Arq Bras Cardiol abc Arquivos Brasileiros de Cardiologia Arq. Bras. Cardiol. 0066-782X 1678-4170 Sociedade Brasileira de Cardiologia - SBC QbkcqdrvVyvK7zBbmFVnMTs S0066-782X2020001300961 abc.20190829 10.36660/abc.20190829 Imagem Stent Ductal Oscilante na Atresia Pulmonar Valvar 0000-0003-1350-8076 Gopalakrishnan Arun 1 Krishnamoorthy Kavassery Mahadevan 1 Kumar Paidi Suresh 1 Sivasubramonian Sivasankaran 1 Sree Chitra Tirunal Institute for Medical Sciences and Technology – Cardiology Thiruvananthapuram Kerala Índia Sree Chitra Tirunal Institute for Medical Sciences and Technology – Cardiology, Thiruvananthapuram, Kerala – Índia Correspondência: Arun Gopalakrishnan • Sree Chitra Tirunal Institute for Medical Sciences and Technology Medical College P O, 695011, Thiruvananthapuram Kerala – Índia. E-mail: arungopalakrishnan99@gmail.com

Contribuição dos autores

Concepção e desenho da pesquisa: Gopalakrishnan A; Obtenção de dados: Gopalakrishnan A, Kumar OS; Análise e interpretação dos dados: Gopalakrishnan A, Krishnamoorthy KM, Kumar PS, Sivasubramonian S; Redação do manuscrito: Gopalakrishnan A, Kumar PS, Sivasubramonian S; Revisão crítica do manuscrito quanto ao conteúdo intelectual importante: Gopalakrishnan A, Krishnamoorthy KM, Sivasubramonian S.

Potencial conflito de interesses

Declaro não haver conflito de interesses pertinentes.

 07 12 2020 11 2020 115 5 961 963 24 11 2019 18 02 2020 08 04 2020 This is an open-access article distributed under the terms of the Creative Commons Attribution License Palavras-chave Cardiopatias Congênitasas Atresia Pulmonar Anomalia de Ebstein Permeabilidade do Canal Arterial Ecocardiografia/métodos

Um recém-nascido a termo de 2,7 kg foi diagnosticado no terceiro dia de vida com anomalia de Ebstein da válvula tricúspide, regurgitação tricúspide grave, atresia pulmonar valvar e circulação pulmonar ducto-dependente. O índice cardiotorácico era de 90% e o escore GOSE de 1,48. A saturação de oxigênio era de 65% e melhorou para 88% com 0,005 mcg/kg/min de prostaglandina. A pressão sistólica do ventrículo direito era de 40mmHg, com uma pressão arterial sistólica de 65mmHg. A criança foi encaminhada para implante de stent no ducto arterial no 7° dia de vida, após confirmação de atresia pulmonar anatômica. O ducto arterial longo recebeu stents de 3,5x16mm e 3,5x8mm, utilizando um acesso 4F na artéria femoral direita. Ambas as extremidades pulmonar e aórtica estavam bem cobertas e a saturação sistêmica melhorou para 85% com ar ambiente e sem prostaglandina.

A ecocardiografia pós-procedimento confirmou stent bem posicionado e com bom fluxo. No entanto, o stent parecia oscilar a cada ciclo cardíaco (figura 1, vídeo 1). Este “sinal de oscilação do stent” foi confirmado na angiografia aórtica pós-implante de stent (vídeo 2). A atresia pulmonar valvar está associada ao “sinal da gaivota” bem descrito na angiografia. O acúmulo de contraste na artéria pulmonar principal que retém uma ligação fibrosa ao tecido anular pulmonar leva ao sinal da gaivota, com os ramos das artérias pulmonares assumindo o formato de asas de “gaivota”. A atresia pulmonar valvar geralmente está associada a um ducto arterial reto inserido no tronco da artéria pulmonar em metade dos casos.1 Mesmo quando o ducto é longo e tortuoso nos outros, estudos sugerem que ele é mais facilmente manejado durante o implante de stent ductal na atresia pulmonar valvar do que na atresia pulmonar de segmento longo.2,3 A oscilação do stent ductal na atresia pulmonar valvar a cada ciclo cardíaco sugere que a extremidade pulmonar do stent está dentro da artéria pulmonar principal, que é uma estrutura intrapericárdica e reflete fielmente as contrações mecânicas cardíacas.4 Sugerimos que este achado ecocardiográfico pode ser utilizado para avaliação da posição do stent em relação à extremidade pulmonar na atresia pulmonar valvar.

