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)

Left atrial thrombosis after heart transplantation.

Left atrial thrombosis in the absence of rheumatic heart disease and atrial fibrillation is a rare occurrence. We report two cases of left atrial pedunculated thrombus formation after orthotopic heart transplantation. Despite an uneventful post-operative course, sinus rhythm and normal contractility of the heart, large thrombi could be found several months following transplantation. Surgical thrombectomy was performed under cardiopulmonary bypass. Operative technique is proposed as one of the main factors that can contribute to left atrial thrombosis after heart transplantation.

[Biphasic graft preservation for lung transplantation]. / Preservación bifásica del injerto en el trasplante pulmonar.

The standard, most widely applied way of preserving a lung for transplantation is infusion through the pulmonary artery (PA) of a pulmonaryplegic solution. In this prospective study, we analyzed the initial function of the pulmonary and cardiac graft after biphasic infusion of a solution introduced retrograde through the left auricle and antegrade through the PA. Twenty-six heart and lung grafts (9 unilateral and 17 bilateral) were preserved by cardioplegia and pulmonaryplegia (biphasic) between January 1996 and March 1997. Indicators of graft viability recorded were the ratio of arterial oxygen pressure (PaO2) to inspired fraction (FiO2), mean systemic pressure (MSP), mean pulmonary artery pressure (MPAP) cardiac output, pulmonary vascular resistance (PVR) and systemic vascular resistance (SVR). The variables were recorded upon arrival of the grafts in the intensive care unit and in the first 24 h. Morbidity and mortality after heart transplants were recorded throughout a follow-up period of one month. After transplantation, most patients had a oxygenation coefficient (PaO2/FiO2) greater than 252 mmHg in the first 48 h. Hemodynamic parameters were also kept within normal ranges immediately after surgery and 24 h later. Mean ischemic time was 245 min for unilateral transplants, 215 for the first lung in double lung transplants, and 300 min for the second lung. In the early postoperative period, 3 patients suffered lung graft dysfunction, which was treated satisfactorily with nitric oxide (NO). No heart transplant patient suffered primary heart failure or left ventricular dilatation. We conclude that biphasic pulmonary preservation achieves satisfactory initial functional viability of the graft. Heart grafts removed simultaneously functioned successfully in the transplanted patient without additional pharmacological or mechanical support.

[Surgical treatment of hypertrophic myocardiopathy using a video-assisted technique. Apropos of a case]. / Tratamiento quirúrgico de la miocardiopatía hipertrófica mediante técnica vídeo-asistida. A propósito de un caso.

The use of endoscopic technology is gaining more and more popularity within cardiac surgery. We present a case employing endoscopic instruments in the resection of the interventricular septum in a patient with hypertrophic cardiomyopathy unresponsive to medical treatment. Advantages of this technique are discussed.

VDD pacing with single lead in a heart transplant recipient: case report.

Permanent pacemaker implantation is required in a large number of transplantation patients principally because of sinus node dysfunction of the donor atrium. The most suitable mode of pacing in these cases is still subject to controversy. We describe one case of a single lead system of VDD stimulation and sensing of the recipient atrial signal in a 32-year-old patient with posttransplant symptomatic sinus node dysfunction. Physiological adaptation of rate was achieved with recovery of normal receptor sinus node function.

[Surgical treatment of atrial fibrillation]. / Tratamiento quirúrgico de la fibrilación auricular.

Atrial fibrillation is the most frequently found sustained arrhythmia. It increases the risk of thromboembolism and adversely affects cardiac performance because of loss of atrial kick. New surgical treatments of atrial fibrillation have been developed to ablate the origin of abnormal impulses on the atrium. The left atrial isolation and the corridor operation restores the regular rhythm, but do not reduce the risk of thromboembolism because the left atrium may continue to fibrillate. The maze operation has proven to be effective in both converting to sinus rhythm and regaining atrial contractility. However, this method is meticulous and time-consuming and takes more cardiac ischemic time, especially when other cardiac procedures are performed simultaneously.

Clinical experience with retrograde lung preservation.

Previous reports and our own experimental work suggest increased vascularity of the tracheobronchial wall when retrograde lung preservation is used. This principle was clinically applied in 21 consecutive lung transplant recipients (10 single and 11 bilateral). Lung preservation was achieved via the left atrial appendage and drainage was obtained through the pulmonary artery. Pneumoplegic preservation was achieved with modified Euro-Collins solution. Cardioplegia was induced by the standard method and the heart, harvested by different teams, did not exhibit left ventricular dilatation. Thirty-two bronchial anastomoses without wrapping were performed. No primary lung graft failure was documented. Cardiopulmonary bypass was instituted in three cases of pulmonary hypertension; however, this was deemed unnecessary in the remainder of the cases of bilateral transplantation while the second organ was being implanted. All bronchial anastomoses were followed between 2 and 28 months. A single instance of bronchial anastomosis dehiscence was observed on the 30th postoperative day. However, no stents were employed in this series, and no strictures or anastomotic granulomas have been reported so far. All the hearts could be used satisfactorily except for one primary graft failure. In conclusion, retrograde lung preservation is feasible in clinical lung transplantation, with simultaneous harvesting of the heart. The impact of retrograde lung preservation on the late clinical outcome remains to be seen.

