Hostname: page-component-586b7cd67f-rcrh6 Total loading time: 0 Render date: 2024-11-25T21:06:47.869Z Has data issue: false hasContentIssue false
  • English
  • Français

Initial clinical manifestations and mid- and long-term results after surgical repair of double-chambered right ventricle in children and adults

Published online by Cambridge University Press:  01 June 2008

Ragiab Telagh ,
Vladimir Alexi-Meskishvili ,
Ronald Hetzer ,
Peter E. Lange ,
Felix Berger  and
Hashim Abdul-Khaliq
Ragiab Telagh
Affiliation:
Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany
Vladimir Alexi-Meskishvili
Affiliation:
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany
Ronald Hetzer
Affiliation:
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany
Peter E. Lange
Affiliation:
Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany
Felix Berger
Affiliation:
Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany
Hashim Abdul-Khaliq*
Affiliation:
Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany Department of Paediatric Cardiology, Saarland University Hospital, Homburg/Saar, Germany
*
Correspondence to: Prof. Hashim Abdul-Khaliq, MD, PhD, Department of Paediatric Cardiology, Saarland University Hospital, 66421 Homburg/Saar, Germany. Tel: +49 6841-1628306; Fax: +49 6841-1628330; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective

By means of retrospective analysis of our institutional experience, we reviewed the clinical manifestation and outcomes of patients subsequent to surgical repair of double-chambered right ventricle.

Methods

Between 1988 and 2005, we performed surgical repair in 35 of 37 patients diagnosed with double-chambered right ventricle. The patients ranged in age from 4 to 69 years, with a mean of 21.3 years. Most presented in infancy, with initial manifestation of a short systolic murmur in 34 (92%) of all cases. Pressure gradients were measured invasively across the right ventricular outflow tract of between 30 and 140 mmHg, with a median of 60 mmHg. An associated ventricular septal defect was present in 26 patients (70%). Of the group, 4 patients were aged over 40 years, and 2 had previously undergone operative closure of a ventricular septal defect.

Results

The operative interval ranged from 2 months to 41 years, with a median of 9 years. In all, we resected muscular bundles and enlarged the right ventricular outflow tract. There was no hospital or late death. Median follow-up subsequent to surgery was 7 years, with a range from 0.4 to 11 years. No patient required further surgery to relieve any obstruction of the right ventricular outflow tract, nor long term medical therapy or pacing because of cardiac arrhythmia.

Conclusions

Surgical repair of a double-chambered right ventricle yields excellent haemodynamic and functional results over the mid to long term.

