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Ann Thorac Surg 2001;72:1520-1522
© 2001 The Society of Thoracic Surgeons

Original article: cardiovascular

Repair of double-chambered right ventricle: surgical results and long-term follow-up

Yoshikazu Hachiro, MD*a, Nobuyuki Takagi, MDa, Tetsuya Koyanagi, MDa, Masayuki Morikawa, MDa, Tomio Abe, MD, PhDa

a Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, Sapporo, Japan

Accepted for publication June 12, 2001.

* Address reprint requests to Dr Hachiro, Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, South 1, West 16, Chuo-ku, Sapporo 060-8543, Japan
e-mail: hachiro{at}rf6.so-net.ne.jp


   Abstract
Top
 Abstract
Introduction
 Patients and methods
 Results
 Comment
 References
 
Background. We reviewed the outcomes of double-chambered right ventricle repair.

Methods. Between 1969 and 1998, 40 patients underwent surgical repair of a double-chamber right ventricle. The patients ranged in age from 3 months to 52 years (mean, 12.8 ± 11.6 years). Right ventricular outflow tract pressure gradients were from 20 to 170 mm Hg (mean, 65.0 ± 38.5 mm Hg) An associated ventricular septal defect was present in 27 patients (67.5%). Four patients were older than 30 years of age.

Results. There were no hospital or late deaths. Mean postsurgical follow-up was 16.5 ± 8.9 years (range, 2.5 to 31 years). No patient required further surgery to relieve obstruction of right ventricular outflow tract.

Conclusions. Surgical repair of a double-chambered right ventricle yields excellent hemodynamic and functional results over both the short and long term.


   Introduction
Top
 Abstract
 Introduction
Patients and methods
 Results
 Comment
 References
 
The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, resulting in double-chambered right ventricle (DCRV). The natural history of this lesion is not well defined, but there is a high association with ventricular septal defect (VSD) and a tendency for the obstruction to progress over time. Typically, DCRV is diagnosed and repaired during childhood or adolescence, and most cases are reported in patients less than 20 years old [1–6].

This report describes our surgical experience with 40 patients, including some adults, who presented with DCRV. The short-term and long-term results of DCRV repair are presented.


   Patients and methods
Top
 Abstract
 Introduction
 Patients and methods
Results
 Comment
 References
 
Patients
Between April 1969 and August 1998, 47 patients underwent repair of a DCRV at the Sapporo Medical University School of Medicine, Sapporo, Japan. Seven patients with incomplete data or who were lost to follow-up were excluded from this study. Twenty patients were female and 20 were male (Table 1). The mean age at the time of the operation was 12.8 ± 11.6 years (range, 3 months to 52 years). Four patients were older than 30 years of age.


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  		Table 1. Clinical Data

 
All patients underwent preoperative cardiac catheterization. The criteria used for the diagnosis of DCRV were as follows: (1) documentation at cardiac catheterization of a systolic pressure gradient between the inflow of the right ventricle and right ventricular outflow tract; (2) a right ventricular angiogram showing a high or low obstruction by an anomalous muscle bundle below the infundibulum; (3) absence of infundibular hypoplasia; and (4) direct observation of intracardiac muscle bundles during surgical repair. Thirty-two patients were diagnosed with DCRV preoperatively, and the remaining patients were diagnosed as having VSD or pulmonary stenosis, or both, by echocardiography; the diagnosis was revised as VSD with DCRV intraoperatively. In the absence of a moderate or large VSD, the criterion for the diagnosis of DCRV and the need for an operation was a systolic pressure gradient between the inflow and outflow chamber of more than 40 mm Hg at rest.

Based on hemodynamic studies, the systolic pressure in the proximal chamber in the right ventricle was 89.9 ± 35.1 mm Hg (range, 44 to 190 mm Hg). The right ventricular systolic pressure was higher than the left ventricular pressure in 42.4% of cases. The systolic pressure gradient across the two chambers was 65.0 ± 38.5 mm Hg (range, 20 to 170 mm Hg). The most frequent associated cardiac anomalies were VSD in 27 patients (67.5%, 23 perimembranous type and four subarterial type), atrial septal defect in 2 patients, subaortic stenosis in 2 patients, aortic valve regurgitation in 2 patients, tricuspid valve regurgitation in 1 patient, and ruptured sinus of Valsalva aneurysm in 1 patient (Table 2).


