Multi-modality Imaging Evaluation of a Rare and Complex Case of Single Ventricle Physiology; the important role of Cardiac MR.

Congenital heart diseases (CHD) represent a major clinical and diagnostic challenge for correct abnormality identification and subsequent successful therapy; even more challenging is following-up patient health after multiple post-interventional corrections often required in complex cardio-vascular abnormalities. We describe a multi-modality imaging evaluation of a complex congenital cardio-vascular diseases, underlining the relevance of cardiac magnetic resonance to non invasively solve some issues related to postsurgical changes.

An unusual case of severe left ventricle outflow tract obstruction due to a coexistence of Takotsubo cardiomyopathy with septal hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a genetic disorder presenting with a pathological increase of left ventricle (LV) wall thicknesses. The most frequent morphological form is characterized by an abnormal LV basal septal hypertrophy. Tako-Tsubo cardiomyopathy (TTC) is a transient left ventricular systolic dysfunction induced by high physical or emotional stress. Its occurrence with HCM is unusual. However, this presentation occurs more often with the classic asymmetrical septal hypertrophy compared with the apical variant. This case demonstrates that the coexistence of TTC with septal HCM in an elderly patient may lead to a severe hemodinamic instability picture.

An unusual interventricular septal bounce in a patient with dermatomyositis: a case report.

BACKGROUND: In literature it has been reported in 1998, for the first time, a case of a 54-year-old man who developed constrictive pericarditis (CP) 12 years after diagnosis of dermatomyositis (DM). To our knowledge, this may be the only case reported. CASE SUMMARY: A 16-year-old man presented to our institution because of symptoms posing a suspicion for an inflammatory disease; after physical examination, lab tests, and other investigations (electromyography, magnetic resonance, and muscular biopsy) was diagnosed as having DM. Patient also showed hepatomegaly and congested jugular veins: after clinical and imaging investigations (transthoracic echocardiography and transoesophageal echocardiography) he was diagnosed as having a CP. Patient underwent pericardial resection and the final outcome consisted of a completely regression of the symptoms. DISCUSSION: Cardiac involvement in patients with DM ranges between 6% and 75%, and it can be clinically manifest or, far more frequently, sub-clinic. Pericardial involvement as a complication of DM is widely reported in the literature, but in almost all cases as acute pericarditis, effusive pericarditis or cardiac tamponade and almost never as a CP.

Aortic Regurgitation as a Risk Factor for Coronary Embolization from Complex Atheromatous Aortic Plaques: A Clinical Case.

Patients with mobile aortic arch atheroma and severe aortic regurgitation may be at higher risk of systemic embolism. We report the case of a 68-year-old male patient with complex aortic arch plaque with superimposed thrombus, in which an acute inferior ST-elevation myocardial infarction occurred. In the reported case, coronary embolism may have been caused by flaked aortic plaque and/or superimposed thrombus, which was possibly carried by aortic regurgitation flow. It is a very rare mechanism by which a coronary embolism is possible from an aortic complex plaque.

Prevalence and Clinical Relevance of Extracardiac Findings in Cardiovascular Magnetic Resonance Imaging.

PURPOSE: To assess the prevalence of extracardiac findings (ECF) during cardiovascular magnetic resonance (CMR) examinations and their downstream effect on clinical management. MATERIALS AND METHODS: We retrospectively identified 500 consecutive patients. Trans-axial balanced steady-state free precession nongated images acquired from the upper thorax to the upper abdomen were evaluated independently by 2 radiologists. ECF were classified as nonsignificant (benign, with no need for further investigation), significant (mandatory to be reported/monitored), and major (clinically remarkable pathology, mandatory to be reported/investigated/treated). Fifteen-month clinical follow-up information was collected through hospital records. RESULTS: Of 500 patients, 108 (21.6%) showed a total of 153 ECF: 59 (11.8% of the entire study population; 38.5% of all ECF) nonsignificant, 76 (15.2%; 49.7%) significant, and 18 (3.6%; 11.8%) major ECF. The most frequent ECF were pleural effusion, hepatic cyst, renal cyst, and ascending aorta dilatation. Of 94 significant and major ECF, 46 were previously unknown and more common in older patients. Newly diagnosed major ECF (n=11, 2.2% of the entire study population, and 7.2% of all ECF)-including 5 tumors (1% of study population)-were confirmed by downstream evaluations and required specific treatment. Patients with major ECF were significantly older than patients without with major ECF. Newly diagnosed clinically significant and major ECF prompted downstream diagnostic tests in 44% and 100% of cases, respectively. CONCLUSIONS: The detection of significant and major ECF is common during CMR reporting. The knowledge and the correct identification of most frequent ECF enable earlier diagnoses and faster treatment initiation of unknown extracardiac pathologies in patients referred to CMR imaging.

