Left circumflex coronary artery from the pulmonary artery in scimitar syndrome.

BACKGROUND: Scimitar syndrome is a rare combination of cardiopulmonary abnormalities found in 1-3 per 1000 live births. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is only found in 1 in 250-400 congenital heart disease patients. OBJECTIVE: We aimed to investigate the incidence of left circumflex ALCAPA within our referral center's cohort of scimitar syndrome patients. MATERIALS AND METHODS: A review of medical records, cardiac imaging and operative notes from all patients diagnosed with scimitar syndrome at our center between 1992 and 2016 was undertaken and all imaging reviewed. RESULTS: Fifty-four patients with scimitar syndrome and imaging were identified. Of these, 3 patients (1 male and 2 female) with ALCAPA were identified, representing an incidence of 5.5% (95% confidence interval [CI] 0-11.67%). In all three cases, the anomalous coronary arising from the pulmonary artery was the left circumflex coronary artery (LCx) and the point of origin was close to the pulmonary arterial bifurcation. CONCLUSION: We hypothesize that the prevalence of LCx-ALCAPA, in the setting of scimitar syndrome, may be greater than previously thought. We suggest that any patient with scimitar syndrome, especially with evidence of ischaemia, should be investigated for ALCAPA. Given its noninvasive nature and simultaneous imaging of the lungs, we suggest that cardiovascular CT is the most appropriate first-line investigation for these patients.

Variants of the scimitar syndrome.

UNLABELLED: Introduction The scimitar syndrome comprises hypoplastic right pulmonary artery and lung, anomalous right pulmonary venous drainage to the inferior caval vein, aortopulmonary collateral(s) to the right lung, and bronchial anomalies. Aim The aim of this study was to describe the morphological and clinical spectrum of variants from the classical scimitar syndrome in a single institution over 22 years. RESULTS: In total, 10 patients were recognised. The most consistent feature was an aortopulmonary collateral to the affected lung (90%), but there was considerable variation in the site and course of pulmonary venous drainage. This was normal in 3 (one with meandering course), anomalous right to superior caval vein in 1, to the superior caval vein and inferior caval vein in 2, and to the superior caval vein and the left atrium in 1; one patient had a right pulmonary (scimitar) vein occluded at the insertion into the inferior caval vein but connected to the right upper pulmonary vein via a fistula. There were two left-sided variants, one with anomalous left drainage to the coronary sinus and a second to the innominate vein. Among all, three patients had an antenatal diagnosis and seven presented between 11 and 312 months of age; 90% of the patients were symptomatic at first assessment. All the patients underwent cardiac catheterisation; collateral embolisation was performed in 50% of the patients. Surgical repair of the anomalous vein was carried out in two patients, one patient had a right pneumonectomy, and one patient was lost to follow-up. There was no mortality reported in the remainder of patients during the study period. CONCLUSION: The heterogeneity of this small series confirms the consistent occurrence of an anomalous arterial supply to the affected lung but considerable variation in pulmonary venous drainage.

Uncommon variants of the scimitar syndrome in two siblings.

The Scimitar syndrome is a complex association of cardiovascular and bronchopulmonary abnormalities, with the main feature a partial or total anomalous right pulmonary venous drainage to the inferior vena cava. A number of cases that lack of all the features of the typical syndrome have been described as Scimitar variant, but the incidence is rare. Familial occurrence is exceptional and limited to few cases in literature. We report two sibling diagnosed with an uncommon variant of the Scimitar syndrome.

Glycopyrrolate and theophylline for the treatment of severe pallid breath-holding spells.

Severe pallid breath-holding spells (BHSs) are based on parasympathetic hyperactivity, leading to cardiac asystole, pallor, brain ischemia, loss of consciousness, and reflex anoxic seizures. In recent years, an increasing number of patients with severe pallid BHSs have been successfully treated with pacemaker implantation. We present the case of a 13-month-old girl suffering from repeated severe pallid BHSs, causing asystole, loss of consciousness, and generalized anoxic seizures. She underwent treatment with oral glycopyrrolate, an anticholinergic drug, and an oral retard preparation of theophylline. The aim of the treatment was to decrease cardiac inhibition with glycopyrrolate and to bring about a positive chronotropic effect with theophylline. In our case, the combined therapy was effective in suppressing syncope and reflex anoxic seizures associated with BHSs This avoided the need for ventricular pacemaker implantation.

