The strange case of congenital mitral stenosis in an adult man with cor triatriatum.

We report the case of a 74-year-old male, with a medical history of cor triatriatum, admitted with a 10-day history of intermittent fever. Three sets of blood cultures were positive for Providencia rettgeri. Transthoracic and transesophageal echocardiogram excluded infective endocarditis, but revealed a congenital accessory tissue adhering to the mitral valve, causing supravalvular mitral stenosis. Cor triatriatum sinistrum is a rare congenital cardiac anomaly, even more uncommon in adults, and quite exceptional when associated with mitral valve disease. Because the patient had no symptoms related to the heart valve disease, no surgical indication was given and he was managed conservatively.

Right Ventricular Hypertrophy, Systolic Function, and Disease Severity in Anderson-Fabry Disease: An Echocardiographic Study.

BACKGROUND: Right ventricular (RV) involvement has been described in Anderson-Fabry disease (AFD), especially in patients with established Fabry cardiomyopathy (FC). However, few and controversial data on RV systolic function are available, and there are no specific tissue Doppler studies. METHODS: Detailed echocardiographic examinations were performed in 45 patients with AFD. FC, defined as maximal left ventricular wall thickness ≥ 15 mm, was present in 12. The Mainz Severity Score Index was calculated for each patient. Pulsed tissue Doppler was applied to the RV free wall at the tricuspid annular level and at the septal and lateral corners at the mitral annular level to obtain systolic tissue Doppler velocities (RV Sa, septal Sa, and lateral Sa, respectively). Twelve patients with amyloid light-chain cardiac amyloidosis were studied as a control group. RESULTS: Echocardiography revealed RV hypertrophy (RVH) in 31% of patients with AFD, all but one of whom were male and all of whom had concomitant left ventricular hypertrophy (LVH). All patients with AFD had normal RV fractional area change (47.9 ± 6.5%) and tricuspid annular plane systolic excursion (21.7 ± 3.2 mm) and all but one also had normal RV Sa (13.2 ± 2.2 cm/sec). RVH positively correlated with indices of LVH (r = 0.8, P = .0001, for all parameters evaluated), as well as with Mainz Severity Score Index (r = 0.70, P = .0001). Septal and lateral Sa were decreased in almost all patients (means, 7.7 ± 1.8 and 7.9 ± 1.9 cm/sec, respectively), irrespective of the presence of LVH. Compared with control subjects with cardiac amyloidosis, patients with FC showed better indices of RV systolic function (P < .001 for all: tricuspid annular plane systolic excursion, RV fractional area change, and RV Sa) despite similar RV wall thickness (6.2 ± 1.2 vs 6.9 ± 1.9 mm, P = NS). CONCLUSIONS: RVH is common in patients with AFD and correlates with disease severity and LVH. RVH, however, does not significantly affect RV systolic function. Patients with FC have better RV systolic function compared with those with cardiac amyloidosis with similar levels of RV thickness. The combination of low LV Sa values and normal RV Sa values might be helpful in the differential diagnosis of infiltrative heart disease.

Presence of 'ghosts' and mortality after transvenous lead extraction.

AIMS: The number of cardiovascular implantable electronic devices has increased progressively, leading to an increased need for transvenous lead extraction (TLE) due to device infections. Previous studies described 'ghost' as a post-removal, new, tubular, mobile mass detected by echocardiography following the lead's intracardiac route in the right-sided heart chambers, associated with diagnosis of cardiac device-related infective endocarditis. We aimed to analyse the association between 'ghosts' assessed by transesophageal echocardiography (TEE) and intracardiac echocardiography (ICE) and mortality in patients undergoing TLE. METHODS AND RESULTS: We prospectively enrolled 217 patients (70 ± 13 years; 164 males) undergoing TLE for systemic infection (139), local device infection (67), and lead malfunction (11). All patients underwent TEE before and 48 h after TLE and ICE during TLE. Patients were allocated to two groups: either with (Group 1) or without (Group 2) post-procedural 'ghost'. Mid-term clinical follow-up was obtained in all patients (11 months, IQR 1-34 months). We identified 30 (14%) patients with 'ghost', after TLE. The significant predictors of 'ghost' were Charlson co-morbidity index (HR = 1.24, 95% CI 1.04-1.48, P = 0.03) and diagnosis of endocarditis assessed by ICE (HR = 1.82, 95% CI 1.01-3.29, P = 0.04). Mortality was higher in Group 1 than in Group 2 (28 vs. 5%; log-rank P < 0.001). Independent predictors of mid-term mortality were the presence of 'ghost' and systemic infection as the clinical presentation of device infection (HR = 3.47, 95% CI 1.18-10.18, P = 0.002; HR = 3.39, 95% CI 1.15-9.95, P = 0.001, respectively). CONCLUSION: The presence of 'ghost' could be an independent predictor of mortality after TLE, thus identifying a subgroup of patients who need closer clinical surveillance to promptly detect any complications.

Thrombus in Transit: A Potentially Life-threatening Complication of Cerebral Sinus Thrombosis.

We report the case of a 41-year-old, 7-weeks-pregnant patient, presenting with headache and generalized seizure due to cerebral venous thrombosis complicated by jugular thrombosis and thrombus dislocation into right cardiac cavities. The patient was treated with intravenous heparin, and underwent embolectomy in extracorporeal circulation. This case illustrates the variability of cerebral venous thrombosis progression and a potentially life-threatening condition even during anticoagulant therapy.

