Objective: Patients with complex congenital heart disease, characterized by right ventricle hypoplasia, had a palliative surgical option with one and a half ventricular repair. Methods: From July 2001 to March 2009, nine patients (mean age 5.2 years, range 3 to 9 years) with hypoplastic right ventricle, underwent correction with one and a half ventricle technique. Preoperative diagnoses included: pulmonary atresia with intact ventricular septum, in six and Ebstein's anomaly, in three cases. Six patients had bidirectional cavo-pulmonary shunt (Glenn operation) previously. The surgical approach was performed with cardiopulmonary bypass to correct intracardiac defects: atrial septal defect closure (nine cases); right ventricle outlet tract reconstruction with porcine pulmonary prosthesis (seven cases); tricuspid valvuloplasty (three cases). Results: There was one (11.1%) hospital death. All the patients left the hospital in good clinical conditions. One patient presented pulmonary stenosis at distal prosthesis anastomosis and needed surgical correction. There was one (12.5%) late deaths after reoperation. At mean follow-up of 39.8 months (range 16 months to 8.4 years) seven patients are alive in functional class I (NYHA). Conclusions: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic right ventricle by means of one and a half ventricle repair has the advantages of reducing the surgical risk of biventricular repair compared to the Fontan circulation; it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation with short and medium-term promising results. Longer follow-up is needed to prove the efficacy of such a repair in the long term.
Objetivo: Pacientes com cardiopatia congênita complexa e caracterizada por hipoplasia do ventrículo direito têm uma opção de correção paliativa com a operação de um ventrículo e meio. Métodos: De julho de 2001 a março de 2009, nove pacientes (idade média de 5,2 anos, variando de 3 a 9 anos), com hipoplasia do ventrículo direito, foram submetidos à correção com a técnica de um ventrículo e meio. Os diagnósticos pré-operatórios foram: atresia pulmonar com septo ventricular intacto, em seis casos, e anomalia de Ebstein, em três casos. Seis pacientes tiveram derivação cavo-pulmonar bidirecional (operação de Glenn) prévia. A abordagem cirúrgica foi realizada com circulação extracorpórea e os defeitos intracardíacos foram corrigidos: fechamento do defeito do septo atrial (nove casos), reconstrução da via de saída do ventrículo direito com prótese pulmonar porcina (sete casos); plástica da valva tricúspide (três casos). Resultados: Houve um (11,1%) óbito. Todos os pacientes receberam alta hospitalar em boas condições clínicas. Um paciente apresentou estenose pulmonar distal à prótese, sendo necessária a reintervenção cirúrgica. Houve um (12,5%) óbito tardio, após a reoperação. Sete (77,8%) pacientes foram seguidos por período médio de 39,8 meses (intervalo de 16 meses para 8,4 anos) e se encontram em boas condições clínicas. Conclusões: O tratamento cirúrgico de cardiopatias congênita, na presença de um ventrículo direito hipoplásico, por meio da operação de um ventrículo e meio, tem menor risco cirúrgico que a correção biventricular, mantendo o átrio direito com baixa pressão, fluxo sanguíneo pulmonar pulsátil e melhora da saturação sistêmica de oxigênio. Os resultados de curto e médio prazo são satisfatórios; porém será necessário um maior tempo de seguimento para provar a eficácia desta abordagem cirúrgica a longo prazo.
The techniques of mitral valve repair are procedures of choice in the surgical treatment of mitral valve disease, and are increasingly used in the surgical management of valvular diseases and preferable to valve replacement when technically feasible.
Knowledge of three-dimensional mitral valve anatomy and its work during the cardiac cycle, associated to the understanding of the mechanisms contributing to the valve dysfunction is critical to the success of the conservative mitral valve surgery.
Until now we could not find uniform results in the literature of mitral valve repair in patients with lesions resulting from rheumatic fever [1-3]. The explanation could be related to the interference of new rheumatic outbreaks in these patients. However, there is agreement on the lowest morbidity and mortality of patients undergoing mitral valve repair compared with valve replacement.
