Congenital Cardiac Malformations Research Articles

Risks of congenital malformations and neonatal intensive care unit admissions with gabapentin use in pregnancy: A cohort study and scoping review with meta-analysis.

The increasing and prevalent use of gabapentin among pregnant people highlights the necessity to assess its neonatal safety. This study aimed to investigate the foetal safety of gabapentin during pregnancy using a cohort study and scoping review with a meta-analysis of published evidence. We conducted a population-based cohort study using the Manitoba health databases between 1995 and 2019. We examined the association between gabapentin use during pregnancy and the prevalence of major congenital malformations, cardiac and orofacial malformations, and neonatal intensive care unit (NICU) admissions using multivariate regression models. We searched the literature in MEDLINE and EMBASE databases from inception to October 2022 to identify relevant observational studies and conducted a meta-analysis using random-effects models, including our cohort study results. Of the 289,227 included pregnancies, 870 pregnant people were exposed to gabapentin. Gabapentin exposure during the First trimester was not associated with an increased risk of any malformations (adjusted relative risk [aRR]) 1.16 (95%confidence interval [CI] 0.92, 1.46), cardiac malformations (aRR 1.29, 95% CI 0.72, 2.29), orofacial malformations (aRR 1.37, 95% CI 0.50, 3.75), and major congenital malformations (aRR 1.00, 95% CI 0.73, 1.36). whereas exposure during any trimester was associated with an increased NICU admission risk (aRR, 1.99 [95% CI 1.70, 2.32]). The meta-analysis of unadjusted results revealed an increased risk of major congenital malformations (RR 1.44, 95% CI 1.28, 1.61, I2 = 0%), cardiac malformations (RR1.66, 95% CI 1.11, 2.47, I2 = 68%), and NICU admissions (RR 3.15, 95% CI 2.90, 3.41, I2 = 10%), and increased trend of orofacial malformations (RR 1.98, 95%CI 0.79, 5.00, I2 = 0%). Gabapentin use was associated with an increased risk of NICU admissions in the cohort study and pooled meta-analysis. Clinicians should prescribe gabapentin with caution during pregnancy and further studies are warranted.

Successful one-stage surgical repair in a rare adult patient with berry syndrome.

Berry syndrome is a rare congenital cardiac malformation, herein we report an adult male patient who was successfully repaired by one-stage surgery. 18-year-old male patient presenting symptoms of chest tightness and shortness for over a year presented to outpatient clinic in our department to have corrective procedure heart. CTA revealed an Aortopulmonary Window (APW) type III, measuring 4.6 cm in maximum diameter. The right pulmonary artery originated from the ascending aorta, and the pulmonary trunk exhibited dilation with dimensions of 8.3 cm × 5.7 cm × 5.9 cm. Additional findings included Interrupted Aortic Arch (IAA) type A, intact ventricular septum, and Patent Ductus Arteriosus (PDA). Echocardiography showed bidirectional shunt at the level of APW in severe pulmonary hypertension. The right heart catheterization indicated a mean pulmonary artery pressure of 70mmHg and a pulmonary artery resistance of 5 Wood units. We evaluated after two weeks of treatment with epoprostenol at a rate of 20 ng/(kg. min) and found a significant improvement in pulmonary-artery pressure. Finally, we communicated with the patient's family and decided to proceed with the procedure. For complex cardiovascular malformations, the ideal treatment strategy must be tailored to the characteristics of the patient to provide maximum efficacy and safety.

Neonatal diagnosis of primary ciliary dyskinesia in a high consanguinity population: a single tertiary center experience.

