HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map 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): Doff B. McElhinney Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by McElhinney, D. B. Articles by Reddy, V. M. Search for Related Content PubMed PubMed Citation Articles by McElhinney, D. B. Articles by Reddy, V. M.

Ann Thorac Surg 2000;70:124-127
© 2000 The Society of Thoracic Surgeons

---

Original articles: Cardiovascular

Double-chambered right ventricle presenting in adulthood

^a Division of Cardiothoracic Surgery, University of California, San Francisco, San Francisco, California, USA
^b Division of Cardiology, University of California, San Francisco, San Francisco, California, USA

Address reprint requests to Dr McElhinney, Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Rm 9557, Philadelphia, PA 19104–4399
e-mail: mcelhinney{at}email.chop.edu

	Abstract

Top Abstract Introduction Material and methods Results Comment References

 
Background. Double-chambered right ventricle is a form of right ventricular outflow tract obstruction that develops over time, often in patients with an abnormally short distance between the moderator band and pulmonary valve. This lesion typically presents in childhood or adolescence and is often accompanied by a ventricular septal defect. Only a handful of previous cases have been described in which double-chambered right ventricle occurred in adulthood.

Methods. Since 1992, three patients more than 30 years old (38, 43, and 66 years of age) have presented at our institution with unusual symptoms or a previous incorrect diagnosis. We reviewed the clinical data in these patients.

Results. Presenting symptoms included syncope, angina, and severe dyspnea resembling pulmonary hypertension. In 1 patient, disease was categorized as New York Heart Association class IV, and in the other 2 as class III. Coexisting anomalies included a patent foramen ovale or secundum atrial septal defect in 2 patients, a small ventricular septal defect in 1 (with a probable history of ventricular septal defect in another), and mild aortic regurgitation in 1. All patients required urgent or emergent operations, with peak pressures in the proximal right ventricular chamber of 135 to 180 mm Hg and severely depressed left ventricular function in 1 patient. Resection of the anomalous right venticular muscle bundles was achieved through a right atrial approach in all patients. All patients were alive with improved functional status at follow-up, which was between 15 and 40 months.

Conclusions. Right ventricular outflow tract obstruction resulting from a double-chambered right ventricle is rare in adults, but when it does occur it can present with unusual symptoms. When evaluating the patient with signs or symptoms of primary right heart failure, cardiologists should make an effort to image the entire right heart complex. Subcostal echocardiography can facilitate adequate visualization of the right ventricle when it is difficult to distinguish the subpulmonary outflow tract from the parasternal and apical windows.

	Introduction

Top Abstract Introduction Material and methods Results Comment References

 
Double-chambered right ventricle (DCRV) is a form of right ventricular outflow tract obstruction characterized by anomalous muscle bands that divide the right ventricle into a proximal high-pressure chamber and a low-pressure distal chamber. Although hemodynamic obstruction of the right ventricular outflow tract appears to be an acquired phenomenon in most cases, the underlying substrate of obstruction is likely congenital [1–3]. The natural history of this lesion is not well defined, but there is a high association with ventricular septal defect and a tendency for the obstruction to progress over time [2–7]. Typically, DCRV is diagnosed and repaired in childhood or adolescence, with most reported cases in patients less than 20 years old [2–7]. Occasionally, however, patients can present with this condition in adulthood [7–11]. When they do, their symptoms can resemble those that occur with other forms of acquired cardiovascular disease. In this report, we describe our experience with 3 patients who presented with DCRV between 38 and 63 years of age with unusual findings or an incorrect diagnosis.

	Material and methods

Top Abstract Introduction Material and methods Results Comment References

 
Between July 1992 and December 1997, 20 patients with DCRV had operations at the University of California, San Francisco. Three of those patients presented with atypical symptoms and were older than 30 years. Diagnostic information was collected by reviewing echocardiograms, cardiac catheterization studies, electrocardiograms, and clinical records. Surgical details were collected by review of operative notes. Follow-up information was obtained by direct contact with the patients’ cardiologists and by reviewing clinical records and imaging studies when applicable.

