Single-stage open repair of severe asymmetric pectus excavatum and mitral valve replacement in connective tissue disease

Pau, C P; Chong, Kee Soon; Yakub, Mohd Azhari; Khalil, Alizan Abdul

doi:10.1177/02184923211014004

Cited by 1 publication

(1 citation statement)

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“…Standard median sternotomy is generally considered the most ideal surgical route for AADA repair. However, for patients suffering from severe thoracic deformity, scoliosis, and other complications, median sternotomy should be avoided as much as possible due to problems with exposure of the surgical field and postoperative lung function (2)(3)(4). However, in this patient, attribute to the severity of aortic root and arch dissection as well as aneurysmal dilatation of the aortic sinus, we attempted a median sternotomy with uncertainty and performed Bentall procedure and Sun's procedure on an MFS patient with severe scoliosis and thoracic deformity.…”

Section: Introductionmentioning

confidence: 99%

Successful management of Stanford type A aortic dissection with severe scoliosis in the setting of Marfan syndrome: a case report

Huang¹,

Yang²,

Liu³

et al. 2022

Ann Transl Med

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Background: Marfan syndrome (MFS) is a connective tissue disorder involving multiple organs. The most severe complications include aortic root dilatation and dissection. In the present report, we provide an uncommon case of acute aortic Stanford type A dissection (AADA) repair with severe scoliosis in an MFS patient and it is even more rare for such surgical treatment to be successfully completed along with holistic management that enables the patient to recover successfully. We offer a reference for future surgical therapy since the specific surgical treatment methods in this case have not been reported in the literature.Case Description: A 40-year-old Chinese female with MFS was rushed to our surgical clinic due to the sudden onset of intense chest pain. Physical examination revealed a diastolic murmur at the aortic valve area, increased arm and pectus carinatum deformity, severe scoliosis, acromicria, arachnodactyly, and planovalgus foot. Subsequently, AADA was discovered through computed tomography scan. In addition, echocardiogram revealed moderate aortic regurgitation and hydropericardium in small amount. Based on revised Ghent criteria, the patient was diagnosed with MFS complicated with aortic dissection. Emergency surgery was successfully performed for repair of the patient's aortic dissection and the diseased aortic valve.Postoperatively, the patient presented with a degree of respiratory insufficiency, However, the respiratory function was not greatly impaired, with good early intervention, such as taking deep breaths and coughing fully, active sputum suction, effective analgesia, ambulation and treadmill exercise. The patient finally recovered completely and was discharged 3 weeks later. Conclusions:We reported on a patient with severe scoliosis who successfully underwent surgical repair of AADA. Our report shows that the application of standard median sternotomy for repairing AADA offers the feasibility of implementation, on the basis of effectively solving various practical problems in the surgery brought about by scoliosis. It has been thoroughly assessed and addressed how the postoperative condition of such patients affects subsequent respiratory function and postoperative recovery. This report further provides a successful clinical reference for the implementation of this type of surgery and the postoperative management of respiratory function.

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Section: Introductionmentioning

confidence: 99%