ABSTRACT
Coração em criss-cross (ou coração entrecruzado) foi descrito pela primeira vez em 1974. Trata-se de uma malformação cardíaca congênita, rara, ocorrendo 8 casos a cada 1.000.000 de crianças, e representando somente 0,1% das malformações congênitas. Os métodos diagnósticos de escolha são o ecocardiograma transtorácico, a ressonância magnética cardíaca (RMC), a angiotomografia (TC) e, eventualmente, o cateterismo cardíaco. Neste relato, descreve-se o caso de um recém-nascido com coração em criss-cross somado à dupla via de saída do ventrículo direito (VD), com vasos mal posicionados, além de comunicação interatrial (CIA), comunicação interventricular (CIV), displasia de valva tricúspide e veia cava superior esquerda persistente. Não se sabe a etiologia exata dessa malformação, mas parece ocorrer pela rotação dos ventrículos em seu eixo longitudinal, não acompanhada das rotações atrial e das valvas atrioventriculares (AV). Esse movimento produz uma alteração das vias de entrada dos ventrículos, determinando que o VD se posicione em plano superior e o esquerdo em plano inferior. Apesar de ainda não se saber a exata causa dessa anomalia, acredita-se que uma alteração genética possa estar levando a esses casos: a mutação do gene Cx43. O diagnóstico do caso em questão foi dado pela ecocardiografia transtorácica e da TC de aorta e artérias pulmonares, que mostraram, além do criss-cross, outras alterações, como dupla via de saída do VD, CIA e CIV amplas.(AU)
Criss-cross heart was first described in 1974. It is a rare congenital heart malformation that occurs in 8 cases per 1,000,000 children, and represents only 0.1% of congenital malformations. The diagnostic methods of choice are transthoracic echocardiography, cardiac magnetic resonance (CMR), computed tomography angiography (CT) and, sometimes, cardiac catheterization. This report describes the case of a newborn with a criss-cross heart in addition to double-outlet right ventricle (RV), with poorly positioned vessels, in addition to atrial septal defect (ASD), interventricular septal defect, tricuspid valve dysplasia and persistent left superior vena cava. The exact etiology of this malformation is not known, but it seems to occur due to rotation of the ventricles in their longitudinal axis, not accompanied by rotation of the atrial and atrioventricular (AV) valves. This movement produces abnormal ventricular inlets, determining that the RV be positioned on a superior plane and the left ventricle on an inferior plane. Although the exact cause of this anomaly is still unknown, it is believed that a genetic abnormality may be leading to these cases: mutation of the Cx43 gene. Diagnosis of the case concerned was given by transthoracic echocardiography and computed CT of the aorta and pulmonary arteries, which showed, in addition to the criss-cross heart, other abnormalities, such as double-outlet RV, large ASD and ventricular septal defect (VSD).(AU)
Subject(s)
Humans , Male , Infant, Newborn , Crisscross Heart/etiology , Crisscross Heart/diagnostic imaging , Heart Defects, Congenital/diagnosis , Heart Ventricles/abnormalities , Double Outlet Right Ventricle/diagnosis , Echocardiography/methods , Cardiac Catheterization/methods , Magnetic Resonance Spectroscopy/methods , Tomography, X-Ray Computed/methods , Persistent Left Superior Vena Cava/diagnosis , Heart Septal Defects, Atrial/diagnosisABSTRACT
Criss-cross heart is an extremely rare anomaly, characterized by an abnormal rotation of the ventricular mass along its major axis. It may be associated with any malformation of the heart segments and connections. Due to the complex structural changes and rarity of the anomaly, the rotation of ventricular axis is often misdiagnosed. In this paper, two cases of criss-cross heart are reported, with emphasis on diagnostic and surgical techniques used to corrected the main defects. A literature review on the subject is also presented which, although sparse, emphasized on the morphologic, diagnostic and surgical aspects of the anomaly.
Coração entrecruzado (criss-cross heart) é uma anomalia extremamente rara, caracterizada por rotação anormal da massa ventricular ao longo do seu eixo maior e pode estar associada com qualquer malformação dos segmentos e das conexões entre as câmaras cardíacas. Devido às alterações estruturais complexas e à raridade da anomalia, essa anomalia de rotação é muitas vezes mal diagnosticada. Neste trabalho são relatados dois casos de coração entrecruzado com ênfase no diagnóstico morfológico e nas técnicas cirúrgicas utilizadas. Foi também realizada revisão da literatura sobre o assunto, que, embora escassa, foi enfatizada quanto à morfologia, diagnóstico, abordagem cirúrgica e possíveis complicações.
