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INTRODUCTION: Aortic stenosis (AS) is the most common valvular heart disease and the most common indication for aortic valve replacement in adults. Aortic valve neocuspidization (AVNeo) with fixed autologous pericardium, also known as the Ozaki procedure, is a possible alternative treatment of AS. Autopericardial valves save the dynamics and physiological anatomy of the aortic root, however, the service life of autopericardial leaflets is limited. There is no data about factors that may influence the development of AVNeo insufficiency. Here, we assessed the effect of autopericardial leaflet symmetry on the development of aortic insufficiency after Ozaki procedure. METHODS: This study included 381 patients with AS who underwent Ozaki procedure. Patients were divided into group 1 (171 patients with symmetric aortic root) and group 2 (210 patients with asymmetric aortic root). RESULTS: The maximum observation period was up to 65 months. Sixteen cases of aortic insufficiency were detected in group 1, and 33 cases were detected in group 2. Based on the results of Cox regression, the predictors of aortic insufficiency in the late postoperative period are age and asymmetry of neocusps. According to results of Kaplan-Meier analysis, insufficiency of AVNeo in the maximum follow-up period after surgical correction of AS for group 1 patients was significantly lower than for group 2 patients (P=0.006). CONCLUSION: Asymmetric neocusps increase the risk of aortic insufficiency in the mid-term period after Ozaki procedure. And the older the patients at the time of surgery, the less likely they develop AVNeo insufficiency.

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We report a 19-year-old woman with a history of percutaneous aortic valve (AV) balloon valvuloplasty 3 years ago. She was admitted with severe symptomatic AV stenosis with narrow aortic annulus. We decided to perform AV reconstruction with autologous pericardium. However, surgical findings revealed a very narrow AV annulus with a unicuspid valve; therefore, an AV enlargement was added.

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AIMS: Left ventricular remodelling occurs during the chronic course of aortic regurgitation (AR) and aortic stenosis (AS), leading to myocardial hypertrophy and fibrosis. Several studies have shown that extracellular volume fraction (ECV) and indexed extracellular volume (iECV) are important surrogate markers of diffuse myocardial fibrosis (MF). Postoperative data on these cardiovascular magnetic resonance (CMR) extracellular expansion parameters for either AS or AR are scarce. This study aimed to demonstrate the postoperative changes that occur in diffuse MF, and the influence of preoperative MF on the reversal of LV remodelling, in patients with AR or AS. METHODS AND RESULTS: Patients with severe AR or AS and indications for surgery were prospectively enrolled. Patients underwent pre- and postoperative CMR, and ECV and iECV were quantified. Data from 99 patients were analysed (32 with AR and 67 with AS). After surgery, the left ventricle mass index decreased in both groups (AR: 110 vs. 91 g/m2; AS: 86 vs. 68 g/m2, both P < 0.001). The late gadolinium enhancement fraction (AR: preoperative 1.9% vs. postoperative 1.7%, P = 0.575; AS: preoperative 2.4% vs. postoperative 2.4%, P = 0.615) and late gadolinium enhancement mass (AR: preoperative 3.8 g vs. postoperative 2.5 g, P = 0.635; AS: preoperative 3.4 g vs. postoperative 3.5 g, P = 0.575) remained stable in both groups. Preoperative iECV and ECV were greater in the AR group (iECV: 30 mL/m2 vs. 22 mL/m2, P = 0.001; ECV: 28.4% vs. 27.2%, P = 0.048). Indexed extracellular volume decreased after surgery in both groups (AR: 30-26.5 mL/m2, AS: 22-18.2 mL/m2, both P < 0.001); it was still greater in the AR group (AR: 26.5 mL/m2 vs. AS: 18.2 mL/m2, P < 0.001). Postoperative ECV remained stable in the AR group (preoperative 28.4% vs. postoperative 29.9%; P = 0.617) and increased in the AS group (preoperative 27.2% vs. postoperative 28.6%; P = 0.033). CONCLUSION: Patients with both AR or AS presented reduction in iECV after surgery, unfolding the reversible nature of diffuse MF. In contrast to patients with AS, those with AR developed postoperative iECV regression with stable ECV, suggesting a balanced reduction in both intracellular and extracellular myocardial components.

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Background: Aortic valve (AV) replacement is the gold standard treatment for severe symptomatic AV disease. Recently, AV reconstruction surgery (Ozaki procedure) emerges as a surgical alternative with good results in the medium term. Methods: We retrospectively analyzed 37 patients who underwent AV reconstruction surgery between January 2018 and June 2020 in a national reference center in Lima, Peru. The median age was 62 years, interquartile range (IQR: 42-68). The main indication for surgery was AV stenosis (62.2%), in most cases due to bicuspid valve (19 patients, 51.4%). Twenty-two (59.4%) patients had another pathology with surgical indication associated to AV disease, 8 (21.6%) had dilatation of the ascending aorta with indication for replacement. Results: One in-hospital death occurred (1/38, 2.7%) due to perioperative myocardial infarction. There was a significant reduction in the medians of the peak (70 mmHg, CI 95% = 50.03-79.86 vs. 14 mmHg, CI 95% = 11.93-17.5, p < 0.0001) and mean (45.5 mmHg CI 95% = 30.6-49.68 vs. 7 mmHg, CI 95% = 5.93-9.6, p < 0.0001) AV gradients when we compared baseline characteristics with first 30-days results. In an average of 19 (± 8.9) months of follow-up, survival, reoperation-free survival for valve dysfunction, and survival free of AV insufficiency ≥ II were 97.3%, 100% and 91.9%, respectively. Significant reduction in the medians of the peak and mean AV gradients was maintained. Conclusions: AV reconstruction surgery showed optimal results in term of mortality, reoperation-free survival, and hemodynamic characteristics of the neo-AV.

