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Acquired Gerbode defect following endocarditis of the tricuspid valve: a case report and literature review

Journal of Cardiothoracic Surgery201510:115

https://doi.org/10.1186/s13019-015-0320-z

Received: 3 May 2015

Accepted: 24 August 2015

Published: 9 September 2015

Abstract

The Gerbode’s defect is a communication between the left ventricle and right atrium. It is usually congenital, but rarely is acquired, as a complication of endocarditis, myocardial infarction, trauma, or after previous cardiac surgery. The acquired Gerbode defect with involvement of the tricuspid valve acquired after bacterial endocarditis can be challenging to repair. We present a rare case of young woman, with endocarditis of the tricuspid valve and acquired Gerbode defect without previous cardiac surgery. She underwent successful surgical closure of the Gerbode defect and reconstruction of the septal leaflet of the tricuspid valve using a an autologous pericardial patch. A total of 20 other cases were reported with acquired Gerbode defect due to endocarditis in patients without previous cardiac surgery. Three other cases presented acquired Gerbode defect due to myocardial infarction and two due to chest trauma. Another series of 62 patients presented acquired Gerbode defect after previous cardiac surgery. Surgical treatment is always feasible with excellent outcome. However the percutanous transcatheter closure remains an excellent option especially in high risk patients.

Keywords

AcquiredGerbodedefect

Introduction

The communication between the left ventricle and right atrium was firstly reported in 1838 by Thurman [1]. In 1957, Gerbode et al. [2] reported the first 5 cases with such a heart defect undergoing successful surgical repair. Such a defect is usually congenital, but rarely is acquired, as a complication of endocarditis [3], myocardial infarction, blunt chest trauma or after previous cardiac surgery [4]. This can be anatomically possible because the normal tricuspid valve is more apically displaced than the mitral valve. Acquired Gerbode defects with large septal destructions and vegetations involving the tricuspid valve can be challenging and might require complex patch repair. We present a case of our patient with this uncommon complication of endocarditis, simulating severe pulmonary hypertension.

Case report

A 40 year old lady from Kosovo, was referred to our hospital for severe pulmonary arterial hypertension and a mass in right atrium suspected for vegetation. About one month before, she was admitted in another hospital and received iv medication. The patient was febrile and the C-reactive protein, white cell count and erythrocyte sedimentation rate were elevated. Blood cultures demonstrated a methacilin sensitive Staphylococcus aureus growth.

Transthoracic echocardiograhy demonstrated a mobile, irregularly shaped, oscillating and highly mobile mass, located above the tricuspid valve septal leaflet (Fig. 1b). A clear jet across a small defect between left ventricle and right atrium consistent with Gerbode type defect was identified. The direction of the Doppler signal also leads to the true diagnosis (Fig. 1a). Cardiac magnetic resonance demonstrated a supravalvular flow associated with infravalvular jet according to the type C acquired Gerbode defect (Fig. 1c and 1d). A normal lung scan excluded pulmonary embolism. The tricuspid regurgitation was considered mild- to- moderate with estimated pulmonary arterial systolic pressure about 60-80 mmHg.
Fig. 1

a Transesophageal echocardiography demonstrating the shunt between the left ventricle and right atrium. b Transthoracic echocardiography demonstrating the vegetation inserted above the septal leaflet of the tricuspid valve. c Cardiac magnetic resonance demonstrating a communication between the left ventricle and right atrium and right ventricle according to (d). C-type acquired Gerbode defect representing a supravalvular combined with n infravalvular communication between the left and right side of the heart

The patients underwent surgery after 2 weeks of antiobiotic therapy. Through a right atriotomy, large vegetation was attached to the septal leaflet and anterior leaflet of tricuspid valve was identified. On removal of the vegetation, a defect was found communicating between the left ventricle and right atrium (Fig. 2a and 2b). This defect represented an acquired Gerbode defect and was closed by two 5/0 pledgeted prolene sutures (Fig. 2c). Then the septal leaflet of tricuspid valve was resected and was replaced with a trimmed autologous pericardial patch. Anteriorly the newly created septal leaflet was attached to the anterior leaflet. Then, two synthetic chorda were employed (Fig. 2d). The hydraulic maneuver demonstrated trivial tricuspid valve regurgitation (Fig. 2c). Then the right atrium was closed. After an uneventfully post-operative period, the patient was discharged home in good clinical condition. Echocardiogram demonstrated trivial tricuspid valve regurgitation and no residual shunt. One year later the patient was doing well. The transthoracic echocardiography at follow-up demonstrated a moderate tricuspid valve regurgitation and no residual shunt.
Fig. 2

a Intraoperative view demonstrating the acquired Gerbode defect after removing the septal leaflet and part of the anterior leaflet of the tricuspid valve. b A diagram representing the extension of the destructed valvular tissue. c Hydraulic maneuver after closure of the acquired Gerbode defect and reconstruction of the septal leaflet of the tricuspid valve. d A diagram demonstrating the final view of the operation