Subject(s)
Child , Female , Humans , Infant, Newborn , Male , Crisscross Heart/pathology , Crisscross Heart/surgery , Diagnosis, Differential , Heart Ventricles/pathology , Medical Illustration , Treatment OutcomeABSTRACT
Objective To probe the clinical application of CT in the diagnosis of criss-cross heart(CCH).Methods Five patients with CCH confirmed by operation were retrospectively analyzed.Enhanced 64-slice spiral CT was performed in 2 patients and enhanced single-slice electron beam CT was performed in 3 patients.Three dimensional reconstructions were applied for the fully display of anatomic malformations,and the results were compared with that of echocardiogram and angiocardiogram with Chi-square test Results(1)Visceroatrial situs solitus,twisted and concordant atrioventricular connection,horizontally oriented ventricular septum,ventricular septum defect and pulmonary stenosis were found in all patients on CT.The ventriculoarterial connection was discordant,including double-outlet right ventricle in 4 patients and complete transposition of great arteries in 1 patient In addition,associated anomalies including persistent left superior vena cava(n=2),coarctation of the aorta(n=1)and right aortic arch with right descending aorta(n=1)were detected as well.(2)Total 33 anomalies in 5 cases were found during operation.The diagnostic accuracy of CT,angiocardiogram and echocardiogram was 93.9%(31/33),81.8%(27/33)and 54.5%(18/33)respectively.There was a significant difference between CT and echocardiogram(X~2=13.39,P＜0.01),and no significant difference between CT and angiocardiogram(X~2=1.29,P＞0.05).Conclusion CT is an excellent imaging technique for the diagnosis of CCH.
ABSTRACT
Objective To explore the value of echocardiography in the diagnosis of the criss-cross heart. Methods The echocardiographic results of 6 patients with the criss-cross heart were reviewed retrospectively. The echocardiographic characteristics were analyzed and compared with the surgery results in 4 operated cases. Results The 6 cases were interpreted as representing a criss-cross heart with solitus atria,D-loop ventricles and concordant atrioventricular connections. The ventriculo-arterial alignments of 5 cases were abnormal including double outlet right ventricle with anterior aorta in 2 and transposition of the great arteries in 3 while 1 patient had concordant connection. The following principal characteristics were tilting the transducer from posterior to anterior could demonstrate the connection of the left-sided left atrium and the right-sided left ventricle through mitral valve at first. The more anterior angulation of the transducer then showed the right-sided right atrium was connected to the left-sided right ventricle through tricuspid imaging displayed the two atrial outflows crossed each other without mixing at atrioventricular valve level.right ventricle often occured. The echocardiographic diagnosis of the criss-cross heart and its associated cardiac abnormalities were confirmed by surgery in 4 cases except 1 persistent left superior vena cava was missed. Conclusions The invisibility of a standard 4-chamber view in any cut was very characteristic in the echocardiographic diagnosis of the criss-cross heart. The definitive appearance was the separate display of the two ventricular inlets and the crossed atrioventricular connections with each atrium emptying into the contralateral ventricle by continuous subxiphoid or apical scanning. The transthoracic echocardiography can diagnose this rare heart disease and associated cardiac abnormalities accurately.
ABSTRACT
Objective To describe the characteristics of the criss-cross heart with comprehensively segmental echocardiographic scanning in order to explore the diagnostic value of the echocardiography.Methods Ten cases of criss-cross heart were retrospectively reviewed with 2-dimensional,color Doppler echocardiography to summarize the main diagnostic methods.Results The right ventricle was located superiorly and the left ventricle inferiorly in 9 patients and paralleled alignment in 1.The solitus situs was observed in 9 patients and dextrocardia in 1;the atrioventricular connections were concordant in 8 patients and discordant in 2.Echocardiography revealed that 5 patients presented with transposition of great artery and 5 with double outlet of right ventricle as well as all with ventricular septum defect.Conclusions Clear visualization is available with echocardiography to show the crossing inflow streams at the level of atrial-ventricular valve,abnormal alignment of the ventricular septum as well as the associated abnormalities.