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OBJECTIVES The role of an additional ventricle in patients with a functional single ventricle undergoing the Fontan operation has been debated due to conflicting data. Our goal was to report our experience with Fontan circulation for complex congenital heart disease, with a focus on the influence that an additional ventricular chamber may have on early and long-term clinical outcomes. METHODS We performed a retrospective clinical study including all patients undergoing the Fontan procedure between 1978 and 2019. Clinical data were retrieved from our institutional database. A ‘biventricular’ Fontan (BVF) was defined as that performed in a patient with single ventricle anomaly where an additional diminutive ventricular cavity was present at echocardiographic evaluation. RESULTS A total of 210 consecutive patients with functional single ventricle were included. Among these, 46 had BVF (21.9%). Early complications occurred in 42 patients (20.0%; 11 in BVF vs 31 in univentricular Fontan; P = 0.53) There were 18 early deaths (8.6%) with no difference between the groups. At a median follow-up of 12.7 years (interquartile range 5.4–20.7), there were no significant differences in late mortality, whereas cardiac rhythm disturbances resulted more frequently in univentricular Fontan (P = 0.018). Statistical analysis showed an equal distribution of BVF across time (P = 0.620), and there were no significant differences in terms of early and late survival (P = 0.53 and P = 0.72, respectively) or morbidity (P = 0.45 and P = 0.80, respectively). CONCLUSIONS A secondary ventricle in Fontan circulation is not significantly related to any clinical disadvantage in terms of survival or onset of complications. However, the immediate postoperative course may be influenced negatively by the presence of an additional secondary ventricle.

SCA from the right sinus is the rarest coronary anomaly. We describe 2 cases: 1 with SCA type-1RI; 2 with SCA type-2RII-A. Appropriate and successful treatment (CABG in case-1; PTCA in case-2) was chosen relying on accurate morphological description provided by MDCT, in order to recognize all the possible mechanisms of myocardial ischemia.

International audience; Background and aim of the study: We sought to evaluate the appropriateness of cardiac anatomy renderings by a new virtual reality (VR) technology, entitled DIVA, directly applicable to raw magnetic resonance imaging (MRI) data without intermediate segmentation steps in comparison to standard three-dimensional (3D) rendering techniques (3D PDF and 3D printing). Differences in post-processing times were also evaluated.Methods: We reconstructed 3D (STL, 3D-PDF, and 3D printed ones) and VR models of three patients with different types of complex congenital heart disease (CHD). We then asked a senior pediatric heart surgeon to compare and grade the results obtained.Results: All anatomical structures were well visualized in both VR and 3D PDF/printed models. Ventricular-arterial connections and their relationship with the great vessels were better visualized with the VR model (Case 2); aortic arch anatomy and details were also better visualized by the VR model (Case 3). The median post-processing time to get VR models using DIVA was 5 min in comparison to 8 h (range 8-12 h including printing time) for 3D models (PDF/printed).Conclusions: VR directly applied to non-segmented 3D-MRI data set is a promising technique for 3D advanced modeling in CHD. It is systematically more consistent and faster when compared to standard 3D-modeling techniques.

We report a case of inflammatory myofibroblastic tumor affecting the pulmonary artery in a 15-year-old male, presenting with a clinical scenario of recurrent pulmonary embolisms. During diagnostic workup for persistent fever, a mass in main pulmonary artery was detected at echocardiography and confirmed at angio-CT scan. The patient underwent a first successful surgical resection and discharged home with no echocardiographic evidence of residual lesions, but, after 5 months, he was admitted for hemoptysis and an angio CT-scan showed a mass in right pulmonary artery with multiple distal perfusion defects, suspicious for both thrombotic and secondary lesions. To prevent further embolisms, the patient was scheduled for a second surgical procedure, which allowed a complete removal of the tumor from major branches of right pulmonary arteries. Our experience highlights that, despite of its intermediate malignancy, inflammatory myofibroblastic tumor may behave as an extremely dangerous condition, requiring multiple surgeries an integrated and multidisciplinary approach.