	Results

Top Abstract Introduction Material and methods Results Comment References

 
Presentation and diagnostic data
The patients were 38, 43, and 66 years of age at the time of presentation. Two were female and the other male. All had lived at least the first 20 years of their life outside of North America and reported symptoms of decreased exercise tolerance dating back to childhood. None had had prior cardiovascular interventions or taken cardiac medications, and there were no significant coexisting medical conditions. All patients had significant cardiopulmonary symptoms; two were in New York Heart Association class III and one was in class IV. Electrocardiographic evidence of right ventricular hypertrophy was present in all patients, with right axis deviation and prominent R-waves in the right precordial leads, but without prominent S-waves in the left precordial leads. There was no evidence of valvar or supravalvar pulmonary stenosis in any patient. Selective coronary angiography showed the coronary arteries to be normal in all patients, and all had normal systemic blood pressure. Clinical data are summarized in Table 1.

The second patient was a 38 year-old woman from Egypt who presented with severe substernal chest pain, increasing dyspnea on exertion, and intermittent dizziness. She reported being diagnosed with "a hole in my heart" in childhood, for which surgical intervention was recommended but refused by her parents. On physical exam she was noted to have a right ventricular heave, a normal S2 with no click and physiologic splitting, and a 3/6 harsh systolic ejection murmur most prominent at the left lower sternal border. Electrocardiography showed right ventricular hypertrophy and right atrial enlargement. Echocardiography and catheterization showed DCRV, severe right ventricular outflow tract obstruction with an estimated gradient of 150 mm Hg and a proximal right ventricular pressure of 170 mm Hg, and mild tricuspid and pulmonary regurgitation. The pulmonary valve was not stenotic, and the pulmonary arteries appeared normal.

The third patient was a 43-year-old man from Yemen who presented with a 6-month history of dyspnea on exertion, which had progressed to dyspnea at rest in the days before presentation. He presented at another institution, where he was found to have clubbing of the fingernails, a right ventricular heave, a 3/6 harsh systolic ejection murmur most prominent at the lower left sternal border, a room air arterial oxygen saturation of 83%, and a hemoglobin level of 21.3 g/dL. A chest radiogram showed cardiomegaly and clear lung fields. An electrocardiogram showed right axis deviation and right atrial enlargement. On echocardiography, he was found to have left and right ventricular hypertrophy, a left ventricular ejection fraction of 25%, a small patent foramen ovale, and an estimated pulmonary arterial pressure of 130 mm Hg. The subvalvar right ventricular outflow tract was not well visualized. The diagnosis of pulmonary hypertension was made, and he was referred to our center for further evaluation. An echocardiogram showed similar findings, and a lung perfusion scan did not show any evidence of pulmonary embolism. After several days of refusal, the patient consented to a cardiac catheterization, which allowed us to make the correct diagnosis of DCRV. On angiography, the pulmonary valve and pulmonary arteries appeared to be normal.

Operative details
All three patients had repair through a right atriotomy approach. Hypertrophied and fibrotic muscle at the infundibular os was resected, as were additional hypertrophied septal and parietal muscle bands. In the first and third patients, the interatrial communication was closed with continuous sutures, and in the second patient the small perimembranous ventricular septal defect was closed with two interrupted pledgetted sutures. There were no surgical complications. Postoperative echocardiography demonstrated residual gradients of 5 to 10 mm Hg across the right ventricular outflow tract.

Outcomes
The first patient had several episodes of asymptomatic, nonsustained supraventricular tachycardia in the early postoperative period. She was placed on digoxin and had no subsequent episodes of arrhythmia. Otherwise, there were no postoperative complications or events. Patients were discharged 5 to 7 days postoperatively.

