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and severe pulmonary hypertension&#46; There was no dysmorphism and no family history of congenital heart disease&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Echocardiography confirmed situs solitus and levocardia&#46; All four pulmonary veins were imaged forming a confluence which opened directly into the right atrium with a mean gradient of 8 mmHg &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figures 1&#8211;3</a>&#41;&#46; There was no vertical vein and the coronary sinus was normal&#46; Individual pulmonary veins were adequately sized&#46; The ASD was of ostium secundum type&#44; measuring 4&#46;7 mm&#44; with mildly restrictive peak and mean gradients of 5 and 1 mmHg&#44; respectively&#46; Surgical exploration revealed all pulmonary veins draining to the right atrium with a shelf over the opening of the left pulmonary veins&#46; The patient underwent primary sutureless TAPVC repair and closure of the ASD with a tanned pericardial patch&#44; and is doing well&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0020" class="elsevierStylePara elsevierViewall">Supracardiac TAPVC draining into the innominate vein or other channels that connect into the systemic venous atrium is the most common type&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> Classical cardiac-type TAPVCs drain into the coronary sinus&#46; TAPVC with direct connection of the pulmonary veins to the morphological right atrium is exceedingly rare except in the setting of isomeric right atrial appendages&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> In our patient&#44; the muscle-deficient confluence formed by the pulmonary veins draining to the right atrial roof was totally unexpected in the setting of normal atrial situs and normal coronary sinus anatomy&#46; The gradient at the pulmonary venous confluence to the right atrium was flow-related&#44; although the ASD was restrictive&#46; The nomenclature of this defect is itself a point of debate &#8211; though anatomically intracardiac&#44; embryologically it drains into the supracardinal venous system&#44; mimicking a supracardiac TAPVC&#46; Computed tomography is useful to delineate the size of individual pulmonary veins and the dimensions of the common chamber and its drainage in TAPVC&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Magnetic resonance imaging is rarely considered in sick neonates&#44; despite its excellent accuracy&#44; owing to the long duration of the study&#46; However the same information can be obtained fairly reliably in most neonates with TAPVC from careful transthoracic echocardiography&#46; In the case presented&#44; the surgeon was confident of the size of the common chamber and the individual pulmonary veins from the echocardiographic images&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In primary sutureless surgical repair&#44; as performed in our case&#44; the proximity of the common chamber to both atria facilitates excellent results with primary surgical repair&#46; While the initial surgical steps are similar&#44; the required pericardial patch to close the original connection to the right atrium is considerably smaller than with other types of TAPVC&#46; It is to be noted that pulmonary venous drainage from the right lung tracks right beneath this patch&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conclusions</span><p id="par0030" class="elsevierStylePara elsevierViewall">Cardiac TAPVC draining to the right atrium is a rare variant with good surgical results&#46; Anomalous connections to the right atrium can be diagnosed if there is no ascending or descending vein&#44; the coronary sinus is of normal size and the pulmonary veins can be followed to their site of entry to the right atrium&#46; Though it connects directly to the right atrium&#44; embryologically it should be considered a supracardiac TAPVC&#46; Systematic preoperative workup of TAPVC by a detailed echocardiographic assessment including the size of all individual pulmonary veins&#44; the common chamber and ASD&#44; screening for potential flow obstruction&#44; can obviate the need for additional imaging&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0035" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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Case report
A rare variant of intracardiac total anomalous pulmonary venous connection
Uma variante rara da conexão venosa pulmonar anómala total intracardíaca
Arun Gopalakrishnana,
Autor para correspondência
arungopalakrishnan99@gmail.com

Corresponding author.