Surgical re-implantation of the left coronary artery (LCA) is the treatment of choice in anomalous origin of left coronary artery from pulmonary artery (ALCAPA). Despite normalization of left ventricular function after surgery, residual coronary lesions or myocardial fibrosis may be found. The aim of this study was to detect regional left ventricular dysfunction predictive of coronary lesions or residual myocardial fibrosis using speckle tracking. We enrolled ten patients treated with surgical re-implantation of LCA for ALCAPA. All patients were asymptomatic, and ejection fraction (EF) was normal. Using S-SR imaging, we studied longitudinal, radial, and circumferential function. A cardiac MRI was performed to assess myocardial fibrosis and the anatomy of the coronaries. In case of suspected coronary stenosis, a coronary angiography was performed. Finally, 20 normal subjects were enrolled as control group. Median age at surgery was 188 days, and mean follow-up was 8.7 ± 4.7 years. Longitudinal and circumferential functions were reduced in LCA territory compared to RCA territory and normal. MRI showed LCA stenosis in three of ten patients, confirmed by coronary angiography: these patients had the lowest longitudinal strain values in LCA territories (-11.7, -14.7 and -14.8%). Radial strain was preserved (Normal 45.6 ± 12.1, ALCAPA 43.5 ± 10.7%, p = ns), while circumferential strain was mildly depressed (-23.5 ± 3.8 vs. -20.3 ± 2.0%, p 0.05). After LCA re-implantation, ALCAPA patients showed a residual sub-endocardial damage in LCA territories. Despite a normal systolic and diastolic function, the prevalence of residual coronary lesions was high. A mean longitudinal strain-15% in LCA territories was able to identify those patients.

Congenital heart defects (CHDs) occur in 1% of live-born infants and frequently are associated with extracardiac malformations. This study aimed to assess the feasibility and accuracy of three-dimensional ultrasonography (3DUS) in fetuses with CHD and to investigate whether 3DUS can add information about the heart and general fetal morphology that shows other congenital malformations or suggests syndromes. For 30 fetuses affected by CHD, 3DUS was performed using a Sonos 7500 ultrasound machine with a cardiac 3D transducer. In 44% of the exams, 3DUS was completely diagnostic for the CHD, providing additional information in 28% of the exams. Furthermore, 3DUS showed 82% of associated malformations, providing the complete diagnosis in 57% of the cases and helping with recognition of syndromes in others. The diagnostic accuracy of 3DUS was superior, with a higher number of acquisitions per exam. Performance was better in fetuses younger than 24 weeks for general morphologic details and in fetuses older than 24 weeks for the heart morphology.

The right ventricle (RV) is the main ventricular chamber in many congenital heart diseases before and after surgical correction, and it is the most important determinant of outcome in postoperative tetralogy of Fallot and other complex malformations. Unfortunately its irregular crescentic shape does not allow the use of the geometric assumption used for the left ventricle. Many methods have been suggested in the literature to overcome this problem, none fully reliable. The introduction of volume-rendered 3-dimensional (3D) reconstruction of echocardiography images provides a tool for the direct measurement of cardiac chambers, not based on geometric assumptions. The aim of this research study was to determine the accuracy of 3D echocardiography (3DE) to measure RV volumes in pediatric patients with secundum atrial septal defects, compared with direct volume measurements performed during the intervention. We performed 3DE study in the operating department, with the patient anesthetized, intubated, and ventilated before the surgical procedure. Sequential 2-dimensional echocardiographic images for subsequent 3D rendering were acquired using an ultrasound machine with a transthoracic 4-MHz rotational or 5-MHz transesophageal omniplane probe; in the last 5 patients a machine was used that was equipped with a 3600-crystal real-time 3D probe. To validate the 3DE measurements, these were compared with the volume of the RV directly measured in the operating department, at the end of the surgical procedure, injecting saline solution through the tricuspid valve, using a graduate syringe. Among 25 pediatric patients enrolled in the study, with an age range of 1 and 14 years (mean 4 years) and a weight range of 8.5 to 57.4 kg (mean 18.6 kg), in 23 a mean of 3 echocardiographic acquisitions were performed and compared with the direct measurement. A close comparison was found between RV volumes measured by 3DE and direct volume measurements (P < .00001). The regression line, shifted toward the y axis, which describes the 3DE volumes, indicated that the echocardiographic measures overestimate the surgical ones. In our study this overestimation had the mean of 9% with values comprised between 3% and 19%. The coefficient of repeatability was 4.79 mL with all the values within this range (2 SD of the mean). We conclude that 3DE provides an accurate measurement of RV volume in pediatric patients with RV volume overload. It is a reliable, noninvasive, and nongeometric method of evaluation of the volume of this chamber, and can be considered a precious tool in the armamentarium of the pediatric cardiologist.

