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heart&#44; auditory system&#44; and extremities &#40;type II MRKH or MURCS association&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">1&#8211;3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Herein&#44; we present percutaneous closure of an isolated secundum atrial septal defect &#40;ASD&#41; in a patient with MRKH syndrome&#46; According to the medical literature&#44; this therapeutic intervention has not previously been reported in this syndrome&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">A 26-year-old female patient was referred to the cardiology department with complaints of increasing shortness of breath&#44; palpitations&#44; and effort dyspnea&#46; The patient had been diagnosed with MRKH syndrome eight years previously&#46; In order to determine the complete anatomy and associated anomalies&#44; abdominopelvic ultrasound and magnetic resonance imaging&#44; radiological vertebral assessment&#44; and temporal computed tomography imaging were performed&#46; These imaging modalities revealed that the location and size of the ovaries were normal&#44; but the uterus and the upper part of the vagina were absent &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46; There was no other imaging abnormality&#46; Audiometric examination was normal&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">During physical examination&#44; a fixed split second heart sound was heard&#46; Electrocardiography was normal&#44; but transthoracic echocardiography &#40;TTE&#41; showed a flattened interventricular septum&#44; dilated right heart chambers &#40;right ventricle 32 mm&#44; right atrium 44 mm&#215;42 mm&#41;&#44; increased systolic pulmonary arterial pressure &#40;50 mmHg&#41;&#44; and mild tricuspid regurgitation&#46; Due to suspicion of left-to-right shunting&#44; transesophageal echocardiography &#40;TEE&#41; was performed&#44; which revealed an ostium secundum ASD with a defect dimension of 17 mm &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#44; <a class="elsevierStyleCrossRef" href="#sec0050">Video 1</a>&#41;&#46; No other congenital heart abnormality was identified&#46; The defect was deemed to be suitable for percutaneous transcatheter closure&#46; The procedure was performed under general anesthesia with TEE guidance&#46; A 26 mm ASD septal occluder device &#40;Lifetech Cera ASD Occluder&#44; Occlutech International AB&#44; Helsingborg&#44; Sweden&#41; was deployed under fluoroscopic and TEE guidance &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#44; <a class="elsevierStyleCrossRef" href="#sec0050">Video 2</a>&#41;&#46; Cessation of flow across the interatrial septum was confirmed by TEE prior to final deployment of the device&#46; TTE was performed 24 hours after implantation and confirmed proper positioning of the device with no significant residual shunting&#46; Recovery was uneventful and the patient was discharged from the hospital under antiplatelet therapy&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">ASD&#44; especially ostium secundum type&#44; is a common congenital heart defect in adults&#46; It occurs as a result of defective development of the interatrial septum&#46; Most cases are isolated and sporadic&#44; but some are associated with genetic syndromes such as MRKH syndrome&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">MRKH syndrome is a clinical condition of M&#252;llerian agenesis&#46; While its etiology remains unclear&#44; there is some evidence concerning its embryogenic and genetic origin&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">1&#8211;3</span></a> It is reported to be associated with cardiovascular disorders&#46; Pittock et al&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> described cardiac anomalies in patients with MRKH syndrome&#44; including truncus arteriosus&#44; patent ductus arteriosus&#44; patent foramen ovale&#44; mitral valve prolapse&#44; and mild mitral regurgitation&#46; Vasquez et al&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a> reported an adult woman with total anomalous pulmonary venous return&#44; ASD and MRKH syndrome&#44; while Kula et al&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a> suggested an association between pulmonary stenosis and MRKH syndrome&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Herein&#44; we present percutaneous closure of isolated ostium secundum ASD in a patient with MRKH syndrome&#46; According to the medical literature&#44; this therapeutic intervention has not previously been reported in this syndrome&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Ethical disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Protection of human and animal subjects</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association &#40;Declaration of Helsinki&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Confidentiality of data</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Right to privacy and informed consent</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors have obtained the written informed consent of the patients or subjects mentioned in the article&#46; The corresponding author is in possession of this document&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A s&#237;ndrome de Mayer-Rokitansky-Kuster-Hauser &#40;MRKH&#41; &#233; uma anomalia cong&#233;nita rara&#44; caraterizada por uma aplasia completa ou parcial do &#250;tero e da parte superior da vagina&#46; Tem sido associado a problemas cardiovasculares&#44; incluindo comunica&#231;&#227;o interauricular&#44; retorno venoso pulmonar an&#243;malo&#44; janela aorto-pulmonar&#44; estenose valvular pulmonar&#44; prolapso da v&#225;lvula mitral&#44; tetralogia de Fallot&#44; tronco arterial e persist&#234;ncia do canal arterial&#46; Pela primeira vez na literatura m&#233;dica&#44; apresentamos um caso de encerramento percut&#226;neo de comunica&#231;&#227;o interauricular tipo <span class="elsevierStyleItalic">ostium secundum</span> isolado nesta s&#237;ndrome&#46;</p></span>"
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Case report
Percutaneous closure of isolated ostium secundum-type atrial septal defect in a patient with Mayer-Rokitansky-Küster-Hauser syndrome
Encerramento percutâneo da comunicação interauricular tipo ostium secundum isolado em doente com síndrome de Mayer-Rokitansky-Kuster-Hauser (MRKH)
Murat Akcaya,
Autor para correspondência
drmuratakcay@hotmail.com

Corresponding author.