This study aims to analyze the 20-year late results of mitral valve repair in rheumatic patients, using various repair techniques.
During the period from January 1985 to December 2005, 330 patients with rheumatic fever diagnosed with mitral failure underwent mitral valve repair at the Heart Institute of the Clinics Hospital of the Faculty of Medicine, University of São Paulo. The study was approved by the Scientific Committee of the Heart Institute and the Ethics Committee of the Clinics Hospital of the Faculty of Medicine, University of São Paulo.
The patients' ages ranged from 5 to 57 years, mean 26.9 ± 15.4 years. One hundred and ninety (57.6%) patients were female and 140 (42.4%) were male.
The techniques of mitral valve repair were: posterior annuloplasty using bovine pericardial patch in 160 (48.4%), Carpentier ring in 75 (22.6%), posterior segmental annuloplasty in 32 (9.7%), quadrangular resection of the posterior leaflet with plication of the ring in 23 (6.9%), posterior Shore annuloplasty in 12 (3.6%), triangular resection of the anterior leaflet in eight (2.4%), Kay in five (1.5%), enlargement of the posterior leaflet using bovine pericardium in four (1.2%), double-U as opposed in four (1.2%), Reed in three (0.9%), cleft suturing in two (0.6%) and Puig-Massana ring in one (0.3%). Associated techniques were used in 182 (55.2%) patients, and the most frequent was cords shortening in 66 (20%) cases. Other techniques used were: multiple papillectomy in 59 (17.8%) cases, commissurotomy in 30 (9.1%), smoothing of the posterior leflet in eight (2.4%), resection of short strings in three (0, 9%), cusp repair in three (0.9%), papillary muscle sliding in two (0.6%) and transposition of chords in one (0.3%).
Associated operations were performed in 219 (66.4%) patients, 90 (27.2%) aortic valve replacements, 88 (26.7%) tricuspid valve repair, 23 (7%) aortic valve repair, 10 (3%) aortic commissurotomies, six (1.8%) CABG and two (0.6%) tricuspid valve replacements.
Regarding the functional class (New York Heart Association), 130 (39.5%) patients were in functional class IV, 179 (54.3%) in functional class III and 21 (6.2%) in functional class II.
The postoperative follow-up was performed through hospital consultations or telephone interviews or using questionnaires sent by mail.
Data will be presented in accordance with the revised standards for data reporting and nomenclature. The survival actuarial and free from events were calculated using the Kaplan Meier  and compared by linear regression analysis. The linearized rates of events are expressed as a percentage of events per patient/year (%/pat.-year).
The hospital mortality was 0.9% (three patients). The causes were low cardiac output in two (0.6%) patients - one was a child who was under rheumatic activity - and multiple organ failure in one (0.3%) patient - with also a child under rheumatic activity.
In the late postoperative period, 177 (63%) patients were in functional class I, 51 (18.1%) were class II, 37 (13.2%) were in functional class III and 16 (5.7%) patients in functional class IV. The period of follow-up was 17,486 months/patients (88.3% full).
Late mortality in this population was 2.8% (nine patients). Five patients died due to heart failure, two, sepsis and the other two the cause of death was unknown.
The linearized rates of the events reoperation, death, endocarditis, and thromboembolism are shown in Table 1. There was no hemolysis in this series of patients.
The actuarial survival in 20 years was 86.4 ± 6.6% (Figure 1). The actuarial curves free from thromboembolism (Figure 2), reoperation (Figure 3) and endocarditis (Figure 4) were 99.7 ± 0.3%, 30.3 ± 11.1% and 95.6 ± 4.3% in 20 years, respectively.
