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Ann Thorac Surg 2005;79:2150-2151
© 2005 The Society of Thoracic Surgeons

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Case report

Pulmonary Autograft for Mitral Valve Replacement in Infants: The Ross-Kabbani Operation

^a Centro Cardiovascolare Malan, Istituto Policlinico San Donato, San Donato Milanese, Italy
^b Divisione Cardiologia Pediatrica, II Università di Napoli, Napoli, Italy
^c Istituto di Clinica Pediatrica, Universita’ di Parma, Parma, Italy
^d Dipartimento Area Critica Medico Chirurgica, Università degli Studi di Firenze, Firenze, Italy

Accepted for publication November 25, 2003.

^_* Address reprint requests to Dr Frigiola, Centro Cardiovascolare Malan, Istituto Policlinico San Donato, San Donato Milanese, Italy (E-mail: alfrigio{at}tin.it).

	Abstract

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We describe the cases of 2 infants aged 6 and 2 months, respectively, affected by mitral valve dysplasia that caused severe valvular insufficiency and heart failure in which a surgical correction was indicated because of critical general conditions. In both patients the anatomic characteristics of the valves were not suitable for repair, and an implant of a mechanical prosthesis was excluded because of the very young age of the infants and the impossibility of maintaining an adequate anticoagulant therapy. Therefore a Ross-Kabbani intervention was performed with an implant of a pulmonary autograft (in the mitral position) and an insertion of a pulmonary homograft. The postoperative course was free of major complications and good function of the autograft was present at short-term follow-up in both cases.

	Introduction

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Mitral valve dysplasia at a young age is a severe anomaly with difficult surgical options. Mitral valve repair is not always feasible and classic mitral valve prosthesis is potentially avoided, because of the young age and difficulties of maintaining an adequate anticoagulant therapy. The Ross-Kabbani operation with a pulmonary autograft has been used for the mitral valve replacement as reported in only a few patients [1] and very rarely in infants [2].

	Case Reports

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Patient 1
A 6-month-old male infant (weight, 4.3 kg) was referred to our center for a second opinion. At 1-month-old, he was hospitalized for the first time for congestive heart failure. Echocardiogram showed severe mitral regurgitation and digoxin and diuretics were started. Since then he required several rehospitalizations for heart failure.

When the baby arrived at our center, he was found to have hepatosplenomegaly without peripheral edema. The echocardiogram showed a dysplastic mitral valve with abnormal chordae apparatus and severe mitral regurgitation with moderate to severe stenosis. Surgery was considered to be a unique therapeutic option because of a severe clinical picture of the infant despite full medications.

At surgery, neither valvular repair nor prosthesis were considered technically feasible and therefore the Ross-Kabbani operation was performed.

In the postoperative course, the infant needed a blood transfusion and intravenous inotropic agents for low cardiac output, and a transient elevation of alanin-amino-transferase was observed. He was discharged on the postoperative day 14 in satisfactory clinical condition. The pre-discharge echocardiogram showed a good contractility and normal functioning of the autograft in the mitral position (with only a mild to moderate regurgitation) and also normal functioning of the pulmonary homograft (with mild regurgitation).

At 2 months follow-up the infant was in good clinical and hemodynamic condition.

Patient 2
A 2-month-old male infant (weight, 3.3 kg) was referred to us for an opinion. He was born at 34 weeks gestation by caesarian section from a twin monochorionic di-amniotic pregnancy complicated during the second trimester by twin-to-twin transfusion syndrome that required a decompressive amniocenteses at 21 weeks gestation. His birth weight was 1,580 g.

At 1 month of age, an echocardiogram revealed a dysplastic mitral valve with thickened leaflets and short papillary muscles with mild to moderate mitral regurgitation, without a need for medications at that time. No relevant cardiac problems were present in co-twin.

One month later the infant presented with polypnea, hepatomegaly, and contraction of the diuresis. The new echocardiogram showed severe mitral regurgitation (see Fig 1). Treatment with furosemide and captopril was started with only a partial improvement of clinical conditions. After arriving at our center, we confirmed the clinical and echocardiographic findings, and because the medical treatment did not improve his clinical conditions, we decided to submit the infant (aged 80 days) to surgical correction.

