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OBJECTIVES Pregnancy-related aortic dissection (AoD) in Marfan syndrome is a lethal catastrophe. Due to its rarity and limited clinical experience, there is no consensus regarding the optimal management strategy. We seek to present our 21-year experience in such patients , focusing on management strategies and early and late outcomes. METHODS Between 1998 and 2019, we managed 30 pregnant women with Marfan syndrome (mean age 30.7 ± 4.3 years) who sustained AoD at a mean of 28.3 ± 8.8 weeks of gestation (GWs). AoD was acute in 21 (70%), type A (TAAD) in 24 (80%) and type B (TBAD) in 6 (20%). Fourteen TAADs (58.3%, 14/24) and 2 TBADs (33.3%, 2/6) occurred in the third trimester or postpartum. The maximal aortic size was < 45 mm in 26.7% (8/30; 3 TAADs, 5 TBADs). Management strategy was based on the types of dissection and GWs (i.e. surgical versus medical treatment, surgery or delivery first). RESULTS TAADs were treated medically in 1 and surgically in 23. The timing of delivery and surgery were caesarean first at 35.4 ± 6.1 GWs in 7 (29.2%), followed by surgery after mean 46 days; single-stage C-section and surgery at 32.0 ± 5.0 GWs in 10 (41.7%); and surgery first at 18.0 ± 5.8 GWs in 6 (25%), followed by C-section after 20 days. Maternal and foetal mortality were 28.6% (2/7) and 14.3% (1/7), 10.0% (1/10) and 20.0% (2/10) and 16.7% (1/6) and 83.3% (5/6), respectively. Five TBADs (83.3%) were managed with C-section followed by surgery in 2 and medical treatment in 3. The respective maternal and foetal mortality were 50% (1/2) and 100% (2/2) and 33.3% (1/3) and 33.3% (1/3), respectively. One TBAD was managed surgically first followed by C-section, resulting in maternal survival and foetal death. Follow-up was complete in 95.8% (23/24) at 3.7 ± 2.9 years. Four late deaths occurred and reoperation was performed in 1 patient. Maternal and foetal survival were 64.3% and 54.1% at 6 years, respectively. CONCLUSIONS Management of AoD in pregnant women with Marfan syndrome should be based on types of dissection (surgical versus medical) and gestational age (delivery or surgery first), which largely determine maternal and foetal survival. Aortic repair should be considered prior to conception in women with Marfan syndrome even at diameters smaller than recommended by current guidelines.

OBJECTIVES To study the perioperative outcomes and long-term survival rates in patients undergoing thoracic endovascular aortic repair (TEVAR) for uncomplicated type B dissection. METHODS A total of 751 patients with uncomplicated type B dissection who underwent TEVAR at our centre between May 2001 and December 2013 were retrospectively reviewed. The mean age of all patients (619 males and 132 females) was 52.8 ± 10.9 years. The follow-up period ranged from 1 to 170 months (median 70 months). RESULTS Five patients died during the perioperative period (mortality rate 0.7%). Four patients (0.5%) developed retrograde type A dissection. Two patients (0.3%) developed paraplegia and 1 patient developed incomplete paralysis (0.1%). There were no postoperative cerebral infarctions. The 5- and 10-year survival rates were 96.5% [95% confidence interval (CI) 95.0–98.0%] and 83.0% (95% CI 77.9–88.4%), respectively. The 5- and 10-year reintervention rates were 4.6% (95% CI 3.0–6.2%) and 7.9% (95% CI 5.3–10.5%), respectively. CONCLUSIONS Although the application of TEVAR for patients with uncomplicated dissection is still under debate, many patients who have undergone TEVAR have benefitted substantially from the treatment. Our data showed that TEVAR had low mortality and complication rates both in the short- and long-term follow-up periods. TEVAR may be considered as a first choice for patients with uncomplicated type B dissection.

OBJECTIVES: The presence of an isolated left vertebral artery (ILVA) remains a challenging issue for thoracic endovascular aortic repair (TEVAR) of type B dissection if the proximal landing zones are inadequate. We retrospectively reviewed our experience of the surgical management of complicated type B dissection with an ILVA using the stented elephant trunk technique. METHODS: Between February 2009 and May 2013, 7 patients with complicated type B dissection (acute = 2 and chronic = 5) underwent the stented elephant trunk procedure under hypothermic cardiopulmonary bypass with selective antegrade cerebral perfusion. All the patients were males with a median age of 53 ± 6 (range 42–59) years. Preoperative lower limb ischaemia was observed in 1 patient, renal dysfunction in 1 patient and visceral ischaemia in 1 patient. RESULTS: There were no in-hospital deaths. The median ventilator support time was 16 ± 3 (range 11–20) h. Ischaemia of the lower limb and viscera was ameliorated after surgical stent-graft implantation. Continuous renal replacement therapy was not required in patients with preoperative renal dysfunction after surgery. No neurological deficits were observed in any patients prior to hospital discharge. One patient underwent TEVAR due to distal aortic dilatation within the mean follow-up period, which was 44 ± 19 months. CONCLUSION: Repair of complicated type B dissection with an ILVA using the stented elephant trunk technique was associated with satisfactory surgical results in patients with inadequate proximal fixation zones. This technique is an alternative to TEVAR for complicated type B dissection with inadequate proximal landing zones.