Therapy Of Cardiac Arrhythmias In Children: An Emerging Role Of Electroanatomical Mapping Systems.

INTRODUCTION: Cardiac arrhythmias are challenging diseases in childhood. Most of them in pediatric subjects (90.2%) are atrioventricular reentrant tachycardias and atrioventricular nodal reentrant tachycardias. The standard 12-lead ECG is a highly accurate diagnostic tool but an invasive electrophysiological study is often required. The main concern about this kind of procedures is their invasive nature and the need of radiations, so antiarrhythmic agents are currently the first line therapy. However, they often show side effects and can be insufficient for the rate control. MATERIALS AND METHODS: We performed a systematic research on Embase and PubMed. We found 563 articles and selected the most representative 50. DISCUSSION: Management of cardiac arrhythmias could be very difficult in several scenarios, especially in children with body weight <15 kg and age <4 years. In general, pediatric subjects show a cumulative risk of malignancy greater than adults, having greater life expectancy. On this basis the guiding principle during radiation delivery in electrophysiological procedures is "as low as reasonably achievable" (acronym: ALARA). The development of 3-dimensional (3D) electroanatomical mapping systems allowed significant reduction of exposure. The most recently reported experiences demonstrate safety and feasibility of fluoroless ablation in the most common arrhythmias in children, even in challenging conditions. CONCLUSION: The first reasonable approach in cardiac arrhythmias involving younger patients seems to be pharmacological. However antiarrhythmic drugs pose problems both in terms of side effects and often have poor efficacy. Expertise in electrophysiological techniques is constantly increasing and the development of new technologies allow us to encourage the use of electroanatomical mapping systems in order to reduce the radiation exposure in children undergoing to catheter ablation, especially for accessory pathways.

A Ruptured Mitral Valve Aneurysm as Complication of a Bicuspid Aortic Valve Endocarditis.

We present a case of a ruptured mitral valve (MV) aneurysm as a complication of a bicuspid aortic valve (BAV) endocarditis. It is about a young 35-year-old man, admitted to Cardiology Unit because of unexpected heart failure picture. We found a BAV endocarditis complicated by anterior MV-anterior leaflet aneurysm formation and subsequent severe MV regurgitation caused by aneurysm perforation. It was a particular and rare situation characterized by an infection of anterior mitral leaflet secondary to an infected regurgitant jet of a primary aortic infective endocarditis due to a BAV. A resulting aneurysm formation on the atrial side of the mitral anterior leaflet leads later to mitral perforation. In this article, we review the more relevant medical literature on this topic.

Rivaroxaban as an effective alternative to warfarin in a patient with atrial fibrillation, thrombophilia, and left atrial appendage thrombus: a case report.

BACKGROUND: Atrial fibrillation is the most common cardiac arrhythmia. It is responsible for up to 20% of all ischemic strokes. Rate control and anticoagulation are crucial for atrial fibrillation management and stroke prevention. CASE PRESENTATION: We present the case of an 84-year-old Italian woman with a left atrial appendage thrombus that developed despite her use of anticoagulant therapy with warfarin for a previous pulmonary embolism. She had atrial fibrillation and heterozygosity for both factor V Leiden and methylenetetrahydrofolate reductase C677T mutation, thus creating resistance to activated protein C. Anticoagulant therapy was switched to heparin for 1 week and then to rivaroxaban. After 3 months of rivaroxaban use, the thrombus disappeared. CONCLUSIONS: This case raises the issue of the ineffectiveness of warfarin therapy in complex cases involving particular thrombophilic conditions and the possibility of using rivaroxaban as a safe and effective alternative.