Introducción: El reemplazo de la válvula aórtica es el tratamiento de elección para la valvulopatía aórtica severa sintomática. Recientemente, la cirugía de reconstrucción valvular aórtica (procedimiento de Ozaki) emerge como una alternativa quirúrgica con buenos resultados a mediano plazo. Métodos: Analizamos retrospectivamente 37 pacientes intervenidos de reconstrucción de válvula aórtica entre enero de 2018 y junio de 2020 en un centro de referencia nacional en Lima, Perú. La mediana de edad fue de 62 años (rango intercuartílico: 42-68). La estenosis de la válvula fue la principal indicación de cirugía (62.2%), en la mayoría de los casos por válvula bicúspide (19 pacientes, 51.4%). 22 (59.4%) pacientes presentaban otra patología con indicación de cirugía, 8 (21.6%) presentaban dilatación de la aorta ascendente con indicación de reemplazo. Resultados: La mortalidad intrahospitalaria fue de 1/38, 2.7%, por infarto de miocardio perioperatorio. Hubo una reducción significativa en las medianas del gradiente máximo (70 mmHg, IC 95% = 50.03-79.86 vs. 14 mmHg, IC 95% = 11.93-17.5, p < 0.0001) y gradiente medio (45.5 mmHg IC 95% = 30.6-49.68 vs. 7 mmHg, IC 95% = 5.93-9.6, p < 0.0001) de la válvula aórtica cuando comparamos las características basales con los resultados de los primeros 30 días. En una media de 19 (± 8.9) meses de seguimiento la sobrevida fue del 97.3%; la sobrevida libre de reoperación por disfunción valvular, 100% y la sobrevida libre de insuficiencia aórtica ≥ II, 91.9%, respectivamente. Se mantuvo una reducción significativa en las medianas de los gradientes máximo y medio de la válvula aórtica. Conclusiones: La cirugía de reconstrucción de válvula aórtica con pericardio autólogo mostró resultados óptimos en términos de mortalidad, supervivencia libre de reoperación y características hemodinámicas de la neo-válvula.

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La sífilis es causada por Treponema pallidum, es reconocida por la variedad de sus manifestaciones clínicas; compromete el sistema nervioso, tegumentario y cardiovascular. Las alteraciones cardiovasculares ocurren en 70% de los casos con sífilis no tratada, el 71% de las aortitis sifilíticas desarrollan aneurismas aórticos y la complicación más frecuente es la insuficiencia aórtica. El tratamiento va dirigido a la enfermedad subyacente, con manejo antibiótico para la sífilis terciaria y las complicaciones tromboembólicas y si requiere reparación quirúrgica. Presentamos un caso poco frecuente de un paciente diagnosticado de infección por el virus de la inmunodeficiencia humana y complicaciones cardiovasculares típicas de la sífilis terciaria, que precisó tratamiento médico y quirúrgico, que fue exitoso. Destacamos la pericia clínica de los profesionales de nuestra institución para diagnosticar e identificar las complicaciones cardiovasculares de la sífilis terciaria.

Syphilis is caused by Treponema pallidum and is recognized by the variety of its clinical manifestations; it involves the nervous, integumentary and cardiovascular systems. Cardiovascular alterations occur in 70% of cases with untreated syphilis, 71% of syphilitic aortitis develop aortic aneurysms and the most frequent complication is aortic insufficiency. Treatment is directed at the underlying disease, with antibiotic treatment for tertiary syphilis and thromboembolic complications and if necessary surgical repair. We present a rare case of a patient diagnosed with human immunodeficiency virus infection and cardiovascular complications typical of tertiary syphilis, who required medical and surgical treatment, which was successful. We highlight the clinical experience of our institution's professionals in the diagnosis and identification of cardiovascular complications of tertiary syphilis.

