Impact of bicuspid aortic valve or tricuspid aortic valve on hemodynamics and left ventricular reverse remodeling in patients after transcatheter aortic valve replacement / 中华心血管病杂志

Objective: To compare the impact of bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV) on hemodynamics and left ventricular reverse remodeling after transcatheter aortic valve replacement (TAVR). Methods: We retrospectively analyzed the clinical data of patients who underwent TAVR in our hospital from January 2019 to March 2021. Patients were divided into BAV group and TAV group according to aortic contrast-enhanced CT. Each patient was followed up by N-terminal pro B-type natriuretic peptide (NT-proBNP) and echocardiography at four time points, namely before TAVR, 24 hours, 1 month and 6 months after TAVR. Echocardiographic data, including mean pressure gradient (MPG), aortic valve area (AVA), left ventricular ejection fraction (LVEF), left ventricle mass (LVM) and LV mass index (LVMi) were evaluated. Results: A total of 41 patients were included. The age was (75.0±8.6) years, and male patients accounted for 53.7%. There were 19 BAV patients and 22 TAV patients in this cohort. All patients undergoing TAVR using a self-expandable prosthesis Venus-A valve. MPG was (54.16±21.22) mmHg(1 mmHg=0.133 kPa) before TAVR, (21.11±9.04) mmHg at 24 hours after TAVR, (18.84±7.37) mmHg at 1 month after TAVR, (17.68±6.04) mmHg at 6 months after TAVR in BAV group. LVEF was (50.42±13.30)% before TAVR, (53.84±10.59)% at 24 hours after TAVR, (55.68±8.71)% at 1 month after TAVR and (57.42±7.78)% at 6 months after TAVR in BAV group. MPG and LVEF substantially improved at each time point after operation, and the difference was statistically significant (all P<0.05) in BAV group. MPG in TAV group improved at each time point after operation, and the difference was statistically significant (all P<0.05). LVMi was (164.13±49.53), (156.37±39.11), (146.65±38.84) and (134.13±39.83) g/m2 at the 4 time points and the value was significantly reduced at 1 and 6 months post TAVR compared to preoperative level(both P<0.05). LVEF in the TAV group remained unchanged at 24 hours after operation, but it was improved at 1 month and 6 months after operation, and the difference was statistically significant (all P<0.05). LVMi in TAV group substantially improved at each time point after operation, and the difference was statistically significant (all P<0.05). NT-proBNP in both two groups improved after operation, at 1 month and 6 months after operation, and the difference was statistically significant (all P<0.05). MPG in TAV group improved better than in BAV group during the postoperative follow-up (24 hours after TAVR: (11.68±5.09) mmHg vs. (21.11±9.04) mmHg, P<0.001, 1 month after TAVR: (10.82±3.71) mmHg vs. (18.84±7.37) mmHg, P<0.001, 6 months after TAVR: (12.36±4.42) mmHg vs. (17.68±6.04) mmHg, P=0.003). There was no significant difference in NT-proBNP between BAV group and TAV group at each time point after operation (all P>0.05). There was no significant difference in paravalvular regurgitation and second prosthesis implantation between the two groups (all P>0.05). Conclusions: AS patients with BAV or TAV experience hemodynamic improvement and obvious left ventricular reverse remodeling after TAVR, and the therapeutic effects of TAVR are similar between BAV and TAV AS patients in the short-term post TAVR.

Rigidez Arterial e Funções Miocárdicas do Ventrículo Esquerdo em Crianças com Válvula Aórtica Bicúspide Funcional / Arterial Stiffness and Left Ventricular Myocardial Function in Children with a Well-Functioning Bicuspid Aortic Valve

Resumo Fundamento: A rigidez arterial é um importante preditor de aortopatia e remodelamento miocárdico em pacientes com válvula aórtica bicúspide, podendo estar aumentada na infância. Objetivo: Avaliar a rigidez arterial e a função miocárdica do ventrículo esquerdo em crianças com válvula aórtica bicúspide funcional. Métodos: Quarenta e quatro crianças com válvula aórtica bicúspide e 41 pares saudáveis com válvula aórtica tricúspide foram incluídos neste estudo caso-controle. Foram obtidos os diâmetros e os escores-z relacionados da raiz aórtica e da aorta ascendente. Quanto à função miocárdica do ventrículo esquerdo, juntamente com as velocidades de fluxo mitral e parâmetros do Modo M, as velocidades miocárdicas e os intervalos de tempo foram avaliados com Doppler tecidual. A análise da onda de pulso foi realizada por aparelho oscilométrico (Mobil-o-Graph). Um valor de p<0,05 foi considerado significativo. Resultados: O índice da massa ventricular esquerda, a velocidade A do fluxo mitral, o diâmetro e o escore z da aorta ascendente e o índice de desempenho miocárdico estavam significativamente maiores nos pacientes (p = 0,04, p = 0,02, p = 0,04, p <0,001 e p <0,001 respectivamente). O índice de desempenho miocárdico correlacionou-se positivamente com o diâmetro da aorta ascendente e a velocidade A (r=0,272; p=0,01, r=356; p=0,001, respectivamente). A análise multivariada revelou que o índice de desempenho miocárdico estava relacionado ao diâmetro da aorta ascendente (p = 0,01). O índice de aumento e a velocidade da onda de pulso foram semelhantes entre os grupos (p> 0,05). Conclusão: De acordo com a análise da onda de pulso oscilométrico, as crianças com válvula aórtica bicúspide funcional apresentam rigidez arterial semelhante a seus pares saudáveis. O diâmetro da aorta ascendente foi estabelecido como preditor independente da função miocárdica do ventrículo esquerdo. A rigidez arterial pode não ser um fator de risco grave em pacientes pediátricos sem dilatação acentuada da aorta ascendente.

