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Ann Thorac Surg 2003;75:1464-1468
© 2003 The Society of Thoracic Surgeons

Original article: cardiovascular

Right ventricular cardiomyoplasty: 10-year follow-up

Juan C Chachques, MD, PhD*,a, Pantelis G Argyriadis, MDa, Guy Fontaine, MD, PhDa, Jean-Louis Hebert, MDa, Robert A Frank, MDa, Nicola D’Attellis, MDa, Jean-N.oël Fabiani, MDa, Alain F Carpentier, MD, PhDa

a Department of Cardiovascular Surgery, Pompidou and Broussais Hospitals, Paris, France

Accepted for publication November 12, 2002.

Keywords 27

* Address reprint requests to Dr Chachques, Hôpital Européen Georges Pompidou, 20 Rue Leblanc, 75015 Paris, France (Email: j.chachques{at}brs.ap-hop-paris.fr).


   Abstract
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 Abstract
Introduction
 Material and methods
 Results
 Comment
 References
 
Background: Chronically depressed right ventricular (RV) function presents an unsolved therapeutic challenge in cardiac surgery. Despite recent advances in medical and surgical therapies, prognosis remains poor and patient’s quality of life and mortality are frequently unacceptable. The aim of this study is to present the first clinical report and long-term results of RV dynamic cardiomyoplasty applied in patients with RV failure caused by isolated RV cardiomyopathies.

Methods: Seven consecutive patients (5 males, 2 females; mean age, 40 ± 9 years; range, 15 to 63 years) from a series of 113 cardiomyoplasty procedures performed at Broussais and Pompidou Hospitals were evaluated. The mean duration of follow-up was 10 ± 3.5 years. All patients had predominant RV dysfunction, associated with tricuspid regurgitation in 6 patients. The cause of RV failure was arrhythmogenic cardiomyopathy (4 patients), ischemic (2 patients), and Uhl’s disease (1 patient), and endomyocardial fibrosis (1 patient). Six patients were in preoperative New York Heart Association functional class III and 1 was in intermittent class III/IV. The mean preoperative ejection fraction (measured by isotopic technique) was 18% ± 5.7% for the right ventricle and 40% ± 13% for the left ventricle. Right ventricular dynamic cardiomyoplasty consists of wrapping the RV free walls with the left latissimus dorsi muscle flap. The distal part of the latissimus dorsi muscle is fixed to the diaphragm and then electrostimulated. Six patients required associated tricuspid valve surgery.

Results: There were no perioperative deaths. The mean duration of follow-up was 10 ± 3.5 years. Six patients are alive with a remarkable quality of life, 4 are in New York Heart Association functional class I and 2 are in class II. One patient who was in New York Heart Association functional class II died in postoperative year 7 caused by stroke. At last follow-up, mean RV ejection fraction was 33% ± 11.8% and left ventricular ejection fraction was 52% ± 12.6%.

Conclusions: The results of this long-term study demonstrate hemodynamic and functional improvements after RV cardiomyoplasty without perioperative mortality, no long-term malignant arrhythmias, and RV dysfunction related deaths. We believe that RV cardiomyoplasty, associated with tricuspid valve surgery when required, could be an effective treatment for severe RV failure.


   Introduction
Top
 Abstract
 Introduction
Material and methods
 Results
 Comment
 References
 
Right ventricular (RV) failure, generally associated with tricuspid valve regurgitation, is a rare disease. There are only a few publications concerning surgical therapy of isolated RV failure, refractory to medical therapy [1–4]. The right ventricle may fail due to primary diseases or may be delayed after left ventricular (LV) failure. Congenital anomalies, arrhythmogenic RV dysplasia, myocarditis, ischemia, and cardiomyopathy are the most frequent causes of primary RV failure.

Based on acquired knowledge on skeletal muscle biology and chronic functional electrostimulation, we applied modifications to the conventional LV cardiomyoplasty procedure [5] by also using the left latissimus dorsi muscle (LDM), to perform a specific RV cardiomyoplasty. Worldwide clinical experience with LV dynamic cardiomyoplasty (CMP) includes more than 1,500 patients (188 of these operations were performed by our group).