Adams-Stokes attack as the first symptom of acute rheumatic fever: report of an adolescent case and review of the literature.

BACKGROUND: Acquired complete heart block, in pediatric age is mainly the results of direct injury to conduction tissue during cardiac surgery or cardiac catheterisation. It can also be observed in different clinical settings as infectious diseases, neoplasia, and inflammatory diseases. It has a wide range of presentation and in some settings it can appear a dramatic event. Although a rare finding during acute rheumatic fever, with a transient course, it may need a specific and intensive treatment. CASE PRESENTATION: We report the case of an Adams-Stokes attack in an adolescent with acute rheumatic carditis and complete atrio-ventricular block. The attack was the first symptom of carditis.We reviewed the literature and could find 25 cases of complete atrio-ventricular block due to rheumatic fever. Ten of the 25 patients experienced an Adams-Stokes attack. Nineteen of the 25 patients were certainly in the pediatric age group. Seven of the 19 pediatric cases experienced an Adams-Stokes attack. In 16/25 cases, the duration of the atrio-ventricular block was reported: it lasted from a few minutes to ten days. Pacemaker implantation was necessary in 7 cases. CONCLUSION: Rheumatic fever must be kept in mind in the diagnostic work-up of patients with acquired complete atrio-ventricular block, particularly when it occurs in pediatric patients. The insertion of a temporary pacemaker should be considered when complete atrio-ventricular block determines Adams-Stokes attacks. Complete heart block during acute rheumatic fever is rare and is usually transient. Along with endocarditis, myocarditis and pericarditis, complete atrio-ventricular block has been recognized, rarely, during the course of acute rheumatic carditis.

Infective endocarditis following body piercing: Presentation of one case due to Gemella morbillorum and review of the literature.

BACKGROUND: In the last decade, body piercing has gained strongly in popularity. There are a few reports in the literature regarding infective endocarditis after body piercing. CASE REPORT: The case of an 18-year-old girl with heart failure and a six-month history of recurring fever is presented. Clinical and laboratory evaluation led to the diagnose of infective endocarditis due to Gemella morbillorum. Oral piercing was recognized to be the cause of bacteremia. Fourteen cases of infective endocarditis related to body piercing were found in the literature and the clinical, etiologic, pathogenic, and preventive aspects of this topic are presented. CONCLUSIONS: Infective endocarditis may be a complication of body piercing and has been observed in patients with heart lesions as well as in previous healthy people. Infective endocarditis can follow body piercing involving either mucous membranes or the skin. This patient is the first case of Gemella morbillorum endocarditis after body piercing reported in the literature. Careful observation for infection at the site of piercing and its treatment as well as professional training for operators seem to be the most effective way to minimize the risk of infective endocarditis after body piercing.

Syncope in children and adolescents: a two-year experience at the Department of Paediatrics in Parma.

BACKGROUND AND AIM OF THE WORK: Syncope occurs frequently in childhood and adolescence. It generally is benign, but may be a sign of pathology. The purpose of this study is to determine the incidence of syncope in children and adolescents in the Parma area, analyse the frequency of syncope due to cardiac, neurological or metabolic diseases, to identify a diagnostic procedure based on careful review, clinical features, and results of investigations. METHODS: The records of 156 children (age 1 to 18 years, mean age 10.5), who were referred to the Paediatric Department for syncope were reviewed. RESULTS: The incidence of syncope in the paediatric population of Parma during a 2-year period (2005-2006) resulted in 86.5/100.000 per year. The cause of syncope was discovered in 154/156 cases. Cardiovascular syncope was diagnosed in 121/156 cases (77.5%). Neurological syncope was diagnosed in 31/156 cases (20%), while metabolic syncope was diagnosed in 2/156 cases (1.25%). The most frequent type was the (benign) neurocardiogenic syncope, but 3 cases were due to arrhythmia, 9 cases were due to epilepsy, 1 case was due to subarachnoid hemorrage and 2 cases were due to hypoglicemia. CONCLUSIONS: Syncope frequently requires medical examination or admission to paediatric departments and, in the majority of cases, does not subtend serious diseases. Nevertheless, syncope always requires a complete medical evaluation in order to exclude underlying pathology. An electrocardiogram must complete the initial evaluation of all patients with syncope. Other investigations are required if clinical data and the ECG do not provide an etiological explanation of the episode.