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Ann Thorac Surg 2005;80:330-331
© 2005 The Society of Thoracic Surgeons


Case report

Ross Procedure and Ventricular Septal Defect Correction With Prolapsed Cusp

Roberto Rocha-e-Silva, MD, PhDa,*, Pablo M.A. Pomerantzeff, MD, PhDa, Robinson T. Munhoz, MD, PhDb, Domingos D. Lourenço Filho, MD, PhDa, Luiz F. Canêo, MD, PhDa, Sérgio A. de Oliveira, MD, PhDa

a Division of Surgery, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil
b Division of Cardiology, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil

Accepted for publication December 29, 2003.

* Address reprint requests to Dr Rocha-e-Silva, R. Leonor Pinheiro da Silva 133, Parque do Colégio, Jundiaí, CEP 13209130, São Paulo, Brazil (Email: rors{at}terra.com.br).


    Abstract
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We present the case of an asymptomatic 31-year-old man with perimembranous ventricular septal defect and aortic insufficiency due to a prolapsed right cusp. The ventricular septal defect was corrected with the rotation of the right cusp, and the Ross procedure was performed for the aortic insufficiency. The patient had an uneventful postoperative course. At immediate and first year follow-up examinations he presented with trivial aortic insufficiency and no residual ventricular septal defect. The patient remains asymptomatic with no medication.


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The ventricular septal defect (VSD) aortic insufficiency (AI) syndrome is congenital. AI does not usually appear until 2 to 5 years of age, but once present it tends to develop into the severe form within 10 years [1, 2]. AI without VSD may be treated by the Ross procedure with low mortality, encouraging intermediate results, and with the advantage of avoiding prosthetic valves requiring anticoagulation[3–8].

A 31-year-old man presented with an initial history of VSD with trivial asymptomatic AI. His AI began to progress by the age of 26. He was admitted to the service without symptoms and not on any medication. Physical examination evidenced a to and fro murmur. Chest roentgenogram demonstrated mild cardiomegaly. Transthoracic echocardiogram revealed a 5.5-mm perimembranous VSD (Fig 1) and AI due to a prolapsed right cusp. The left ventricular diastolic diameter was 6.3 cm, the left ventricular systolic diameter was 4.8 cm, and the left ventricular ejection fraction was 54%. Systolic dysfunction was minimal and mitral valve insufficiency was trivial.



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Fig 1. Transthoracic echocardiogram revealed a 5.5-mm perimembranous ventricular septal defect (CIV).

 
Ventricular septal defect correction and the Ross procedure were instituted. Access was made through a median sternotomy. Cardiopulmonary bypass was established at 28°C using a single large, two-stage venous cannula and venting of the left atrium. Tepid blood cardioplegia was performed every 20 minutes. After transecting the aorta, the VSD was visualized beneath the right cusp. Aortic insufficiency was due to a thickened and prolapsed right coronary cusp. Valvar prolapse is usually correctable through a conservative procedure in infancy, but as expected in an adult, it required replacement [9]. The right cusp was rotated toward the VSD and was attached to its borders with a continuous 5-0 Prolene suture (Ethicon, Somerville, NJ) (Fig 2). The other cusps were removed and the Ross procedure was performed. The mini aortic root was replaced with an autograft pulmonary valve cylinder, and its proximal root was secured with separate 3-0 Mersilene (Ethicon) stitches over a Teflon patch (Biomedical, Sao Paulo, Brazil) followed by a second line of continuous 5-0 Prolene suture (Ethicon). Coronary ostia were reinserted with continuous 7-0 Prolene suture (Ethicon). The distal root of the cylinder was connected to the aortic trunk with a continuous 6-0 Prolene suture (Ethicon). The right ventricular outflow was reestablished through an allograft pulmonary valve; the proximal and distal sutures were completed with 5-0 and 6-0 Prolene (Ethicon), respectively. The total ischemic and cardiopulmonary bypass times were 195 and 224 minutes, respectively.



