Prenatal diagnosis of criss-cross heart - case series and review of the literature.

OBJECTIVE: This study aimed to improve the accuracy of prenatal diagnosis by analyzing fetal echocardiographic features of criss-cross heart (CCH), to provide an effective basis for the development of management strategies and improve the prognosis of patients. METHODS: A retrospective analysis was performed on CCH cases diagnosed prenatally at our center between July 2016 and June 2022. Clinical data and prenatal fetal echocardiographic images were reviewed. Literature on prenatal diagnosis of CCH was searched from January 2000 to December 2023 in the PubMed database. RESULTS: Fourteen (0.03%) CCH cases were diagnosed from a database of fetal echocardiograms of 41354 cases at our center. The prenatal genetic testing results were normal in 10 cases and 4 cases didn't check. All cases underwent termination of pregnancy. All cases showed crossed ventricular inflow tracts and combined with other cardiac structural abnormalities. A total of eight articles containing 25 cases were found in the literature review and all cases were associated with other cardiac structural abnormalities. CONCLUSION: Prenatal echocardiography is the primary tool for fetal diagnosis of CCH. Continuous scanning helps avoid missing data and misdiagnosis.

Coração em Criss-Cross: um relato de caso / Criss-Cross Heart: a case report

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)

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.

OBJECTIVE: Criss-cross heart (CCH) is a rare congenital cardiac defect defined by crossing of ventricular inflow streams contributing to less than 0.1 % of all congenital heart anomalies. Due to its rarity and complexity, prenatal diagnosis in these patients remains challenging. We sought to describe prenatal findings and postnatal course in eight cases of prenatally diagnosed CCH. METHODS: This is a retrospective case series of prenatally diagnosed CCH in three centers between 2010-2017. We reviewed fetal echocardiograms as well as postnatal clinical charts and surgical reports. RESULTS: 8 cases of CCH were included. The median gestational age at diagnosis was 27 weeks. 7 patients were found with situs solitus, one fetus with situs ambiguous. In all patients, the four-chamber view was abnormal. There was atrioventricular discordance in half of the patients, while all patients showed ventriculoarterial discordance. All patients were found with additional cardiac anomalies, including ventricular septal defect, double outlet right ventricle, right aortic arch, atrial septal defect and pulmonary stenosis. Three patients underwent amniocentesis without pathological findings. All patients were born alive at a median gestational age of 38 + 2 weeks and survived our median follow-up of 181 days. CONCLUSION: CCH can be diagnosed prenatally by detailed fetal echocardiography when observing an abnormal four-chamber view with crossing of inflow streams into both ventricles and a lack of parallel orientation of the atrioventricular valve axis due to a clockwise or counterclockwise rotation of the ventricular mass along its axis. With the help of prenatal ultrasound, parental guidance and counselling as well as postnatal pediatric cardiac management can be warranted.

Imaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography.

AIM: To evaluate the imaging characteristics and associations in patients with twisted atrioventricular connections on multidetector computed tomography (CT) angiography. MATERIAL AND METHODS: We retrospectively reviewed 2605 CT angiography studies performed for suspected congenital heart diseases in our institution from January 2014 to December 2018. Twisted atrioventricular connections were diagnosed in 12 patients. Segmental sequential approach developed by Anderson et al was employed to characterize the complex congenital heart disease. Ventricular topology was also determined. CTA was also assessed to look for any associated intra- and extracardiac anomalies. RESULTS: Out of 12 patients with twisted atrioventricular connections, usual viscero-atrial arrangement was seen in nine patients, two patients had mirror-imaged viscero-atrial arrangement and one patient had mirror-imaged atrial arrangement. Right-sided heart was seen in four patients. Two patients had discordant atrioventricular connections, seven had concordant atrioventricular connections while three showed double-inlet right ventricle. Ventriculoarterial connections were concordant in two patients, discordant in one patient while nine patients had double-outlet right ventricle. Superior-inferior ventricular morphology with near-horizontal interventricular septum was seen in four patients. Left-sided aortic arch with normal branching pattern was observed in nine patients. No coronary anomaly was seen in any patient. Other associations included ventricular and atrial septal defects, pulmonic stenosis, hypoplasia/atresia of tricuspid valve, straddling of mitral valve, and duplicated superior caval veins. CONCLUSION: CT angiography is useful in diagnosis of twisted atrioventricular connections with accurate identification of viscero-atrial arrangement, atrioventricular and ventriculoarterial connections, and orientation and presence or absence of associated anomalies.

