Aortic surgery in Marfan patients with severe pectus excavatum

Raffa, Giuseppe Maria; Kowalewski, Mariusz; Malvindi, Pietro Giorgio; Bertani, Alessandro; Romano, Giuseppe; Sciacca, Sergio; Turrisi, Marco; Armaro, Alessandro; Stringi, Vincenzo; Montalbano, Giuseppe; Mattiucci, Gabriella; Follis, Fabrizio; Pilato, Michele

doi:10.2459/jcm.0000000000000394

Cited by 13 publications

(9 citation statements)

References 27 publications

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“…2 Correction of both cardiac and chest wall pathology in a single stage can be performed safely; however, there are differing techniques and lack of consensus regarding the best timing for surgery. 3 It is an uncommon procedure and most of the literature consists of case reports. 4,5 In this case, severe asymmetrical pectus excavatum and close proximity of right inferior pulmonary vein behind the sternum render median sternotomy unsafe.…”

Section: Discussionmentioning

confidence: 99%

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

Pau

Chong

Yakub

et al. 2021

Asian Cardiovasc Thorac Ann

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We present a 14-year-old boy with Loey–Dietz syndrome with severe mitral regurgitation, pectus excavatum and scoliosis. The Haller index was 25. The heart was displaced into the left hemithorax. The right inferior pulmonary vein was very close to the sternum and vertebral body. Single-stage surgery was performed. An osseo-myo-cutaneous pedicled flap was created by sterno-manubrial junction dislocation and rib resection with bilateral internal mammary arteries supplying the flap. Cardiopulmonary bypass and mitral valve replacement was performed. The defect was bridged with three straight plates. The flap was laid on top and anchored. Early outcome at three months was good.

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Section: Discussionmentioning

confidence: 99%

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

Pau

Chong

Yakub

et al. 2021

Asian Cardiovasc Thorac Ann

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“…El Índice de Haller se utiliza para evaluar la gravedad de la deformidad del PE en la TAC (Imagen N°5). Tradicionalmente, un Índice de Haller de >3.25 se consideraba una indicación para la cirugía en pacientes con una deformidad del PE 13,14 .…”

Section: Discussionunclassified

Pectus excavatum como debut de síndrome de Marfan: reporte de caso

Sánchez

Cueva²,

López³

et al. 2021

RFCM

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Introducción: el Síndrome de Marfan (SM) es un trastorno autosómico dominante caracterizado por alteraciones del tejido conectivo; en el que la talla alta, las extremidades y dedos alargados se asocian con anormalidades en los sistemas cardiovascular, esquelético y ocular. Su diagnóstico se basa en los criterios de Ghent, que reúne hallazgos clínicos y factores hereditarios. Su principal complicación catastrófica es la dilatación y disección de aorta. Mediante el análisis del presente caso clínico se espera recabar las principales características relacionadas a esta importante patología que aporte argumentos claves a la literatura médica para un diagnóstico precoz y seguimiento oportuno que disminuya la morbimortalidad de esta enfermedad. Caso clínico: presentamos un paciente masculino en seguimiento por parte de Medicina Interna, por su pectus excavatum (PE), además presenta estrías cutáneas, signos de la muñeca y pulgar, escoliosis dorsal, proporción reducida del segmento superior/inferior, índice brazada/estatura aumentada y prolapso de válvula mitral, sumando un puntaje total de 8 en la escala de Ghent se confirma diagnóstico de SM. Conclusiones: el SM presenta una clínica heterogénea, cuyo diagnóstico se basa en los criterios de Ghent. Existe un alto riesgo de complicaciones aórticas, con alta tasa de mortalidad por lo que el diagnóstico temprano es fundamental.

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“…Aortic and pectus excavatum surgery in Marfan patients have been carried out with many surgical techniques and approaches [1]. The Ravitch and the Nussprocedures are the most common techniques for the treatment of pectus excavatum, they are not simply esthetical and both have advantages and disadvantages:…”

Section: Discussionmentioning

confidence: 99%

Concomitant Tirone David and Ravitch Procedure in Marfan Syndrome: A Case Report

Brega¹,

Braham²,

Pisani³

et al. 2019

OALib

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Pectus excavatum is a known manifestation of Marfan disease and, when coexisting with aortic diseases imposing surgical intervention, its treatment is performed in simultaneous surgery. We describe a 29-year old man affected by Marfan syndrome, who underwent a Tirone David intervention for Valsalva sinuses dilatation and in concomitance a Ravitch procedure was needed. The patient had already undergone an esthetical intervention for the thoracic deformity, but the preoperatory CT found a severe form of pectus, which imposed the aortic and thoracic correction in one single time. Surgery and postoperative outcome were satisfactory.

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Aortic surgery in Marfan patients with severe pectus excavatum

Abstract: Aortic surgery in Marfan patients with pectus excavatum was carried out according to a variety of strategies, surgical techniques and accesses with low complications rate and no mortality. Many of these were well tolerated with minimal complications and no mortality.

Cited by 13 publications

References 27 publications

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

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

Pectus excavatum como debut de síndrome de Marfan: reporte de caso

Concomitant Tirone David and Ravitch Procedure in Marfan Syndrome: A Case Report

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