Usefulness of intracardiac echocardiography for the diagnosis of cardiovascular implantable electronic device-related endocarditis.

OBJECTIVES: The goal of this study was to compare transesophageal echocardiography (TEE) and intracardiac echocardiography (ICE) for the diagnosis of cardiac device-related endocarditis (CDI). BACKGROUND: The diagnosis of infective endocarditis (IE) was established by using the modified Duke criteria based mainly on echocardiography and blood culture results. No previous studies have compared ICE with TEE for the diagnosis of IE. METHODS: We prospectively enrolled 162 patients (age 72 ± 11 years; 125 male) who underwent transvenous lead extraction: 152 with CDI and 10 with lead malfunction (control group). Using the modified Duke criteria, we divided the patients with infection into 3 groups: 44 with a "definite" diagnosis of IE (group 1), 52 with a "possible" diagnosis of IE (group 2), and 56 with a "rejected" diagnosis of IE (group 3). TEE and ICE were performed before the procedure. RESULTS: In group 1, ICE identified intracardiac masses (ICM) in all 44 patients; TEE identified ICM in 32 patients (73%). In group 2, 6 patients (11%) had ICE and TEE both positive for ICM, 8 patients (15%) had a negative TEE but a positive ICE, and 38 patients (73%) had ICE and TEE both negative. In group 3, 2 patients (3%) had ICM both at ICE and TEE, 1 patient (2%) had an ICM at ICE and a negative TEE, and 53 patients (95%) had no ICM at ICE and TEE. ICE and TEE were both negative in the control group. CONCLUSIONS: ICE represents a useful technique for the diagnosis of ICM, thus providing improved imaging of right-sided leads and increasing the diagnostic yield compared with TEE.

[Body mass index and cardiovascular events: the "obesity paradox"]. / Recenti acquisizioni sui rapporti tra indice di massa corporea e danno cardiovascolare: il "paradosso dell'obesità".

Excessive body mass among healthy subjects carries an increased risk of subsequent cardiovascular events. Excess weight implies the presence of white, viscero-abdominal fat, that promotes insulin-resistance, is infiltrated by macrophages, and is less differentiated compared to subcutaneous or brown fat. Conversely, among patients with cardiovascular disease, slim patients have a greater risk of recurrent atherothrombotic events than fatter patients ("obesity paradox"). Lean patients with cardiovascular disease, on average, have more comorbidities and haemorrhagic complications than their heavier counteparts, and probably they conceal predisposing factors that are still unknown and therefore difficult to treat.

Entrapment of a floating thrombus in the right atrium by persistent Chiari's network: a barrier to massive pulmonary embolism.

Syncope may be a manifestation of pulmonary embolism. Occlusion of a pulmonary artery with cardiac output drop, sometimes resulting in cardiac arrest, is the main underlying mechanism. In this letter we describe the case of a patient in which the persistence of a long remnant of the embryonal Chiari's network could have worked as an anatomic barrier preventing massive pulmonary embolism and cardiac arrest.

Transient global amnesia during transoesophageal echocardiogram.

Transient global amnesia (TGA) is a common paroxysmal disorder of episodic memory. The aetiology of TGA is still unknown. Brain ischaemia, migraine, epileptic seizure, venous congestion and psychological disturbances have been proposed as pathological mechanisms. Moreover, different precipitating events are recognised in most TGA patients including physical activity, severe emotional stress, painful experiences, immersion in cold water and sexual intercourse. We describe a 54-year-old woman who presented a TGA immediately after right-left shunt of saline contrast during the execution of transoesophageal echocardiography. Aetiopathological considerations for this uncommon presentation are discussed.

Characterization of the electroanatomic substrate in a case of noncompaction left ventricle.

An apparently healthy 43-year-old man was submitted to cardiological evaluation for sport activity. Two-dimensional echocardiography led to suspicion of noncompaction deformity, later confirmed by magnetic resonance imaging (MRI), left ventricular catheterization and endomyocardial biopsies. To exclude life-threatening arrhythmias, the patient was submitted to an electrophysiological study and to a real-time three-dimensional electroanatomic reconstruction of left ventricle. The electroanatomic map revealed extensive area of electrical abnormalities. Extent and localization of scar areas mostly corresponded to the areas of enhancement observed at MRI. The present report is the first on electroanatomic substrate evaluation in a noncompaction left ventricle. Our findings show that ventricular noncompaction is characterized by electrical abnormalities including low voltage and scar areas, mainly related to the presence and extent of myocardial fibrosis rather than noncompacted myocardium. Electroanatomic mapping may contribute to detect and quantify fibrotic areas in patients presenting this rare cardiomyopathy.

Combined percutaneous pulmonary valvuloplasty and patent foramen ovale closure in an adult with recurrent transient ischemic attacks.

We report the case of a 60-year-old man with a history of recurrent transient ischemic attacks, effort syncope, cyanosis, erythrocytosis and a systolic murmur. Echocardiography and catheterization showed severe pulmonary stenosis and a patent foramen ovale with a right-to-left shunt. The patient was submitted to combined percutaneous pulmonary valvuloplasty and patent foramen ovale closure using the Amplatzer device.