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Fig 2. Diagram of surgical procedure. (A) Perimembranous ventricular septal defect (VSD) beneath a prolapsed right coronary cusp (RCC). (B) De-insertion and down-rotation of RCC over VSD. (C) Removal of excess tissue and closure of VSD with continuous Prolene suture (Ethicon, Somerville, NJ).

 
The patient had an uneventful postoperative course and was discharged from the hospital 10 days postoperatively. The immediate and 1 year follow-up transthoracic echocardiograms revealed a trivial AI, no residual VSD, a left ventricular diastolic diameter of 5.9 cm, a left ventricular systolic diameter of 4.5 cm, a left ventricular ejection fraction of 55%, and an unaltered trivial mitral valve insufficiency. The patient remains asymptomatic with no medication.


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The VSD–AI syndrome is not common and is usually corrected in the first years of life because of its symptomatic clinical course. This case remained asymptomatic into adult life, which is rare. Due to the enlarged ventricular cavity and a large VSD, surgery was indicated as an adult. Aortic insufficiency was not correctable through a conservative procedure, as expected in an adult [9]. To avoid the introduction of an artificial patch into the left ventricular outflow tract, we chose to rotate down the prolapsed aortic cusp over the VSD. This technique is simple as the cranial margin of the cusp is already in place, and in this case there was enough tissue to cover the VSD without tension. The Ross procedure was then performed by replacing the mini aortic root with an autograft pulmonary valve cylinder. The aortic occlusion time was 195 minutes, which is shorter than the average time of 215 ± 15 minutes as obtained from a previously described series of 44 adult patients submitted to the Ross procedure whose authors claim that "the complexity of the Ross procedure is balanced by its advantages of excellent hemodynamics, the potential for a permanent replacement for the aortic valve, and no need for anticoagulation" [8]. The immediate and 1 year follow-up echocardiograms confirmed good outcomes.

This case report highlights the fact that the VSD–AI syndrome can be successfully treated in adults without the use of artificial or heterologous patches through the rotation of the prolapsed aortic cusp over the VSD and the Ross procedure.


    References
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  1. Chung KJ, Manning JA. Ventricular septal defect associated with aortic insufficiencymedical and surgical management. Am Heart J 1974;87:435.[Medline]
  2. Dimich I, Steinfeld L, Litwak RS, Park S, Silvers N. Subpulmonic ventricular septal defect associated with aortic insufficiency Am J Cardiol 1973;32:325.[Medline]
  3. Costa FDA, Poffo R, Matte E, et al. A five-year experience with the Ross operationwhat have we learned?. Rev Bras Cir Cardiovasc 2000;15(2):109-128.
  4. Pinto Jr VC, Carvalho Jr W, Barroso HB, et al. Ross procedure in children Rev Bras Cir Cardiovasc 1999;14(2):114-120.
  5. Braun J, Hazekamp MG, Schoof PH, Ottenkamp J, Huysmans HA. Short-term follow up of the Ross operation in children J Heart Valve Dis 1998;7(6):615-619.[Medline]
  6. Marino BS, Wernovsky G, Rychik J, Bockoven JR, Godinez RI, Spray TL. Early results of the Ross procedure in simple and complex left heart disease Circulation 1999;100(Suppl 19):II-162-II-166.
  7. Joyce F, Tingleff J, Pettersson G. Expanding indications for the Ross operation J Heart Valve Dis 1995;4(4):352-363.[Medline]
  8. Fullerton DA, Fredericksen JW, Sundaresan RS, Horvath KA. The Ross procedure in adultsintermediate-term results. Ann Thorac Surg 2003;76:471-477.[Abstract/Free Full Text]
  9. Kirklin JW, Barratt-Boyes BG. Cardiac Surgery. 2nd ed.. New York, NY: Churchill Livingstone Inc; 1993. pp. 807-808.




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