Though PCD usually presents after birth in term neonates, diagnosing PCD during the neonatal and infancy stages is uncommon, particularly in children who do not exhibit laterality defects. We report our recent experience with the diagnosis of PCD in the neonatal and early infantile period in a highly consanguine population. This was achieved by implementing a novel genetic-based diagnostic approach based on direct testing for recognized regional genetic variants. We conducted a retrospective analysis of children diagnosed with PCD at Soroka University Medical Center during the neonatal or early infantile period between 2020 and 2023. We included children under 3 months of age who had a genetic confirmation of PCD, as evidenced by the presence of two pathogenic variants in recognized genes. Genetic testing targeted regional genetic variants in previously identified PCD genes. Eight patients were included. The median age at diagnosis was 12.5 days. Three (38%) were born prematurely < 34 weeks gestational age. All patients were presented with respiratory distress and hypoxemia after birth. The median duration of oxygen support was 23 days, and upper lobe atelectasis was present in five patients (63%). Congenital cardiac malformation was present in four patients. Organ laterality defects were present in four patients. Genetic mutations identified were in the DNAAF5, DNAL1, DNAAF3, and DNAH1 genes. Conclusion: Neonatal diagnosis of PCD is uncommon, especially in atypical presentations such as children without laterality defects or preterms. Focusing on a genetic diagnosis of the local tribal pathogenic variants promotes a potential cost-efficient test leading to earlier diagnosis. There is a need for a standardized protocol for earlier diagnosis of PCD in high-consanguinity areas. What is Known: • Primary ciliary dyskinesia (PCD) typically presents after birth in term neonates. • Diagnosing PCD during neonatal and infancy stages is challenging, particularly in children without laterality defects. What is New: • A novel genetic-based diagnostic approach was implemented on the neonatal population in a highly consanguine community, focusing on direct testing for regional genetic variants, leading to early and rapid diagnosis of PCD.

Identification of miR-20b-5p as an inhibitory regulator in cardiac differentiation via TET2 and DNA hydroxymethylation

BackgroundCongenital heart disease (CHD) is a prevalent congenital cardiac malformation, which lacks effective early biological diagnosis and intervention. MicroRNAs, as epigenetic regulators of cardiac development, provide potential biomarkers for the diagnosis and treatment of CHD. However, the mechanisms underlying miRNAs-mediated regulation of cardiac development and CHD malformation remain to be further elucidated. This study aimed to explore the function of microRNA-20b-5p (miR-20b-5p) in cardiac development and CHD pathogenesis.Methods and resultsmiRNA expression profiling identified that miR-20b-5p was significantly downregulated during a 12-day cardiac differentiation of human embryonic stem cells (hESCs), whereas it was markedly upregulated in plasma samples of atrial septal defect (ASD) patients. Our results further revealed that miR-20b-5p suppressed hESCs-derived cardiac differentiation by targeting tet methylcytosine dioxygenase 2 (TET2) and 5-hydroxymethylcytosine, leading to a reduction in key cardiac transcription factors including GATA4, NKX2.5, TBX5, MYH6 and cTnT. Additionally, knockdown of TET2 significantly inhibited cardiac differentiation, which could be partially restored by miR-20b-5p inhibition.ConclusionsCollectively, this study provides compelling evidence that miR-20b-5p functions as an inhibitory regulator in hESCs-derived cardiac differentiation by targeting TET2, highlighting its potential as a biomarker for ASD.

Postoperative Atrioventricular Block in Pediatric Patients: Impact of Congenital Cardiac Malformations and Medications.

Postoperative atrioventricular block may occur after pediatric cardiac surgery. A small proportion of those who develop atrioventricular block will require pacemaker placement. The primary aim of this study was to determine factors associated with postoperative atrioventricular block. Secondary aims included determining factors associated with pacemaker placement in those with atrioventricular block. Data from the PHIS data were utilized to identify patients under 18 years of age who underwent cardiac surgery. Those who did and did not develop atrioventricular block. Univariable analyses and regression analyses were conducted to determine factors associated with postoperative atrioventricular block. Similar analyses were conducted to determine factors associated with pacemaker placement in those with atrioventricular block. A total of 43,716 admissions were identified. Of these, 2093 (5%) developed atrioventricular block and 480 (1% of total admissions) underwent pacemaker placement. Approximately 70% of those with atrioventricular block received steroids but this was not associated with a decrease in pacemaker placement. Risk factors (congenital malformations of the heart, comorbidities, medications) associated with increased risk of atrioventricular block and pacemaker placement were identified. Postoperative atrioventricular block occurred in 5% of pediatric admissions for cardiac surgery. Of these admissions with postoperative atrioventricular block, 23% required pacemaker placement. Isoproterenol and steroids were not associated with a reduction in the likelihood of pacemaker placement.

