scholarly journals Aortic Valve Stenosis with Black Pigmentation Due to Alkaptonuria: A Case Report

Author(s):  
Takahiro Shojima ◽  
Yasuyuki Zaima ◽  
Kazuyoshi Takagi ◽  
Tohru Takaseya ◽  
Hiroyuki Otsuka ◽  
...  

We report about a 78-year-old woman with severe aortic valve stenosis (AS). She had heavily calcified and thickened aortic valve leaflets with black pigmentation. After excision of the aortic valve leaflets, biological aortic valve replacement (AVR) was performed. Alkaptonuria was diagnosed and confirmed based on increased urinary excretion of homogentisic acid. She has had no cardiovascular system-related symptoms for 3 years and no evidence of structural valve deterioration. Data on the long-term outcomes of AVR associated with structural valve deterioration that could regulate prosthetic valve selection in alkaptonuria are limited. Therefore, further research on the natural evolution of AS and the rate of structural valve deterioration after AVR is needed to provide an optimal prosthesis for these patients.

2020 ◽  
Vol 75 (11) ◽  
pp. 2150
Author(s):  
Nicolas Isaza ◽  
Milind Y. Desai ◽  
Samir R. Kapadia ◽  
Amar Krishnaswamy ◽  
L. Leonardo Rodriguez ◽  
...  

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Avinee ◽  
E Durand ◽  
T Levesque ◽  
P Y Litzler ◽  
J N Dacher ◽  
...  

Abstract Background Since the first-in-man transcatheter aortic valve implantation (TAVI) performed in 2002, the number of procedures has dramatically increased. However, long-term data regarding outcome and valve durability remain poor. Purpose We aimed to evaluate the evolution of 30-day outcomes over years and long-term mortality and valve durability after TAVI. Methods All consecutive patients presenting with severe symptomatic aortic stenosis treated by TAVI in our center were included prospectively. Clinical and echocardiographic follow-up was performed at 30 days and annually thereafter. Survival curves were constructed using Kaplan-Meier analysis. We also evaluated valve durability according to the European standardized definition of structural valve deterioration. Results Between 2002 and 2018, 1530 consecutive patients underwent TAVI including 1285 (84.0%) patients via a femoral approach. A balloon-expandable transcatheter heart valve was predominantly used (1421 patients; 92.9%). The annual transfemoral approach rate increased progressively to reach 93.3%. Age of patients remained stable over time with a global mean age of 83.7±6.5 years old. Logistic EuroSCORE decreased from 49.2±8.2% to 14.3±8.6% (p<0.0001). Thirty-day mortality dramatically decreased below 3% since 2015 and was 0% in 2018. Similarly, major vascular complications decreased from 50.0% in the first year to less than 1% since 2017 (p=0.001). The length of hospital-stay progressively shortened up to a median of 2 days in 2018. The Kaplan-Meier survival estimation was 82.3%, 60.3%, 33.0%; 11.7% and 8.9% respectively at 1, 3, 5, 8 and 10 years. On long-term follow-up the mean aortic gradient remained unchanged (Figure), and only five patients presented a severe prosthetic valve deterioration. Among them, four patients successfully benefited from a valve in valve TAVI procedure. The competing risk analysis at 10 years estimates risk for severe and moderate-or-severe valve deterioration of 1.9±0.9% and 4.3±1.3% respectively. TEE mean transaortic gradient Conclusions Long-term (up to 10 years) follow-up of our large pioneer series of patients treated by TAVI shows a dramatic improvement of outcomes and no warning signs of valve deterioration suggesting very encouraging valve durability, using predominantly, a balloon expandable prosthesis. Further studies are warranted to study valve durability after TAVI before extension to lower risk patients.


2020 ◽  
Vol 110 (6) ◽  
pp. 1967-1973 ◽  
Author(s):  
Razan Salem ◽  
Andreas Zierer ◽  
Afsaneh Karimian-Tabrizi ◽  
Aleksandra Miskovic ◽  
Anton Moritz ◽  
...  

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