Comment

Gerbode described such a defect as a congenital atrioventricular shunt originating from the interventricular membranous septum with regurgitation into the right atrium through a defect or cleft in the tricuspid valve leaflet [2]. Less common is the acquired form of a Gerbode defect, which is often associated with bacterial endocarditis [524], myocardial infarction [2527], blunt chest trauma [28, 29] or post previous cardiac surgical procedures [30, 31].

After a careful revision of the literature we found 25 other reported cases with acquired Gerbode defect without previous cardiac surgery. In 22 of them, including our case, the cause was endocarditis. Only 4 patients were females. 7 out 21 cases presented endocarditis due to Staphylococcus aureus, usually involving the aortic valve extending below the aortic annulus onto the upper part of the interventricular septum. Infective tissue destruction leads to a perforation of the septum creating a communication between the left ventricle and the right atrium. However 8 out of 21 cases including our case presented tricuspid valve endocarditis causing an acquired Gerbode defect (Table 1). In difference to the endocarditis of the left side, in the tricuspid valve endocarditis the vegetations and destructed tissue are located in the right side so, it might be more than enough the closure of the communication only on the right side, if healthy tissue is present as in our case. The mortality was almost 9 % in patients with endocarditis. Also the postoperative complications such as renal failure was identified in 3 patients (13.6 %) and complete atrioventricular block in 3 patients (13.6 %). The high incidence of the complete atrioventricularf block might be explained with the closed vicinity of the Gerbode defect with the conduction system and atrioventricular node. Interestingely in none of the cases with Gerbode defect without prior cardiac surgery undergoing surgical correction is reported recurrence of the communication between the left ventricle and right atrium or endocarditis recurrence.
Table 1

Patients with acquired Gerbode defect without prior cardiac surgery

Author (Ref)

Year

Gender/Age

Location

Bacteria

Diagnosis

Treatment

Outcome

1. Battin [5]

1991

Male/15

na

na

TTE

Surgery

Survived

2. Saiki [6]

1994

Male/42

MV,AV

Streptococcus hemolyticus

TTE,

Surgery

Survived

3. Katoh [7]

1994

Male/58

TV

na

na

Surgery

Survived

4. Elian [8]

1995

Male/64

TV

Staphylococcus aureus

TTE, TEE, CC

Surgery

Survived

5. Velebit [9]

1995

Male/ 30

BAV

Staphylococcus aureus

TEE, CC

Surgery

Survived(AVB)

6. Winslow [10]

1995

Male/ 30

AV

Staphylococcus aureus

TTE, TEE

Surgery

Survived

7. Michel [11]

1996

Male/52

AV

Streptococcus viridans

TTE, TEE

Conservative

Survived

8. Alphonso [12]

2003

Male/ 63

AV

Culture negative

TTE

Surgery

Survived

9. Raja [13]

2006

Male/47

RA

Staphylococcus aureus

TTE, TEE

Surgery

Survived(RF)

10. Fukui [14]

2007

Male/57

TV, AV, MV

na

TEE

Surgery

Survived

11. Tatewaki [15]

2008

Female/7

TV, AV, MV

Staphylococcus aureus

TEE, CT

Surgery

Survived

12. Inouel [16]

2009

Female/21

AV

Culture negative

TTE, TEE

Surgery

Survived

13. Cortez-Dias [17]

2009

Male/59

MV

Staphylococcus aureus

TTE, TEE

Conservative

Died(AVB, RF)

14. Mendoza [18]

2009

Female/52

AV

Streptococcus mutans

TTE, CT

Surgery

Survived

15. Hori [19]

2010

Male/41

BAV

na

TTE

Surgery

Survived

16. Matt [20]

2010

Male/35

AV

Hemophilus aphrophilus

TTE,TEE

Surgery

Survived(AVB)