An Unusual Presentation of Partial Atrioventricular Canal Defect in an Elderly Woman

A partial atrioventricular canal defect (PAVCD) is a form of endocardial cushion defect. It’s a rare congenital cardiac malformation, few reported patients have survived into the seventh decade of life. A 71-year-old female with a history of diabetes and hypertension was admitted for heart failure (HF) symptoms. Transthoracic echocardiography revealed a left to right shunt in the basal portion of the interatrial septum, 19mm ostium primum atrial septal defect (ASD), a mitral valve cleft, and severe mitral valve regurgitation. PAVCD consists of an ostium primum ASD and the dividing of the common atrioventricular (AV) valve into 2 separate AV orifices, resulting in a so-called cleft in the mitral valve. The ventricular septum is intact. Echocardiography is the key tool for diagnosis and anatomic classification. Surgical repair is recommended in adults with primum ASDs causing impaired functional capacity, right atrial or right ventricular enlargement, and left-to-right shunt without cyanosis, as long as systolic pulmonary artery pressures are lower than 50% of systemic pressures. Reconstructive surgery includes, in addition to the closure of the interatrial septum, a suturing of the mitral cleft. The estimated mean age at death of the patients with ostium primum ASD is about 40 years. The objective of this clinical case is to highlight the good clinical tolerance of atrial shunts and the anatomical particularities in the PAVCD.

Ebstein's anomaly: an electrophysiological perspective.

Ebstein's anomaly of the tricuspid valve (EA) is an uncommon congenital cardiac malformation. It can present with atrioventricular tachycardia (AVRT), atrioventricular nodal re-entrant tachycardia (AVNRT), atrial arrhythmias, and rarely with ventricular tachycardia. The 12-lead electrocardiogram (ECG) is critically important and often diagnostic even prior to an electrophysiology study (EPS). Due to its complex anatomy, it poses particular challenges for mapping and ablation, even for an experienced electrophysiologist. In this review, we aim to provide insight into the electrophysiological perspective of EA and an in-depth analysis of the various arrhythmias encountered in diverse clinical scenarios.

Isolation of the left innominate artery: When to operate?

ABSTRACT A right aortic arch with an isolated left innominate artery from the pulmonary artery is an exceedingly rare congenital cardiac malformation. We describe the management and complex surgical timing considerations in two such cases, successfully operated on day 4 and 7 months of age, including the use of cranial ultrasound as a helpful tool to guide decision-making. We also describe the first reported association of this defect with a 4q25 deletion encompassing the LEF1 gene.

Prenatal features and postnatal follow-up of congenital ventricular outpouching: A retrospective study of two centers in China.

Congenital ventricular outpouching (CVO) is a rare cardiac malformation that can manifest as congenital ventricular aneurysm (CVA) and/or congenital ventricular diverticula (CVD). In this study, we describe the prenatal features and postnatal follow-up of 27 cases of CVO. The clinical data of 27 patients with CVO who attended Sir Run Run Shaw Hospital Affiliated to the Medical College of Zhejiang University (Zhejiang Province, China) and Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University (Zhejiang Province, China) from April 2013 to October 2022 were retrospectively analyzed. The patients were also followed up by telephone. The prenatal characteristics and postnatal outcomes of the patients with CVO were evaluated. CVO was detected in 26 cases prenatally, 14 (51.85%) were diagnosed with CVA, nine (33.33%) were diagnosed with CVD, three (11.11%) were equivocal for CVA/CVD, and one (3.70%) was detected with CVA postnatally. Six patients underwent follow-up fetal echocardiography approximately 4weeks after the initial echocardiography examination, and a significant difference in CVO size was observed between the two examinations (P=.02). Eight patients (29.63%) demonstrated cardiovascular dysfunction, and the median CVO size in fetuses with and without cardiovascular dysfunction was 205 (range: 169-396) mm2 and 124 (range: 92-154.5) mm2 , respectively (P=.01). Correlation was found between CVO size and fetal cardiac dysfunction (p=.000, r=.778). Eight patients (29.63%) had cardiac/extracardiac defects. Thirteen patients were live born, 12 were terminated pregnancies, and two were lost to follow-up. The postpartum size of the CVOs remained stable in six patients, decreased in two patients, dissolved in three patients, and were surgically removed in two patients. With the exception of one patient with CVA complicated with complex congenital cardiac malformation who underwent surgical treatment after birth and who had postoperative left ventricular dysfunction (Case 1), the prognosis of all of the patients was good. CVO is often associated with cardiac malformations. The size of prenatal CVOs can increase with gestational development, and cardiovascular dysfunction is significantly related to CVO size. The postpartum prognosis of patients with CVO is good. Echocardiography plays a key role in the diagnosis of congenital ventricular outpouching. Prenatal counseling should be cautious regarding the diagnosis and the prognosis although our cases had a favorable prognosis.