Fig. 1 - Actuarial survival at 20 years
Fig. 2 - Actuarial curve free from thromboembolism
Fig. 3 - Actuarial curve free from reoperation
Fig. 4 - Actuarial curve free from endocarditis
A degenerative disease of the mitral valve is the most common cause of mitral failure in industrialized countries. In such cases, the mitral valve repair is the procedure of choice, with excellent late results [5,6]. However, in our country, rheumatic etiology is prevalent and, in this population, the long-term outcomes may be affected by the appearance of new outbreaks of the disease . Despite this fact, every effort must be made in order to perform conservative treatment, since a large proportion of these patients are young and therefore subject to greater number of surgical interventions during evolution [8,9]. Other factors to be considered are the difficulty of anticoagulation of patients in our country and early calcification of bioprostheses in this age group.
Mitral valve repair in rheumatic patients have shown better outcomes than mitral valve replacement . On the other hand, conservative surgery of the mitral valve in rheumatic patients is technically more difficult than in patients with degenerative valvular heart disease  and its outcomes depend on the degree of impairment of the valve at the time of surgical approach, that is, the worse the conditions at the time of reconstruction, the worse the outcome [12,13].
The valve repair requires the surgeon a perfect knowledge of anatomy and the multiplicity of existing techniques. Moreover, the evaluation of the leaflets, the subvalvular plane, the tendinae cords and papillary muscles should be performed in a systematic manner during surgery. The test with saline solution complements this analysis. Transesophageal Doppler echocardiography has contributed to the evaluation and intraoperative decision.
The choice of technique to be used for annuloplasty should take into account the valve change. Usually, the dilation of the mitral orifice affects only the posterior two thirds of the annulus, which corresponds to the area of the posterior leaflet. Several rings have been described, rigid or flexible, to remodel the mitral valve [2,14]. Loop et al.  prefer the use of the Carpentier ring to reduce the annulus. Although widely used, the rigid rings may have some problems, such as systolic anterior motion in 4% to 10% of cases, causing obstruction of the outflow tract of the left ventricle . In our Service, we have used this technique preferably in the large dilated left ventricle and, in other situations where it is necessary to reduce the annulus, we used a bovine pericardium patch from trigone to trigone, with good late outcomes . A bovine pericardium patch is flexible and does not interfere with the normal anatomy of the mitral annulus, which we now know is not flat and is changed during the different phases of the cardiac cycle.
However, other authors have used grafts from autologous tissue, such as the posterior leaflet of the tricuspid valve  and pericardium , in mitral valve repair in rheumatic patients, on which the shrinkage and low mobility are the primary findings.
The late results of mitral valve repair also depend on a good coaptation of the leaflets, that is, an area of adequate coaptation after valve repair. This good coaptation can be achieved with the help of the shortening of the chordae tendinae  or shortening of papillary muscles. Recently, in children, we have increased the use of sliding of both papillary muscles in order to proceed to the shortening of all the cords of the anterior leaflet.
The hospital mortality was 0.9%, a low value when compared to international literature, which ranges from 2.6% to 3.6% [2,21]. In our country, Petrucci Jr et al.  reached a mortality rate of 0% in a small group of 23 rheumatic patients undergoing mitral valve repair. The late mortality of 3.2% with linear rate of 0.5% patient/years was also lower than that found in the literature [11,21].
The linearized rate of reoperation found of 3.5% patients-years was similar to the literature  and higher when compared to patients with degenerative etiology . A higher linearized rate of reoperation is expected, since the rheumatic etiology is a risk factor for reoperation due to outbreaks of rheumatic fever, which continually provoke structural changes in the mitral valve apparatus .
The rheumatic patients undergoing mitral valve repair showed low rates of complications (thromboembolism, endocarditis, and hemolysis) in the long-term. Such outcomes are similar to those found in the literature [11,25].
In conclusion, mitral valve repair is feasible in the correction of mitral failure of rheumatic etiology, with low operative mortality.
1. Billingsley AM, Laks H, Boyce SW, George B, Santulli T, Williams RG. Definitive repair in patients with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg. 1989;97(5):746-54. [MedLine]
2. Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg. 1993;105(3):406-23.