Criss-cross heart with double-outlet right ventricle, subpulmonary ventricular septal defect, and bicuspid pulmonary valve.

Criss-cross heart (CCH) is an extremely rare complex congenital heart malformation. It accounts for less than 0.1% of congenital heart diseases. Here, we describe a unique case of CCH with double-outlet right ventricle, huge subpulmonary ventricular septal defect, bicuspid pulmonary valve, and right-hand aortic arch. The anatomic features were observed with echocardiography, and the diagnosis was confirmed at surgery. Many variations of CCH have been described. The present case expands the spectrum of this entity and may provide new insight into this complex anatomy.

Never trust first impressions when assessing the heart of a pregnant woman.

We describe the echocardiographic finding of a 35-year-old pregnant woman, referred for a clinical consultation at our institution because of congestive heart failure. She underwent echocardiography with only little information about a history of cardiac surgery during infancy. At the first sight, parasternal long axis view demonstrates normal structures, but on the apical view we diagnosed a very rare condition with typical imaging findings: Criss Cross heart.

Prenatal Diagnosis of Criss-Cross Heart Using 4-Dimensional Color Doppler Rendering.

Criss-cross heart is a rare congenital cardiac anomaly characterized by the crossing of two ventricular inflow streams. We have demonstrated the utility of 4-dimensional color Doppler rendering in diagnosing the criss-cross heart in a fetus. Four-dimensional color Doppler rendering can demonstrate the relative direction of intracardiac blood flows and facilitate recognition of the crossover of inflow streams in the same plane, confirming the criss-cross heart diagnosis in the fetus.

Crisscross heart with situs invesus in pregnancy: A rare case report and literature review.

Crisscross heart(CCH) is a rare congenital anomaly. CCH is always with rare complex congenital heart malformations, and for this reason it is difficult to diagnose. We present an asymptomatic 29-year-old pregant woman with history of cardiac murmur at 28 weeks of gestation. Transthoracic echocardiography revealed a CCH with situs inversus, concordant atrioventricular, double outlet right ventricle, ventricular septum defect and pulmonary stenosis. The anomalies were demonstrated by two-dimensional echocardiography and confirmed by color Doppler imaging. The patient was stable and did not require surgical interventions. She underwent successful term vaginal delivery without complication and her baby had a normal heart. Patients with CCHs can be diagnosed by a careful, systematic study with two-dimensional and Doppler echocardiography to identify the ventricular morphology and position, determine the sequence of connections and spatial relationships of the cardiac chambers and identify other abnormalities.

Prenatal Diagnosis of the Criss-Cross Heart.

We sought to describe the prenatal features and postnatal outcomes of the "criss-cross heart" (CCH) with crossing atrioventricular connections as encountered in 5 cases and to review the literature regarding fetal diagnoses of this lesion. We reviewed the clinical history and fetal echocardiograms of 5 patients with a diagnosis of CCH encountered in our institution from 2010 to 2015. Affected pregnancies were identified through the University of Alberta fetal cardiology database. Fetal and postnatal echocardiograms, prenatal and postnatal clinical records, autopsy, and surgical reports were reviewed. Over the past 5 years, major fetal congenital heart disease was identified in 448 pregnancies in the province of Alberta. Of these, 5 had CCH. All 5 were referred for suspected congenital heart disease, and none had significant extracardiac pathology. Detailed methodical 2-dimensional and color Doppler fetal echocardiography defined the complex diagnoses that were subsequently confirmed postnatally (n = 4) and at fetal autopsy (n = 1). Prenatal diagnosis correctly predicted postnatal presentation and outcomes in 1 with double outlet right ventricle and coarctation, 2 presenting with complete (D) transposition of the great arteries physiology, and 1 presenting as corrected (L) transposition. Three required single ventricle palliation and 1 underwent an arterial switch procedure. In conclusion, detailed evaluation of fetal CCH can result in correct anatomical and pathophysiological diagnoses. It demands a full understanding of the anatomy and pathophysiology to predict accurately the presentation at birth for accurate counseling and planning perinatal management.