The goal of this study is to present our 10-year clinical experience concerning the RV CMP procedure, used alone or associated with tricuspid valve repair or replacement, for right ventricular failure.


   Material and methods
Top
 Abstract
 Introduction
 Material and methods
Results
 Comment
 References
 
Patient population
Among 188 patients who underwent CMP by our team (113 operations at Broussais and Pompidou Hospitals and 75 operations by our team abroad), 7 consecutive patients underwent CMP for RV failure at our institution. This retrospective study includes 5 males and 2 females with a mean age of 40 ± 9 years (range, 15 to 63 years). Cause of RV failure was arrhythmogenic right ventricular cardiomyopathy (4 patients), ischemic (2 patients), and Uhl’s disease (1 patient). Six patients also had tricuspid valve regurgitation. Two patients had atrial fibrillation and 1 patient had atrioventricular (A-V) block.

Hepatic cirrhosis caused by right heart failure and chronic anemia was also present in 1 patient. All patients had advanced heart failure, with 6 patients in New York Heart Association functional class III and 1 in intermittent class III/IV (Table 1). Mean pulmonary artery wedge pressure was 28.7 ± 8 mm Hg. The mean preoperative isotopic RV ejection fraction was 18% ± 5.7%, and the mean LV ejection fraction was 40% ± 13%.


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  		Table 1 Preoperative Patient Characteristics
 
Surgical technique for RV CMP
The left LDM is used to perform an RV CMP because the muscle mass can be placed anteriorly to the RV free wall and the direction of muscular fibers are orientated perpendicularly to the long-axis of the RV. In this manner the entire RV cavity can be better compressed during systole. After LDM dissection, implantation of pacing leads and transposition into the chest, the LDM flap is placed onto the anterior, lateral, and diaphragmatic RV free walls. Afterward its distal end is secured to the pericardial sac as far posteriorly as possible at the junction between the diaphragmatic and the posterior parts of the pericardium with interrupted 4-0 Ethibond sutures (Ethicon, Inc, Somerville, NJ). In order to synchronize LDM and heart contractions, two sensing epicardial leads are inserted in the LV wall. These leads should not be placed in the RV because of diseased myocardium. During the electrically induced contraction of the LDM, the dilated RV is compressed between the LDM and the diaphragm, obtaining hemodynamic effects comparable with external heart massage (Fig 1). At the end of surgery, a specific myostimulator is placed in a subcutaneous epigastric pocket. The Transform pulse generator (Medtronic Inc, Minneapolis, MN) was used in 6 patients and the Myos pulse generator (Biotronik, Berlin, Germany) in 1 patient.


Figure 1
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  		Fig 1. Surgical technique for right ventricular (RV) cardiomyoplasty. The latissimus dorsi muscle (LDM) flap is positioned anteriorly to the RV and fixed distally to the diaphragm.

 
Postoperative muscle electrostimulation
Electrical stimulation of the skeletal muscle flap followed a progressive muscle conditioning protocol. The LDM electrostimulation started at postoperative week 2. After a 6-week muscle conditioning period the patients were stimulated with a six-pulse burst and a mean pulse amplitude of 4.2 ± 0.8 volts. To avoid LDM fibrosis due to overstimulation [6], the LDM was maintained under a 1:2 stimulation mode in relation to the heart rate. The delay between the ventricular-sensed event and the muscle burst was adjusted to provide an exact synchronization between the muscle flap contraction and the RV systole. This was done by echocardiographic assessment of the tricuspid valve closure.

Associated surgical procedures
Five patients underwent a dynamic CMP combined with a tricuspid valve repair, and 1 patient had the tricuspid valve replaced by a bioprosthesis. In the patients that required tricuspid valve surgery, procedures were performed under cardiopulmonary bypass and moderate systemic hypothermia. Tricuspid valve repair was performed using a Carpenter-Edward’s annuloplasty ring. In 1 patient a papillary muscle and chordal transposition were performed. Only 1 patient had an RV CMP procedure without cardiopulmonary bypass. In 6 patients this procedure was a first-time operation and for 1 patient this was redo surgery (previously the latter patient had surgery for atrial septal defect closure and mitral valve repair). The mechanism of tricuspid valve regurgitation was by dilation of the valvular annulus and traction of the subvalvular apparatus by the dilated RV in all patients.


