Rare case of total anomalous pulmonary venous return into the right atrium in situs solitus.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein's ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

Calmodulin mutation in long QT syndrome 15 associated with congenital heart defects further complicated by a functional 2:1 atrioventricular block: Management from foetal life to postpartum.

We report a long QT syndrome 15 whose diagnosis was suspected during foetal life and confirmed at birth and was associated with congenital heart disease. Genetic testing revealed a rare mutation associated with the CALM2 gene. At 23 weeks of gestation, severe foetal sinus bradycardia (∼100 bpm) was detected. In the third trimester, the foetus developed severe right ventricular hypertrophy. At birth, the electrocardiogram showed a long QT interval of 640 ms, and after 1 hour, the newborn showed functional 2:1 atrioventricular block at ventricular rate of 50 bpm. After further pharmacological therapies, epicardial wires were surgically implanted for transient pacing in VVI mode at 90 bpm. Echocardiogram showed aneurysmatic left atrial appendage, dilated right segments, hypertrophied right ventricle, ostium secundum type atrial septal defect, and muscular ventricular septal defect. At two weeks of postpartum, a permanent dual-chamber pacemaker was implanted in the DDD mode and the patient was discharged with a prescription of beta-blockers and calcium therapy.

Anomalous venoatrial connections - CT and MRI assessment.

Abnormal venous atrial (VA) connections present a congenital heart disease (CHD) challenge for pediatric cardiologists. Fully anatomical evaluation is very difficult in prenatal and perinatal follow-up, but it has a profound impact on surgical correction and outcome. The echocardiogram is first-line imaging and represents the gold standard tool for simple abnormal VA connection. CT and MRI are mandatory for more complex heart disease and "nightmare cases". 3D post-processing of volumetric CT and MRI acquisition helps to clarify anatomical relationships and allows for the creation of 3D printing models that can become crucial in customizing surgical strategy. Our article describes a ten-year (2013-2022) tertiary referral CHD center of abnormal AV connections investigated with CT and MRI, illustrating most of these complex diseases with the help of volume rendering (VR) or multiplanar reconstructions (MPR). The nightmarish cases will also be addressed due to the complex cardiovascular arrangement that requires a challenging surgical solution for correction along with the post-surgical complications.

Multiple ventricular septal defects associated to anomalous venous returns, mitral valve disease, myocardium hypertrophy, and right outflow obstruction: a multimodality imaging assessment.

We report a rare congenital heart disease characterized by multiple ventricular septal defects associated to anomalous systemic and pulmonary venous returns, marked apical myocardial hypertrophy of both ventricles and of right outflow, and hypoplastic mitral anulus. Multimodality imaging is mandatory to assess anatomical details.

Anatomically corrected malposition of the great arteries (S,L,D) with mutation of Nodal gene.

Anatomically corrected malposition of the great arteries is a rare CHD, involving alignment and position of the great arteries. We report an infant with situs solitus, atrioventricular discordance, and ventriculoarterial concordance with the aorta arising anteriorly and to the right of the pulmonary artery. A mutation of Nodal gene, implicated in the pathogenesis of human left-right patterning defects, was found.

Anomalous right ventricular muscle bands obstructing a large apical muscular ventricular septal defect: From fetal to post-natal three-dimensional assessment.

Anomalous right ventricle muscle bands and apical ventricular septal defect are two anomalies sometimes associated. We report a fetal diagnosis of a large apical ventricular septal defect, right intraventricular obstruction caused by anomalous muscle bands; consequently, the high right intraventricular pressure resulted in a right-to-left bulging of ventricular septum and moderate tricuspid regurgitation. Postnatal echocardiogram confirmed the fetal diagnosis and defined accurately the right ventricular anatomy through the three-dimensional echocardiographic assessment.

Double outlet right ventricle in the setting of hypoplastic left ventricle, mitral atresia, interruption of aortic arch, and uncommon intra-atrial anomalies in Trisomy 18.

We present the case of a 1-day-old newborn, with prenatal diagnosis of Trisomy 18 and complex congenital heart disease. Echocardiography at birth showed double outlet right ventricle with non-committed interventricular communication in the setting of mitral atresia, hypoplastic left ventricle, and patent aortic root with bicuspid aortic valve and type A interrupted aortic arch. Adding anomalies were the typical congenital polyvalvular disease, Chiari network, and left intra-atrial shelf dividing morphologically left atrium. This is a rare combination of cardiac anatomical malformations in Trisomy 18.

Atrioventricular Discordance with Double-Outlet Right Ventricle in Mirror Imagery and Levocardia: A Very Rare Case Report.

A newborn without prenatal diagnosis, with bronchial and abdominal situs inversus in levocardia, was referred to our hospital for accurate evaluation; echocardiography showed venoatrial connections in mirror-image arrangement, atrioventricular (AV) discordance, and double-outlet right ventricle (DORV). Additional cardiac malformations were double upper caval district, atrial communication, subpulmonary interventricular communication, and moderate subvalvular and valvular pulmonary stenosis. Few days after birth, the patient presented low oxygen saturation and the heart team decided for a palliative surgery. We describe a very rare case in a newborn with bronchial-abdominal mirror imagery, AV discordance, and DORV in levocardia.

