservatively. Immediate TTE showed new severe aortic regurgitation(AR) with probable left coronary cusp(LCC) perforation and preserved LVEF.After stabilization, he was scheduled for TEE which showed tear in the LCC with complex fragmented jet of holodiastolic AR filling the entire LVOT.There was new late diastolic to early systolic MR.3DTEE Enface view of the aortic valve was evident of ovoid shaped laceration in LCC extending till the free margin (Fig 1C). There were additional tears in Non-coronary cusp(NCC) extending till the free margin of right coronary cusp(RCC).3DTEE colour was notable for regurgitant jet origin from LCC/NCC (Fig 2B). Based on above findings,He was offered aortic valve intervention but was reluctant initially.He presented 2 months later with exertional dyspnoea, mild LV dilatation and drop in LVEF to 50%.He consented for surgery and underwent bioprosthetic Aortic valve replacement because of extensive cusp tear and deformation of the valve along with complete revascularisation of the remaining diseased coronaries.Post-operative course was complicated by vasoplegic arrest and acute renal failure which was successfully managed conservatively. Discussion We report this case for the rarity of this post-PCI iatrogenic complication and the incremental role of 3DTEE in identifying the exact pathology.It was proposed that during PCI multiple stalling of rotatbur in calcified LAD caused traction and eventually disengagement of guide causing rotabur to freely hang in ascending aorta close to the cusps.Possibly this rotablator with very high speed(180000rpm) would have momentarily come in contact with the aortic cusps causing cuspal perforation and heart failure in Cath lab.3DTEE correlated very well with the anatomo-pathology and matched with intraoperative finding as shown in figures 2A & 2C.These findings prior to aortic cross clamp could significantly reduce time of surgery as he had an additional CABG procedure to be performed.There are few isolated case reports of post PCI aortic valve perforation but probably ours is the first one secondary to the use of rotablator with near involvement of all the cusps and reasonably accurate 3DTEE-anatomic characterisation. Abstract P249 Figure.

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Heart valve disease (aortic valve disease): anatomy and pathology of the aortic valve

10.1093/med/9780198726012.003.0031 ◽

2016 ◽

Author(s):

Cristina Basso ◽

Gaetano Thiene ◽

Siew Yen Ho

Keyword(s):

Aortic Valve ◽

Aortic Wall ◽

Ascending Aorta ◽

Valve Disease ◽

Heart Valve Disease ◽

Distinctive Features ◽

Aorta Aneurysm ◽

Rheumatic Valve Disease ◽

Relationship Of ◽

The Relationship

The gross features of the aortic valve apparatus, consisting of three semilunar leaflets, three interleaflet triangles, three commissures, and the aortic wall, are discussed both in terms of normal and pathological anatomy. The concept of aortic annulus and the relationship of the aortic valve with the coronary arteries, the membranous septum, and conduction system and the mitral valve are addressed. When dealing with pathology, the chapter focuses on the main distinctive features of aortic valve stenosis and aortic valve incompetence. Regarding the former, the abnormalities reside in the cusps, either two or three in number, with cusp thickening, and calcification with or without commissural fusion (thus distinguishing senile and chronic rheumatic valve disease); in the latter, the gross changes can affect either the cusps (infective endocarditis with tissue perforation/laceration and rheumatic valve disease with tissue retraction) or the aortic wall (ascending aorta aneurysm either inflammatory or degenerative). The distinctive gross abnormalities in the various conditions are illustrated.

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Midterm results of aortic valve repair with the pericardial cusp extension technique in rheumatic valve disease

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2003.09.087 ◽

2004 ◽

Vol 77 (4) ◽

pp. 1272-1276 ◽

Cited By ~ 34

Author(s):

Nilgün Bozbuga ◽

Vedat Erentug ◽

Kaan Kirali ◽

Esat Akinci ◽

Ömer Isik ◽

...