Transposition of Great Vessels: Latin-American Postoperative Experience

The transposition of great vessels (TGV) is a congenital cardiac malformation where atrioventricular concordance is conserved with ventriculoarterial discordance. Regarding the management of this pathology, surgery is the cornerstone treatment. We aimed to describe the surgical characteristics of the pediatric population with transposition of great vessels surgically managed by our cardiovascular surgery group.

Long-Term Results Following Combined Repair of Atrioventricular Septal Defect with Tetralogy of Fallot.

Atrioventricular septal defect (AVSD) in association with tetralogy of Fallot (TOF) is a rare and complex congenital cardiac malformation. We report our institutional experience and outcomes following surgical correction over a 20-year period. Patients who underwent combined surgical AVSD and TOF correction between October 2001 and February 2020 were included for analysis. All patients underwent primary repair. The study data were prospectively collected and retrospectively analyzed. Primary outcomes were in-hospital mortality and long-term freedom from reoperation. During the study period, a total of 10 consecutive patients underwent combined surgical AVSD and TOF correction. Median age at operation was 307days (IQR 228-457) and median weight was 7.7kg (IQR 6.7-9.5). Down Syndrome was present in six of the patients. In-hospital mortality was 0%. One patient required re-exploration due to bleeding. Median follow-up was 11years (IQR 11months -16years). There was one case of reoperation due to significant residual ventricular septal defect after 2 months. None of the patients died during follow-up. Combined primary AVSD and TOF repair can be performed with low early mortality and morbidity, as well as a high long-term freedom from reoperation.

Abstract 17786: Transcatheter Atrial Septal Defect With Deficient Retro-Aortic Rim Closure Success Rate and Complication is Similar to Other Anatomical Variants: Meta-Analysis

Introduction: Atrial septal defect (ASD) is a common congenital cardiac malformation resulting from incomplete fusion of one of the multiple embryological structures. Though surgical repair when indicated had been the norm, technological advances over the years have led to the current availability of transcatheter closure of the ASD. This alternative approach has been associated with lower complications and mortality but the procedural success and outcome are variable dependent upon anatomical features. Hypothesis: Several reports have linked deficiency of retro-aortic rim to higher complications, particularly aortic erosion in patients undergoing transcatheter ASD closure. Recent retrospective studies have however suggested otherwise. We herein performed meta-analysis of existing data on the success rate and adverse events following transcatheter closure of ASDs in patients with deficient retro-aortic rims (DRAR) in an attempt to provide general recommendations on the optimal approach for this common anatomic variant. Methods: Twelve relevant studies of ASD with DRAR were identified utilizing PubMed. Each of the twelve studies was closely reviewed for major adverse events including cardiovascular mortality, mortality from non-cardiovascular causes, device erosions, device embolization, residual shunting post-ASD repair, and other complications. The incident of each major adverse events was subsequently tabulated. Five of these reports directly compared the failure rates of ASD closure between patients with and without DRAR. A Mantel-Haenszel random effect meta-analysis was performed on these five publications. Results: Of the total 4,621 transcatheter-based approach ASD closures included, the rate of major adverse events was 5.14%. There was no significant difference in the procedural failure rate between those with and without DRAR ( relative risk =1.14, 95% CI[0.39 3.35], p=0.811 ), (heterogeneity, I 2 =74.75%). Conclusions: Our meta-analysis suggests that transcatheter ASD closure could be performed in patients with deficient retro-aortic rim ASD with expected similar rates of adverse events and success rates as in other anatomical variants.

Transplacental non-steroidal anti-inflammatory drugs versus expectant management in fetal Ebstein anomaly with circular shunt: Systematic review and meta-analysis.