   Results
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 Abstract
 Introduction
 Material and methods
 Results
Comment
 References
 
There were no perioperative deaths. Mean postoperative intensive care unit stay was 4.3 ± 1.8 days. In 1 patient with a preoperative RVEF of 12% and LV ejection fraction of 26%, an intraaortic balloon pump was placed intraoperatively. The duration of counterpulsation was 36 hours. Inotropic support was necessary in 2 patients avoiding high-dose inotropic and vasoactive drugs to preserve LDM flap viability. Previous studies have demonstrated an important vasoreactivity of the LDM artery to different drugs used postoperatively [7]. For this reason our policy consists of early placement of an intraaortic balloon pump. The hospital mortality of 0% is apparently related to a careful patient selection (excluding patients with end-stage heart failure and in permanent New York Heart Association functional class IV) and skilled intensive care unit management. The use of the extracardiac suture technique, which avoids heart manipulation during cardiac wrapping, contributed to the successful results.

The mean duration of follow-up was 10 ± 3.5 years. No patient was lost to follow-up, and no malignant cardiac arrhythmias were recorded during the follow-up period. Six patients are alive with good quality of life, 4 are in New York Heart Association functional class I, and 2 are in class II. Only 1 patient died while in functional class II in postoperative year 7, which was caused by stroke. Ventricular function was annually evaluated (Fig 2). At last control the mean RV ejection fraction was 33% ± 11.8% (18 to 51) and the mean LV ejection fraction was 52% ± 12.6% (28 to 58) (Table 2).


Figure 2
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  		Fig 2. Echocardiographic study 4 months after right ventricular cardiomyoplasty in a patient with arrhythmogenic dysplasia. The contractility of the right ventricular lateral wall improves during latissimus dorsi muscle electrostimulation (burst).

 

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  		Table 2 Hemodynamic Profile
 

   Comment
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 Abstract
 Introduction
 Material and methods
 Results
 Comment
References
 
The importance of right ventricular function has been underestimated in the past, especially as a determinant of cardiac symptoms, exercise tolerance, and survival in patients with valvular disease [1, 2]. Pump function of the right ventricle has been thought not to be relevant for the overall function of the heart. However, scientific understanding of the important role of the right ventricle has changed dramatically. Dilation of the right ventricle induces chamber remodeling with a resultant drop in ejection fraction. As a consequence, the tricuspid valve annulus dilates inducing tricuspid regurgitation [3]. The main problem in RV failure, generally associated with tricuspid valve regurgitation, is the elevated mortality rate when a tricuspid valve replacement is performed. Ratnatunga and coworkers [8] have demonstrated that tricuspid valve replacement carries a high 30-day mortality and poor long-term survival. Tricuspid valve annuloplasty is generally agreed as the preferred treatment for tricuspid regurgitation. Tricuspid valve replacement is reserved for patients with significant organic valvular disease that is unlikely to improve if more conservative measures are taken. Glower and coworkers [9], in their series of tricuspid surgery, report an operative mortality rate of 14% in patients undergoing first-time, isolated tricuspid valve replacement, and 19% in patients undergoing redo surgery for isolated tricuspid valve replacement. The most common cause of death, both in hospital and after discharge, was congestive heart failure. Overall mortality at 10 years was 32% for patients less than 50 years of age. Van Nooten and coworkers [10] report an operative mortality rate of 16.4% and a 30-month mortality rate of 57.4%. Scully and Armstrong [11] report a hospital mortality rate of 27% and a mortality rate of 32% for a mean follow-up of 75 ± 45 months. Sugimoto and coworkers [12] showed that long-term outcome after tricuspid valve surgery results in poor improvement in quality of life. Kaplan and coworkers [13] reported 129 tricuspid valve replacements (mean age, 35 ± 11 years) with an early mortality of 24.5% and a late mortality of 9.7%. Our experience in tricuspid valve surgery at Broussais Hospital includes 982 patients (63% females, mean age 42 ± 8 years) operated between January 1990 and June 2000. Hospital mortality was 12%, and 3% of patients required reoperations caused by valve dysfunction.