A sífilis é causada pelo Treponema pallidum e é reconhecida pela variedade de suas manifestações clínicas; ela afeta os sistemas nervoso, tegumentar e cardiovascular. As alterações cardiovasculares ocorrem em 70% dos casos de sífilis não tratada, 71% dos casos de aortite sifilítica desenvolvem aneurismas aórticos e a complicação mais frequente é a insuficiência aórtica. O tratamento é direcionado para a doença subjacente, com tratamento antibiótico para sífilis terciária e complicações tromboembólicas e, se necessário, reparo cirúrgico. Apresentamos um caso raro de um paciente diagnosticado com infecção pelo vírus da imunodeficiência humana e complicações cardiovasculares típicas da sífilis terciária, que precisou de tratamento médico e cirúrgico, que foi bem-sucedido. Apresentamos um caso raro de um paciente com diagnóstico de infecção pelo vírus da imunodeficiência humana e complicações cardiovasculares típicas da sífilis terciária, que necessitou de tratamento médico e cirúrgico, o qual foi bem-sucedido. Destacamos a experiência clínica dos profissionais de nossa instituição no diagnóstico e na identificação das complicações cardiovasculares da sífilis terciária.

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Marfan syndrome is an autosomal dominant hereditary disease that compromises various systems that usually require a multidisciplinary approach. The cause of Marfan syndrome is unknown, but recent genetic studies have related this disease to an extracellular microfibrillar defect located on chromosome 15q15-q21.3. Due to the severity of the signs and symptoms the diagnosis is usually at a very young age. We report a patient with extreme Marfan syndrome with all the particulars that this syndrome offers.

El síndrome de Marfan es una enfermedad hereditaria autosómica dominante que compromete diversos sistemas que habitualmente requieren un enfoque multidisciplinario. La causa del síndrome de Marfan es desconocida, pero recientes estudios genéticos han relacionado esta enfermedad a un defecto microfibrilar extracelular localizado en el cromosoma 15q15-q21,3. Debido a la severidad de los signos y síntomas el diagnóstico suele ser a edad muy temprana. Reportamos un paciente con síndrome de Marfan extremo con todas las particulares que éste síndrome ofrece.

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OBJECTIVE: In recent years, transcatheter aortic valve replace (TAVR) has revolutionized the interventional treatment of aortic stenosis, however, only scarce evidence considers it as treatment for Aortic Regurgitation (AR). At present, the treatment of Pure AR of Native Valve with TAVR does not fall within the recommendations of international guidelines, as it poses multiple challenges with immediate and long-term variable and unpredictable results. The objective of this paper is to present the case of a patient with prohibitive operative risk that benefited of TAVR treatment for AR. MATERIALS AND METHODS: We present the case of a 79-year-old male patient who has severe Pure Native Aortic Valve Regurgitation, considered inoperable. Based on the heart team's decision, TAVR was planned with the use of an Edwards SAPIEN 3 valve. After TAVR, the patient developed complete heart block and a pacemaker was implanted. He improved to NYHA II functional class. At 15-month follow-up, he suffered intracranial hemorrhage and passed away. RESULTS AND CONCLUSIONS: Management of patients with severe symptomatic AR with high surgical risk continues to be a special challenge. They have high mortality if left untreated with valvular change, despite medical treatment. Even though it is strictly off-label, TAVR might be a reasonable solution for a select type of patients who are considered inoperable due to surgical high mortality risk.

OBJETIVO: En años recientes, el reemplazo valvular aórtico vía percutánea (TAVI) ha revolucionado el tratamiento de los pacientes con estenosis aórtica, sin embargo, poca evidencia la considera como opción de tratamiento en pacientes con insuficiencia aórtica (IA), especialmente en pacientes con Insuficiencia Aórtica Pura de Válvula Nativa (IAPVN), dado que esta patología confiere múltiples retos con resultados muy variables tanto a corto como a largo plazo. Dada su alta mortalidad en caso de permanecer sin tratamiento, la primera opción de tratamiento es la cirugía de cambio valvular. MATERIAL Y MÉTODOS: Presentamos el caso de un masculino de 79 años con IAPVN grave considerado inoperable dadas las comorbilidades. De acuerdo con la decisión del equipo de Cardiología, se decidió realizar TAVI con la colocación de una Válvula Edwards SAPIEN 3. El paciente desarrolló bloqueo AV completo como única complicación y se colocó marcapasos permanente. Permaneció en clase funcional NYHA II. A los 15 meses de seguimiento presentó hemorragia intracraneal y falleció. RESULTADOS Y CONCLUSIONES: El manejo de los pacientes con IAPVN grave sintomática con riesgo quirúrgico alto sigue siendo un reto especial. Tienen alta mortalidad si no se realiza cambio valvular a pesar de tratamiento médico. A pesar de que no se recomienda en las guías actuales, la TAVI puede ser una opción razonable para un grupo muy selecto de pacientes que son considerados inoperables.

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INTRODUCTION: Coronary artery occlusion due to fusion of a leaflet to the sinotubular junction is a rare finding that we should consider in the differential diagnosis of young patients who have aortic regurgitation and angina. PATIENT AND METHOD: We present a young female with severe aortic regurgitation due to right coronary fusion who underwent mini-invasive aortic valve reconstruction. RESULTS: Postoperative evolution was satisfactory. The patient was discharged on the 5th postoperative day and after 3.5 years of follow-up he remains in functional class I, without anticoagulant treatment and with mild aortic regurgitation. COCNCLUSION: The Ozaki technique can be used in patients with aortic regurgitation due to single leaflet dysfunction.

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