17. Ota [21]

2011

Male/71

AV

Streptococcus pneumonia

TTE,TEE

Surgery

Survived

18. Pillai [22]

2011

Male/12

TV

Culture negative

TEE

Surgery

Survived

19. Carpenter [23]

2012

Male/22

TV

Staphylococcus lugdunensis

TEE, CT

Surgery

Survived

20. Hsu [24]

2014

Male/40

BAV

Cardiobacterium hominis

TEE,

Surgery

Died(RF)

21. Prifti et al.

2015

Female/40

TV

Staphylococcus aureus

TTE, TEE

Surgery

Survived

 

Area of myocardial infarction

 

22. Hole [25]

1995

Male/63

Inferior myocardial infarction

TTE

Surgery

Survived

23. Jobic [26]

1997

Female/72

Inferior myocardial infarction

TTE, TEE

Surgery

Died (RF)

24. Newman [27]

1996

Male/72

Inferior myocardial infarction Trauma

TTE, TEE

Surgery

Died

25. Venkatesh [28]

1996

Male/16

Blunt trauma

TTE, TEE

Surgery

Survived

26. Selinger [29]

1998

Male/70

Bullet, trauma

TTE,TEE,CC

Surgery

Survived

Legend: TTE Transthoracic echocardiography, TEE Transesophageal echocardiography, CC Cardiac catheterization, CT Cardiac tomography, na not available, AV Aortic valve, BAV Bicuspid Aortic Valve, MV Mitral valve, TV Tricuspid valve, RF Renal Failure, AVB Complete atrioventricular block

Three other cases acquired Gerbode defect post myocardial infarction were found in the literature and all of them presented inferior myocardial infarction. 2 of them died after surgery. Two other patients were found with acquired Gerbode defect due to blunt chest trauma or bullet penetration. The overall mortality in 26 patients without prior cardiac surgery was 15.4 %. The postoperative hospital stay was less than 2 weeks in the survived cases.

Interestingely, acquired Gerbode defect after previous cardiac surgery was found in 62 other patients (Table 2). 26 of them underwent surgical closure of the defect and 18 percutaneous closure employing different occlude devices. 11 patients did not undergo any interventional procedure, probably due to small shunt or high operative risk. Most of the patients were undergone previously aortic valve surgery or mitral valve surgery. However the mortality, in this group of patients despite all of them were redo operations, was almost 3.2% extremely lower than patients undergoing first time cardiac surgical procedure (Table 1).
Table 2

Patients with acquired Gerbode defect undergoing previous cardiac surgery

Author

Year

Gender

Age

Diagnostic tool

Previous procedure

Treatment

Outcome

1. Katta et al.

1994

Male

54

TTE,TEE

Endomyocardial biopsy

Conservative

Survived

2. Dzwonczyk et al.

1995

Male

25

TTE

ASD repair

na

na

3. Dzwonczyk et al.

1995

Female

72

TTE

AVR, VSD repair

na

na

4. Fukui et al.

2000

Male

53

TEE

MVR x 2

Surgery

Survived

5. Benisty et al.

2000

Male

72

TTE, TEE

MVR

Surgery

n.a.

6. Benisty et al.

2000

Male

73

TTE, TEE

MVR x 3, AVR

Surgery

n.a.