Causes of RV failure can be congenital (eg, Ebstein’s anomaly) [14], ischemic, idiopathic cardiomyopathy, or arrythmogenic dysplasia, which are characterized by the progressive replacement of RV myocardium by fat and fibrotic tissue [4, 15]. In addition, recent studies demonstrate that apoptotic myocardial cell death occurs in the arrythmogenic dysplasia [16].

Clinical presentation of arrhythmogenic RV cardiomyopathy is usually related to ventricular tachycardias with a left bundle branch block pattern or ventricular fibrillation leading to cardiac arrest, mostly in young people and athletes. Later in the disease evolution, progression and extension of RV muscle disease and LV involvement may result in severe right or biventricular heart failure with risk of thromboembolic complications. In cases of refractory congestive heart failure, patients may become candidates for heart transplantation [4, 15]. Recently a total right ventricular exclusion surgical procedure was proposed for this pathology and was performed in 5 patients. In this maxi-invasive approach the entire RV free wall is resected, the orifice of the tricuspid valve is closed, and the cavopulmonary connections are constructed [17].

Dynamic CMP was proposed as a potential therapeutic option in heart failure because of its several advantages: (1) It represents an autologous source of circulatory assistance; (2) it can be performed electively, without waiting for a donor organ, and (3) it represents reduced costs compared with mechanical assist devices and transplantation [5, 18].

In the present study our approach consisted of performing a CMP surgical procedure to improve right ventricular function associated in some patients with tricuspid valve repair. In the past, right ventricular CMP was investigated in experimental models [19–21]. Macoviak and coworkers [22] demonstrated that a pedicled diaphragmatic muscle graft used to replace a portion of the RV and chronically stimulated retains its ability to contract synchronously with the heart 1 month after implant. Millner and coworkers [23] have undertaken a study of RV dynamic CMP in sheep. The data presented suggests that the transformed LDM would be able to provide an adequate power source to assist the right atrium or ventricle. From experimental studies in which the RV was functionally excluded with a Fontan-type procedure, Chachques and coworkers [24] concluded that atrial CMP provides a pulsatile flow pattern in the pulmonary artery and increases cardiac output.

Experimental and clinical studies have demonstrated the potential of CMP to improve systolic contractility, to counteract the ventricular dilatation, and to ensure reverse remodeling of the LV [25]. Recent experimental studies based on response to volume loading after 6 months of CMP showed functional recovery from active electrostimulated girding and prevention of functional deterioration from passive nonstimulated cardiac wrapping [26].

The RV myocardial wall and chamber appeared to be better adapted than the LV when assisted by an electrostimulated LDM. In fact, the anatomical and hemodynamic characteristics and thickness of the RV myocardium can be more easily compressed during systole by the paced LDM. Moreover, the RV can be extensively wrapped by the left LDM, which can be positioned so that the muscular fibers are oriented perpendicularly to the ventricular septum.

In contrast with the high mortality and poor quality of life found in the series of patients treated with single tricuspid valve repair or replacement [8–13], the results of our RV CMP clinical trial demonstrated no perioperative mortality without RV dysfunction related deaths, as well as hemodynamic and clinical improvements without malignant ventricular arrhythmias. We believe that surgical indications for RV failure could be expanded, thus associating RV CMP with tricuspid valve repair [27].


   References
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 

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  8. Ratnatunga CP, Edwards MB, Dore CJ, Taylor KM. Tricuspid valve replacement. UK heart valve registry mid-term results comparing mechanical and biological prostheses. Ann Thorac Surg 1998;66:1940-1947.[Abstract/Free Full Text]
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  12. Sugimoto T, Okada M, Ozaki N, Hatakeyama T, Kawahira T. Long term results of treatment for functional tricuspid regurgitation with regurgitant volume. characteristic differences based on primary cardiac lesion. J Thorac Cardiovasc Surg 1999;117:463-471.[Abstract/Free Full Text]
  13. Kaplan M, Kut MS, Demirtas MM, Cimen S, Ozler A. Prosthetic replacement of tricuspid valve. bioprosthetic or mechanical. Ann Thorac Surg 2002;73:467-473.[Abstract/Free Full Text]
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