Keywords

Congenital heart diseasesobstructionmuscle bandcorrection
Type
Original Article
Information
Cardiology in the Young , Volume 18 , Issue 3 , June 2008 , pp. 268 - 274
Copyright
Copyright © Cambridge University Press 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Lacour-Gayet, F. Congenital Heart Surgery Nomenclature and Database Project: right ventricular outflow tract obstruction-intact ventricular septum. Ann Thorac Surg 2000; 69: S83–S96.CrossRefGoogle ScholarPubMed
2.Forster, JW, Humphries, JO. Right ventricular anomalous muscle bundle. Clinical and laboratory presentation and natural history. Circulation 1971; 43: 115–127.CrossRefGoogle ScholarPubMed
3.Keith, A. The Hunterian Lecture on malformations on the heart. Lancet 1909: 359.Google Scholar
4.Lucas, RV Jr, Varco, RL, Lillehei, CW, Adams, P Jr, Anderson, RC, Edwards, JE. Anomalous muscle bundle of the right ventricle. Hemodynamic consequences and surgical considerations. Circulation 1962; 25: 443–455.CrossRefGoogle ScholarPubMed
5.Pongiglione, G, Freedom, RM, Cook, D, Rowe, RD. Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect: an angiocardiographic study. Am J Cardiol 1982; 50: 776–780.CrossRefGoogle ScholarPubMed
6.Gallucci, V, Scalia, D, Thiene, G, Mazzucco, A, Valfre, C. Double chambered right ventricle: surgical experience and anatomical considerations. Thorac Cardiovasc Surg 1980; 28: 13–17.CrossRefGoogle ScholarPubMed
7.Rowland, TW, Rosenthal, A, Castaneda, AR. Double-chamber right ventricle: experience with 17 cases. Am Heart J 1975; 89: 455–462.CrossRefGoogle ScholarPubMed
8.Alva, C, Ho, SY, Lincoln, CR, Rigby, ML, Wright, A, Anderson, RH. The nature of the obstructive muscular bundles in double chambered right ventricle. J Thorac Cardiovasc Surg 1999; 117: 1180–1189.CrossRefGoogle ScholarPubMed
9.Hindle, WV Jr, Engle, MA, Hagstrom, JW. Anomalous right ventricular muscles: a clinicopathologic study. Am J Cardiol 1968; 21: 487–495.CrossRefGoogle ScholarPubMed
10.Hartmann, AF Jr, Goldring, D, Ferguson, TB, et al. The course of children with the two-chambered right ventricle. J Thorac Cardiovasc Surg 1970; 60: 72–83.CrossRefGoogle ScholarPubMed
11.Barnes, RJ, Kwong, KH, Cheung, AC. Aberrant muscle bundle of the right ventricle. Br Heart J 1971; 33: 546–551.CrossRefGoogle ScholarPubMed
12.Gale, GE, Heimann, KW, Barlow, JB. The double chambered right ventricle. A report of five cases. Isr J Med Sciences 1969; 5: 574–579.Google ScholarPubMed
13.Hui, W, Abd El Rahman, MY, Dsebissowa, F, et al. Comparison of modified short axis view and apical four chamber view in evaluating right ventricular function after repair of tetralogy of Fallot. Int J Cardiol 2005; 105: 256–261.CrossRefGoogle ScholarPubMed
14.Anderson, RH, Wilkinson, JE, Becker, AE. The Bulbus cordis – a misunderstood region of developing human hearts: its significance to the classification of congenital cardiac malformation. In: Rosenquist, GC, Bergsma, D (eds). Morphogenesis and malformation of the cardiovascular system. Alan R. Liss, New York, 1978, pp. 1–16.Google Scholar
15.Anderson, RH, Razavi, R, Taylor, AM. Cardiac anatomy revisited. J Anat 2004; 205: 159–177.CrossRefGoogle ScholarPubMed
16.Goitein, KJ, Neches, WH, Park, SC, Mathews, RA, Lenox, CC, Zuberbuhler, JR. Electrocardiogram in double chamber right ventricle. Am J Cardiol 1980; 45: 604–608.CrossRefGoogle ScholarPubMed
17.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.CrossRefGoogle ScholarPubMed
18.Pan, TC, Zhang, BG, Ge, YX, Zhao, JP, Shen, YZ. Surgical treatment of double-chamber right ventricle and its associated anomalies. J Tongji Medical University=Tong ji yi ke da xue xue bao 1992; 12: 250–252.Google ScholarPubMed
19. Shubhayan S. Double chambered right ventricle. http://www.emedicine.com/ped/topic612.htm. Accessed on April 2006.Google Scholar
20.Vogel, M, Smallhorn, JF, Freedom, RM, Coles, J, Williams, WG, Trusler, GA. An echocardiographic study of the association of ventricular septal defect and right ventricular muscle bundles with a fixed subaortic abnormality. Am J Cardiol 1988; 61: 857–860.CrossRefGoogle ScholarPubMed
21.Fisher, CH, James, AE Jr, Humphries, JO, Forster, J, White, RI Jr. Radiographic findings in anomalous muscle bundle of the right ventricle. An analysis of 15 cases. Radiology 1971; 101: 35–43.CrossRefGoogle ScholarPubMed
22.Simpson, WF Jr, Sade, RM, Crawford, FA, Taylor, AB, Fyfe, DA. Double chambered right ventricle. Ann Thorac Surg 1987; 44: 7–10.CrossRefGoogle ScholarPubMed
23.Pena, R, Cabrera, A, Pastor, E, et al. The double chambered right ventricle: the surgical results in 28 cases. Rev Esp Cardiol 1992; 45: 183–187.Google ScholarPubMed
24.Alva, C, Ortegon, J, Herrera, F, et al. Types of obstructions in double chambered right ventricle: mid-term results. Arch Med Res 2002; 33: 261–264.CrossRefGoogle ScholarPubMed
25.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.Google ScholarPubMed
26.Tsuchikane, E, Kobayashi, T, Kirino, M, et al. Percutaneous myocardial ablation in double-chamber right ventricle. Catheter Cardiovasc Interv 2000; 49: 97–101.3.0.CO;2-2>CrossRefGoogle ScholarPubMed
27.Hachiro, Y, Takagi, N, Koyanagi, T, Morikawa, M, Abe, T. Repair of double chambered right ventricle: surgical results and long-term follow-up. Ann Thorac Surg 2001; 72: 1520–1522.CrossRefGoogle ScholarPubMed
28.Ford, DK, Bullaboy, CA, Derkac, WM, Hopkins, RA, Jennings, RB Jr, Johnson, DH. Transatrial repair of double chambered right ventricle. Ann Thorac Surg 1988; 46: 412–415.CrossRefGoogle ScholarPubMed
29.Penkoske, PA, Duncan, N, Collins-Nakai, RL. Surgical repair of double chambered right ventricle with or without ventriculotomy. J Thorac Cardiovasc Surg 1987; 93: 385–393.CrossRefGoogle ScholarPubMed