[Primary cardiac lymphoma: a case report]. / Linfoma primitivo cardiaco: un caso clinico.

Primary cardiac lymphomas are rare entities (1.3% of all primary cardiac tumors) of difficult clinical identification. We report a case of a primitive cardiac lymphoma in a 35-year-old immunocompetent patient, presenting with signs and symptoms of cardiac tamponade. Echocardiography revealed a lateral atrioventricular mass associated with large pericardial effusion. After pericardiocentesis, surgical excision was performed. Chemotherapy regimens were administered according to established protocols and were effective in inducing complete remission at 6 months.

Thrombophilia-Related Complications in the Treatment of a Left Atrial Appendage Thrombus: A Case Report.

Trans-esophageal echocardiography (TEE) revealed a left atrial appendage (LAA) thrombus in an 84-year-old woman with nonvalvular atrial fibrillation not known before our evaluation. In her medical history, there were hypertension, dyslipidemia and a previous pulmonary embolism. She was taking warfarin at time of our evaluation and presented signs and symptoms of heart failure. Together with heart failure treatment, intravenous anticoagulation with unfractionated heparin was initiated. Treatment was complicated by additional right lower limb embolic event and the LAA thrombus remained unchanged. Testing revealed heterozygosity for both the factor V Leiden and the methylenetetrahydrofolate reductase C677T mutations inducing resistance to activated protein C. The patient refused transcatheter closure of the left atrial appendage.

[Echo-Doppler evaluation of recent onset chronic venous insufficiency in elderly patients: does the heart have a role?]. / Valutazione eco-Doppler dell'insufficienza venosa cronica di recente insorgenza in soggetti anziani: il cuore potrebbe avere un ruolo?

The onset in elderly subjects of clinical signs of chronic venous insufficiency (CVI), without a previous history of venous disease of the lower limbs, raises questions about the etiology. In our study we evaluated the possible causes investigating the venous system of the lower limbs and right heart function in elderly subjects with signs of CVI. The alterations found were on the reduction of TAPSE, a significantly higher body mass index and a reduced ability to walk compared to the control group. The differences described could explain edema and skin changes of recent onset. If it is CVI functional type or of congestive heart failure in the preclinical stage will be clarified only by adequate follow-up.

[Congenital valvular heart disease with high familial penetrance]. / Valvulopatia congenita ad elevata penetranza familiare.

Bicuspid valve aortic (BVA) is one of the most common congenital malformations. Only 20% of patients preserves a normal valve function throughout life. There are sporadic and familial forms, the latter to autosomal dominant. We present a case of familiarity of BVA high penetrance. Patient with aortic stenosis by BVA, is the father of two children with BVA.

[Correlation between insulin resistance and endothelial dysfunction assessed by flow-mediated dilation]. / Correlazione tra insulino-resistenza e disfunzione endoteliale valutata mediante vasodilatazione flusso-mediata.

Insulin resistance and endothelial dysfunction are major cardiovascular risk factors. The purpose of this study was to determine whether only patients with insulin resistance also have an alteration of endothelial function assessed by ultrasound flow-mediated (FMD). In a population of 165 patients, 30 subjects had only a family history of diabetes and/or sporadic findings of hypoglycemia, and they were assessed for FMD, the glucose and insulin curve. Of these 30 subjects, 20 patients had an alteration of the parameters measured and were related with 20 healthy patients. The study group and the control group showed a markedly significant statistical difference in the performance of the curve insulin, in the calculation of FMD and HOMA. FMD allows to study populations with no apparent cardiovascular risk and to monitor the effectiveness of possible endothelial protective therapy.