Eventos aórticos en el embarazo de pacientes con síndrome de Marfan. Lecciones de un estudio multicéntrico / Aortic events in pregnant patients with Marfan syndrome. Lessons from a multicenter study

Introducción y objetivos: El embarazo en el síndrome de Marfan (SM) incrementa el riesgo de eventos aórticos. La evidencia clínica actual es escasa y no existe un consenso específico sobre el tratamiento óptimo de estas pacientes. Se presenta nuestra experiencia multicéntrica. Métodos: Entre enero de 2004 y enero de 2020, 632 pacientes con SM mantuvieron revisiones periódicas en unidades de Marfan. Durante este periodo se identificó a todas las mujeres gestantes y se analizó la incidencia de eventos aórticos durante el embarazo y el puerperio. Resultados: Se hallaron 133 embarazos de 89 mujeres (8 con cirugía de aorta previa). No hubo mortalidad materna. Cinco mujeres sufrieron eventos aórticos durante el tercer trimestre del embarazo y el puerperio (2 disecciones tipo A, 1 disección tipo B y 2 crecimientos significativos de la aorta (≥ 3 mm). La incidencia de eventos aórticos fue del 3,7%. Se evidenció una mayor tendencia a eventos con diámetros aórticos pregestacionales ≥ 40 mm (p=0,058). La mortalidad fetal fue del 3%. El 37,6% de los partos se realizaron mediante cesárea. Conclusiones: Las mujeres con SM tienen un incremento del riesgo de eventos aórticos en el embarazo, especialmente durante el tercer trimestre y el periodo posparto. Se debería valorar, en centros de referencia, la cirugía aórtica profiláctica pregestacional con diámetros aórticos ≥ 40 mm. Es importante un diagnóstico precoz, un estudio pregestacional de toda la aorta, la administración de bloqueadores beta y un estrecho seguimiento durante el embarazo, especialmente durante el último trimestre y el posparto (AU)

Introduction and objectives: Pregnancy in women with Marfan syndrome (MS) is associated with an increased risk of aortic events. The clinical evidence on pregnant patients with MS is limited and there is no specific consensus on their optimal management. We report our multicenter experience. Methods: From January 2004 to January 2020, 632 patients with MS underwent periodic monitoring in Marfan units. During this period, we identified all pregnant women with MS and analyzed the incidence of aortic events during pregnancy and puerperium. Results: There were 133 pregnancies in 89 women with MS (8 women with prior aortic surgery). There were no maternal deaths, but 5 women had aortic events during the third trimester and puerperium (type A dissections in 2, type B dissection in 1, and significant [≥ 3mm] aortic growth in 2). The aortic event rate was 3.7%. Pregestational aortic diameter≥ 40 mm showed a nonsignificant association with aortic events (P=.058). Fetal mortality was 3% and 37.6% of births were cesarean deliveries. Conclusions: Women with MS have an increased risk of aortic events during pregnancy, especially in the third trimester and postpartum period. Patients with MS and aortic diameters ≥40mm should be assessed in experienced centers for prophylactic aortic surgery before pregnancy. It is important to provide early diagnosis, prepregnancy study of the aorta, beta-blocker administration, and close monitoring during pregnancy, especially during the last trimester and postpartum (AU)

Aspergillus endocarditis in lung transplant recipients: case report and literature review.

We present a case of endocarditis caused by Aspergillus in a lung transplant (LT) patient and review 6 previously reported cases. All cases were caused by Aspergillus fumigatus. Five patients (71%) were transplanted due to cystic fibrosis. None of the patients had any previously known valvular heart disease. The time that elapsed between LT surgery and the diagnosis of Aspergillus endocarditis was 8±6 months. Large peripheral emboli were observed in 6 patients (85%); blood cultures were negative in all. Transthoracic echocardiography did not detect the presence of vegetations in 3 patients (43%); the vegetations measured >1.5 cm in all cases. Five patients (71%) died from disseminated disease. Both survivors underwent combined therapy with antifungal drugs and surgical treatment.

High incidence of severe infections in heart transplant recipients receiving tacrolimus.

BACKGROUND: Tacrolimus (FK) is being increasingly used as an alternative to cyclosporine (CyA) in heart transplantation (HTx). It is believed to engender slightly more powerful protection against acute rejection. However, the increased immunosuppression could result in an excess of infectious complications. METHODS: Our study compared the incidence of major infections (MInf), defined as life-threatening infectious episodes requiring admission and intravenous (IV) antimicrobial therapy, among a series of HTx recipients treated with either FK (n=30) or CyA (n=84). RESULTS: A total of 21 patients received FK in an elective protocol and 9 patients initially treated with CyA were converted to FK. Tacrolimus was combined with azathioprine and prednisone in 21 cases, and with mycophenolate mofetil and steroids in 8 recipients. After a follow-up between 6 and 37 months, 11 patients (37%) in the FK group developed 13 episodes of MInf, most (85%) occurring during the first posttransplant year. Conversely, CyA patients (n=84), a group with similar characteristics and follow-up, showed a MInf incidence of 12% (P<.05). Among the FK group, the most common site of MInf was pulmonary (69%). A variety of opportunistic agents caused MInf in 54% of cases, whereas the remaining ones were attributed to nosocomial bacteria. There were three deaths (27% of all MInf), all in azathioprine-treated patients with initial FK therapy. CONCLUSIONS: Tacrolimus therapy seems to be associated with an increased incidence of severe infections in HTx recipients. We recommend aggressive diagnostic and therapeutic approaches for patients on FK who develop signs or symptoms of infection in the first year after HTx.