, Venkateshwaran Subramaniana, Bijulal Sasidharana, Deepa Sasikumara, Kavassery Mahadevan Krishnamoorthya, Baiju S. Dharanb, Ajitkumar Valaparambila
a Sree Chitra Tirunal Institute for Medical Sciences and Technology, Department of Cardiology, Thiruvananthapuram, India
b Sree Chitra Tirunal Institute for Medical Sciences and Technology, Department of Cardiovascular and Thoracic Surgery, Division of Congenital Heart Surgery, Thiruvananthapuram, India
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and severe pulmonary hypertension&#46; There was no dysmorphism and no family history of congenital heart disease&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Echocardiography confirmed situs solitus and levocardia&#46; All four pulmonary veins were imaged forming a confluence which opened directly into the right atrium with a mean gradient of 8 mmHg &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figures 1&#8211;3</a>&#41;&#46; There was no vertical vein and the coronary sinus was normal&#46; Individual pulmonary veins were adequately sized&#46; The ASD was of ostium secundum type&#44; measuring 4&#46;7 mm&#44; with mildly restrictive peak and mean gradients of 5 and 1 mmHg&#44; respectively&#46; Surgical exploration revealed all pulmonary veins draining to the right atrium with a shelf over the opening of the left pulmonary veins&#46; The patient underwent primary sutureless TAPVC repair and closure of the ASD with a tanned pericardial patch&#44; and is doing well&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0020" class="elsevierStylePara elsevierViewall">Supracardiac TAPVC draining into the innominate vein or other channels that connect into the systemic venous atrium is the most common type&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> Classical cardiac-type TAPVCs drain into the coronary sinus&#46; TAPVC with direct connection of the pulmonary veins to the morphological right atrium is exceedingly rare except in the setting of isomeric right atrial appendages&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> In our patient&#44; the muscle-deficient confluence formed by the pulmonary veins draining to the right atrial roof was totally unexpected in the setting of normal atrial situs and normal coronary sinus anatomy&#46; The gradient at the pulmonary venous confluence to the right atrium was flow-related&#44; although the ASD was restrictive&#46; The nomenclature of this defect is itself a point of debate &#8211; though anatomically intracardiac&#44; embryologically it drains into the supracardinal venous system&#44; mimicking a supracardiac TAPVC&#46; Computed tomography is useful to delineate the size of individual pulmonary veins and the dimensions of the common chamber and its drainage in TAPVC&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Magnetic resonance imaging is rarely considered in sick neonates&#44; despite its excellent accuracy&#44; owing to the long duration of the study&#46; However the same information can be obtained fairly reliably in most neonates with TAPVC from careful transthoracic echocardiography&#46; In the case presented&#44; the surgeon was confident of the size of the common chamber and the individual pulmonary veins from the echocardiographic images&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In primary sutureless surgical repair&#44; as performed in our case&#44; the proximity of the common chamber to both atria facilitates excellent results with primary surgical repair&#46; While the initial surgical steps are similar&#44; the required pericardial patch to close the original connection to the right atrium is considerably smaller than with other types of TAPVC&#46; It is to be noted that pulmonary venous drainage from the right lung tracks right beneath this patch&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conclusions</span><p id="par0030" class="elsevierStylePara elsevierViewall">Cardiac TAPVC draining to the right atrium is a rare variant with good surgical results&#46; 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Informação do artigo
ISSN: 08702551
Idioma original: Inglês
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2020 Outubro 54 14 68
2020 Setembro 75 8 83
2020 Agosto 65 8 73
2020 Julho 112 18 130
2020 Junho 70 19 89
2020 Maio 58 4 62
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2020 Janeiro 54 8 62
2019 Dezembro 59 12 71
2019 Novembro 53 9 62
2019 Outubro 54 8 62
2019 Setembro 71 6 77
2019 Agosto 45 15 60
2019 Julho 68 13 81
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2019 Maro 123 10 133
2019 Fevereiro 123 14 137
2019 Janeiro 79 5 84
2018 Dezembro 74 14 88
2018 Novembro 106 8 114
2018 Outubro 184 25 209
2018 Setembro 38 14 52
2018 Agosto 36 12 48
2018 Julho 19 5 24
2018 Junho 35 8 43
2018 Maio 36 10 46
2018 Abril 36 10 46
2018 Maro 37 8 45
2018 Fevereiro 23 7 30
2018 Janeiro 32 15 47
2017 Dezembro 108 40 148
2017 Novembro 8 19 27
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