Imagens estáticas da ecocardiografia transtorácica do paciente em projeção paraesternal de eixo curto modificada na sístole (painel 1a) e diástole (painel 1b) mostram diferença marcante no eixo longo do stent no ducto arterial. – A ecocardiografia transtorácica na projeção paraesternal de eixo curto modificada mostra o stent oscilante no ducto arterial. A extremidade aórtica do stent é fixa, enquanto a movimentação vigorosa do segmento da artéria pulmonar principal na atresia pulmonar valvar é responsável pela oscilação. link: <ext-link ext-link-type="uri" xlink:href="http://abccardiol.org/supplementary-material/2020/11505/2019-0829-video-1.mp4">http://abccardiol.org/supplementary-material/2020/11505/2019-0829-video-1.mp4</ext-link> – Fluoroscopia em loop na projeção oblíqua anterior esquerda mostrando o ducto arterial com stent e o “sinal da gaivota” e “sinal do stent oscilante” da atresia pulmonar valvar. link: <ext-link ext-link-type="uri" xlink:href="http://abccardiol.org/supplementary-material/2020/11505/2019-0829-video-2.mp4">http://abccardiol.org/supplementary-material/2020/11505/2019-0829-video-2.mp4</ext-link>

O risco potencial de movimento cíclico do stent foi avaliado e o bebê foi mantido em acompanhamento rigoroso. Ele está bem aos 5 meses de acompanhamento, com bom fluxo de stent, artérias pulmonares de bom tamanho e os movimentos oscilatórios do stent persistem.

Fontes de financiamento

O presente estudo não teve fontes de financiamento externas.

Vinculação acadêmica

Não há vinculação deste estudo a programas de pós-graduação.

 Referências Haddad RN Hanna N Charbel R Daou L Chehab G Saliba Z Ductal stenting to improve pulmonary blood flow in pulmonary atresia with intact ventricular septum and critical pulmonary stenosis after balloon valvuloplasty Cardiol Young 2019 04 29 4 492 498 1. Haddad RN, Hanna N, Charbel R, Daou L, Chehab G, Saliba Z. Ductal stenting to improve pulmonary blood flow in pulmonary atresia with intact ventricular septum and critical pulmonary stenosis after balloon valvuloplasty. Cardiol Young. 2019 Apr;29(4):492–8. Schneider M Zartner P Sidiropoulos A Konertz W Hausdorf G Stent implantation of the arterial duct in newborns with duct-dependent circulation Eur Heart J 1998 09 19 9 1401 1409 2. Schneider M, Zartner P, Sidiropoulos A, Konertz W, Hausdorf G. Stent implantation of the arterial duct in newborns with duct-dependent circulation. Eur Heart J. 1998 Sep;19(9):1401–9. Mortera C Rissech M Bartrons J Carretero J Prada F Ductus Arteriosus Patency With Stenting in Critical Pulmonary Stenosis and Pulmonary Atresia With Intact Interventricular Septum Rev Esp Cardiol 2005 05 01 58 5 592 595 3. Mortera C, Rissech M, Bartrons J, Carretero J, Prada F. Ductus Arteriosus Patency With Stenting in Critical Pulmonary Stenosis and Pulmonary Atresia With Intact Interventricular Septum. Rev Esp Cardiol. 2005 May 1;58(5):592–5. Standring S Gray's Anatomy, The Anatomical Basis of Clinical Practice 41st ed Philadelphia Elsevier Limited 2016 1584 p 4. Standring S. Gray's Anatomy, The Anatomical Basis of Clinical Practice. 41st ed. Philadelphia: Elsevier Limited; 2016. 1584 p. Image Oscillating Ductal Stent in Valvar Pulmonary Atresia 0000-0003-1350-8076 Gopalakrishnan Arun 1 Krishnamoorthy Kavassery Mahadevan 1 Kumar Paidi Suresh 1 Sivasubramonian Sivasankaran 1 Thiruvananthapuram Kerala Índia Sree Chitra Tirunal Institute for Medical Sciences and Technology – Cardiology, Thiruvananthapuram, Kerala – Índia Mailing Address: Arun Gopalakrishnan • Sree Chitra Tirunal Institute for Medical Sciences and Technology Medical College P O, 695011, Thiruvananthapuram Kerala – Índia. E-mail: arungopalakrishnan99@gmail.com

Author Contributions

Conception and design of the research: Gopalakrishnan A; Acquisition of data: Gopalakrishnan A, Kumar OS; Analysis and interpretation of the data: Gopalakrishnan A, Krishnamoorthy KM, Kumar PS, Sivasubramonian S; Writing of the manuscript: Gopalakrishnan A, Kumar PS, Sivasubramonian S; Critical revision of the manuscript for intellectual content: Gopalakrishnan A, Krishnamoorthy KM, Sivasubramonian S.

