Infant Barlow’s Disease in Association with Atrial Septal Defect

Clinical data: Female, seven years old, referred to our service complaining about congestive heart failure symptoms due to mitral valve regurgitation and atrial septal defect. Technical description: Echocardiographic findings compatible with Barlow’s disease and atrial septal defect, ostium secundum type. Operation: She was submitted to mitral valvuloplasty with chordal shortening and prosthetic posterior ring (Gregori-Braile®) along with patch atrioseptoplasty. Comments: Mitral valve regurgitation is a rare congenital heart disease and Barlow’s disease is probably rarer. Mitral valve repair is the treatment of choice.

Keywords:
Mitral Valve Insufficiency; Mitral Valve Annuloplasty; Congenital Heart Defect; Atrial Septal Defect; Thoracic Surgery

Authorship

Isaac Azevedo Silva

Substantial contributions to the conception or design of the work

or the acquisition

analysis

or interpretation of data for the work

drafting the work or revising it critically for important intellectual content

final approval of the version to be published

CardioPedBrasil® - Centro do Coração da Criança at Hospital da Criança e Maternidade São José do Rio Preto, São Paulo, Brazil (FUNFARME/FAMERP)Hospital da Criança e Maternidade São José do Rio PretoBrazilSão Paulo, BrazilCardioPedBrasil® - Centro do Coração da Criança at Hospital da Criança e Maternidade São José do Rio Preto, São Paulo, Brazil (FUNFARME/FAMERP)

Larissa Ales Leite Matos

Substantial contributions to the conception or design of the work

or the acquisition

analysis

or interpretation of data for the work

drafting the work or revising it critically for important intellectual content

final approval of the version to be published

Carolina Sant’Anna

Substantial contributions to the conception or design of the work

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analysis

or interpretation of data for the work

drafting the work or revising it critically for important intellectual content

final approval of the version to be published

Ulisses Alexandre Croti Correspondence Address: Ulisses Alexandre Crotihttps://orcid.org/0000-0002-1127-4782, CardioPedBrasil - Centro do Coração da Criança, Hospital da Criança e Maternidade de São José do Rio Preto - FUNFARME/FAMERP. Av. Jamil Feres Kfouri, 60 - Jd. Panorama - São José do Rio Preto, SP, Brazil, Zip Code: 15091-240, E-mail: ulissesacroti@gmail.com

Substantial contributions to the conception or design of the work

or the acquisition

analysis

or interpretation of data for the work

revising the work

final approval of the version to be published

https://orcid.org/0000-0002-1127-4782

Correspondence Address: Ulisses Alexandre Crotihttps://orcid.org/0000-0002-1127-4782, CardioPedBrasil - Centro do Coração da Criança, Hospital da Criança e Maternidade de São José do Rio Preto - FUNFARME/FAMERP. Av. Jamil Feres Kfouri, 60 - Jd. Panorama - São José do Rio Preto, SP, Brazil, Zip Code: 15091-240, E-mail: ulissesacroti@gmail.com

No conflict of interest.

SCIMAGO INSTITUTIONS RANKINGS

Figures | Tables

Figures (6)
Tables (1)

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Fig. 1
Chest radiography with prominent pulmonary vascular markings and mild cardiomegaly.

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Fig. 2
Apical four-chamber transesophageal echocardiogram, two-dimensional view. A) Imaging with probe rotated toward right-sided structures, showing dropout in mid-septum between the left atrium and right atrium consistent with ostium secundum atrial septal defect (ASD). B) Addition of color Doppler shows left-to-right shunt through the ASD.

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Fig. 3
Transesophageal echocardiogram, two-dimensional view of mitral valve. A) Prolapsed, thickened, and elongated chordae tendineae. B) Failure of coaptation and billowing anterior segments (A2 and A3) and posterior scallops (P2 and P3). C) Addition of color Doppler shows severe mitral regurgitation.

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Fig. 4
Three-dimensional echocardiogram image of the mitral valve from the left atrial perspective. Color Doppler demonstrates valve regurgitation (arrow) (A) and left-to-right shunt through the atrial septal defect (arrow) (B).

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Fig. 5
Three-dimensional image from the left atrial perspective demonstrating features typical of Barlow’s disease with severe mitral regurgitation, annular dilation, leaflet redundancy, and multisegmental prolapsing/billowing mitral valve components.

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Fig. 6
Surgical aspect of the mitral valve from atrial view. A) After atriotomy, with ring repair sutures in place (note diffuse thickening of the anterior leaflet with failure of coaptation between A2-A3 and P2-P3). B) Imaging after implantation of a rigid 26 mm posterior ring (Gregori-Braile®). C) Final aspect with ring in place and proper coaptation of the leaflets after testing with saline solution (NaCl 0.9%).

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Abbreviations, Acronyms & Symbols ASD = Atrial septal defect BD = Barlow’s disease MV = Mitral valve

Fig. 1 Chest radiography with prominent pulmonary vascular markings and mild cardiomegaly.

Fig. 2 Apical four-chamber transesophageal echocardiogram, two-dimensional view. A) Imaging with probe rotated toward right-sided structures, showing dropout in mid-septum between the left atrium and right atrium consistent with ostium secundum atrial septal defect (ASD). B) Addition of color Doppler shows left-to-right shunt through the ASD.

Fig. 3 Transesophageal echocardiogram, two-dimensional view of mitral valve. A) Prolapsed, thickened, and elongated chordae tendineae. B) Failure of coaptation and billowing anterior segments (A2 and A3) and posterior scallops (P2 and P3). C) Addition of color Doppler shows severe mitral regurgitation.

Fig. 4 Three-dimensional echocardiogram image of the mitral valve from the left atrial perspective. Color Doppler demonstrates valve regurgitation (arrow) (A) and left-to-right shunt through the atrial septal defect (arrow) (B).

Fig. 5 Three-dimensional image from the left atrial perspective demonstrating features typical of Barlow’s disease with severe mitral regurgitation, annular dilation, leaflet redundancy, and multisegmental prolapsing/billowing mitral valve components.

Fig. 6 Surgical aspect of the mitral valve from atrial view. A) After atriotomy, with ring repair sutures in place (note diffuse thickening of the anterior leaflet with failure of coaptation between A2-A3 and P2-P3). B) Imaging after implantation of a rigid 26 mm posterior ring (Gregori-Braile®). C) Final aspect with ring in place and proper coaptation of the leaflets after testing with saline solution (NaCl 0.9%).

Abbreviations, Acronyms & Symbols
ASD	= Atrial septal defect
BD	= Barlow’s disease
MV	= Mitral valve

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Infant Barlow’s Disease in Association with Atrial Septal Defect

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[1] Correspondence Address: Ulisses Alexandre Crotihttps://orcid.org/0000-0002-1127-4782, CardioPedBrasil - Centro do Coração da Criança, Hospital da Criança e Maternidade de São José do Rio Preto - FUNFARME/FAMERP. Av. Jamil Feres Kfouri, 60 - Jd. Panorama - São José do Rio Preto, SP, Brazil, Zip Code: 15091-240, E-mail: ulissesacroti@gmail.com