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  		Table 2. Associated Lesions With Double-Chambered Right Ventricle

 
Operative procedure
All patients underwent surgical correction through a median sternotomy with cardiopulmonary bypass under moderate hypothermia (28°C to 32°C). Since 1977, cold crystalloid or blood cardioplegia was used for myocardial protection with topical cold saline solution infused into the pericardium. All associated cardiac anomalies were corrected simultaneously. Surgical approaches included a longitudinal right ventriculotomy, a right atriotomy, and a combined pulmonary arteriotomy and right atriotomy. A longitudinal right ventriculotomy was used in most patients before 1987. Since then, a right atriotomy was performed, or a combined pulmonary arteriotomy and right atriotomy were performed if possible.

Operative repair was accomplished through a right ventriculotomy in 29 patients (72.5%), through a right atriotomy in 8 patients, and through a combined pulmonary arteriotomy and right atriotomy in 3 patients. The ventriculotomy was closed directly or with a patch depending on the surgeon’s impression of the right ventricular cavity size after completion of the resection. When a VSD was present, it was closed with a Dacron patch (DuPont, Wilmington, DE) (10 patients) or a direct suture (17 patients). In 1 patient with subaortic stenosis, this resection was carried out through the aortic valve. One patient had a connection between the right coronary sinus and the right ventricle. Direct suture of this ruptured aneurysm was performed with interrupted mattress sutures. Other associated procedures included aortic valve replacement with an Omnicarbon bioprosthesis (29-mm valve, Medical CV, Inc, Minneapolis, MN) in 1 patient with severe aortic regurgitation, tricuspid valve replacement with a Hancock T-6 bioprosthesis (33-mm valve, Medtronic Heart Valve, Inc, Santa Ana, CA) in one patient with severe degenerative tricuspid regurgitation, and atrial septal defect closure in 2 patients.


   Results
Top
 Abstract
 Introduction
 Patients and methods
 Results
Comment
 References
 
The patients’ postoperative courses were uneventful and no major complications occurred. There were no hospital or late deaths.

Persistent cardiac residua and hemodynamic sequelae generally were mild. Among the 20 patients (50%) who underwent postoperative cardiac catheterization for suspected hemodynamic significant residua, the residual right ventricular outflow tract gradient was 14.5 ± 9.3 mm Hg (range, 2 to 30 mm Hg). A mild right ventricular outflow tract gradient (peak systolic gradient of 30 mm Hg) was present in 3 patients. A residual VSD was documented in 4 patients by echocardiography. However, these were hemodynamically insignificant. One patient required tricuspid valve replacement with a Hancock T-6 bioprosthesis (29-mm valve) for severe tricuspid regurgitation 10 years after the initial repair. The cause of tricuspid regurgitation was distortion of the tricuspid valve as a result of the VSD closure. Ten years later this patient underwent repeat tricuspid valve replacement with a Carpentier-Edwards bioprosthesis (31-mm valve, Baxter Healthcare, Irvine, CA) because primary tissue failure occurred. Another patient required repeat tricuspid valve replacement with a Hancock II bioprosthesis (31-mm valve, Medtronic Heart Valve, Inc, Santa Ana, CA) for primary tissue failure 7 years after the initial operation. All patients were in sinus rhythm; however, 30 patients (75%) had complete or incomplete right bundle branch block postoperatively. One patient who underwent right ventriculotomy required antiarrhythmia agents to treat ventricular tachycardia in the late postoperative period.

Mean follow-up time was 16.5 ± 8.9 years (range, 2.5 to 31 years). No patient required further operation for obstruction of the right ventricular outflow tract. All patients are alive and asymptomatic.


   Comment
Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
References
 
Primary DCRV is an uncommon congenital anomaly consisting of one or more anomalous muscle bundles that divide the right ventricle into a proximal high-pressure chamber and a distal low-pressure chamber. However, it is well documented that right ventricular outflow tract obstruction caused by anomalous muscle bundles may be an acquired phenomenon in some patients with ventricular septal defect and valvar pulmonary stenosis [2], and that obstruction may progress with time [7].

None of the patients in this study required reoperation for residual right ventricular outflow tract obstruction. In some patients, clinical evaluation suggested or postoperative cardiac catheterizations documented trivial to mild residual right ventricular outflow tract obstruction or minor leakage through a residual VSD. However, none of these problems were clinically significant. We believe that an excellent outcome should be the rule, not the exception, after complete surgical repair of DCRV.

Double-chambered right ventricle is rare in adults, and adult cardiologists will not often see this anomaly in their practice. However, patients can present with this condition in adulthood [6, 8–10]. McElhinney and associates [10] reported 3 patients with DCRV, aged 38 to 63 years old who presented with unusual findings or an incorrect diagnosis initially. We also treated 4 patients older than 30 years of age. We recommend repair in almost all adults when symptoms exist, an associated lesion is present, or there is a significant degree of asymptomatic obstruction. The right ventricular outflow tract obstruction in DCRV is likely to progress, and eventually the patients will become symptomatic.

