Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study.

OBJECTIVE: To investigate pregnancy outcomes, especially the risk of pregnancy-related aortic dissection (AD), in patients with Marfan syndrome (MFS) after prophylactic aortic root replacement (ARR). DESIGN: Retrospective case series study. SETTING: Tertiary perinatal care centre at a university hospital. POPULATION: Pregnant women fulfilling the revised Ghent nosology (2010) criteria for MFS who were managed at our institute. METHODS: The pregnancy outcomes of all patients with MFS managed at our institute between 1982 and September 2016 were reviewed retrospectively based on medical records. MAIN OUTCOME MEASURES: Obstetrical management and complication including the incidence of AD throughout the peripartum period. RESULTS: Among 22 patients (28 pregnancies) who had been managed as potential MFS or related disorders, 14 (17 pregnancies) fulfilled the revised Ghent nosology (2010) criteria for MFS and were enrolled in this study. Five patients (five pregnancies) had received ARR before conception: three (60%) developed type B aortic dissection [AD(B)] during the peripartum period, compared with only one of 10 patients (12 pregnancies) without ARR (P < 0.05, Chi-square test). CONCLUSIONS: Our study results suggest that MFS patients after prophylactic ARR are still at high risk of AD(B) during the peripartum period. Careful pre-pregnancy counselling and multidisciplinary care throughout the peripartum period are essential for the management of MFS, even after surgical repair of an ascending aortic aneurysm. TWEETABLE ABSTRACT: MFS patients after prophylactic ARR are still at high risk of type B aortic dissection during the peripartum period.

[Short-term outcome of extracardiac lateral tunnel using the atrial free wall and autologous pericardium].

BACKGROUND: Several modifications to the original Fontan procedure have been proposed in order to decrease postoperative morbidity. Lateral tunnel and extracardiac total cavo-pulmonary connection are 2 such modifications. PATIENTS: Between August 2005 and December 2005, the extracardiac lateral tunnel procedure was performed in 5 patients. The age at operation ranged from 19 to 59 months (median 24 months) and the weight ranged from 9.2 to 16.1 kg (median 11.4 kg). RESULTS: There was no mortality. The mean operation time was 466 +/-118 minutes. The mean cardiopulmonary bypass time was 198 +/- 61 minutes. The mean durations of intubation, intensive care unit stay, drainage tube use, and hospital stay were 1 +/- 1, 7 +/- 3, 12 +/- 5 and 30 +/- 2 days, respectively. Postoperative catheterization findings demonstrated that the mean superior venous caval pressure, inferior venous caval pressure, ventricular volume and ventricular ejection fraction were 10.0 +/- 1.4 mmHg, 11.0 +/- 2.4 mmHg, 140 +/- 47% of normal and 58.0 +/- 6.8% , respectively. CONCLUSIONS: The short-term results of the extracardiac lateral tunnel compared favorably with the results of different types of Fontan operation. In addition this procedure has the potential for growth and anticoagulation therapy is unnecessary.