[The heart transplant in congenital cardiopathies. The surgical considerations]. / El trasplante cardíaco en las cardiopatías congénitas. Consideraciones quirúrgicas.

Structural congenital heart disease accounts for a small number of patients who undergo heart transplantation. Orthotopic heart transplantation in this group of patients necessitates special operative modifications and may be technically challenging. However, offer an effective therapeutic alternative for persons with end-stage myocardial failure in conjunction with congenital heart disease.

[Donor selection. Marginal donors]. / Selección de donantes. Donantes marginales.

The number of heart transplant candidates has increased steadily in the past several years because of improved survival and sustantial decrease in the incidence of rejection and serious infections. Unfortunately the number of available donors was remained insufficient, the method of expanding the donors pool may be to liberate the criteria for an acceptable donors heart (age, size, inotropic drugs ...). We analyzed the incidence of this factors.

Efficacy of low-dose OKT3 as cytolytic induction therapy in heart transplantation.

BACKGROUND: The need for prophylactic cytolytic treatment in heart transplantation is a controversial issue. Its use, however, might prevent the onset of cellular rejection in the immediate postoperative period, facilitating patient management. It has recently been suggested that the administration of these products at low doses might have the same immunologic impact and would reduce secondary effects and the cost of treatment. METHODS: In a nonrandomized retrospective study, we assessed 45 consecutive patients who underwent orthotopic heart transplantation in 1992 and 1993. Six patients who died before receiving the complete OKT3 dose were excluded. Twenty-three patients were treated with 5mg/day doses of OKT3 for 7 consecutive days. Another 16 patients received 2.5 mg of OKT3 for 7 consecutive days. RESULTS: There were no significant differences between the two groups with respect to CD3 counts on days 2 (0.1% +/- 0.3% versus 0.04% +/- 0.25%; p > 0.05) and 6 (0.2% +/- 0.45% versus 0.1% +/- 0.3%; p > 0.05), number of rejection episodes (1.45% +/- 0.8% per year of follow-up versus 1.7% +/- 1.2%, p = 0.66), number of infectious complications (8 versus 3, p > 0.05), total methylprednisolone dose used to treat rejection crises (3900 +/- 2765 versus 3600 +/- 1963 mg; p = 0.71), adverse effects attributed to OKT3 (two versus none), or length of the postoperative hospital stay (36.8 +/- 19 versus 30.2 +/- 20.9 days). CONCLUSIONS: As cytolytic induction therapy in heart transplantation, a daily regimen of 2.5 mg of OKT3 for 7 days achieves the same clinical and immunologic effect as the conventional 5 mg/day dose. In addition, it results in a considerable reduction in the cost of treatment.

[Right ventricular arrhythmogenic dysplasia. The role of heart transplantation in its management]. / Displasia arritmogénica ventricular derecha. El papel del trasplante cardíaco en su manejo.

Arrhythmogenic right ventricular dysplasia is a rare disease that usually presents with ventricular arrhythmias and sometimes with heart failure. Rarely symptoms become severe and refractory to conventional therapy. We present the case of a 36-year-old man with this disease who had sustained ventricular arrhythmias and severe right heart failure. Because these symptoms were poorly controlled with medical therapy, cardiac transplantation was finally chosen as definitive treatment. The patient had a favorable course and now leads near-normal life.

[A heart transplant in a patient with doxorubicin-induced myocardiopathy as a complication of the treatment of a lymphoma]. / Trasplante cardíaco en un paciente con miocardiopatía por doxorrubicina como complicación del tratamiento de un linfoma.

We present the case of a young man who developed severe cardiomyopathy eight years after receiving high-dose doxorubicin therapy as treatment for diffuse lymphoblastic lymphoma. He underwent cardiac transplantation and eighteen months later the tumor has not recurred and the patient has no cardiac symptoms.

The hemodynamic responses to upright exercise after orthotopic cardiac transplant.

The study was undertaken to examine the heart rate (HR) and stroke volume (SV) responses to upright exercise in patients after orthotopic cardiac transplantation (Group A). The findings were compared to data obtained from post-coronary artery bypass surgery patients (Group B) and from healthy subjects (Group C). All three groups (n = 12 in each group) were matched for age and gender. The preexercise HR and blood pressure were significantly higher in Group A (p < 0.05), whereas the SV was significantly lower (41 +/- 4mL.beat-1 vs 63 +/- 3 and 65 +/- 2 mL.beat-1 in Group B and C respectively, p < 0.05). In Group A, at submaximal work loads, the SV was consistently lower than in Groups B and C. During the early phases of exercise, the HR was consistently higher in Group A also. Systemic vascular resistance remained significantly higher in Group A throughout exercise (p < 0.05).

Heart transplantation in a patient with liver hydatidosis.

The human can serve as an accidental intermediate host to Echinococcus granulosus, a parasite that targets dogs as its definitive hosts. We present the case of a 27-year-old man with liver hydatidosis, who underwent heart transplantation because of ischemic cardiomyopathy. The immunosuppressor treatment had no effect on the size of the cysts, which were removed surgically 14 months after heart transplantation; good results persist after 3 years of follow-up.