Potential Conflict of Interest

No potential conflict of interest relevant to this article was reported.

 Keywords Heart Defects, Congenital Pulmonary Atresia Ebstein Anomaly Ductus Arteriosus, Patent Echocardiography/methods

A 2.7 kg term neonate was diagnosed on day 3 of life with Ebstein's anomaly of the tricuspid valve, severe tricuspid regurgitation, valvar pulmonary atresia and duct-dependent pulmonary circulation. The cardiothoracic ratio was 90%, and GOSE score was 1.48. Oxygen saturation was 65% and improved to 88% on 0.005 mcg/kg/min of prostaglandin. The right ventricular systolic pressure was 40mmHg against a systolic blood pressure of 65mmHg. The child was taken up for stenting of the arterial duct on day 7 of life after the confirmation of anatomic pulmonary atresia. The long arterial duct was stented with 3.5x16mm and 3.5x8mm stents, using a 4F right femoral artery access. Both the pulmonary and aortic ends were well covered and systemic saturation improved to 85% on room air and off prostaglandin.

The post-procedure echocardiography confirmed a well-positioned stent with good flow. However, the stent appeared to oscillate with every cardiac cycle (figure 1, video 1). This “oscillating stent sign” was confirmed on the post-stenting aortic angiogram (video 2). Valvar pulmonary atresia is associated with the well-described “seagull sign” on angiogram. Pooling of contrast in the main pulmonary artery that retains a fibrous attachment to pulmonary annular tissue leads to the seagull sign, with the branch pulmonary arteries assuming the shape of the wings of the ‘seagull’. Valvar pulmonary atresia is generally associated with a straight arterial duct inserted into the main pulmonary artery in half of the cases.1 Even when the duct is long and tortuous in the others, studies suggest that it is more readily negotiated during ductal stenting in valvar pulmonary atresia as opposed to long segment pulmonary atresia.2,3 The oscillation of the ductal stent in valvar pulmonary atresia with every cardiac cycle suggests that the pulmonary end of the stent is within the main pulmonary artery, which is an intrapericardial structure and faithfully reflects the mechanical contractions of the heart.4 We suggest that this echocardiographic finding may be utilized for the assessment of stent position with respect to the pulmonary end in valvar pulmonary atresia.

Transthoracic echocardiographic still images of the patient in modified parasternal short axis projection in systole (panel 1a) and diastole (panel 1b) shows marked difference in the long axis of the stent in the arterial duct. – Transthoracic echocardiography in the modified parasternal short axis projection shows the oscillating stent in the arterial duct. The aortic end of the stent is fixed, while the vigorous movement of the main pulmonary artery segment in valvar pulmonary atresia is responsible for the oscillation. link: <ext-link ext-link-type="uri" xlink:href="http://abccardiol.org/supplementary-material/2020/11505/2019-0829-video-1.mp4">http://abccardiol.org/supplementary-material/2020/11505/2019-0829-video-1.mp4</ext-link> – Transthoracic echocardiography in the modified parasternal short axis projection shows the oscillating stent in the arterial duct. The aortic end of the stent is fixed, while the vigorous movement of the main pulmonary artery segment in valvar pulmonary atresia is responsible for the oscillation. link: <ext-link ext-link-type="uri" xlink:href="http://abccardiol.org/supplementary-material/2020/11505/2019-0829-video-2.mp4">http://abccardiol.org/supplementary-material/2020/11505/2019-0829-video-2.mp4</ext-link>

The potential risk of cyclic stent movement was assessed, and the baby was kept on close follow up. He is doing well at 5 months’ follow up with good stent flow, good sized pulmonary arteries and the oscillatory movements of the stent persist.

Sources of Funding

There were no external funding sources for this study.

Study Association

This study is not associated with any thesis or dissertation work.