Clinical, electrocardiographic, and echocardiographic follow-up was obtained in all patients between 15 and 40 months postoperatively. No patient required reintervention. The first patient remained on digoxin only and was free of symptoms. The second patient was readmitted 16 months postoperatively with nonexertional chest pain radiating to the left shoulder concurrent with symptoms of an upper respiratory infection. On continuous electrocardiographic monitoring, she was noted to have a single 20-beat run of ventricular tachycardia that was accompanied by palpitations. She was placed on amiodarone after refusing to consent for an electrophysiologic study. At the time of most recent follow-up, she was scheduled to have an electrophysiologic study. The third patient had almost complete relief of symptoms, with only occasional dyspnea on exertion, and was taking no medications. On echocardiography, he had mild residual left ventricular dysfunction, which was substantially improved relative to the preoperative state.

	Comment

Top Abstract Introduction Material and methods Results Comment References

 
We have reviewed our experience with DCRV in three adults who presented with unusual symptoms or with a previous incorrect diagnosis. One presented with syncope, one with angina pectoris, and one with a prior diagnosis of pulmonary hypertension. All of these presentations are characteristic of acquired forms of cardiovascular disease, which highlights the importance for the adult cardiologist to be attuned to this condition, despite its rarity in patients beyond the second decade of life. All of our patients had operations, with complete relief of obstruction, no significant operative morbidity, and substantial improvement in symptoms and functional status.

Patients with DCRV seldom present in adulthood. We are aware of only seven previously published cases of this lesion in patients more than 30 years of age (Table 2) [7–11]. Four of these patients had operations, and follow-up of 20 years or more was reported in 3, who were all well with no significant recurrence of obstruction (1 had systemic hypertension and aortic regurgitation and was in NYHA class II). In addition to these 7 patients, 3 others were mentioned in the discussion following the report by Warden and colleagues [9], including a 63-year-old patient mentioned by Dr Bentall, and 2 patients in their 40s described by Dr Bigelow. All 3 of those patients had repair, but the 2 mentioned by Dr Bigelow died in the early postoperative period of low output syndrome. An autopsy specimen from a patient with DCRV who died at age 84 years was included in the study by Wong and associates [1], but no clinical details were reported for that patient. There may have been other adult patients with DCRV reported in early series of ventricular septal defect or tetralogy of Fallot repair, as DCRV can develop in the context of both of those lesions [3, 4, 12].

When DCRV is diagnosed in the adult patient, we recommend repair in almost all cases where symptoms or associated lesions are present or when there is asymptomatic obstruction of a significant degree (> 40-mm Hg gradient). The right ventricular outflow tract obstruction in DCRV is likely to progress, and patients can develop severe and varied symptoms. Moreover, patients do well after correction. Although the 2 patients less than 40 years old mentioned by Dr Bigelow in the discussion to Warden and colleagues [9] died of low output, they were operated on before the era of myocardial protection, and perioperative care has improved dramatically since then. Age should not be a major consideration if the patient has significant symptoms, as studies have shown that patients with symptomatic heart disease in their 80s or 90s can benefit substantially from repair, without a dramatically higher operative mortality rate [16]. Long-term follow-up studies have shown that patients do well after repair of DCRV, and that the right ventricular outflow tract obstruction generally does not recur [10], although cases with recurrent obstruction have been described [12].

	References

Top Abstract Introduction Material and methods Results Comment References

 

