CARDIOVASCULAR JOURNAL OF AFRICA • Volume 34, No 1, January–April 2023 AFRICA 55 Left thoracotomy approach for aortic root surgery Roman Komarov, Alisher Ismailbaev, Ashot Simonyan, Inoyat Ermetov, Ivan Ivashov Abstract Marfan syndrome is an autosomal dominant disease of connective tissue affecting approximately one in 5 000 people. However, the majority of patients (60–80%) with Marfan syndrome have some degree of aortic root dilation or thoracic aortic aneurysm that can lead to aortic dissection and rupture, which has long been recognised as the leading cause of death in Marfan syndrome. The funnel breast (pectus excavatum), often seen in patients with Marfan syndrome, radically complicates the access during aortic root or arch interventions, forcing cardiosurgery practitioners to seek alternative approaches. We present a series of clinical observations of two patients with Marfan syndrome and aneurysm of the aortic root and ascending aorta. A left-sided thoracotomy provides the possibility of successful implementation of extensive surgical interventions, decreasing the risks of intra-operative damage to the cardiac structures adjacent to the sternum in this complex cohort of patients. Keywords: Marfan syndrome, aortic root dilation, aortic aneurysm, funnel breast, approach, aortic root surgery Submitted 8/1/21; accepted 14/3/22 Published online 29/6/22 Cardiovasc J Afr 2023; 34: 55–58 www.cvja.co.za DOI: 10.5830/CVJA-2022-014 It is known that the access of choice in aortic root and arch surgery is a median sternotomy.1 The cohort of patients suffering from Marfan syndrome (MS), associated with severe connective tissue dysplasia, is known for its high risk (increasing with age) of aortic events (60–80%), which require timely surgical treatment.2 The funnel breast (pectus excavatum), often seen in MS patients, radically complicates the access during aortic root or arch interventions, forcing cardiosurgery practitioners to seek alternative approaches. In these two clinical cases, we provide the technique and results of using left-sided thoracotomy access in aortic root surgery in Marfan syndrome patients. Case reports Case 1 was a male patient, B, aged 23 years, who was admitted to the clinics with symptoms of shortness of breath upon moderate physical activity, and a sense of discomfort in the chest area. Pre-hospital diagnostics revealed the presence of MS, aneurysm of the ascending aorta, as well as significant kyphoscoliosis of the thoracic spine, due to which the patient had undergone thoracoplasty at the age of five years. The anthropometrical data matched with signs of MS: body length was 205 cm and weight was 50 kg. It is worth noting that thoracoplasty conducted in early childhood had not given the desired result: the chest deformation progressed to a severe degree (Fig. 1A). Transthoracic echocardiography (TTE) showed that the patient had an aneurysmatic dilation of the aortic root and ascending aorta (up to 7.5 cm), annulodilation (up to 29 mm) and regurgitation in the aortic valve (up to degree two) with no significant structural changes in the leaflets. No contractility disorders were detected in the ventricular myocardium and no signs of other valvular abnormalities were found (Fig. 1B). The results of computed tomography (CT) showed a significant funnel-shaped chest deformation with a distance of not more than 10 cm between the posterior surface of the sternum and the vertebral column, and dislocated mediastinal organs to the left of the median line. From the CT findings, aortic dissection could not be ruled out, as the images showed only localised intimal dissection at the area of the left coronary artery (LCA) orifice. Therefore, taking into consideration the patient presented with absolute indications for surgical treatment due to the presence of aortic root abnormality, as well as the high risk of using an interventional approach of median sternotomy, the decision was to perform surgery using left-sided thoracotomy access. The thoracotomy procedure provided an approximately 9-cm-long approach along the fifth intercostal space on the left side. Cardiopulmonary bypass (CPB) was applied by cannulation of the femoral vessels, with the access providing a nicely exposed aortic root, allowing better visualisation of the operating field compared to a median sternotomy (since there was dislocation of the mediastinum to the left). Surgical intervention procedures were conducted after a selective intubation of the right primary bronchus while excluding the left lung. Taking into consideration the significant asymmetry of the valve leaflets, which were also thinned, the decision was to avoid the valve-sparing procedure. Therefore, the procedures in this patient included standard prosthetic replacement of the aortic valve and aortic root using the Bentall method (mechanical prosthetic device size 27) with re-implantation of the coronary arteries in various areas, repair of the dissected orifice of the LCA and forming a perigraft-toright atrial shunt (Cabrol) (Fig. 1C). The duration of CPB was 150 minutes, the aortic cross-clamp was applied for 100 minutes, and the duration of intensive care unit (ICU) stay was 24 hours. The patient was discharged on day six after surgery with no complications. Satisfactory haemodynamic data for the aortic root and aortic valve prothesis were obtained on TTE and multi-slice CT at five years of follow up. Case 2 was a male patient, Z, aged 30 years, who was suffering from MS. He had undergone a supra-coronary prosthetic IM Sechenov First Moscow State Medical University, Moscow, Russia Roman Komarov, MD Alisher Ismailbaev, MD Ashot Simonyan, MD Inoyat Ermetov, MD Ivan Ivashov, PhD, MD, i.ivashov@yandex.ru
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