, Okan Gulelb, Korhan Soylub, Murat Mericb, Muzaffer Elmalic
a Cardiology Clinic, Terme State Hospital, Samsun, Turkey
b Department of Cardiology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey
c Department of Radiology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey
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abdominopelvic ultrasound and magnetic resonance imaging&#44; radiological vertebral assessment&#44; and temporal computed tomography imaging were performed&#46; These imaging modalities revealed that the location and size of the ovaries were normal&#44; but the uterus and the upper part of the vagina were absent &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46; There was no other imaging abnormality&#46; Audiometric examination was normal&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">During physical examination&#44; a fixed split second heart sound was heard&#46; Electrocardiography was normal&#44; but transthoracic echocardiography &#40;TTE&#41; showed a flattened interventricular septum&#44; dilated right heart chambers &#40;right ventricle 32 mm&#44; right atrium 44 mm&#215;42 mm&#41;&#44; increased systolic pulmonary arterial pressure &#40;50 mmHg&#41;&#44; and mild tricuspid regurgitation&#46; Due to suspicion of left-to-right shunting&#44; transesophageal echocardiography &#40;TEE&#41; was performed&#44; which revealed an ostium secundum ASD with a defect dimension of 17 mm &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#44; <a class="elsevierStyleCrossRef" href="#sec0050">Video 1</a>&#41;&#46; No other congenital heart abnormality was identified&#46; The defect was deemed to be suitable for percutaneous transcatheter closure&#46; The procedure was performed under general anesthesia with TEE guidance&#46; A 26 mm ASD septal occluder device &#40;Lifetech Cera ASD Occluder&#44; Occlutech International AB&#44; Helsingborg&#44; Sweden&#41; was deployed under fluoroscopic and TEE guidance &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#44; <a class="elsevierStyleCrossRef" href="#sec0050">Video 2</a>&#41;&#46; Cessation of flow across the interatrial septum was confirmed by TEE prior to final deployment of the device&#46; TTE was performed 24 hours after implantation and confirmed proper positioning of the device with no significant residual shunting&#46; Recovery was uneventful and the patient was discharged from the hospital under antiplatelet therapy&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">ASD&#44; especially ostium secundum type&#44; is a common congenital heart defect in adults&#46; It occurs as a result of defective development of the interatrial septum&#46; Most cases are isolated and sporadic&#44; but some are associated with genetic syndromes such as MRKH syndrome&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">MRKH syndrome is a clinical condition of M&#252;llerian agenesis&#46; While its etiology remains unclear&#44; there is some evidence concerning its embryogenic and genetic origin&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">1&#8211;3</span></a> It is reported to be associated with cardiovascular disorders&#46; Pittock et al&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> described cardiac anomalies in patients with MRKH syndrome&#44; including truncus arteriosus&#44; patent ductus arteriosus&#44; patent foramen ovale&#44; mitral valve prolapse&#44; and mild mitral regurgitation&#46; Vasquez et al&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a> reported an adult woman with total anomalous pulmonary venous return&#44; ASD and MRKH syndrome&#44; while Kula et al&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a> suggested an association between pulmonary stenosis and MRKH syndrome&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Herein&#44; we present percutaneous closure of isolated ostium secundum ASD in a patient with MRKH syndrome&#46; According to the medical literature&#44; this therapeutic