View larger version (157K): [in this window] [in a new window]   Fig 1. Preoperative echocardiogram of patient 2 in apical 4-chamber view. Severe mitral regurgitation in color Doppler with the regurgitant jet reaching the top of the left atrium is well evident.

In the postoperative course, nitrous oxide and inotropic agents were used for 4 and 7 days, respectively. At 2 months follow-up the infant was clinically well with a mild gradient of the autograft in the mitral position.

Surgical Technique
The heart was approached through a median sternotomy and normothermic cardiopulmonary bypass was established. The pulmonary trunk was isolated to the beating heart. Distally the trunk was sectioned just proximal to its bifurcation, and at proximal level it was cut "under" the valvular cusps, leaving a rim of infundibular muscle of about 3 mm. The right ventricular outflow tract was reconstructed with a homograft. Next, the mitral substitute was constructed by encasing the autologous pulmonary valve inside an armed Gore-Tex prosthesis (IMPRA, C. R. Bard, Murray Hill, NJ) (Fig 2). The pulmonary valve was attached to the tube with proximal and distal running 7–0 Prolene sutures (Ethicon, Somerville, NJ). The circumference of the prosthesis was interrupted by performing one longitudinal incision in patient 1 and two longitudinal incisions in the patient 2. The incisions were performed through the whole height of the prosthesis, thus obtaining a cleft about 1 to 2 mm in width; in this way we hope to ensure growth of the encased autograft with the growth of the child. The size of the prosthesis was chosen according to the size of the pulmonary autograft (ie, 15 mm in the patient 1 and 14 mm in patient 2).

View larger version (147K): [in this window] [in a new window]   Fig 2. Top view of the Gore-Tex prosthesis with the sutured encased autograft.

	Comment

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In conclusion, the Ross-Kabbani operation [3] was a unique solution for these 2 infants, because the surgical treatment was mandatory and neither a mitral valve repair nor a mitral valve replacement with a prosthetic valve were technically feasible for the small dimension of the left atrium. In addition, the young age of these infants did not allow an adequate anticoagulant therapy.

The technique we used on our infant patients is relatively new and complex. We believe that we can improve the technique with experience in time. However, the late efficacy of this approach for small infants remains uncertain and long-term follow-up is necessary [4].

	References

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1. Ross DN. Replacement of aortic and mitral valves with a pulmonary autograft Lancet 1967;2(523):956-958.[Medline]
2. Sarraf A, Bruniaux J, Planche C. Ross mitral procedure for massive congenital mitral insufficiency Arch Mal Coeur Vaiss 2001;94(5):509-512.[Medline]
3. Kabbani SS, Jamil H, Hammoud A. Technique for replacing the mitral valve with a pulmonary autograftthe Ross-Kabbani operation. Ann Thorac Surg 2001;72:947-950.[Abstract/Free Full Text]
4. Kabbani SS, Jamil H, Hammoud A, et al. Use of the pulmonary autograft for mitral replacementshort- and medium-term experience. Eur J Cardiothorac Surg 2001;20:257-261.[Abstract/Free Full Text]

This article has been cited by other articles:

				S. S. Kabbani, N. A. Sabbagh, A. Y. Kudsi, F. Nabhani, and H. Jamil Update on the mitral pulmonary autograft Asian Cardiovasc Thorac Ann, June 1, 2011; 19(3-4): 253 - 259. [Abstract] [Full Text] [PDF]

				S. Kabbani, H. Jamil, F. Nabhani, A. Hamoud, K. Katan, N. Sabbagh, A. Koudsi, L. Kabbani, and G. Hamed Analysis of 92 mitral pulmonary autograft replacement (Ross II) operations. J. Thorac. Cardiovasc. Surg., October 1, 2007; 134(4): 902 - 908.e7. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Alessandro Frigiola Marisa Di Donato Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Frigiola, A. Articles by Di Donato, M. Search for Related Content PubMed PubMed Citation Articles by Frigiola, A. Articles by Di Donato, M. Related Collections Congenital - acyanotic