Keyword(s):

Aortic Valve ◽

Valve Disease ◽

Aortic Valve Repair ◽

Valve Repair ◽

Midterm Results ◽

Rheumatic Valve Disease

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von Willebrand factor abnormalities in aortic valve stenosis: Pathophysiology and impact on bleeding

Thrombosis and Haemostasis ◽

10.1160/th10-10-0634 ◽

2011 ◽

Vol 106 (07) ◽

pp. 58-66 ◽

Cited By ~ 36

Author(s):

Sandro Sponga ◽

Elena Pontara ◽

Maria Grazia Cattini ◽

Cristina Basso ◽

Gaetano Thiene ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Valve Stenosis ◽

Von Willebrand Factor ◽

Severe Aortic Stenosis ◽

Valve Disease ◽

Minor Bleeding ◽

Acquired Von Willebrand Syndrome ◽

Rheumatic Valve Disease ◽

Von Willebrand ◽

Willebrand Factor

SummaryAcquired von Willebrand syndrome (AVWS) may complicate severe aortic valve stenosis, due to a reduction in the haemostatically more efficient large von Willebrand factor (VWF) multimers. This study was designed to analyse the relevance of VWF abnormalities and haemorrhagic diathesis in severe aortic valve stenosis. Forty-one consecutive patients undergoing valve replacement were investigated: seven had minor bleeding symptoms in their recent history; 10 (24.3%) had a reduced VWF collagen binding (VWF:CB) to VWF antigen ratio, and 33 (80.5%) had a decrease in large VWF multimers. The shortage of large multimers was not associated with any accumulation of small VWF multimers (apparently ruling out any increased VWF proteolysis), nor was there any increase in VWF propeptide, which excludes a shorter VWF survival. The risk of developing VWF abnormalities was higher in patients with rheumatic valve disease than in degenerative cases (p=0.025) and in valves with <50% of residual endothelial cells (p=0.004). Bleeders differed from non-bleeders in that they had a higher mean transvalvular gradient and a more marked decrease in large VWF multimers. VWF abnormalities did not exacerbate peri-operative blood loss, however – a finding consistent with the full correction of these VWF abnormalities, seen already on the first postoperative day and persisting for up to six months after surgery. According to the data obtained in our cohort of patients VWF abnormalities are common in severe aortic stenosis, particularly in cases of rheumatic valve disease, but loss of the largest multimers does not seem to cause clinical bleeding in most patients.

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P6501Recurrent genetic aberrations in bicuspid aortic valve disease patients with isolated severe aortic regurgitation

European Heart Journal ◽

10.1093/eurheartj/ehz746.1091 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

Y Wang ◽

B Wu ◽

J Li ◽

C Wang ◽

X Shu ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Bicuspid Aortic Valve ◽

Genetic Variants ◽

Early Onset ◽

Aortic Valve Disease ◽

Valve Disease ◽

Genetic Defects ◽

Severe Aortic Regurgitation ◽

Rare Genetic Variants

Abstract Background The etiology of bicuspid aortic valve disease (BAVD) is still unclear. Recent studies have demonstrated elevated prevalence of genetic defects in BAV patients with root phenotype, which includes aortic regurgitation and root-predominant dilatation. Purpose The present study intended to illustrate the feature of genetic defects among early-onset BAV patients with isolated severe aortic regurgitation. Methods From June 2015 to December 2017, whole exome sequencing was performed upon 27 BAVD patients with isolated severe aortic regurgitation under 45 years in our institution. Patients were categorized into right-left (R-L, n=16) and non-RL (n=11) cusp fusion types, and those with complex cardiac defects were excluded from the present study. Results Among 27 patients with a median age of 30.5 (18–44) years, only one was female with a rare left-non-coronary cusp fusion type. The prevalence of root phenotype was markedly higher in RL patients (56.3% vs 9.1%, p=0.018). In RL patients, the numbers of rare genetic variants (RGVs) were 15 in extracellular matrix genes, 8 in TGF-β signaling pathway genes, 2 in smooth muscle cell contraction apparatus genes, and 3 in familiar BAV related genes. In non-RL patients, the number of RGVs were 15, 3, 4, and 5, respectively. On the other hand, the number of RGVs in above gene clusters were 9, 6, 3, 2 in patients with a root phenotype, and 21, 5, 3, 6 in those without. Eight recurrent genetic variants were identified in 6 genes (see Table). An interesting observation was that ADAMTS2 variants were exclusively found among non-RL patients without root phenotype, as FBN2 variants among RL patients with root phenoype. Recurrent Rare Genetic Variants Gene Reference sequence Variant 1000G 1000G-East Asia Patients TGFBR2 NM_001024847.2 p.Val216Ile/c.646G>A 0.004 0.018 A16, A23 TGFBR2 NM_001024847.2 p.Thr340Met/c.1019C>T 0.003 0.015 A03, A05, A07 ADAMTS2 NM_014244.4 p.Gly1169Val/c.3506G>T 0.0044 0.021 A03, A15 FBN2 NM_001999.3 p.Gly475Val/c.1424G>T 0.0004 0.002 A19, A24 ELN NM_001278939.1 p.Pro93Leu/c.278C>T 0.0014 0.0069 A22, A26 COL4A5 NM_033380.2 p.Gly953Val/c.2858G>T 0.0079 0.03 A11, A17 MYLK NM_053025.3 p.Ser243Trp/c.728C>G 0.0002 0.001 A01, A02 MYLK NM_053025.3 p.Asp717Tyr/c.2149G>T 0.0024 0.011 A04, A21 Conclusion Recurrent genetic variants could be identified in a cohort of early-onset BAVD patients with isolated severe aortic regurgitation and staggering male predominance. The incidence and clinical relevance of these variants should be validated in an extended real-world BAV cohort.

