Case report: a novel approach for the emergency repair of acute aortic rupture associated with congenital aortic Coarctation

A 28-year-old female who was pregnant (20 weeks gestation) presented to the emergency department with complaints of the sudden onset of severe back pain and dyspnoea.

She had a history of hypertension for more than 14 years, without blood pressure control. As this was her first pregnancy, obstetrical consultations were performed regularly in a local hospital. No other abnormality was recorded, except systemic hypertension.

On emergency physical examination, her vital signs were unstable. Her heart rate was 118 beats/min. The blood pressure in her upper limbs was 80/50 mmHg, and the blood pressure in her lower limbs was too low to calculate. The breath sounds in the left lung disappeared during chest auscultation. Laboratory tests showed that her haemoglobin level had dropped to 63 g/dL.

Echocardiography revealed that the cardiac structures and function were normal, the ascending aorta was normal, and there was a massive left pleural effusion. Unfortunately, it was diagnosed as intrauterine foetal death by pelvic ultrasound.

Aortic CT angiography revealed congenital aortic coarctation associated with pseudoaneurysm formation distal to the coarcted segment with a large left-sided haemothorax. Hence, we highly suspected pseudoaneurysm rupture. Moreover, total aortic hypoplasia was observed.

Considering this patient had haemorrhagic shock, conventional open repair was deemed excessively risky. Moreover, the removal of the dead foetus as soon as possible was required to prevent secondary coagulation disorders. The patient was immediately transferred to the hybrid operating room, and general anaesthesia was performed. Right femoral artery access was established percutaneously, and a 5F sheath was inserted. A calibrated pigtail catheter (Cook Group Inc., Bloomington, Ind) was introduced through the right femoral artery and was advanced over a hydrophilic guidewire (0.035,180 cm, Radiofocus guidewire M, Terumo Europe, N.V., Leuven, Belgium) up to the ascending aorta. Left radial artery access was established to help the catheter pass through the coarcted segment. Angiography of the entire aorta was obtained, and the diagnosis was confirmed to be congenital aortic coarctation-associated aortic hypoplasia and a ruptured pseudoaneurysm distal to the coarcted segment, which caused a large left-sided haemothorax and left lung collapse.

A series of accurate measurements were performed. The diameter of the most coarcted segment was 1.8 mm, and the aortic diameters of the proximal and distal coarcted segments were only 9.2 mm and 8.6 mm, respectively. The diameters of the bilateral femoral arteries were only 3.8 mm and 3.8 mm, respectively. The diameters of the bilateral iliac arteries were only 4.1 mm, respectively (Fig. 1). The extremely narrow access to the vessel and severe aortic hypoplasia made therapeutic decisions difficult. It was impossible to perform a routine endovascular operation, such as covered-stent grafts or covered Cheatham-Platinum stent implantation (at least a 14F delivery system was required, 1F ≈ 0.33 mm).

Then, a novel approach was proposed. In the first step, a right-side axillary-femoral bypass was performed. An 8 mm * 60 mm vascular graft (GORE INTERING Vascular Graft, GORE, USA) was sutured at the right infraclavicular region, with a right femoral end-to-side anastomosis. The graft was tunneled subcutaneously along the midaxillary line to prevent graft entanglement due to torso flexion. In the second step, transcatheter aortic occlusion was performed via left femoral access. Left femoral access was obtained percutaneously, and then, a 10F long sheath (Cook, Bloomington, Indiana, USA) was advanced from the left femoral artery over a 0.035 in. superstiff guidewire (Amplatz Super Stiff, Boston Scientific, USA) through the lesion segment. Two 18/16 mm PDA occluders (Starway Medical Technology Inc., Beijing, China) were placed proximal and distal to the lesion segment to block flow. The proximal occluder was placed exactly between the origin of the left subclavian artery and the narrowest segment, and the distal occluder was placed 30 mm beyond the site of the pseudoaneurysm, where the aortic wall was morphologically normal. Postoperative angiography demonstrated patent bypass and the satisfied position of the occluders. Ruptured pseudoaneurysm was successfully excluded. No residual leakage was found. The upper abdominal aorta still filled in an anterograde manner by extensive collateral vessels. The perfusion of the abdominal visceral arteries was sufficient. The right iliac artery was filled in a retrograde manner by the bypass vessel. The anterograde and retrograde blood flows intermixed at the distal abdominal aorta (Fig. 2). After the procedure, the vital signs became stable, and the blood pressure was 125/71 mmHg in the upper limbs and 116/73 mmHg in the lower limbs. A 6F Perclose ProGlide suture closure device (Abbott Vascular, Abbott Park, Ill) was deployed at the access point. In the third step, the hysterotomy procedure was performed by an obstetrician/gynaecologist.

The patient was then transferred to the intensive care unit, and the pleural effusion was drained intermittently (300 mL every other day). She was extubated on the 3rd day after the procedure and then received low-dose antiplatelet therapy (aspirin 100 mg/daily). Her recovery was uneventful, with an upper limb blood pressure of 130/80 mmHg and a lower limb blood pressure of 120/75 mmHg. The left lung progressively re-expanded. She was discharged on the 10th day after the operation in good clinical condition.

The patient was followed up for 18 months after discharge. During the follow-up, she underwent physical examination, including blood pressure (BP) measurements and CT angiography of the total aorta. Daily antiplatelet therapy was continued until now. Her blood pressure was approximately 130/70 mmHg in the upper limbs and 116/68 mmHg in the lower limbs (the pressure gradient was approximately 10–20 mmHg).

The first-year follow-up computed tomography angiography revealed that the positions of the occluders were satisfied, the coarcted segment and ruptured pseudoaneurysm were excluded completely, and the bypass vessel was patent (Fig. 3). The patient also underwent abdominal ultrasonography at the 18-month follow-up. Adequate blood flow of visceral arteries and bilateral lower limbs was revealed, and the mixed level of anterograde and retrograde blood flow was still at the distal abdominal aorta.