Prenatal diagnosis of criss-cross heart: sonographical and pathological features of five cases.

OBJECTIVE: To describe the sonographical and pathological features of fetal criss-cross heart (CCH). STUDY DESIGN: All cases of fetal CCH diagnosed by fetal echocardiogram from May 2003-May 2011 were identified at a single referral center using an established perinatal database. Demographic and genetic information, sonographical images and autopsy reports were reviewed. Sonographical and pathological features are described. RESULT: Five cases of fetal CCH were identified, all of which were confirmed by autopsy. Characteristic sonographical findings include: (1) the inability to obtain four-chamber view at standard transverse plane through the fetal chest; (2) appreciation of the misaligned spatial atrial-ventricle connection with the interventricular septum in a 'spiraling' orientation; (3) orientation of the two ventricular inlets in a superior-inferior and crossing position; and (4) a four-chamber-like view seen in the sagittal plane of the fetal chest. Doppler ultrasound demonstrates the 'criss-cross' arrangement of the inflow tracts into the two ventricles simultaneously in the transverse plane of the fetal chest. CONCLUSION: CCH is a rare developmental disorder that can be accurately diagnosed prenatally. Early diagnosis will allow for more targeted counseling and early intervention.

Crisscross heart with tricuspid atresia diagnosed in utero.

Crisscross heart is a rare congenital cardiac anomaly in which systemic and pulmonary venous streams cross without mixing at atrioventricular level. We report a case of crisscross heart with tricuspid atresia, double outlet right ventricle, and pulmonary stenosis, which was diagnosed prenatally.

Deciphering the mysteries of crisscross heart by transthoracic echocardiography.

BACKGROUND: Accurate diagnosis of crisscross heart and its associated anomalies is important but problematic for cardiologists. This study aimed at identifying unique transthoracic echocardiographic features and common associated lesions of this complex condition. METHOD: Clinical and echocardiographic features of 10 patients with crisscross anatomy were studied. Echocardiographic findings were verified by cardiac magnetic resonance imaging or surgical inspection. RESULTS: Crisscross anatomy (10 patients, age at diagnosis ranged from 1 month to 25 years, five female) was identified in 0.076% of patients with congenital heart diseases from 1985 to 2006. All patients had cyanosis and 80% of them were underweight. Superior-inferior ventricles (SIV) and crossed ventricular inflow streams were seen in 90% and 100% of patients, respectively. All patients had abnormal ventriculo-arterial (VA) connections: five with transposition of great artery (L-type: n = 2; D-type: n = 3) and five with double outlet right ventricle. Commonly associated anomalies included ventricular septal defects (100%), right ventricular outflow tract obstruction (60%), atrioventricular valves straddling or overriding (50%), atrial septal defect (40%), and right ventricular hypoplasia (30%). Seven patients received cardiac surgery for the relief of cyanosis. CONCLUSIONS: SIV and crossed inflow streams are important diagnostic features for crisscross heart by transthoracic echocardiogram. The hemodynamic consequences of abnormal VA connections and associated defects impact surgical management.

Echocardiographic characteristics of the criss-cross heart.

