Mitral valve replacement with a pulmonary autograft: long-term follow-up in an infant.

A 7-month-old boy with a complete atrioventricular septal defect presented with severe left atrioventricular valve regurgitation 4 months after complete repair. As the valve was unsuitable for the repair and the annulus was too small to accommodate a mechanical prosthesis, the modified mitral Ross operation was performed. The long-term outcome was uneventful for 12 years. The mitral Ross procedure is an old-described technique in which classically the pulmonary autograft is encased in a prosthetic conduit preventing any growth potential. On the contrary, the modified technique used in this case shows that the long-term function can be obtained. This procedure may be a valuable option when mitral valve replacement is necessary in infants.

Hybrid access to atria via the Guiraudon Universal Cardiac Introducer for arrhythmia ablation after total cavopulmonary derivation.

We report the first use of a new platform, the Guiraudon Universal Cardiac Introducer (GUCI), in humans for accessing the left atrium for catheter-based ablations in patients with resistant atrial arrhythmias after total cavopulmonary derivation. The GUCI was originally designed for intracardiac access for closed, beating instrumental intracardiac surgery.The patient was a 29-year-old man with problematic atrial arrhythmias resistant to antiarrhythmic drugs because of severe uncontrolled bradycardia and because his pacemaker was explanted for infection.The GUCI was attached to the left atrial appendage via an anterior left thoracotomy. The GUCI was modified to accommodate introduction and manipulation of multiple catheters. This allowed electrophysiologists to perform catheter-based exploration and ablation. A DDD pacemaker was implanted, with an atrial endocardial lead introduced via the GUCI cuff and a ventricular epicardial lead.Postoperative atrial arrhythmias were controlled using amiodarone and atrial pacing. At the 12-month follow-up, the patient was arrhythmia- and drug-free and returned to full employment.This new access offers an additional new alternative atrial access to treat resistant arrhythmia after total cavopulmonary derivation. The current state-of-the-art makes patient selection difficult and uncomfortable for the surgeons because of incomplete preoperative electrophysiological data, such as a return to the beginning of surgery for arrhythmia; however, more cumulative experience with intraoperative electrophysiological data and new mapping technologies should address these limitations.

Long-term results of the REV (réparation à l'ètage ventriculaire) operation.

OBJECTIVE: Despite its innovative features, the réparation à l'ètage ventriculaire (REV) procedure has not gained large popularity in the treatment of transposition of the great arteries, ventricular septal defect, pulmonary stenosis, and related anomalies, and thus the Rastelli operation remains the preferred type of repair. We try to obviate the alleged lack of long-term results that has been suggested to explain this reluctance to change. METHODS: We reviewed a series of 205 patients who underwent the REV procedure between 1980 and 2003. RESULTS: Hospital mortality was 12% (24 patients). Ten of 181 early survivors residing in distant countries could not be traced for follow-up. There were 13 late deaths (2 of noncardiac causes). Overall survival and freedom from any reoperation at 25 years were 85% and 45%, respectively, as determined by using the Kaplan-Meier method. Reoperation was commonly required because of recurrent right ventricular outflow tract obstruction (36 patients) and rarely by left ventricular outflow tract stenosis (3 patients). By using cumulative method analysis, at the 25-year follow-up interval, the probability of being alive without reoperation was 45%, that of reoperation for right ventricular outflow tract obstruction was 33%, and that of reoperation for left ventricular outflow tract obstruction was 5%. Clinical status is excellent: 137 patients are in New York Heart Association class I (87%), and 131 patients are free of arrhythmias. CONCLUSIONS: These results represent a considerable improvement over those of the Rastelli operation in terms of survival and need for reoperation for right or left ventricular outflow tract obstruction.

Surgery for malposition of the great arteries: the REV procedure.

The REV procedure was introduced in 1980 to treat transposition of the great arteries with ventricular septal defect (VSD) and pulmonary stenosis and malpositions similar to transposition of the great arteries (TGA). It aims at overcoming the drawbacks and limitations of the classic Rastelli operation, such as subaortic stenosis, late ventricular deterioration, arrhythmias and sudden death. In particular, the resection of the infundibular septum allows for the placement of a straighter, smaller ventricular patch, bulging much less in the right ventricular cavity. The extensive mobilization of the main pulmonary branches permits a direct connection with the right ventricular incision, thus avoiding the need for an extracardiac conduit. The procedure was performed in 205 patients as of December 2003 with 12% hospital mortality. Patients for whom the Rastelli operation would have been contraindicated, were accepted for REV repair. Late results show a clear improvement over those reported with the Rastelli operation in terms of overall survival (85% at 25-year follow-up interval) and prevalence of reoperation for right ventricular obstruction. Obstruction of the left ventricle-to-aorta tunnel is exceedingly rare. This operation should be considered the gold standard when new surgical options are considered for this complex form of transpositions/malpositions of the great arteries.