We report a case of a 3-month-old child, presenting with signs of myocardial ischemia and hemodynamic instability, requiring extracorporeal membrane oxygenation support. Due to the unclear preoperative instrumental findings, a surgical exploration was performed revealing the presence of coronary ostia stenosis and an intramural course of both coronary arteries. The child underwent a successful plasty of the coronary artery ostia by resecting the excessive obstructing fibrous tissue followed by unroofing of the intramural coronary artery course with restoring of a normal myocardial perfusion and function.

A 1-day-old newborn was referred with prenatal echocardiographic diagnosis of cardiac mass. Born by caesarean section at 38+3 weeks of pregnancy, he presented with cyanosis and bradycardia at birth, requiring immediate intubation and mechanical ventilation. Chest x-ray demonstrated cardiomegaly with a cardiothoracic index of 0.7 (Figure 1). Echocardiographic examination (Figure 2 and Movie I in the online-only Data Supplement) confirmed the diagnosis of a large cardiac mass located at the posterior wall and at the apex of the left ventricle (LV). The child presented with several episodes of paroxysmal supraventricular tachycardia, which were treated with DC shock and various pharmacological agents, such as amiodarone, adenosine, flecainide, digoxin, and metoprolol, and 1 episode of ventricular fibrillation with cardiac arrest requiring resuscitation. MRI showed a single large mass (5.5×5 cm) arising from the left atrioventricular groove and extending to the LV lateral wall and apex, up to the interventricular septum, with no LV inflow or outflow obstruction, and was suggestive …

Background Arterial Tortuosity Syndrome (ATS) is a very rare autosomal recessive connective tissue disorder (CTD) characterized by tortuosity and elongation of the large- and medium-sized arteries and a propensity for aneurysm formation and vascular dissection. During infancy, children frequently present the involvement of the pulmonary arteries (elongation, tortuosity, stenosis) with dyspnea and cyanosis. Other CTD signs of ATS are dysmorphisms, abdominal hernias, joint hypermobility, skeletal abnormalities, and keratoconus. ATS is typically described as a severe disease with high rate of mortality due to major cardiovascular malformations. ATS is caused by mutations in the SLC2A10 gene, which encodes the facilitative glucose transporter 10 (GLUT10). Approximately 100 ATS patients have been described, and 21 causal mutations have been identified in the SLC2A10 gene. Case presentation We describe the clinical findings and molecular characterization of three new ATS families, which provide insight into the clinical phenotype of the disorder; furthermore, we expand the allelic repertoire of SLC2A10 by identifying two novel mutations. We also review the ATS patients characterized by our group and compare their clinical findings with previous data. Conclusions Our data confirm that the cardiovascular prognosis in ATS is less severe than previously reported and that the first years of life are the most critical for possible life-threatening events. Molecular diagnosis is mandatory to distinguish ATS from other CTDs and to define targeted clinical follow-up and timely cardiovascular surgical or interventional treatment, when needed. Electronic supplementary material The online version of this article (doi:10.1186/s12881-014-0122-5) contains supplementary material, which is available to authorized users.