Ebstein anomaly (EA) is a rare congenital cardiac malformation associated with high perinatal mortality. In this systematic review and meta-analysis, we aimed to investigate the outcomes of pregnancies affected by EA or tricuspid valve dysplasia (TVD) with circular shunt, focusing on two prenatal management approaches: (1) expectant management (EM) and (2) transplacental non-steroidal anti-inflammatory drugs (NSAID) therapy. We searched PubMed, Scopus, and Web of Science systematically from its inception until June 2023. The random-effect model was used to pool the data. Heterogeneity was assessed using the I2 value. Twenty-one studies with a total of 610 fetuses with EA/TVD with circular shunt were included in the synthesis, of which 17 studies (583 fetuses) were on EM and 4 studies (27 fetuses) used transplacental NSAID therapy. The NSAID group had higher rates of moderate to severe tricuspid regurgitation, hydrops, and pericardial effusion on prenatal ultrasound compared with the EM group. However, ductal constriction was achieved in 81% of NSAID cases, mitigating the disease pathophysiology, although 65% of them experienced oligohydramnios. Notably, the NSAID group showed significantly higher rates of live birth (86%) and survival to hospital discharge (89%) compared with the EM group (67% and 43%, respectively). Despite these promising results, it's important to acknowledge that the number of cases treated with NSAIDs was small, with limited safety data. Therefore, caution is advised in interpreting these findings, and patients considering NSAID therapy should be informed about these limitations. Future multicenter studies are necessary to further explore the safety and effectiveness of NSAID therapy in this particular population.

Long-term results following atrioventricular septal defect repair

BackgroundAtrioventricular septal defects (AVSD) represent 4–7% of congenital cardiac malformations. Definitive early repair is favored over prior pulmonary artery banding and delayed definitive repair in many centers. The aim of this study was to analyze long-term outcomes following AVSD repair over a 21-year period.MethodsA total of 202 consecutive patients underwent surgical AVSD correction between June 1999 and December 2020. Surgery was performed using the double-patch technique. The study data were prospectively collected and retrospectively analyzed. Primary outcomes were In-hospital mortality and overall long-term freedom from reoperation.ResultsMedian age at operation was 120 days (IQR 94–150), median weight was 5.0 kg (4.2–5.3). None of the patients died within the first 30 postoperative days. In-hospital mortality was 0.5% (1/202 patients). Median follow-up was 57 months (11–121). Overall freedom from reoperation at 5, 10 and 15 years was 91.8%, 86.9% and 86.9%, respectively.ConclusionAVSD repair with the double-patch technique is a safe and effective procedure with good early postoperative outcomes and low long-term reoperation rates.

Left femoral hypoplasia associated with congenital fibular agenesis – A rare case report

Background and objectives. Unilateral or bilateral femoral hypoplasia represents a rare clinical entity and it can be isolated or associated with otherlimb malformations such as: congenital absence of the fibula (the most common association), the absence of lateral foot rays or clubfoot, or other organ defects such asspinal dysraphism, or congenital cardiac malformations. Only a few cases have been detected by antenatal ultrasonography so far. Materials and methods. We present the case of a newborn admitted at birth in the Neonatology Department of County Emergency Clinical Hospital of Constanta, diagnosed prenatally with left femur hypoplasia, confirmed postnatally. Results. The subject is a full-term female neonate, born at 38 weeks gestational age, with a birth weight of 2600 g, Apgar Score of 9. The mother is 39-year-old, Gravida-I, Para-I, with no medical history of consanguinity, genetic or congenital abnormalities, exposure to toxins, teratogen drugs, or viral infections. The 14 weeks and 16 weeks ultrasound revealed a shortened left femur, below the 5th percentile, with no other facial morphology, cardiac, neurologic, gastrointestinal, or genitourinary system abnormalities mentioned. A cesarean section was performed at 38 gestational weeks (premature rupture of membranes, breech presentation). The diagnosis was confirmed postpartum by X-ray. Conclusions. The presented case emphasizes the importance of prenatal care and early detection of skeletal abnormalities for parental education and genetic counseling, offering an informed choice whether to continue with the pregnancy and planning for postnatal care with a multidisciplinary team: obstetrics and gynecology, neonatology, and pediatric orthopedic surgery.