7. Weinrich et al.

2001

Female

58

TEE, CC

MVRx 2

Surgery

Survived

8. Wasserman et al.

2002

Male

78

TTE, TEE,

AVR

Surgery

Survived

9. Cabalka et al.

2005

Female

70

TTE, TEE

MVR x 2

Percutaneous

Survived

10. Lorber et al.

2006

Female

78

TTE, CC

MVR

Percutaneous

Survived

11. Ramasubbu et al.

2006

Male

41

TEE

Aortic root reconstruction

Surgery

Survived

12. Ramasubbu et al.

2006

Female

44

TEE

Aortic root reconstruction

Conservative

Survived

13. Trehan et al.

2006

Male

22

TTE, MRI, CC

VSD + sinus valsalva repair

Percutaneous

Survived

14. Martinez et al.

2007

Female

70

TTE

MVR

Percutaneous

Survived

15. Martinez et al.

2007

Male

67

TTE

AVR

Percutaneous

Survived

16. Uslu et al.

2007

Male

54

TTE

MVR

Surgery

Survived

17. Hilberath et al.

2007

Male

68

TEE

AVR + endocarditis

Surgery

Survived

18. Frigg et al.

2008

Female

77

TEE, CC

AVR

Surgery

Survived

19. Moaref et al.

2008

Female

51

TEE

MVR

Surgery

na

20. Aoyagi et al.

2008

Female

71

TTE, CC

MVR, TV repair

Surgery

Survived

21. Rothman et al.

2008

Male

86

TTE, CC

MVR

Percutaneous

Survived

22. Hansalia et al.

2009

Female

46

TTE

AVR

Surgery

Survived

23. Yared et al.

2009

Male

60

TTE, TTE

AVR+ endocarditis

na

na

24. Gorki et al.

2009

Female

69

na

AVR + endocarditis

na

na

25. Subramaniam et al.

2009

Male

60

TEE, CT

AVR

Surgery

Survived

26. Amirghofran et al.

2009

Female

51

TEE

MVR

Surgery

Survived

27. Silbiger et al.

2009

Female

30

TTE, CC

VSD repair

Conservative

Survived

28. Cheema et al.

2009

Female

31

MRI

VSD repair

Conservative

Survived

29. Can et al.

2009

Male

72

TTE

AV nod ablation

Conservative

Survived

30. Can et al.

2009

Male

68

Autopsy

AV nod ablation

na

Died

31. Dadkhah et al.

2009

Female

73

TEE

TV repair

Conservative

Survived

32. Mohapatra et al

2009

Female

22

TEE

MVR (RF)

Surgery

Survived

33. Sun et al.

2010

na

na

na

MVR

Surgery

na

34. Sun et al.

2010

na

na

na

MVR

na

na

35. Pursnani et al.

2010

Male

78

TTE, TEE

AVR

Surgery

Survived

36. Sharma et al.

2011

Male

80

TTE

AV nod ablation

Conservative

Survived

37. Kumar et al.

2011

Female

59

TEE

AVRx2 + endocarditis

Surgery

Survived

38. Zhu et al.

2012

Baby

6 months

TTE, TEE

ASD, VSD repair

Percutaneous

Survived

39. Bochard-Villanueva

2012

Male

63

TEE, CT

AVR+ endocarditis

Surgery

Survived

40. Vallakati et al.

2012

Female

53

TTE

AVR

Conservative

Survived

41. Elmistekawy et al.

2012

Male

59

TEE

AVR

Surgery

Survived

42. Dores et al.

2012

Male

50

TTE, TEE

AVR, MVR

Surgery

Survived

43. Yurdakul et al.

2012

Male

68

TEE

AVR

Surgery

Survived

44. Mousavi et al.

2012

Female

76

TEE, MRI

AVR

Conservative

Survived

45. Ozdogan et al.

2012

Female

31

TTE, TEE

MVRx2 + endocarditis

Surgery

Died

46. Anderson et al.

2012

na

na

na

AVR

na

na

47. Toprak et al.

2013

Male

32

TTE, TEE

AVR

Conservative

Survived

48. Notarangelo et al.

2013

n.a.