1. Wong P.C., Sanders S.P., Jonas R.A., et al. Pulmonary valve-moderator band distance and association with development of double-chambered right ventricle. Am J Cardiol 1991;68:1681-1686.[Medline]
2. 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]
3. Pongiglione G., Freedom R.M., Cook D., Rowe R.D. Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect. Am J Cardiol 1982;50:776-780.[Medline]
4. Maron B.J., Ferrans V.J., White R.I. Unusual evolution of acquired infundibular stenosis in patients with ventricular septal defect. Circulation 1973;48:1092-1103.[Abstract/Free Full Text]
5. Cabrera A., Martinez P., Rumoroso J.R., et al. Double-chambered right ventricle. Eur Heart J 1995;16:682-686.[Abstract/Free Full Text]
6. Cil E., Saraclar M., Ozkutlu S., et al. Double-chambered right ventricle. Int J Cardiol 1995;50:19-29.[Medline]
7. Forster J.W., Humphries J.O. Right ventricular anomalous muscle bundles. Circulation 1971;43:115-127.[Abstract/Free Full Text]
8. Osborn R.C., Taylor J., Soto B., Burnum J.F. Double chambered right ventricle in a 70-year-old woman. Alabama J Med Sci 1984;21:73-77.[Medline]
9. 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]
10. 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]
11. Blery M., Gaux J.C., Rosenthal D., et al. An abnormal right ventricular muscular band associated with a normal pulmonary artery and a normal septum. Ann Radiol (France) 1972;15:879-885.
12. Moran A.M., Hornberger L.K., Jonas R.A., Keane J.F. Development of double-chambered right ventricle after repair of tetralogy of Fallot. J Am Coll Cardiol 1998;31:1127-1133.[Abstract/Free Full Text]
13. Silverman N.H. Right heart obstructive lesions. In: Silverman N.H., ed. Pediatric echocardiography. Baltimore: Williams & Wilkins, 1993:327-359.
14. Fogel M.A., Rychik J. Right ventricular function in congenital heart disease. Prog Cardiovasc Dis 1998;40:343-356.[Medline]
15. Rein A.J., Gomori J.M., Gilon D. Magnetic resonance and echocardiographic imaging of double chamber right ventricle. J Comput Assist Tomogr 1995;19:329-330.[Medline]
16. Khan J.H., McElhinney D.B., Hall T.S., Merrick S.H. Cardiac valve surgery in octogenarians. Arch Surg 1998;133:887-893.[Abstract/Free Full Text]

This article has been cited by other articles:

				J. J. Kardux, H. W. van Es, and B. J. W. M. Rensing Case 119: Double-chambered Right Ventricle Radiology, August 1, 2007; 244(2): 617 - 619. [Full Text] [PDF]

				M. Alvarez, L. Tercedor, J. M Lozano, and J. Azpitarte Sustained monomorphic ventricular tachycardia associated with unrepaired double-chambered right ventricle Europace, October 1, 2006; 8(10): 901 - 903. [Abstract] [Full Text] [PDF]

				M. Nagashima, T. Tomino, H. Satoh, T. Nakata, T. Ohtani, and H. Saito Double-Chambered Right Ventricle in Adulthood Asian Cardiovasc Thorac Ann, June 1, 2005; 13(2): 127 - 130. [Abstract] [Full Text] [PDF]

				P Hoffman, A W Wojcik, J Rozanski, H Siudalska, E Jakubowska, E K Wlodarska, and M Kowalski The role of echocardiography in diagnosing double chambered right ventricle in adults Heart, July 1, 2004; 90(7): 789 - 793. [Abstract] [Full Text] [PDF]

				J. M. Oliver, A. Garrido, A. Gonzalez, F. Benito, M. Mateos, A. Aroca, and E. Sanz Rapid progression of midventricular obstruction in adults with double-chambered right ventricle J. Thorac. Cardiovasc. Surg., September 1, 2003; 126(3): 711 - 717. [Abstract] [Full Text] [PDF]

				Y. Hachiro, N. Takagi, T. Koyanagi, M. Morikawa, and T. Abe Repair of double-chambered right ventricle: surgical results and long-term follow-up Ann. Thorac. Surg., November 1, 2001; 72(5): 1520 - 1522. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map 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): Doff B. McElhinney Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by McElhinney, D. B. Articles by Reddy, V. M. Search for Related Content PubMed PubMed Citation Articles by McElhinney, D. B. Articles by Reddy, V. M.