3. Carpentier A, Chauvaud S, Macé L, Relland J, Mihaileanu S, Marino JP, et al. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1988;96(1):92-101. [MedLine]
4. Rowlatt JF, Rimoldi JHA, Lev M. The quantitative anatomy of the normal child's heart. Pediatr Clin North Am. 1963;10:499. [MedLine]
5. Hanley FL. The one and a half ventricle repair: we can do it, but should we do it? J Thorac Cardiovasc Surg. 1999;117(4):659-61. [MedLine]
6. Corno AF, Chassot PG, Payot M, Sekarski N, Tozzi P, Von Segesser LK. Ebstein's anomaly: one and a half ventricular repair. Swiss Med Wkly. 2002;132(33-34):485-8. [MedLine]
7. Stellin G, Vida VL, Milanesi O, Rubino M, Padalino MA, Secchieri S, et al. Surgical treatment of complex cardiac anomalies: the 'one and one half ventricle repair'. Eur J Cardiothorac Surg. 2002;22(3):431-6.
8. de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg. 1988;96(5):682-95. [MedLine]
9. Maluf MA, Andrade JCS, Catani R, Carvalho AC, Negrini N, Buffolo E. Avaliação dos resultados tardios da operação de derivação cavo-pulmonar bidirecional, no tratamento paliativo de cardiopatias congênitas com câmara ventricular única. Rev Bras Cir Cardiovasc. 1994;9(2):95-103. View article
10. Fantini FA, Gontijo Filho B, Martins C, Lopes RM, Heiden E, Vrandecic E, et al. Cirurgia de Glenn bidirecional: importância da manutenção de fluxo "pulsátil" na artéria pulmonar. Rev Bras Cir Cardiovasc. 1995;10(1):25-33. View article
11. Pinto WC, Carvalho W, Barroso HB, Mejia JAC, Mesquita FA, Sardenberg CR, et al. Anastomose cavo-pulmonar total sem uso de material protético. Rev Bras Cir Cardiovasc. 1998;13(3):67-72. View article
12. Gontijo Filho B, Fantini FA, Lopes RM, Martins C, Castro MF, Drumond LF, et al. Resultados a médio prazo da anastomose de Glenn bidirecional. Rev Bras Cir Cardiovasc. 1999;14(1):39-45. View article
13. Westphal FL, Maluf MA, Silva CMC, Carvalho AC, Buffolo E. Estudo angiográfico comparativo da artéria pulmonar no pré e pós-operatório de pacientes submetidos a operação de derivação cavopulmonar bidirecional. Rev Bras Cir Cardiovasc. 2001;16(4):305-20. View article
14. Fonseca L, Silva JP, Franchi SM, Castro RM, Comparato DO, Baumgratz JF. Operação de Glenn bidirecional no tratamento estagiado da síndrome de hipoplasia do coração esquerdo: resultados imediatos e tardios. Rev Bras Cir Cardiovasc. 2005;20(1):34-7. View article
15. Fantini FA, Gontijo B, Martins C, Lopes RM, Vrandecic EC, Goulart E, et al. Operação de Fontan: uma técnica em evolução. Rev Bras Cir Cardiovasc. 2009;24(4):154-7. View article
16. Kreutzer C, Mayorquim RC, Kreutzer GO, Conejeros W, Roman MI, Vasquez H, et al. Experience with one and a half ventricle repair. J Thorac Cardiovasc Surg. 1999;117(4):662-8. [MedLine]
17. Uemura H, Yagihara T,Adachi I, Kagisaki K, Shikata F. Conversion to total cavopulmonary connection after failed one and one-half ventricular repair.Ann Thorac Surg. 2007;84(2):666-8. [MedLine]
18. Miyaji K, Shimada M, Sekiguchi A, Ishizawa A, Isoda T, Tsunemoto M. Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair". Ann Thorac Surg. 1995;60(6):1762-4. [MedLine]
Article receive on Friday, August 20, 2010