69

TTE, TEE

MVR

Percutaneous

Survived

49. Sinisalo et al.

2013

Male

75

TTE, TEE, CC

AVR

Percutaneous

Survived

50. Sinisalo et al.

2013

Female

23

TEE, CC

VSD repair

Percutaneous

Survived

51. Sinisalo et al.

2013

Male

10

TEE, CC

ASD, VSD repair

Percutaneous

Survived

52. Sinisalo et al.

2013

Male

8

TEE, CC

VSD repair

Percutaneous

Survived

53. Dangol et al.

2013

Male

6 months

TTE,TEE,CC

ToF repair

Percutaneous

Survived

54. Lee et al.

2013

Male

3 months

TTE, CC

ASD, PDA, VSD repair

Percutaneous

Survived

55. Poulin et al.

2013

Female

75

TTE,TEE

MVR

Percutaneous

Survived

56. Primus et al.

2013

Female

76

TTE,TEE

AVR

Conservative

Survived

57. Chaturvedi et al.

2013

Male

62

TTE, MRI

AVR

Percutaneous

Survived

58. Tayama et al.

2014

Male

75

TTE, CC

MV and TV repair

Surgery

Survived

59. Hussain et al.

2014

Male

45

TTE, TEE

AVRx2

Surgery

Survived

60. Chamsi-Pasha et al

2014

Male

67

TTE, TEE

MVR, TVR

Surgery

Survived

61. Taskesen et al.

2014

Male

74

TTE, TEE

AVRx2

Percutaneous

Survived

62. Fanari et al

2015

Female

50

TTE, CT

AVR

Percutaneous

Survived

Patients with acquired Gerbode defect undergoing previous cardiac surgery

Legend: TTE Transthoracic echocardiography, TEE Transesophageal echocardiography, CC Cardiac catheterization, CT Cardiac tomography, MRI Magnetic resonance, na-not available, AVR Aortic valve replacement, MVR Mitral valve replacement, TV Tricuspid valve, ASD Atrial septal defect, VSD Ventricular septal defect, ToF Tetralogy of Fallot, PDA Patent ductus arteriosum

The diagnosis was made in most of the cases by transthoracic and transesophageal echocardiography. It seems that echocardiographic examination is the most frequently diagnostic tool employed in these patients. Identification of an actual communication is often extremely difficult, so a careful and meticulous echocardiogram should be done in order to prevent echocardiographic misinterpretation of this defect as pulmonary arterial hypertension. The large systolic pressure gradient between the left ventricle and the right atrium would expectedly result in a high velocity systolic Doppler flow signal in right atrium and it can be sometimes mistakably diagnosed as tricuspid regurgitant jet simulating pulmonary arterial hypertension. However cardiac catheterization, cardiac tomography or magnetic resonance such as in our case offers valuable information. Interestingely our case after been diagnosed with Gerbode defect underwent cardiac magnetic resonance which revealed a class C acquired Gerbode defect as previously described [4].

Treatment of the acquired Gerbode defect depends on symptoms, magnitude of shunt, flow volume, concomitant anatomic abnormalities and co-morbidities. Asymptomatic, chronic, small defects can be managed conservatively.

Percutaneous transcatheter closure techniques have been employed in almost 25% of patients, mostly in high risk surgical candidates due to previous valve replacement, advanced age, anti-coagulation, and multiple comorbidities. Advanced cardiac imaging techniques such as transesophageal echocardiography provide excellent images for guidance in device sizing and deployment. The Amplatzer duct occluder device is a mainstay in treatment as it provides less radial force [30] than the muscular ventricular septal defect closure device causing fewer complications [31].

In most of the cases with acquired Gerbode defect a simple direct suture might be enough to close the defect [12] such as in our case, although large Gerbode defect associated with partial or total distruction of the tricuspid valve can be much more challenging. In such cases reconstruction or replacement of the tricuspid valve might be required. Tatewaki et al. [15] describe a pericardial patch closure with sutures from the ventricular side of the tricuspid valve through the leaflets. Others reported a Dacron patch closure with septal leaflet reimplantation onto the patch [9, 12], an annuloplasty ring implantation, or tricuspid valve replacement [5, 8, 9, 12]. Matt et al. [20] presented a double plicated patch combining a defect closure and reconstruction of the tricuspid valve annulus and septal leaflet. In our case we closed the defect from the right side using two single pledgeted prolene suture and reconstruct the septal and anterior tricuspid valve leaflets using an autologous pericardial patch. This technique allowed us to perform a complex right-sided defect repair with one patch that might be advantageous in an infective situation. Such a technique might allow an extensive reconstruction of the tricuspid valve, if necessary.

As conclusion, the acquired Gerbode defect a rare form of intracardiac shunt, but its incidence has been increasing during the last decades. Increased numbers of invasive and repeat cardiovascular procedures and infective endocarditis have led to this increase in acquired Gerbode defect. Surgical treatment is always feasible with excellent outcome. However the percutanous transcatheter closure remains an excellent option especially in high risk patients.

Conclusion

The acquired Gerbode defect a rare form of intracardiac shunt, but its incidence has been increasing during the last decades. Increased numbers of invasive and repeat cardiovascular procedures and infective endocarditis have led to this increase in acquired Gerbode defect. Surgical treatment is always feasible with excellent outcome. However the percutanous transcatheter closure remains an excellent option especially in high risk patients.

Consent

Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of the Journal of Cardiothoracic Surgery.

Declarations

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Authors’ Affiliations

(1)
Division of Cardiac Surgery, University Hospital Center of Tirana
(2)
Division of Cardiology, Regional Hospital of Gjakovo
(3)
Division of Heart Disease, Gjakovo Hospital, Rr. Prizren

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