OBJECTIVE: To assess the ultrasonic characteristics of the criss-cross heart, and explore the value of echocardiography in the diagnosis of this rare congenital cardiac defect. METHODS: We reviewed the echocardiographic findings in 4 patients having criss-crossed atrioventricular connections at our hospital, and compared the findings with observations at surgery in 3 of the patients. RESULTS: In all 4 patients, there was usual atrial arrangement, right hand ventricular topology, and concordant atrioventricular connections. The inlet components of the ventricular mass, however, crossed such that the apical component of the morphologically right ventricle was situated anteriorly and superiorly, and extended to the left relative to the apex of the morphologically left ventricle. The ventriculo-arterial connections were concordant in 1 patient, double outlet from the morphologically right ventricle in 2, and discordant in the other. In all 4 patients, it proved impossible to obtain the standard 4-chamber view showing simultaneously all four chambers and both atrioventricular valves. A series of apical 4-chamber or subcostal coronal views, obtained by tilting the transducer from posterior to anterior, demonstrate initially the connection of the left-sided left atrium and the right-sided left ventricle through the mitral valve. More anterior angulation of the transducer then showed the right-sided right atrium to be connected to the left-sided right ventricle through the tricuspid valve, confirming the presence of twisted atrioventricular connections. Color Doppler imaging displayed the crossing of the atrioventricular connections without mixing of the streams. Short-axis views across the ventricular mass confirmed that the right ventricle was superior, anterior, and to the left of the left ventricle, and demonstrated the horizontal position of the ventricular septum. When viewed subcostally, the distance between the tricuspid valve and the orifice of the inferior vena cava was significantly increased relative to normal findings. The echocardiographic findings were confirmed during surgical interventions in 3 patients, apart from the failure to diagnose one instance of persistent patency of the left superior vena cava. CONCLUSIONS: The failure to obtain a characteristic 4-chamber view in any cut was diagnostic for recognition of the criss-crossed atrioventricular junctions. Transthoracic echocardiography provides definitive images of this rare arrangement, and accurately defines the associated cardiac abnormalities.

[Echocardiographic evaluation of crisscross heart].

OBJECTIVE: To explore atrioventricular connection and atrioventricular segmental situs in patients with crisscross heart (CCH) and to evaluate the diagnostic value of echocardiography for this anomaly. METHODS: Ten consecutive patients with crisscross heart were enrolled into this retrospective study. Their echocardiographic data were analyzed and compared with the results of X-ray angiocardiography and 64-slice multi-detector row computed tomography (MDCT) or MRI. RESULTS: The crossing of atrioventricular valves could be seen in each case by scanning in a subxiphoid or apical 4-chamber view. Both the positive rate and the specificity were 100%. Horizontal ventricular septum was in 9 cases and vertical (sagittal) ventricular septum in 1 case. The segmental set of 8 patients with concordant atrioventricular connection was {S. D. L} in 5 cases, {S. D. D} 1 case, {S. D. S} 1 case and {S. L. D} 1 case. The segmental set of 1 case with discordant atrioventricular connection was {I. D. D} and another 1 case with ambiguous atrioventricular connection was {A. L. L}. In 1 case, the atrioventricular connection was inconsistent with the atrioventricular segmental situs. Ventriculoarterial connections were concordant in 1, DORV in 6, TGA in 2 and C-TGA in 1. CONCLUSION: Echocardiography is proven quite helpful in diagnosis of CCH, and continuous sweeps in subxiphoid long-axis plane or apical 4-chamber view play a key role. Both the atrioventricular connection and the atrioventricular segmental situs are complicated so that they are not always concordance with each other. It is necessary to account for separately.

Echocardiographic segmental analysis in patients with an atrioventricular criss-cross.

The aim of this study was to describe the echocardiographic characteristics of patients with an atrioventricular criss-cross who underwent segmental cardiac morphological analysis. The study involved five patients with a criss-cross heart (0.6% of the studies). The patients' average age was 6 years and 3 months. All patients presented with situs solitus. Three had a discordant atrioventricular connection and two had a concordant connection. In addition, four patients had a ventriculoarterial connection with a double-outlet right ventricle while one had a concordant connection. Two patients had superior-inferior ventricles. Other associated lesions included ventricular septal defect in all patients, pulmonary stenosis, persistent ductus arteriosus and atrial septal defect in three, pulmonary perforation in one, and a hypoplastic aortic arch in one. In these patients, segmental analysis can be carried out satisfactorily using transthoracic echocardiography.