Aortic events in pregnant patients with Marfan syndrome. Lessons from a multicenter study.

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≥ 40mm 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.

The influence of Marfans and bicuspid valves on outcomes following aortic valve reimplantation.

BACKGROUND: We analyzed our early and midterm results with aortic valve reimplantation surgery to determine the influence of Marfan syndrome and bicuspid valves on outcomes with this technique. METHODS: Between March 2004 and December 2015, 267 patients underwent aortic valve reimplantation operations. The mean diameter of the sinuses of Valsalva was 50 ± 3 mm and moderate/severe aortic regurgitation was present in 34.4% of these patients. A bicuspid aortic valve was present in 21% and 40% had Marfan syndrome. RESULTS: Overall 30-day mortality was 0.37% (1/267). Mean follow-up was 59.7 ± 38.7 months. Overall survival at 1, 3, and 5 years was 98 ± 8%, 98 ± 1%, and 94 ± 2%, respectively. Freedom from reoperation and aortic regurgitation >II was 99 ± 5%, 98 ± 8%, 96.7 ± 8%, and 99 ± 6%, 98 ± 1%, 98 ± 1%, respectively at 1, 3, and 5 years follow-up, with no differences between Marfan and bicuspid aortic valve groups. (p = 0.94 and p = 0.96, respectively). No endocarditis or thromboembolic complications were documented, and 93.6% of the patients did not receive any anticoagulation therapy. CONCLUSIONS: The reimplantation technique for aortic root aneurysms is associated with excellent clinical and functional outcomes at short and mid-term follow-up.

Early thrombosis of a tricuspid annuloplasty ring and mild hyperhomocysteinemia.

Tricuspid annuloplasty ring thrombosis is an extremely infrequent pathology, and no evidence-based antithrombotic management has been described. We present the case of a 40-year-old female with isolated mild hyperhomocysteinemia and early ring thrombus formation after surgical primary closure of an atrial septal defect and ring tricuspid annuloplasty. Clinical management, antithrombotic treatment, and hyperhomocysteinemia implications are discussed.

Predictors of mortality and reoperation in acute type-a aortic dissection surgery: 18 years of experience.

INTRODUCTION AND OBJECTIVES: To describe our experience with acute type-A aortic dissection surgery, including an analysis of the effect of cerebral protection on outcome. METHODS: Between March 1990 and October 2007, 98 consecutive patients underwent surgery for acute type-A aortic dissection. Of these, 85 had an ascending aorta replacement, while the entire arch was replaced in 13. The aortic valve was replaced in 34 patients but preserved in the rest. An intimal tear was observed in 83 patients. RESULTS: The in-hospital mortality rate was 15%. Risk factors for in-hospital mortality were age > or = 70 years and preoperative cardiogenic shock (P< .05). Antegrade cerebral perfusion was used in the last 16 consecutive patients, whose in-hospital mortality rate was 6%. The proportions of patients who survived and who did not require reoperation at 1, 5 and 10 years of follow-up were 98.6%+/-1.3%, 86.2%+/-4.6% and 68.2%+/-8.9%, and 97.2%+/-1.9%, 82.5%+/-4.8% and 55.9%+/-7.9% for the two outcomes, respectively. The risk factors for reoperation were found to be severe preoperative aortic regurgitation and preservation of the aortic valve (P< .05). The only risk factor for late mortality was not using antegrade cerebral perfusion (P< .05). CONCLUSIONS: Despite its seriousness, surgery for acute aortic dissection produces good early and long-term results. Antegrade cerebral perfusion improves the prognosis of these patients and should be the technique of choice for cerebral protection.