Identification of Greb1l as a genetic determinant of crisscross heart in mice showing torsion of the heart tube by shortage of progenitor cells.

Despite their burden, most congenital defects remain poorly understood, due to lack of knowledge of embryological mechanisms. Here, we identify Greb1l mutants as a mouse model of crisscross heart. Based on 3D quantifications of shape changes, we demonstrate that torsion of the atrioventricular canal occurs together with supero-inferior ventricles at E10.5, after heart looping. Mutants phenocopy partial deficiency in retinoic acid signaling, which reflect overlapping pathways in cardiac precursors. Spatiotemporal gene mapping and cross-correlated transcriptomic analyses further reveal the role of Greb1l in maintaining a pool of dorsal pericardial wall precursor cells during heart tube elongation, likely by controlling ribosome biogenesis and cell differentiation. Consequently, we observe growth arrest and malposition of the outflow tract, which are predictive of abnormal tube remodeling in mutants. Our work on a rare cardiac malformation opens novel perspectives on the origin of a broader spectrum of congenital defects associated with GREB1L in humans.

Physiologic Repair of Congenitally Corrected Transposition of the Great Arteries in a Criss-Cross Heart.

Congenitally corrected transposition of great arteries is an uncommon congenital heart disease characterized by discordance at both the atrioventricular and ventriculoarterial connections. A rare subgroup of patients with congenitally corrected transposition of the great arteries also has a criss-cross heart. The morphology of these patients represents a diagnostic challenge that requires critical analysis to perform a satisfactory surgical procedure. We present a case of a 12-year-old patient with the above mentioned anatomy who underwent physiologic repair.

[Arterial Switch Operation for the Criss-cross Heart Complicated by Muscular Ventricular Septal Defect:Report of a Case].

Criss-cross heart is an extreamly rare anomaly characterized by abnormal rotation of the heart on its long axis. Almost always there are associated cardiac anomalies such as pulmonary stenosis, ventricular septal defect (VSD) and ventriculoarterial connection discord, and most cases are candidates for Fontan procedure due to hypoplasia of right ventricle or straddling atrioventricular valve. We report a case of arterial switch operation for criss-cross heart with muscular ventricular septal defect. The patient was diagnosed with criss-cross heart, double outlet right ventricle, subpulmonary VSD, muscular VSD and patent ductus arteriosus (PDA). PDA ligation and pulmonary artery banding (PAB) was performed in the neonatal period, and an arterial switch operation (ASO) was planed at 6 months of age. Preoperative angiography showed nearly normal right ventricular volume and echocardiography showed normal subvalvular structures of atrioventricular valves. ASO, intraventricular rerouting and muscular VSD closure by sandwitch technique were successfully performed.

Criss cross heart: A challenging diagnosis.

Crisscross heart (CCH) is a rare anomaly characterized by twisting of the inflow streams of right and left ventricles. The etiology of CCH is not known. Its frequency is less than 8/10, 00,000 live births. Cyanosis is common in neonatal age, while shortness of breath is the main presenting complaint in older children. We present a case series of CCH with array of associations, diagnosed by echocardiography. CCH is a rare heart defect that can be diagnosed by echocardiography by a vigilant operator. Early diagnosis is the key to successful management resulting in increased survival rate, better outcomes and improved quality of life.

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.

Criss-cross heart three-dimensional printed models in medical education: A multicenter study on their value as a supporting tool to conventional imaging.

The utility of three-dimensional (3D) printed models for medical education in complex congenital heart disease (CHD) is sparse and limited. The purpose of this study was to evaluate the utility of 3D printed models for medical education in criss-cross hearts covering a wide range of participants with different levels of knowledge and experience, from medical students, clinical fellows up to senior medical personnel. Study participants were enrolled from four dedicated imaging workshops developed between 2016 and 2019. The study design was a non-randomized cross-over study to evaluate 127 participants' level of understanding of the criss-cross heart anatomy. This was evaluated using the scores obtained following teaching with conventional images (echocardiography and magnetic resonance imaging) versus a 3D printed model learning approach. A significant improvement in anatomical knowledge of criss-cross heart anatomy was observed when comparing conventional imaging test scores to 3D printed model tests [76.9% (61.5%-87.8%) vs. 84.6% (76.9%-96.2%), P < 0.001]. The increase in the questionnaire marks was statistically significant across all academic groups (consultants in pediatric cardiology, fellows in pediatric cardiology, and medical students). Ninety-four percent (120) and 95.2% (121) of the participants agreed or strongly agreed, respectively, that 3D models helped them to better understand the medical images. Participants scored their overall satisfaction with the 3D printed models as 9.1 out of 10 points. In complex CHD such as criss-cross hearts, 3D printed replicas improve the understanding of cardiovascular anatomy. They enhanced the teaching experience especially when approaching medical students.

Intra-atrial Pulmonary Venous Conduit Leak in Criss-Cross Heart: Role of Three-Dimensional Modeling.

Three-dimensional (3D) modeling has become an invaluable tool for operative planning in the continually evolving complex field of adult congenital heart surgery. We present a case of an intra-atrial conduit leak after multiple repairs of common atrium and criss-cross morphology. 3D modeling was critical in preoperative evaluation and operative planning for complicated intracardiac anatomy after an uncommon initial approach to preserve a biventricular circulation. In the setting of complex or rare congenital cardiac anomalies, advanced imaging and 3D modeling are helpful with preoperative planning.

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.

Criss-cross heart: Transthoracic echocardiographic features.

OBJECTIVE: To study the echocardiographic features of criss-cross heart (CCH), a congenital cardiac anomaly characterized by crossed ventricular inflow streams, in Indian patients. METHODS: In this retrospective observational study, all pediatric echocardiograms performed in a single tertiary care institution in South India over a three-year period were scrutinized for a diagnosis of CCH. Demographic, clinical and echocardiographic data were collected from patients' medical records and echocardiographic database. Crossed ventricular inflow streams was identified when there was inability to visualize both atrio-ventricular valves in a single imaging plane in cardiac four chamber view. RESULTS: CCH was diagnosed in five patients from 10,500 pediatric echocardiographic studies. The age at diagnosis ranged from one month to 8 years. Cyanosis was present in all but one of the five cases. Crossed ventricular inflow streams was present by definition in all cases, whereas superior-inferior ventricular relationship was present in only three cases. All cases were associated with ventricular septal defects. Atrio-ventricular discordance was seen in three cases and concordance in two. Ventriculo-arterial discordance was seen in three cases, concordance in one and double outlet right ventricle in one. Three cases had pulmonary stenosis and the other two had pulmonary arterial hypertension. Straddling of AV valve was observed in four cases and hypoplastic aortic arch in one case. CONCLUSION: CCH is an extremely rare congenital cardiac anomaly. Superior-inferior ventricular relationship often co-exists with CCH, but is not necessarily present in all cases. CCH requires early diagnosis because of its common association with diverse cardiac anomalies.

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.

Crisscross heart-Morphology, clinical diagnosis, and management options.

Crisscross heart is a rare cardiac malformation characterized by crossing of the inflow streams of the two ventricles due to a twisting of the heart about its long axis. The developmental mechanisms and causes of crisscross heart remain unknown. Neonates present with cyanosis and a systolic murmur. In this study, we report a case of crisscross heart with situs inversus, concordant atrioventricular and ventriculo arterial connection with ventricular septal defect and pulmonary stenosis, and review the literature on this rare cardiac malformation.