Primary cardiac tumours in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies. Owing to their rarity, there is very little literature available, and most knowledge is based on collections of case reports. Therefore, we reviewed retrospectively our 27 years of clinical experience on the overall management of cardiac tumours among children in order to improve not only our knowledge but also to provide others with information about the incidence, clinical presentation, management, and long-term outcome of this rare disease. Between April, 1982 and April, 2009, 52 children were diagnosed with cardiac tumours at our Institution. Medical records and follow-up echocardiographic evaluations were studied. The diagnosis was prenatal in 35% of the patients. The most frequent tumour types were rhabdomyomas (61.5%), fibromas (15.4%), and myxomas (5.8%). There were no cases of primary malignant tumours. All diagnoses were achieved using two-dimensional echocardiography, and for 12 patients a pathological analysis was carried out. A total of 41 patients (79%) were managed medically, whereas 11 (21%) patients underwent surgical treatment. At a mean follow-up of 7.2 ± 5.4 years, two patients died of complications related to cardiac transplantation; all the remaining patients are in excellent clinical condition. In conclusion, cardiac tumours in paediatric practice are usually clinically and histologically benign. Only a few cases need surgery. Up to one-third of the cardiac masses are detectable prenatally. Rhabdomyoma is the most common histotype, followed by fibroma and myxoma. The long-term prognosis is generally good.

OBJECTIVES We report our 8-year experience with surgery for congenital heart disease in adults in a medium-volume surgical center. METHODS We collected data of 161 consecutive patients who underwent 230 procedures from January 1997 to December 2004. Surgical procedures were divided into two groups: repair (85.7%) and reoperation (14.3). RESULTS There was only one in-hospital death (Fontan revision, in reoperation group). At a mean follow-up time of 56 +/- 30 months, overall freedom from any kind of complication is 76.5%. Cox analysis showed that incremental risk factors were preoperative cyanosis, reoperation, and length of ICU stay (hazard ratio = 4.47, 3.34 and 1.49, respectively; P < 0.001 in all), whereas decremental risk factors were preoperative New York Heart Association class 1 (hazard ratio = 0.328, P < 0.001) and surgery for 'septal defect' (hazard ratio = 0.26, P = 0.02). CONCLUSION Surgery for congenital heart disease in adults seems to be an overall well-tolerated and a low-risk treatment, with overall good mid-term clinical results.

Background The optimal management for critical aortic stenosis in early infancy continues to challenge cardiologists and cardiac surgeons. We present a review of our experience with the surgical treatment of residual aortic valve disease after percutaneous balloon dilation for critical aortic stenosis in early infancy. Methods Since 1989, 11 of the 38 patients who survived aortic balloon dilation (28.9%) have undergone surgical treatment for residual aortic valve dysfunction. Median time from aortic balloon dilation to surgical intervention was 7 months (range 1 to 56 months). Residual aortic stenosis was the predominant problem in 8 patients and aortic regurgitation was predominant in 2 patients. Results Aortic valvuloplasty was possible in 5 children; pulmonary autograft replacement of the aortic valve was performed in 6 children. Two children underwent a Ross-Konno procedure because of annulus hypoplasia and severe left ventricular outflow tract obstruction. Two early deaths occurred after a Ross-Konno procedure, both with findings of severe left ventricular fibroelastosis at the pathologic examination. Median follow-up time was 5 years (range 1 month to 11.9 years). No late deaths occurred. One patient with moderate-severe aortic valve regurgitation after aortic valvuloplasty underwent a successful Ross operation. All 9 patients are asymptomatic and are in good clinical condition. Conclusions We are convinced that the best aortic valve in the pediatric age group is the native one, provided it can function acceptably. However, in cases where conservative surgical treatment fails to yield a functional aortic valve, replacement of the valve is indicated, and the best aortic valve substitute in infants is the pulmonary autograft because of its potential for growth.