[Complex right cervical aortic arch repair: less is more]. / Riparazione di arco aortico cervicale destroposto: "less is more".

Cervical aortic arch is a rare malformation that often has anatomical abnormalities of the supra-aortic trunks and may also be associated with aortic stenosis, aneurysms, or cardiac malformations. To correct them, symptomatic patients undergo surgery, which usually consists of a prosthetic graft repair, aortoplasty patch, or an end-to-end anastomosis. In addition, circulatory arrest and deep hypothermia are often required, as in aortic arch surgery. We report the case of a 13-year-old patient who underwent correction of a right cervical aortic arch stenosis with a post-stenotic aneurysm between the origin of the right carotid artery and the right subclavian artery. The anatomy of the aortic branches was abnormal. The surgical procedure consisted of an extensive resection with direct end-to-end anastomosis, without the use of a prosthetic graft, using moderate hypothermic cardiopulmonary bypass and without circulatory arrest.

Post-Myocardial Infarction Ventricular Septal Defect Closure by a Percutaneous Septal Occluder Device After Unsuccessful Surgical Closure: Never Lose Hope.

Post myocardial infarction ventricular septal defect (VSD) is a life-threatening complication following ST elevation myocardial infarction (STEMI). Current guidelines recommend the urgent VSD closure for its significant mortality. Despite VSD is generally treated by surgical repair, surgeons often refrain from early surgery due to extremely poor results. We report the case of a 76-year-old women admitted to our hospital for a subacute myocardial infarction complicated by acute heart failure with VSD and apical thrombosis. The patient underwent an urgent surgical repair of VSD with a bovine pericardium patch and concomitant double saphenous vein graft for the left anterior descending and the first diagonal branch. After two days an early surgical patch dehiscence was observed and a percutaneous closure was planned. Due to the particular morphology of the unnatural anatomy of the septum generated by the dehiscence, we decided to close the defect using an off-label device for ventricular rupture. A 30/30 mm Amplatzer ASD-MF occluder was successfully implanted. At one-year follow up the patient was alive without significant residual shunt.

Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study.

BACKGROUND: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies. METHODS: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database. RESULTS: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p < 0.001). Coronary unroofing was the most frequent procedure (56.4%). Operative mortality was 1.3% (2 patients with preoperative severe heart failure). At a median follow up of 18 months (range 0.1-23 years), 89.9% of survivors are in NYHA ≤ II, while only 3 elderly surgical patients died late. Return to sport activity was significantly higher in Surgical patients (48.1% vs 18.2%, p < 0.001). CONCLUSIONS: Surgery for AAOCA is safe and with low morbidity. When compared to Medical patients, who remain on exercise restriction and medical therapy, surgical patients have a benefit in terms of symptoms and return to normal life. Since the long term-risk of sudden cardiac death is still unknown, we currently recommend accurate long term surveillance in all patients with AAOCA.

Left ventricular giant rhabdomyoma in an infant with no tuberous sclerosis: accidental finding and complex management.

The accidental finding of a giant left ventricular rhabdomyoma in a female infant with no tuberous sclerosis is described herein. This is the first report of a huge cardiac rhabdomyoma occluding the left ventricular cavity, which was not associated with tuberous sclerosis. The clinical management of the baby and the difficult therapeutical choices involved both pediatricians and pediatric cardiologists.

Supraventricular tachycardia in neonates: antiarrhythmic drug choice dilemma.

Supraventricular tachycardia (SVT), being atrioventricular re-entry the underlying mechanism, is the most frequent tachyarrhythmia requiring a medical treatment in infants with no cardiac disease. The acute treatment of a single episode of SVT has generally an excellent prognosis. An antiarrhythmic prophylaxis of SVT recurrences is usually recommended during the first year of life. Although many efficient drugs are available for the SVT treatment, a careful risk-benefit analysis of each single case should suggest the correct drug choice.

Evaluation of regional myocardial function by Doppler tissue imaging in univentricular heart after successful Fontan repair.

BACKGROUND: The univentricular heart (UVH) corrected by Fontan repair is characterized by a single dilated pumping chamber, which is both hypertrophic and hypocontractile. The complex geometrical distortion and asynchronous contraction of this organ prevents assessment of systolic and diastolic function by traditional echocardiographic procedures. METHODS: Sixteen children (10 males, 6 females) aged 12-31 years, who had undergone UVH Fontan repair were enrolled in the study. A transthoracic echocardiography was performed. Twelve different myocardial wall segments from single ventricles were studied by Doppler tissue imaging to measure peak systolic velocity (S), isovolumetric relaxation time (IRT), isovolumetric contraction time (ICT), and E'/A' ratio. RESULTS: S and E'/A' ratio were significantly lower in the UVH group than in controls (P < 0.01 and P < 0.05, respectively). IRT and ICT were significantly longer in UVH than in controls (P < 0.001 and P < 0.005, respectively). In the UVH, both systolic and diastolic deficits were generally registered in the apical segments and also randomly distributed between the basal and middle segments of the single ventricle. CONCLUSIONS: It is an acknowledged fact that compared to normal children ventricular function is impaired in patients with UVH. Tissue Doppler imaging at multiple points of the single ventricle provides a complete, accurate assessment of systolic and diastolic function after Fontan repair, overcoming problems posed by geometrical distortion and limitations of conventional echocardiographic methods.

Cor triatriatum and lipomatous hypertrophy of the interatrial septum in the elderly: a case report.

Cor triatriatum is a rare congenital heart defect characterized by the presence of a fibromuscular membrane dividing the left atrium into two distinct chambers. Lipomatous hypertrophy of the atrial septum is an infrequently observed benign abnormality caused by large fatty tissue deposits in the interatrial septum. An increased incidence of atrial arrhythmias is described in both pathologies, while a significant obstruction of blood flow mimicking mitral stenosis is typically manifested in cor triatriatum. We report the case of a 75-year-old woman with a previously undescribed association of the above stated abnormalities detected by both transthoracic and transeosophageal echocardiography. Diagnosis was confirmed by means of computed tomography. The singular physiologic and anatomic factors underlying survival until such a late age are described. The diagnostic, therapeutic and surgical management is discussed and a short review of the literature performed.

COTS technologies for telemedicine applications.

PURPOSE: To demonstrate a simple low-cost system for tele-echocardiology, focused on paediatric cardiology applications. METHODS: The system was realized using open-source software and COTS technologies. It is based on the transmission of two simultaneous video streams, obtained by direct digitization of the output of an ultrasound machine and by a netcam showing the examination that is taking place. These streams are then embedded into a web page so they are accessible, together with basic video controls, via a standard web browser. The system can also record video streams on a server for further use. RESULTS: The system was tested on a small group of neonatal cases with suspected cardiopathies for a preliminary assessment of its features and diagnostic capabilities. Both the clinical and technological results were encouraging and are leading the way for further experimentation. CONCLUSIONS: The presented system can transfer clinical images and videos in an efficient way and in real time. It can be used in the same hospital to support internal consultancy requests, in remote areas using Internet connections and for didactic purposes using low cost COTS appliances and simple interfaces for end users. The solution proposed can be extended to control different medical appliances in those remote hospitals.