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P780 Left ventricular torsion in severe valvular disease

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.439 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

A Azul Freitas ◽

J Ferreira ◽

C Ferreira ◽

J Milner ◽

P Alves ◽

...

Keyword(s):

Aortic Valve ◽

Left Ventricle ◽

Ejection Fraction ◽

Aortic Valve Disease ◽

Left Ventricular ◽

Valvular Disease ◽

Valve Disease ◽

Disease Group ◽

Severe Aortic Regurgitation ◽

Significant Difference

Abstract Introduction Left Ventricular (LV) torsion is an important component of LV performance. With the development of speckle tracking echocardiography, it became possible and feasible to measure rotation and twisting with a high degree of accuracy. No standard normal values are defined for peak torsion, although mean values around 10° are found in normal subjects with a slight increase with age. Purpose In this study we aimed to evaluate torsion in the different types of severe valvular disease. Methods We conducted a retrospective, observational study including patients with severe valvular disease with suitable images for torsion analysis. We included 61 patients (21 with severe aortic stenosis (AS), 20 with severe aortic regurgitation (AR) and 20 with severe mitral regurgitation (MR). Circumferential basal and apical strain was performed, and peak torsion was calculated. Results were compared between groups and were related with echocardiographic parameters, including left ventricle ejection fraction (LVEF). Results Mean age was 70.3 ± 13.6 years with a male preponderance (66%). Mean LVEF was within normal range in the aortic valve disease group; no significant difference was found in LVEF between AS and AR patients (57 ± 7.7% vs 55 ± 9.7%, p = 0.57). In comparison with the aortic disease group, MR patients had a reduced LVEF (48 ± 17.3% vs 56 ± 8.7%, p = 0.05). Mean peak torsion was 8.9 ± 5.1° in AS, 12.6 ± 4.9° in AR and 7.9 ± 3.2° in MR (p = 0.004). Comparing with aortic valve disease patients, MR patients had a reduced mean peak torsion (7.9 ± 3.2° vs 10.7 ± 5.3°, p = 0.03). In relation with patients with AS, those with AR had a higher peak torsion (12.6 ± 4.9° vs 8.9 ± 5.1°, p = 0.024) and a higher left ventricle end-diastolic volume (87.3 ± 29.1 mL.m-² vs 64.5 ± 24.9 mL.m-², p = 0.011). Circumferential apical strain showed a negative correlation with peak torsion (r²=0.203, p = 0.006) and with LVEF (r²=0.290, p < 0.001). Peak torsion did not demonstrate any significant correlation neither LVEF nor circumferential basal strain. Conclusion LV function and peak torsion are more associated with apical than basal circumferential movement. Aortic valve disease is responsible for LV torsion variations in patients with normal ejection fraction, showing an increase in AR and a reduction in AS. In MR patients a reduced LVEF could entails a decrease in peak torsion.

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