intervention has not previously been reported in this syndrome&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Ethical disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Protection of human and animal subjects</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association &#40;Declaration of Helsinki&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Confidentiality of data</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Right to privacy and informed consent</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors have obtained the written informed consent of the patients or subjects mentioned in the article&#46; The corresponding author is in possession of this document&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Mayer-Rokitansky-K&#252;ster-Hauser &#40;MRKH&#41; syndrome is a rare congenital anomaly characterized by complete or partial aplasia of the uterus and the upper part of the vagina&#46; It is reported to be associated with cardiovascular disorders including atrial septal defect&#44; anomalous pulmonary venous return&#44; aortopulmonary window&#44; pulmonary valve stenosis&#44; mitral valve prolapse&#44; tetralogy of Fallot&#44; truncus arteriosus&#44; and patent ductus arteriosus&#46; Herein&#44; for the first time in the medical literature&#44; we present percutaneous closure of an isolated ostium secundum atrial septal defect in this syndrome&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A s&#237;ndrome de Mayer-Rokitansky-Kuster-Hauser &#40;MRKH&#41; &#233; uma anomalia cong&#233;nita rara&#44; caraterizada por uma aplasia completa ou parcial do &#250;tero e da parte superior da vagina&#46; Tem sido associado a problemas cardiovasculares&#44; incluindo comunica&#231;&#227;o interauricular&#44; retorno venoso pulmonar an&#243;malo&#44; janela aorto-pulmonar&#44; estenose valvular pulmonar&#44; prolapso da v&#225;lvula mitral&#44; tetralogia de Fallot&#44; tronco arterial e persist&#234;ncia do canal arterial&#46; Pela primeira vez na literatura m&#233;dica&#44; apresentamos um caso de encerramento percut&#226;neo de comunica&#231;&#227;o interauricular tipo <span class="elsevierStyleItalic">ostium secundum</span> isolado nesta s&#237;ndrome&#46;</p></span>"
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                      "titulo" => "The Mayer-Rokitansky-K&#252;ster-Hauser syndrome &#40;congenital absence of uterus and vagina&#41; &#8211; phenotypic manifestations and genetic approaches"
                      "autores" => array:1 [
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                            0 => "D&#46; Guerrier"
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2021 Setembro 38 30 68
2021 Agosto 54 44 98
2021 Julho 28 26 54
2021 Junho 46 20 66
2021 Maio 42 34 76
2021 Abril 63 84 147
2021 Maro 79 19 98
2021 Fevereiro 67 21 88
2021 Janeiro 53 15 68
2020 Dezembro 45 6 51
2020 Novembro 38 20 58
2020 Outubro 26 16 42
2020 Setembro 79 12 91
2020 Agosto 27 9 36
2020 Julho 55 10 65
2020 Junho 58 17 75
2020 Maio 45 10 55
2020 Abril 48 9 57
2020 Maro 48 9 57
2020 Fevereiro 195 16 211
2020 Janeiro 80 10 90
2019 Dezembro 62 12 74
2019 Novembro 39 9 48
2019 Outubro 63 13 76
2019 Setembro 50 4 54
2019 Agosto 56 13 69
2019 Julho 49 9 58
2019 Junho 39 9 48
2019 Maio 62 9 71
2019 Abril 31 19 50
2019 Maro 119 14 133
2019 Fevereiro 40 10 50
2019 Janeiro 43 10 53
2018 Dezembro 78 19 97
2018 Novembro 108 9 117
2018 Outubro 228 16 244
2018 Setembro 57 11 68
2018 Agosto 33 17 50
2018 Julho 35 2 37
2018 Junho 70 7 77
2018 Maio 67 11 78
2018 Abril 104 11 115
2018 Maro 85 6 91
2018 Fevereiro 62 2 64
2018 Janeiro 83 6 89
2017 Dezembro 95 14 109
2017 Novembro 49 12 61
2017 Outubro 38 13 51
2017 Setembro 35 10 45
2017 Agosto 41 14 55
2017 Julho 37 11 48
2017 Junho 38 11 49
2017 Maio 35 8 43
2017 Abril 23 4 27
2017 Maro 42 10 52
2017 Fevereiro 33 4 37
2017 Janeiro 51 13 64
2016 Dezembro 88 52 140
2016 Novembro 7 14 21
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