The surgical repair of DCRV consists of resecting the obstructing muscle bundles. This has been accomplished through a right ventriculotomy [11, 12], transatrially [13], or through a combination of transatrial and transpulmonary approaches [14]. Right ventriculotomy depresses right ventricular function [15] and increases the risk of ventricular arrhythmias [16]. Accordingly, alternative approaches to ventriculotomy are currently preferred [4, 17]. However, ventriculotomy may still be necessary in some patients with severe right ventricular obstruction and in some older patients. Regardless of the approach, it is important to avoid damage to the tricuspid valve and its accessory structures.

The long-term prognosis for patients after intracardiac repair of DCRV is excellent. At a mean follow-up of 16 years, there were no premature late deaths and most patients were asymptomatic.


   References
Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 

  1. Alva C., Ho S.Y., Lincoln C.R., Rigby M.L., Wright A., Anderson R.H. The nature of the obstructive muscular bundles in double-chambered right ventricle. J Thorac Cardiovasc Surg 1999;117:1180-1189.[Abstract/Free Full Text]
  2. Pongiglione G., Freedom R.M., Cook D., Rowe R.D. Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect: an angiocardiographic study. Am J Cardiol 1982;50:776-780.[Medline]
  3. Maron B.J., Ferrans V.J., White R.I. Unusual evolution of acquired infundibular stenosis in patients with ventricular septal defect. Clinical and morphologic observations. Circulation 1973;48:1092-1103.[Abstract/Free Full Text]
  4. Cabrera A., Martinez P., Rumoroso J.R., et al. Double-chambered right ventricle. Eur Heart J 1995;16:682-686.[Abstract/Free Full Text]
  5. Cil E., Saraclar M., Ozkutlu S., et al. Double-chambered right ventricle: experience with 52 cases. Int J Cardiol 1995;50:19-29.[Medline]
  6. Forster J.W., Humphries J.O. Right ventricular anomalous muscle bundle. Clinical and laboratory presentation and natural history. Circulation 1971;43:115-127.[Abstract/Free Full Text]
  7. Fellows K.E., Martin E.C., Rosenthal A. Angiocardiography of obstructing muscular bands of the right ventricle. AJR Am J Roentgenol 1977;128:249-256.[Abstract]
  8. Warden H.E., Lucas R.V., Varco R.L. Right ventricular obstruction resulting from anomalous muscle bundles. J Thorac Cardiovasc Surg 1966;51:53-65.[Medline]
  9. Kveselis D., Rosenthal A., Ferguson P., Behrendt D., Sloan H. Long-term prognosis after repair of double-chamber right ventricle with ventricular septal defect. Am J Cardiol 1984;54:1292-1295.[Medline]
  10. McElhinney D.B., Chatterjee K.M., Reddy V.M. Double-chambered right ventricle presenting in adulthood. Ann Thorac Surg 2000;70:124-127.[Abstract/Free Full Text]
  11. Hartmann A.F., Goldring D., Ferguson T.B., et al. The course of children with the two-chambered right ventricle. J Thorac Cardiovasc Surg 1970;60:72-83.[Medline]
  12. Li M.D., Coles J.C., McDonald A.C. Anomalous muscle bundle of the right ventricle. Its recognition and surgical treatment. Br Heart J 1978;40:1040-1045.[Abstract/Free Full Text]
  13. Wallsh E., Weinstein G., Franzone A.J., Kania H., Cucci C. Anomalous right ventricular muscle bundle. Repair through the right atrium. J Thorac Cardiovasc Surg 1975;69:830-834.[Abstract]
  14. Penkoske P.A., Duncan N., Collins-Nakai R.L. Surgical repair of double-chambered right ventricle with or without ventriculotomy. J Thorac Cardiovasc Surg 1987;93:385-393.[Abstract]
  15. Shimazaki Y., Kawashima Y., Mori T., Beppu S., Yokota K. Angiographic volume estimation of right ventricle. Re-evaluation of the previous methods. Chest 1980;77:390-395.[Abstract/Free Full Text]
  16. Kobayashi J., Hirose H., Nakano S., Matsuda H., Shirakura R., Kawashima Y. Ambulatory electrocardiographic study of the frequency and cause of ventricular arrhythmia after correction of tetralogy of Fallot. Am J Cardiol 1984;54:1310-1313.[Medline]
  17. Galal O., Al-Halees Z., Solymar L., et al. Double-chambered right ventricle in 73 patients. Spectrum of the disease and surgical results of transatrial repair. Can J Cardiol 2000;16:167-174.[Medline]



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