Introduction

Pseudoaneurysm is an infrequent but life-threatening complication of aortic coarctation repair. It occurs in 5-9% of patients and, left untreated, carries poor medium-term prognosis. Further complications such as pseudoaneurysm rupture or Aorto-Pulmonary Fistula (APF) carry a high mortality rate and are a therapeutic as well as a surgical challenge, not least because they require immediate attention, accurate diagnosis and decisive action. The standard management approach of such complications is open surgery. Yet, this can become particularly delicate because these complications tend to occur in patients who already underwent prior open surgery in this location, and because most such patients are still young, having undergone the initial surgery in infancy or in their teenage years. This might partly explain that the intra-operative mortality during open surgical management is reported to be non-negligible (around 15%) [1]. Furthermore, the infrequent occurrence of such complications makes them rarities, best managed by a multidisciplinary team, and to this day devoid of generally agreed-upon guidelines. In this case report, we wish to present our choice of a hybrid management technique, which proved surgically adequate and clinically successful.

Case Report

A 42-year-old man was transferred to our hospital following several episodes of hemoptysis that progressively increased over a week and were accompanied by cough and dyspnea (NYHA class 2). He reported neither chest pain nor palpitations and had no known exposure to tuberculosis. The patient had undergone two aortic coarctation repairs, 26 and 37 years earlier. Regular follow-up every other year was managed by his cardiologist. The patient was not under medication, had no history of drug abuse, never smoked and had no family history of systemic diseases. At initial presentation, the patient was hemodynamically stable and his cardiovascular evaluation showed no sign of respiratory distress. The laboratory analyses yielded results for hemoglobin level at 160g/l (normal range: 133-177 g/l) and white blood cell count at 10.7 G/l (normal range: 4.0-10-0 G/l). Cardiac enzymes were all within normal range. The chest X-ray showed a widening of the mediastinum and multidetector contrast-enhanced Computed Tomography (CT) demonstrated a bulky (79 x 69 mm) thoracic aortic pseudoaneurysm at the aortic isthmus starting 7 mm distally to the left subclavian artery origin and ending 6 cm lower (Figure 1a-c). Examination of the lung window revealed peri-aneurysmal infiltration in the upper left lung parenchyma with multiple pulmonary ground glass opacities in the apex (Figure 1d). Because of the radiological signs of alveolar hemorrhage, the presence of the bulky aneurysm and the clinical presentation, we suspected an aorto-bronchopulmonary fistula, a rare complication of aortic pseudoaneurysms. Because the patients had a history of two prior thoracic surgeries, and because of the proximal localization of the pseudoaneurysm, a multidisciplinary decision was made to exclude the aneurysm using a thoracic endograft that would ultimately cover the left CCA and subclavian arteries. The surgical procedure was performed using a transverse incision over the anterior border of the right sternomastoid, with the patient positioned in dorsal decubitus to expose the right CCA. A second left supraclavicular incision was made to expose the left CCA. The dissection of the left subclavian artery was carried out all the way to the origin of the vertebral and internal mammary arteries. To establish the bypass, a 7mm Dacron tube was connected end-to-side to the right CCA and end-to-end to the Left Subclavian Artery (LSA) proximally to the vertebral and to the internal mammary arteries. The prosthetic bypass was tunneled in the retropharyngeal and retroesophageal space. Figure 2 shows the reimplantation of the left CCA on the Dacron graft.

Immediately after the bypass was established, a 170mm E-VITA thoracic 3D Jotec® endograft was expanded just distally to the brachiocephalic trunk in zone 1, using bilateral femoral approaches. The final completion angiogram showed a type Ib endoleak. The endoleak was immediately managed using a second E-VITA thoracic Jotec® endograft ending around the vertebral T9 level. A final selective angiogram demonstrated the complete exclusion of the aneurysm and the absence of endoleak. The brachio-cephalic trunk and the bypass were permeable (Figure 3). The total operative time was 221 minutes, clamping times were 7, 15 and 17 minutes for the right CCA, the left CCA and the vertebral artery, respectively. Total blood loss was 200 ml. The patient was extubated immediately after surgery and transferred to the intensive care unit for 24 hrs. At post-operative day 2, the angioCT scan showed a permeable graft and supra-aortic debranching (Figure 4). Doppler pulse ultrasound examination confirmed good flow in the supra-aortic trunks with triphasic and symmetric flow (Q 500ml/min) in the subclavian arteries with no steal syndrome. The neurovascular examination of the arm was normal, with both radial and ulnar pulses well detectable. The patient was discharged from hospital and allowed to go home on post-operative day 5 without any complication or recurrence of hemoptysis.

Discussion

This case illustrates a rare occurrence of aorto-bronchial fistula (ABF) caused by a pseudoaneurysm many years after coarctation repair. Hemoptysis was the main clinical sign and diagnosis was confirmed by CT scan. Management was discussed in a multidisciplinary team including vascular, cardiac and thoracic surgeons. Kodolitsch and al [4] studied 25 pseudoaneurysms following aortic coarctation repair. In their cohort, none of them presented with ABF. Advanced age at coarctation repair and patch graft technique independently predicted local aneurysmal formation. Considering the large diameter of the pseudoaneurysm located in a field that had already been operated on twice, open surgery was deemed too dangerous, even considering the young age of the patient and the absence of medical comorbidities. This opinion was aligned with reports by other authors, which published relatively high rates of intra-operative deaths (15%) for similar indications, and 100% mortality with a conservative treatment and no surgery [1]. In another study, Mosquera and al [2] retrospectively reviewed 18 aorto-bronchial fistulas, of which five were secondary to aortic open repair. These authors studied two management techniques: open surgery and thoracic endovascular aortic repair (TEVAR) for patients in whom open surgery was not possible because of comorbidities. They assessed outcome predictors of aorto-bronchial fistulas and reported that with both surgical approaches, outcomes were mainly conditioned by the etiology of the fistula. These authors also reported that the worst prognosis was found among patients with fistulas caused by mycotic aneurysms and that the overall in-hospital mortality was 50 % (n=9), mostly caused by sepsis. Recently, endograft repairs became an alternative to open surgery with encouraging results even in patients suitable for open surgery. Bailey and al [3] studied 11 cases (mean age 69.8 years old, range: 30-89) of endovascular treatment of ABF. These authors reported a 91% technical success rate during an average follow-up of 8.8 months. Endoleaks (9%) and infection (18%) were the most prevalent complications of this technique requiring long-term follow-up [4].

Conclusion

Pseudoaneurysm with ABF following aortic coarctation repair is a rare etiology of ABF with relatively non-specific clinical manifestations. Surgically, it is particularly challenging because it is frequently located at the aortic isthmus and it occurs in a young population that has already been operated at least once. Those characteristics require a durable and safe treatment in a patient with delicate ground and no alternative non-surgical options. No guidelines have been established as yet for the management of this rare complication. Our choice of a hybrid management technique was successful and outcomes at one month were positive.

Figures

References

1. Picichè, Marco, De Paulis R, Fabbri A, Chiariello L (2003) “Postoperative Aortic Fistulas into the Airways: Etiology, Pathogenesis, Presentation, Diagnosis, and Management.” The Annals of Thoracic Surgery 75: 1998-2006.
2. Mosquera, Victor X, Milagros Marini, Pombo-Felipe F, Gómez-Martinez P, et al. (2014) “Predictors of Outcome and Different Management of Aortobronchial and Aortoesophageal Fistulas.” The Journal of Thoracic and Cardiovascular Surgery 148: 3020-26.e1-2.
3. Bailey, Charles J, Seth Force, Ross Milner, Karthikeshwar Kasirajan, et al. (2011) “Thoracic Endovascular Repair as a Safe Management Strategy for Aortobronchial Fistulas.” Journal of Vascular Surgery 53: 1202-1209.
4. Von Kodolitsch Y, Aydin MA, Koschyk DH, Loose R, Schalwat I, et al. (2002) “Predictors of Aneurysmal Formation after Surgical Correction of Aortic Coarctation.” J Am Coll Cardiol. 39: 617-624.