Cardiac Surgery Resident University of Western Ontario, London Health Sciences Centre London, Ontario, Canada
Case background: A 57-year-old male with a history of smoking, obesity, COPD, type 2 diabetes, remote bowel perforation and a new stage 4 follicular lymphoma with a history of chest radiation presented to our center with NYHA class 4 heart failure. He was admitted to our cardiac care unit and started on IV milrinone, furosemide infusion and a 7-day course of oral prednisone and ceftriaxone for potential COPD exacerbation. He had large, bilateral pleural effusions which were drained with bilateral 14-French pleural catheters. For his lymphoma, he was seen by hematology and initially planned to undergo CHOP-R regimen. However, given his heart failure, this was delayed.
A transesophageal echocardiogram revealed a pseudoaneurysm and fistula of the posterior aortic root and left sinus of Valsalva with perforation into the left atrium and left ventricle, disrupting the aorto-mitral curtain (Figure 1). As a result, there was severe mitral regurgitation. Significant flow from the pseudoaneurysm was noted into the LA and LV with diastolic flow reversal in the descending thoracic aorta. A moderately stenotic bicuspid aortic valve was noted with moderate aortic insufficiency. No signs of aortic root abscess were observed, but the ascending aorta was aneurysmal, measuring 4.9 cm x 4.7 cm.
Preoperative CT heart confirmed the initial finding of a large pseudoaneurysm centred within the aorto-mitral curtain and central fibrous trigones. It was hypothesized that this aneurysm originated from the aortic annulus. The pseudoaneurysm measured approximately 5.8cm on the long axis and 2.5cm on the short axis view.
Given the complexity of his condition, hematology, radiation oncology, and cardiac surgery were involved in a multidisciplinary discussion. It was determined that surgical repair of the pseudoaneurysm and sinus of the Valsalva aneurysm would be necessary before initiating chemotherapy. Therefore, we planned to repair his pseudoaneurysm, aorto-ventricular fistula, ascending aorta, and aortic and mitral valves.
Sinus of Valsalva aneurysms (SVAs) are rare. They typically originate from the right aortic sinus and often rupture into the right atrium or ventricle. Left-sided SVAs are even rarer, and when they rupture into the left heart, they constitute a surgical emergency. Due to the limited documentation of surgical approaches for this pathology, we describe a multivalve repair strategy incorporating concomitant aorto-mitral curtain reconstruction and fistula repair.
Management Challenges: Managing this patient posed multiple challenges. First and foremost, the patient had an exceptionally high operative risk, with an STS-predicted mortality of 16% and morbidity/mortality of 64%. Despite these risks, he was determined to undergo surgery, understanding that this was his only opportunity to eventually receive treatment for his lymphoma.
Intraoperatively, we encountered significant obstacles. Extensive tissue friability and adhesions, likely due to his history of chest radiation, complicated dissection, repair, and made hemostasis challenging to achieve. Given the rarity of this pathology and the lack of standardized approaches in the literature, meticulous surgical sequencing was crucial. We began by repairing the mitral valve perforation with an autologous pericardial patch, followed by reconstruction of the aorto-mitral curtain and closure of the aorto-atrial fistula using a bovine pericardial patch. Once root reconstruction and mitral valve repair were complete, we proceeded with bioprosthetic aortic valve replacement and replacement of the aneurysmal ascending aorta with a Dacron graft. Due to his friable, poor-quality tissues, cardiopulmonary bypass (CPB) time was prolonged, but he was ultimately weaned off CPB successfully on moderate-dose inotropes.
Postoperative challenges were even more formidable. The patient had a prolonged ICU stay with high-dose inotropic support. After initial stabilization and transfer to the ward, he developed cardiac tamponade, necessitating pericardiocentesis and ICU readmission. His course was further complicated by MSSA bacteremia and bioprosthetic valve endocarditis, requiring six weeks of IV antibiotics. During this period, he experienced delirium, respiratory failure requiring reintubation, and eventually, percutaneous tracheostomy.
After three months in hospital, he made it through all postoperative complications and was eventually discharged home. He is currently being treated for his lymphoma with chemotherapy. His positive outcome is a testament to the multidisciplinary team that worked together to give him the best outcome possible. Several discussions were made as a group. Hematology's decision to delay chemotherapy and immunosuppressive therapy to allow for the treatment of his endocarditis and encourage proper healing. Coordinating physiotherapy, nutrition and rehabilitation for long-term recovery. All involved played a key role in our patient's success.
Despite these complications, after three months in the hospital, he was successfully discharged home and is now undergoing chemotherapy for his lymphoma. His survival is a testament to the multidisciplinary effort that enabled his recovery. Key discussions with hematology led to delaying chemotherapy and immunosuppression to resolve infection and optimize wound healing. Coordinated physiotherapy, nutritional support, and rehabilitation were essential for his long-term recovery. Every team member was critical in ensuring the best possible outcome in this highly complex case.
Left-sided Sinus of Valsalva (SoV) aneurysm rupture is a rare and poorly documented entity in cardiac surgery, particularly when complicated by atrioventricular fistula and multi-valvular destruction. Our submission introduces an innovative multivalve repair strategy incorporating aorto-mitral curtain reconstruction and fistula repair using bovine pericardium. By detailing a stepwise, reproducible surgical technique, we provide valuable insights into managing these high-risk cases and contribute to the advancement of complex cardiac surgery.