Abstract Background: Arterial stiffness is an important predictor factor of aortopathy and myocardial remodeling in patients with a bicuspid aortic valve and it might be increased in childhood. Objective: To assess the arterial stiffness and left ventricular myocardial function in children with a well-functioning bicuspid aortic valve. Methods: Forty-four children with a bicuspid aortic valve and 41 healthy peers with a tricuspid aortic valve were included in this case-control study. Diameters and the related z-scores of the aortic root and ascending aorta were obtained. As for the left ventricular myocardial function, along with the mitral inflow velocities and M-Mode parameters, myocardial velocities and time intervals were assessed with tissue Doppler imaging. A pulse wave analysis was performed by oscillometric device (Mobil-o-Graph). A p value <0.05 was considered significant. Results: The left ventricular mass index, mitral inflow A velocity, diameter and z-score of the ascending aorta, and myocardial performance index were significantly higher in patients (p=0.04, p=0.02,p=0.04, p<0.001,and p<0.001 respectively). The myocardial performance index was positively correlated with the diameter of the ascending aorta and A velocity (r=0.272;p=0.01, r=356;p=0.001, respectively). The multivariate analysis revealed that the myocardial performance index was related to the ascending aorta diameter (p=0.01). The augmentation index and pulse wave velocity were similar between the groups (p>0.05). Conclusion: According to the oscillometric pulse wave analysis, the children with a well-functioning bicuspid aortic valve had similar arterial stiffness to that of the healthy peers. The ascending aorta diameter was established as an independent predictor of left ventricular myocardial function. Arterial stiffness may not be a severe risk factor in pediatric patients without marked ascending aorta dilation.

Válvula aórtica bicúspide: ¿qué debo conocer? Revisión actualizada de sus aspectos clínicos y fisiopatológicos / Bicuspid aortic valve: what should I know? Updated review of its clinical and pathophysiological aspects

Resumen La válvula aórtica bicúspide es la cardiopatía congénita más frecuente en la población general. Lejos de ser solo una malformación valvular inocua, supone una enfermedad compleja y heterogénea. A menudo es identificada como un hallazgo incidental en personas sanas, cursando de manera asintomática. Sin embargo, en un alto porcentaje de pacientes conduce a lo largo de su vida a complicaciones valvulares (estenosis, insuficiencia, endocarditis) o aórticas (dilatación o disección). Con frecuencia estas manifestaciones suceden a una edad temprana y causan una elevada morbimortalidad. A pesar de que en los últimos años se ha producido una intensa investigación en este campo, la fisiopatogenia de la enfermedad no es del todo conocida y muchas preguntas siguen abiertas. En este artículo se revisan de forma actualizada los aspectos clínicos y fisiopatológicos más novedosos y relevantes sobre esta cardiopatía congénita.

Abstract The most common congenital heart disease in the general population is the bicuspid aortic valve. Far from being just a harmless valve malformation, it is a complex and heterogeneous disease. It is often identified as an incidental finding in healthy people. However, in a high percentage of patients it leads throughout their life towards valvular (stenosis, insufficiency, endocarditis) or aortic (dilatation or dissection) complications. Frequently, manifestations occur at an early age, being responsible for high morbidity and mortality. Even though in recent years intense research has been carried out in this field, the pathophysiogenesis of the disease is not fully known and many questions remain open. In this article, we review the most innovative and relevant clinical and pathophysiological aspects of this congenital heart disease.

Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital

Abstract Background: Coarctation of the aorta is a congenital segmental narrowing of the aortic arch with severe hemodynamic repercussions and increased cardiovascular mortality. Early surgical correction and life-time echocardiographic follow-up must be performed to improve prognosis. However, this goal has been challenged by high rates of underdiagnosis, which delay surgical correction, and by recoarctation in up to one third of operated patients. Objectives: The objectives of this study were: (i) to register the frequency of common clinical signs at diagnosis of coarctation of the aorta; (ii) to describe the course of echocardiographic parameters before and during the follow-up of coartectomized subjects; (iii) to analyze the clinical prognosis of patients according to baseline characteristics, occurrence of recoarctation and associated malformations. Methods: Case-series of 72 patients coarctectomized between June 1996 and November 2016 in a tertiary care hospital. Clinical, echocardiographic and surgical variables were considered. All patients were submitted to coarctectomy by posterolateral thoracotomy and end-to-end anastomosis. Data were classified as parametric or non-parametric by Kolmogorov-Smirnov test. Parametric data were expressed as mean and standard deviation, and non-parametric data as median and interquartile range. Continuous variables were analyzed using paired t-tests, and categorical variables were compared by chi-square test. For all analysis, a p-value of less than 0.05 was considered statistically significant. Statistical analysis was performed using SPSS, version 20.0 (IBM, Chicago, IL, USA). Results: The mean follow-up time was 5.8 years (range: 0-20 years). At diagnosis, most patients had heart murmur (88%), non-palpable pulse in the lower limbs (50%), left ventricular hypertrophy (78%), and bicuspid aortic valve (33%), with a mean aortic peak gradient of 55 mmHg. After surgical correction, those without recoarctation were less symptomatic (60 vs 4.5%; p < 0.001), had lower aortic peak gradient (54 ± 3.8 vs 13 ± 0.8; p = 0.01) and left ventricle mass (95 ± 9.2 vs. 63 ± 11; p = 0.01), and the most common complications were late hypertension (39.2%), and recoarctation (27.6%). Recoarcted patients did not show improvement of neither clinical nor echocardiographic variables. Age at repair and bicuspid aortic valve groups had comparable results with controls. Surgical procedure was safe; mean time of hospitalization was 10 days and mean surgery time 2.3 hours. Conclusions: Coarctectomy improves cardiac symptoms and left ventricular hypertrophy, with a slight effect on the incidence of hypertension. Recoarctation occurs in one-third of patients and draws attention for the need of lifelong surveillance by echocardiography.

Patrón de flujo en "4D Flow" y su vínculo con la aortopatía bicúspide / 4D Flow pattern and its role in bicuspid aortic valve aortopathy

ABSTRACT: Bicuspid aortic valve (BAV) disease is generally associated with thoracic aortic dilatation (TAD). Related factors include; genetical, morphological (valvular phenotype) and most recently, hemodynamic profiles associated with flow pattern and wall shear stress. Cardiac magnetic resonance 4D Flow (4DF) can give an integral evaluation of these later flow variables. Remarkable, different spectrums of flow and vortex direction exist in BAV that are related to the site of TAD (proximal or distal). Therefore, we present a 57 years old patient with BAV (Sievers 0) with anteroposterior leaflets distribution in which 4DF depicted an anteriorly and righthand oriented jet that correlated with the zone of grater AD; also, vortex rotation was counterclockwise, corresponding to the most frequent vortex type in BAV. In conclusion, 4DF is a powerful and ground-breaking tool that enhances our knowledge of BAV related aortopathy.

Hipoplasia de la valva posterior de la válvula mitral y válvula aórtica bicúspide. Una muy rara asociación para encontrar en un paciente adulto / Hypoplasia of the posterior mitral valve leaflet and bicuspid aortic valve. A very rare combination to find in an adult patient

Resumen Las cardiopatías congénitas representan un reto diagnóstico para el clínico, sobre todo si su presentación es en el adulto. Las anomalías de este tipo que se observan sobre la válvula mitral pueden presentarse de forma aislada o en conjunto con otros defectos cardiacos. Se expone un caso de hipoplasia severa de la valva posterior de la válvula mitral, entidad que en condiciones normales es fatal antes de nacer o muy sintomática durante la niñez, pero cuya presentación tardía es extremadamente rara y difícil de encontrar en el adulto. Este es el primer caso reportado no solo en nuestro país sino en todo Suramérica.

Abstract The congenital heart diseases are a diagnostic challenge for the clinician, particularly when they are presented in an adult. These types of anomalies that are observed in the mitral valve can be presented in isolated form or together with other heart defects. The case is presented of a patient with severe hypoplasia of the posterior mitral valve leaflet. This anomaly is, under normal conditions, fatal before birth or very symptomatic during childhood, and its delayed presentation is extremely rare and difficult to find in the adult. This the first case reported, not only in this country, but in the whole of South America.

Endocarditis infecciosa fatal por Corynebacterium pseudodiphtheriticum / Letal endocarditis due to Corynebacterium pseudodiphtheriticum

We present a case report of a infective endocarditis by Corynebacterium pseudodiphtheriticum in a young patient eight years old with bicuspid aortic valve that led to a severe neurological embolism and death. Corynebacterium pseudodiphtheriticum is part of the nasopharyngeal and skin flora. However, there are cases reported of endocarditis usually associated with immunocompromised patients that mostly presented a poor clinical outcome.

Se presenta el caso clínico de una endocarditis infecciosa por Corynebacterium pseudodiphtheriticum en un niño de 8 años, portador de una valvulopatía aórtica bicúspide, que falleció por una embolia cerebral grave. Corynebacterium pseudodiphtheriticum forma parte de la microbiota de la piel y nasofaringe. Sin embargo, se han descrito casos de endocarditis en pacientes inmunocomprometidos, asociados, en su mayoría, a una mala evolución clínica.