Mortality in Patients With Ebstein Anomaly

BackgroundLow birth prevalence and referral bias constitute significant obstacles to elucidating the natural history of Ebstein anomaly (EA). ObjectivesAn extensive 2-country register-based collaboration was performed to investigate the mortality in patients with EA. MethodsPatients born from 1970 to 2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries. Each patient was matched by birth year and sex with 10 control subjects from the general population. Cumulative mortality and HR of mortality were computed using Kaplan-Meier failure function and Cox proportional regression model. ResultsThe study included 530 patients with EA and 5,300 matched control subjects with a median follow-up of 11 years. In the EA cohort, 43% (228) underwent cardiac surgery. Cumulative mortality was lower for patients diagnosed in the modern era (the year 2000 and later) than for those diagnosed in the prior era (P < 0.001). Patients with isolated lesion displayed lower cumulative mortality than patients with complex lesions did (P < 0.001). Patients with a presumed mild EA anatomy displayed a 35-year cumulative mortality of 11% (vs 4% for the matched control subjects; P < 0.001), yielding an HR for mortality of 6.0 (95% CI: 2.7-13.6), whereas patients with presumed severe EA demonstrated an HR of 36.2 (95% CI: 15.5-84.4) compared with control subjects and a cumulative mortality of 18% 35 years following diagnosis. ConclusionsMortality in patients with EA is high irrespective of presence of concomitant congenital cardiac malformations and time of diagnosis compared with the general population, but overall mortality has improved in the contemporary era.

Congenital cardiac malformations in children with down syndrome: An echocardiographic based study.

Objective: To find out the prevalence and pattern of congenital heart diseases (CHDs) in children with Down syndrome (DS). Study Design: Cross-sectional study. Setting: Department of Pediatrics, Rai Medical College Teaching Hospital, Sargodha, Pakistan. Period: January 2021 to December 2022. Material &amp; Methods: A total of 165 children of either gender, aged 1 month to 12 years with DS diagnosed with the help of typical clinical features and confirmation by cytogenetic studies were analyzed. Children diagnosed with DS were further evaluated for cardiac assessment as per 2-D echocardiographic and Doppler examinations. The prevalence of CHDs was noted among children with DS. Results: In a total of 165 children with DS, 86 (52.1%) were male. The mean age was noted to be 1.8±1.2 years while 106 (64.2%) children were aged below 1 year. The existence of CHDs was found to be in 56 (33.9%). Among 56 children with CHDs, ventricular septal defect (VSD), atrial septal defect (ASD) and tetralogy of fallot (TOF) were the commonest forms of CHDs noted in 16 (28.6%), 12 (21.4%) and 9 (16.1%) children respectively. Conclusion: High prevalence of CHDs in children with Down syndrome was noted. The VSD and ASD were the most common forms of CHDs among children with Down syndrome.

Di George syndrome revealed by hypocalcemia: A case report

Di George syndrome or (the 22q11.2 deletion syndrome) is due to a chromosomal abnormality namely a genetic abnormality caused by a microdeletion of chromosome 22. The main manifestations are cardiac congenital malformations, thymic hypoplasia, and hypoparathyroidism with hypocalcemia, psychomotor retardation, craniofacial dysmorphies and sometimes immunodeficiency. The diagnosis can be made antenatally, during fetopathological examination or after birth. We report the case of a 49-year-old female patient who was initially followed for iatrogenic hypoparathyroidism under Levothyrox replacement therapy and whose evolution was marked by the discovery of a Di George syndrome during follow-up.

A revised terminology for the pharyngeal arches and the arch arteries.

The pharyngeal arches are a series of bulges found on the lateral surface of the head of vertebrate embryos. In humans, and other amniotes, there are five pharyngeal arches and traditionally these have been labelled from cranial to caudal-1, 2, 3, 4 and 6. This numbering is odd-there is no '5'. Two reasons have been given for this. One is that during development, a 'fifth' arch forms transiently but is not fully realised. The second is that this numbering fits with the evolutionary history of the pharyngeal arches. Recent studies, however, have shown that neither of these justifications have basis. The traditional labelling is problematic as it causes confusion to those trying to understand the development of the pharyngeal arches. In particular, it creates difficulties in the field of congenital cardiac malformations, where it is common to find congenital cardiac lesions interpreted on the basis of persistence of the postulated arteries of the fifth arch. To resolve these problems and to take account of the recent studies that have clarified pharyngeal arch development, we propose a new terminology for the pharyngeal arches. In this revised scheme, the pharyngeal arches are to be labelled as follows-the first, most cranial, the mandibular (M), the second, the hyoid (H), the third, the carotid (C), the fourth, the aortic (A) and the last, most caudal, the pulmonary (P).

A rare ventriculoarterial connection: double outlet of both ventricles.

Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling.