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two of partial anomalous pulmonary venous connection &#40;PAPVC&#41; with an indolent course&#44; and a neonate with TAPVC requiring urgent surgical intervention&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case reports</span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Case 1 &#40;partial anomalous venous return with infracardiac drainage&#41;</span><p id="par0015" class="elsevierStylePara elsevierViewall">We describe the case of a 14-year-old girl assessed for an incidental finding on chest X-ray &#40;pulmonary asymmetry&#41;&#46; The chest X-ray revealed right pulmonary hypoplasia as well as an anomalous pulmonary vein descending below the diaphragm creating a curved shape on the right side&#44; the scimitar sign&#46; Echocardiography showed dextrocardia with apex on the left&#44; at least two pulmonary veins draining into the left atrium&#44; intact atrial and ventricular septa&#44; no dilation of the cardiac chambers&#44; preserved global biventricular systolic function and no signs of pulmonary hypertension&#46; Computed tomography &#40;CT&#41; confirmed and better characterized these imaging findings &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>A&#41;&#46; Magnetic resonance imaging &#40;MRI&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>B&#41;&#44; in addition to angiographic evaluation&#44; was also important for excluding associated congenital heart disease&#44; and for assessment of right ventricular &#40;RV&#41; systolic function and volume&#44; as well as left-to-right shunting &#40;Qp&#58;Qs 1&#58;2&#41;&#46; Given that the patient was asymptomatic and there was no evidence of cardiac functional impairment&#44; a conservative strategy was adopted&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Case report 2 &#40;partial anomalous venous connection with supracardiac drainage&#41;</span><p id="par0020" class="elsevierStylePara elsevierViewall">A nine-year-old girl was referred to the pediatric cardiology department for a cardiac murmur and Turner syndrome&#46; She was asymptomatic and presented an ejection systolic murmur at the left sternal border&#46; The electrocardiogram &#40;ECG&#41; showed right bundle branch block and the echocardiogram showed no intracardiac shunts&#44; right ventricular dilatation with abnormal motion of the ventricular septum&#44; and normal biventricular function&#46; Cardiac catheterization revealed a normal heart with Qp&#47;Qs 1&#58;1&#46; She continued to be followed in the pediatric cardiology clinic&#46; At the age of 13 years she still presented right ventricular dilatation with an intact interatrial septum&#46; MRI &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>A and B&#41; showed a partial anomalous pulmonary venous connection to the brachiocephalic vein&#44; intact interatrial septum&#44; right ventricular dilatation and Qp&#47;Qs 1&#46;6&#58;1&#46; She underwent cardiac surgery with a good outcome&#46; This case highlights the potential role of MRI in these anomalies and is a reminder that although it is still the gold standard&#44; cardiac catheterization when performed incorrectly may give inaccurate information&#46; One of the key points is to take venous blood samples at various levels in order to obtain precise results&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Case report 3 &#40;total anomalous pulmonary venous connection&#41;</span><p id="par0025" class="elsevierStylePara elsevierViewall">A six-month-old male infant was referred to our hospital for respiratory distress and failure to thrive&#46; Physical examination showed transcutaneous oxygen saturation 90&#37;&#44; respiratory rate 50 cpm&#44; and subcostal and inferior intercostal retractions&#46; Symmetrical and wide peripheral pulses were observed&#44; a faint systolic murmur was audible over the left sternal border&#44; and the liver was palpable 2 cm below the right costal margin&#46; The chest X-ray &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>A&#41; showed cardiomegaly and moderate pulmonary venous congestion&#46; Echocardiography &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>B and C&#41; showed a dilated right atrium and ventricle&#44; a small left atrium and a small ostium secundum type atrial septal defect &#40;ASD&#41;&#46; No pulmonary venous connection to the left atrium could be identified&#44; and there was evidence of an abnormal pulmonary venous confluence behind the left atrium&#46; A diagnosis of TAPVC was made&#46; The patient underwent surgery in order to redirect pulmonary vein flow to the left atrium and to close the ASD&#46; Since then&#44; his growth and development have been appropriate for his age&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Discussion</span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Pulmonary venous developmental anomalies</span><p id="par0030" class="elsevierStylePara elsevierViewall">The development of the pulmonary veins starts early in embryonic life and involves several complicated processes&#46; Pulmonary venous developmental anomalies occur if any of these processes fails to progress properly&#46; Thus&#44; abnormal venous pulmonary drainage may be partial &#40;PAPVC&#41; in cases where only part of the pulmonary venous anatomy is abnormal&#44; or it can involve all the pulmonary veins &#40;TAPVC&#41;&#44; resulting in complete drainage of the pulmonary venous return into the systemic venous circulation&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">TAPVC&#44; as in case report 3&#44; accounts for approximately 1-5&#37; of congenital cardiovascular anomalies&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> This condition is a cause of neonatal cyanosis and may rapidly result in death when blood is not shunted from the pulmonary to the systemic circulation&#46; This shunting typically occurs through an ASD or patent foramen ovale or&#44; less commonly&#44; a patent ductus arteriosus&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">PAPVC is also a relatively uncommon congenital anomaly&#44; found in only 0&#46;5&#8211;0&#46;7&#37; of the general population&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a> Anomalies in veins from the right lung are twice as common as from the left lung&#46; The most common form is one in which a right superior pulmonary vein connects to the right atrium or the superior vena cava&#46; This form is almost always associated with a sinus venosus type ASD&#46; PAPVC with an intact atrial septum&#44; as in case report 2&#44; is a very rare finding&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Anomalous pulmonary venous connections are classified on the basis of the location of pulmonary venous drainage as one of four types&#58; supracardiac&#44; cardiac&#44; infracardiac&#44; or mixed&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">In case report 1&#44; we describe an infracardiac pulmonary drainage known as scimitar syndrome&#44; in which part of the right lung is drained by a pulmonary vein connecting to the inferior vena cava&#46; This anomaly is usually found in combination with hypoplasia of the right lung&#44; pulmonary hypertension and other cardiac defects&#46; Overall&#44; 19-31&#37; of patients with scimitar syndrome have associated cardiac anomalies&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">5</span></a> Its name refers to the tubular opacity typically following the right cardiac border&#44; which resembles the curved Turkish sword known as a scimitar&#46; This syndrome can present early in the neonatal period or later in life with a wide clinical spectrum&#46; When diagnosis is established beyond the neonatal period&#44; symptoms are usually milder or even absent&#44; depending on the degree of lung hypoplasia&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Patients with PAPVC are typically acyanotic and most commonly only mildly symptomatic or asymptomatic&#46; Some authors have suggested that PAPVC becomes clinically significant only when 50&#37; or more of the pulmonary blood flow returns anomalously&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">6</span></a></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Imaging techniques</span><p id="par0060" class="elsevierStylePara elsevierViewall">Imaging techniques&#44; especially CT and MRI&#44; have a pivotal role in the accurate characterization of these abnormalities&#44; predicting outcomes and establishing appropriate preoperative planning&#46; Echocardiography is the initial imaging technique of choice for congenital heart disease&#44; but it has several limitations in the detection and assessment of these anomalies&#46; Besides&#44; pulmonary angiography by right heart catheterization may not reveal the anatomical details of small accessory and anomalous vessels&#46; CT offers the possibility of noninvasive and rapid acquisition with high resolution&#46; Both axial and three-dimensional reconstructed images depict anomalous pulmonary venous structures clearly&#44; with statistically similar detection rates that approach 100&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a> Cardiac gating is not required for the assessment of pulmonary venous structures&#44; although it may prove useful if the patient is being assessed specifically for cor triatriatum or central pulmonary vein hypoplasia or stenosis&#46; The primary disadvantage of CT is that it requires the use of ionizing radiation&#44; which is a major concern particularly in young patients&#46; The radiation exposure from a single diagnostic procedure is usually harmless&#46; However&#44; because of the increased lifetime risk per unit dose for children&#44; radiation can lead to a small&#44; but non-negligible&#44; increase in risk of cancer&#46; The use of ECG-gated dose modulation may limit exposure during the less informative parts of the cardiac cycle&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">MRI has the advantages of not using ionizing radiation and the ability to acquire multiple imaging phases using a single intravenous bolus of gadolinium contrast&#44; and is capable of depicting associated cardiac defects&#46; Moreover&#44; MRI can quantify cardiac volumes and cardiac function&#44; being particularly useful for the assessment of RV function&#44; as well as valve dynamics&#46; A variety of MRI techniques can be used to evaluate the pulmonary venous system&#46; High-resolution double inversion-recovery fast &#40;or turbo&#41; spin echo &#40;or black blood&#41; images are useful for assessing anatomy&#46; Gradient-recalled echo and two-dimensional balanced steady-state free precession provide important information about cardiac chamber and valvular function&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a> Gadolinium-enhanced MRI angiography is an extremely useful technique since it is independent of ECG gating and allows rapid dynamic imaging of thoracic vascular structures&#46; In addition&#44; phase-contrast MRI has been validated as an accurate method for noninvasive quantification of intracardiac shunting&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">7</span></a> Disadvantages of MRI include the amount of time required for image acquisition&#44; the frequent need for patient sedation&#44; and its susceptibility to metal-related artifacts&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a> Therefore&#44; overall&#44; MRI is the preferred imaging technique for the assessment of pulmonary venous anomalies&#44; although the better anatomic detail of CT could prove decisive when choosing which exam to use&#46; Although the standard technique for the assessment of these anomalies has long been cardiac catheterization&#44; they can also be accurately assessed by MRI or CT&#44; and so this invasive exam is frequently unnecessary&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Treatment</span><p id="par0070" class="elsevierStylePara elsevierViewall">Unlike PAPVC&#44; no catheter-corrective treatment is possible for TAPVC&#44; although atrial septostomy is used in some patients when corrective surgery is delayed&#46; In all cases&#44; the goal of surgery is to redirect pulmonary vein flow entirely to the left atrium and to repair associated anomalies&#46; TAPVC is still associated with significant morbidity and mortality&#44; due to the severe hemodynamic and metabolic compromise at presentation&#46; Moreover&#44; 10-15&#37; of patients undergoing repair of TAPVC require multiple interventions due to recurrent stenosis after initial successful correction&#44; with an increasingly poor outcome at each representation&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">8</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">In pediatric patients&#44; PAPVC is usually treated if they have Qp&#58;Qs of 1&#58;1&#46;5 or more as they are more likely to develop pulmonary hypertension and right ventricular failure&#44;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">9</span></a> although this cutoff has not been subject to rigorous study&#46; In adult patients&#44; the criteria for surgical repair are less clear cut&#46; Those who have already developed symptoms due to shunting&#44; or have evidence of right-sided volume overload&#44; can be considered for surgery&#46; However&#44; in asymptomatic patients with a low shunt fraction and no clinical or evidence of right heart overload or pulmonary hypertension&#44; surgery may be unnecessary&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">In cases of PAPVC&#44; there are a number of correction procedures&#44; depending on the number and the site of the anomalous vein or veins&#46; Among several techniques&#44; the Warden method or modified Warden method are usually used&#46; The optimal time for intervention is preschool age&#46; A European Congenital Heart Surgeons Association multicenter study aimed to analyze the surgical results and outcomes of patients who underwent surgery for scimitar syndrome&#44; either by baffling the anomalous drainage into the left atrium via a tunnel or transecting the &#8216;scimitar drainage&#8217; near its entrance into the inferior vena cava and then reimplanting it directly into the left atrium&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a> Their analysis demonstrated that corrective surgery can be done safely with low mortality and morbidity&#44; independently of the type of surgical technique used&#44; especially if conducted prior to the development of pulmonary hypertension&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Percutaneous transcatheter occlusion of an anomalous pulmonary venous connection using coils or an Amplatzer occlusion device has also been reported&#44; although this is only feasible when the anomalous pulmonary veins connect to both the left atrium and the systemic veins&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">11</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Thus&#44; for patients with PAPVC who have already developed mild to moderate pulmonary hypertension&#44; surgical repair is usually safe and effective&#44; although catheter-guided and medical therapies may play an increasing role&#46; Finally&#44; in patients who have already progressed to severe pulmonary hypertension&#44; lung or heart-lung transplantation may be necessary&#46;</p></span></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conclusion</span><p id="par0095" class="elsevierStylePara elsevierViewall">There is a wide spectrum of pulmonary venous developmental anomalies&#46; Their exact drainage pattern and associated complications can be identified by different imaging techniques&#46; CT and MRI are noninvasive imaging techniques that play an increasingly important role in the assessment of these patients&#46;</p><p id="par0100" class="elsevierStylePara elsevierViewall">Most patients with an anomalous pulmonary venous connection are treated surgically if they are symptomatic or if they have significant left-to-right shunting&#46; Percutaneous treatment is occasionally possible in PAPVC&#46; Physicians who diagnose and treat adult patients with pulmonary hypertension&#44; right chamber dilatation with signs of volume overload and an intact atrial septum should always consider abnormal venous pulmonary drainage as a potential etiology&#46;</p></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Ethical disclosures</span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Protection of human and animal subjects</span><p id="par0105" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Confidentiality of data</span><p id="par0110" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Right to privacy and informed consent</span><p id="par0115" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Conflicts of interest</span><p id="par0120" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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            0 => "Conex&#227;o an&#243;mala das veias pulmonares"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Anomalous pulmonary venous connection is an uncommon congenital anomaly in which all &#40;total form&#41; or some &#40;partial form&#41; pulmonary veins drain into a systemic vein or into the right atrium rather than into the left atrium&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">The authors present one case of total anomalous pulmonary venous connection and two cases of partial anomalous pulmonary venous connection&#44; one of supracardiac drainage into the brachiocephalic vein&#44; and the other of infracardiac anomalous venous drainage &#40;scimitar syndrome&#41;&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Through the presentation of these cases&#44; this article aims to review the main pulmonary venous developmental defects&#44; highlighting the role of imaging techniques in the assessment of these anomalies&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A conex&#227;o an&#243;mala das veias pulmonares &#233; uma anomalia cong&#233;nita rara na qual todas as veias pulmonares &#40;forma total&#41; ou algumas &#40;forma parcial&#41; drenam numa veia sist&#233;mica ou na aur&#237;cula direita&#44; em vez da aur&#237;cula esquerda&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Os autores apresentam um caso de conex&#227;o an&#243;mala total das veias pulmonares e dois casos de retorno an&#243;malo parcial das veias pulmonares&#44; uma drenagem supracard&#237;aca ao nivel da veia braquiocef&#225;lica e uma infracard&#237;aca &#40;s&#237;ndrome da cimitarra&#41;&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Atrav&#233;s da apresenta&#231;&#227;o destes casos&#44; este artigo pretende fazer uma revis&#227;o dos principais defeitos do desenvolvimento venoso pulmonar e real&#231;ar a import&#226;ncia das t&#233;cnicas de imagem na avalia&#231;&#227;o destas anomalias&#46;</p></span>"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Coronal-reformatted contrast-enhanced computed tomography showing the scimitar vein draining into the inferior vena cava &#40;arrow&#41; as well as right pulmonary hypoplasia with left lung expansion&#59; &#40;B&#41; sagittal four-dimensional magnetic resonance imaging angiographic image also demonstrating the scimitar vein &#40;orange arrow&#41;&#46;</p>"
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          "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Three-dimensional and &#40;B&#41; four-dimensional magnetic resonance angiographic images showing the anomalous left superior pulmonary vein &#40;arrow&#41; draining into the brachiocephalic vein &#40;BV&#41;&#46; Ao&#58; aorta&#59; JV&#58; jugular vein&#59; SVC&#58; superior vena cava&#46;</p>"
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          "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Chest X-ray showing cardiomegaly and moderate pulmonary venous congestion&#59; &#40;B&#41; dilatation of the right chambers&#59; &#40;C&#41; total anomalous pulmonary venous connection&#44; with no communication between the pulmonary veins and left atrium and a common vertical vein &#40;arrow&#41;&#46; la&#58; left atrium&#59; lv&#58; left ventricle&#59; ra&#58; right atrium&#59; rv&#58; right ventricle&#46;</p>"
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                            0 => "U&#46; Gudjonsson"
                            1 => "J&#46;W&#46; Brown"
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                        "tituloSerie" => "Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu"
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                        "volumen" => "9"
                        "paginaInicial" => "56"
                        "paginaFinal" => "62"
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                      "titulo" => "Congenital heart disease&#58; prevalence at livebirth&#58; the Baltimore-Washington Infant Study"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "C&#46; Ferencz"
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                            2 => "R&#46;J&#46; McCarter"
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                        ]
                      ]
                    ]
                  ]
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