Double aortic arch with hypoplastic right aortic arch and type C atresia of left aortic arch.

We report the case of a 2-month-old baby with a double aortic arch, type C atresia of the left arch and severe hypoplasia of the right aortic arch between the right carotid and subclavian arteries, resulting in systemic obstruction, left ventricular dysfunction and congestive heart failure. Surgical augmentation of the right aortic arch ameliorated the obstruction with improvement in left ventricular function and symptoms.

Erythrocytosis and severe asphyxia: two different causes of neonatal myocardial infarction.

Neonatal acute myocardial infarction is a rare event that carries a high mortality rate. We describe the cases of two newborns who survived acute myocardial infarction and discuss the management. The first neonate was born with severe asphyxia and left ventricular myocardial infarction with ventricular tachycardia. In this patient, systemic flow was maintained by right-to-left shunting through the patent ductus arteriosus. The second neonate presented with a haematocrit of 80% and an inferolateral myocardial infarction. Intensive treatment of low cardiac output syndrome led to survival of both high-risk neonates. In the follow-up, at 48 and 4 months, respectively, ventricular function recovered in both patients.

Clinical and electrophysiological characteristics, and relatively benign outcome, of typical atrioventricular nodal reentrant tachycardia in children and adolescents.

BACKGROUND: Atrioventricular nodal re-entrant tachycardia is an uncommon arrhythmia in children. The natural history of this disturbance is poorly known in young patients. METHODS: We analyzed the clinical and electrophysiological features, and the final outcome, in 19 children affected by typical atrioventricular nodal re-entrant tachycardia diagnosed by a transoesophageal electrophysiological study. RESULTS: Of the cohort, 12 patients were female and 7 male, with a mean age of 11 years. Dual atrioventricular nodal physiology was demonstrated in 14 children (73%). The mean length of the tachycardia cycle was 297 milliseconds, with periods of 2 to 1 atrioventricular block during tachycardia noted in 5 children (26%). The mean cycle length was significantly shorter in the children who presented episodes of 2 to 1 atrioventricular block than in those who did not. After diagnosis, 12 children were not treated, 6 were treated with medical therapy, and 1 was submitted to radiofrequency transcatheter ablation. During a mean follow-up period of 41 months, 2 children with rare, but sustained, episodes of tachycardia that initially had not been treated were submitted to radiofrequency transcatheter ablation. Among children treated pharmacologically, 1 teenager was submitted to radiofrequency transcatheter ablation on the basis of parental choice, 3 children have discontinued medical therapy recording only sporadic episodes of tachycardia, and 2 children are still treated with antiarrhythmic drugs. At the last follow-up visit, 13 children (68%) were without any treatment, 4 had been successfully ablated, and 2 were still on medical treatment. CONCLUSIONS: Our data indicates a relatively benign outcome in this group of children and adolescents with atrioventricular nodal re-entrant tachycardia.

Combined endothelin receptor antagonist and transcatheter interventional therapy of patent ductus arteriosus with severe pulmonary artery hypertension.

The natural history of congenital heart disease (CHD) with left to right shunt and high pulmonary blood flow, is characterized by development of severe Pulmonary Artery Hypertension (PAH); this condition usually contraindicates any type of surgical or interventional cardiac correction because of bad results. We here report the case of an adult patient with a patent ductus arteriosus and severe PAH, treated uneventfully with a staged combined therapy: Bosentan for 3 months and then percutaneous closure with amplatzer duct occluder (AGA Med. Co. Golden Valley, MN). The patient showed a dramatic improvement both of pulmonary hemodynamics and functional capacity at 8 months follow up confirming the efficacy of pulmonary vasodilator therapy in PAH associated to congenital heart disease.

Percutaneous left atrial appendage transcatheter occlusion in patients with chronic nonvalvular atrial fibrillation: early institutional experience.

OBJECTIVE: The percutaneous left atrial appendage transcatheter occlusion system (PLAATO, ev3 Inc., Plymouth, Minnesota, USA) is a novel technique of interventional cardiology, which has recently been proposed for the nonpharmacological prophylaxis of thromboembolic events in patients with chronic nonvalvular atrial fibrillation ineligible for oral anticoagulation therapy with vitamin K antagonists. The aim of this study was to assess the safety, feasibility and mid-term efficacy of this procedure. METHODS: We conducted a preliminary institutional experience on a selected population of seven patients (two male, five female, mean age 64 +/- 7 years) with chronic nonvalvular atrial fibrillation at high risk for thromboembolic events with contraindications for oral anticoagulation therapy with vitamin K antagonists. RESULTS: All procedures were successfully performed without any complications at a mean follow-up of 7 +/- 4 months and no thromboembolic or adverse cardiac events occurred. CONCLUSIONS: Percutaneous left atrial appendage transcatheter occlusion is safe and feasible, without any complications at mid-term follow-up.