Mitral valvar anomalies and discrete subaortic stenosis.

On the basis of our clinical experience, we hypothesized that the role of mitral valvar anomalies in the development and recurrence of discrete subaortic stenosis might be underestimated. From January 1994 to October 2000, the anatomy of the mitral valve and its relationship to the other components of the left ventricular outflow tract were studied by echocardiography in a series of 73 consecutive patients referred to our institution for surgical correction of discrete subaortic stenosis. In all patients for whom it was considered advisable, surgical correction of the mitral anomaly was performed, together with resection of the fibro-muscular subaortic stenosis. One or more mitral valvar anomalies were found in 35 patients (48%). They could be grouped into five categories: insertion of a papillary muscle into the aortic leaflet, insertion of a papillary muscle into the ventricular wall, "muscularization" of the subaortic portion of the aortic leaflet, anomalous insertion of the valvar tissue into the ventricular wall, and accessory valvar tissue. In all cases with anomalous mitral valvar anatomy, surgical correction was feasible. It consisted of transection of the anomalous papillary muscle or its attachment, resection of accessory valvar tissue, and/or patch enlargement of the aortic leaflet. The incidence of mitral valvar anomalies associated with subaortic stenosis is probably underestimated. Our data suggest that they should be systematically searched for during the evaluation of all cases of subaortic stenosis. Their surgical correction is generally feasible, and might improve the mid and long term results.

Estratégia para o tratamento cirúrgico das anomalias da conexäo ventrículo-arterial com comunicaçäo interventricular / Strategy for the surgical treatment of the anomalies of ventriculo-arterial connections with intraventricular septal defects

Designamos anomalias da conexao ventrículo-arterial toda a conexao que difere daquela de um coraçao normal. Dentro desta abordagem, nao utilizamos os termos ventrículo direito ou esquerdo com dupla via de saída, Taussig-Bing e transposiçao das grandes artérias, quando estes estao associados a uma comunicaçao interventricular. Nestas anomalias, o objetivo da correçao é o de conectar o ventrículo esquerdo com a aorta e o ventrículo direito com a artéria pulmonar. A estratégia que escolhemos é baseada na hipótese de que a correçao mais simples é aquela que nao exige a utilizaçao de um tubo protético, a transferência de coronárias, ou a septaçao complexa da cavidade ventricular. Baseados na experiência de 162 correçoes para as anomalias da conexao ventrículo-arterial, em uma série de 197 pacientes, utilizamos três tipos de correçao anatômica: a correçao intraventricular (tunelizaçao ventrículo esquerdo-aorta) em 35 pacientes, o REV (tunelizaçao ventrículo esquerdo-aorta associada a translocaçao do tronco pulmonar sobre o ventrículo direito) em 78 pacientes, e a operaçao de Jatene associada ao fechamento da comunicaçao interventricular em 49 pacientes. O tipo de correçao ideal é a correçao intraventricular, na qual a simples tunelizaçao ventrículo esquerdo-aorta estabelece uma conexao ventrículo-arterial normal. Ouando a correçao intraventricular nao é possível, nós indicamos o REV em presença de estenose pulmonar e a operaçao de Jatene na ausência desta. A questao principal é saber quando uma correçao intraventricular é realizável. A realizaçao desta é funçao da distância entre a valva tricúspide e a valva pulmonar. Se esta distância é suficientemente grande (igual ou superior ao diâmetro do orifício aórtico), o túnel intraventricular é realizável; se nao, outra modalidade de correçao é indicada. Nossa experiência atual sugere que a exploraçao pré-operatória das distâncias entre a valva tricúside e as válvulas semilunares é um critério essencial para a escolha da correçao apropriada para as anomalias da conexao ventrículo-arterial associadas a uma comunicaçao interventricular. Esta estratégia nao se opoe às outras classificaçoes usuais, baseadas na posiçao das grandes artérias, ou na situaçao da comunicaçao interventricular, e ela nos fornece informaçoes precisas quanto à possibilidade de realizar uma correçao intraventricular.