Arrhythmogenic right ventricular cardiomyopathy/dysplasia.

We describe the case of a 51-year-old woman with a 10-year history of dyspnoea and fatigue on slight effort, presyncopal episodes, and ventricular extrasystolic arrhythmia. Tests were negative for coronary artery disease, valvular disease, or left ventricular dysfunction. The patient fulfilled the clinical criteria for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) and the diagnosis was confirmed histologically with an endomyocardial biopsy. During 5-year follow up she also exhibited significant structural progression to the left ventricle. This is a rare case of ARVC/D manifested in middle age, with a negative family history, negative test for desmosome mutations, and negative myocardial immunohistochemical analysis, evidence that tends to suggest an acquired form of the disease. We also present a brief review of the clinical, electrocardiographic, structural, pathological/anatomical and genetic characteristics of the disease, the diagnostic criteria, prognosis, management, and sudden death prevention, as well as the way we have managed our patient until the present day.

Doppler tissue imaging unmasks right ventricular function abnormalities in HIV-infected patients.

BACKGROUND: We sought to investigate right ventricular (RV) function with Doppler tissue imaging (DTI) in human immunodeficiency virus (HIV)-infected patients receiving highly-active antiretroviral treatment, without any heart-related symptoms. METHODS: We studied 38 asymptomatic HIV patients (aged 44.5 ± 9.2 years, 22 of them men) and 25 age-matched and sex-matched controls. All subjects underwent conventional and DTI estimation of left ventricular (LV) systolic and diastolic function, measuring peak systolic and diastolic myocardial velocities at the mitral annulus (Sm, Em, Am). Two-dimensional (2-D) echocardiographic study of the right ventricle (RV) was performed from the four-chamber view, and RV end-diastolic dimensions were measured. DTI recordings from the RV free wall at the tricuspid annulus were used to determine systolic (SmRV) and diastolic function (EmRV and AmRV). RESULTS: HIV-infected patients compared to controls exhibited significantly lower peak systolic velocities at the septal-SmIVS (7.9 ± 1.3 vs 9.1 ± 1.4 cm/s, p = 0.002) and lateral mitral annulus - SmLAT (9.8 ± 1.7 vs 11.2 ± 1.3 cm/s, p = 0.025); no difference was observed regarding conventional 2-D examination of LV systolic and diastolic function and DTI-derived Em and Am. No significant difference occurred between HIV patients and controls regarding RV end-diastolic dimensions and pulmonary artery systolic pressure. However, SmRV (13.8 ± 1.6 vs 14.9 ± 2.2 cm/s, p = 0.040), EmRV (11.6 ± 3 vs 13.5 ± 2.6 cm/s, p = 0.028) and AmRV (10.9 ± 2.5 vs 13.8 ± 4 cm/s, p = 0.003) were significantly reduced in HIV patients as compared to controls. CONCLUSIONS: DTI unmasks subtle and otherwise undetectable abnormalities of the longitudinal LV systolic function and both RV systolic and diastolic function, in asymptomatic HIV patients receiving highly-active antiretroviral treatment.

Acute inferior ST-segment elevation myocardial infarction treated with primary angioplasty using only a Pronto aspiration catheter.

In a patient with acute myocardial infarction treated with primary angioplasty, a large amount of thrombus was removed from the middle segment of the right coronary artery using a Pronto V3 extraction catheter (Vascular Solutions, Inc., Minneapolis, Minnesota). Repeat angiography revealed no significant residual stenosis and no further intervention was undertaken.

Clinical value of B-type natriuretic peptide for the assessment of left ventricular filling pressures in patients with systolic heart failure and inconclusive tissue Doppler indexes.

Invasive hemodynamic monitoring with Swan-Ganz catheterization to guide treatment decisions in heart failure may be hazardous and may lack prognostic value. We assessed the clinical utility of B-type natriuretic peptide (BNP) in estimating left ventricular filling pressures in patients with inconclusive tissue Doppler indexes. In this study, 50 patients with systolic heart failure and an early transmitral velocity to early diastolic mitral annular velocity ratio (E/Ea) between 8 and 15 were studied. Among them, 25 had been admitted for acutely decompensated heart failure (group A) and the remainder were clinically stable outpatients (group B). All patients underwent simultaneous invasive pulmonary capillary wedge pressure (PCWP) determination, BNP measurement, and echocardiography. In group A, BNP correlated with PCWP (r = 0.803, P < 0.001), deceleration time (DT, r = -0.602, p = 0.001), and end-systolic wall stress (SWS, r = 0.565, P = 0.003). In multivariate analysis, BNP was the only parameter independently associated with PCWP (P = 0.023). In group B, no correlation was found between BNP and PCWP or SWS, while DT correlated significantly with both PCWP (r = -0.817, P < 0.001) and BNP (r = -0.8, P < 0.001). We conclude that BNP may be a useful noninvasive tool for the assessment of left ventricular filling pressures in patients with acutely decompensated heart failure and inconclusive tissue Doppler indexes.

Traumatic dissection of the left circumflex artery after a fall from height.

The authors present the case of a healthy 40-year-old man who developed a myocardial infarction because of traumatic dissection of the left circumflex coronary artery following a fall from height. To our knowledge, this is the first time that a traumatic lesion of the left circumflex artery is being reported after a fall.

Primary myxofibrosarcoma of the left atrium: case report and review of the literature.

A 63-year-old woman with progressive dyspnea underwent transthoracic echocardiography and was found to have a large multilobed mass in the left atrium that was attached to lateral wall. On inspection during surgery, the tumor was found to infiltrate the posterior mitral annulus and leaflet. The patient underwent surgical resection of the tumor and mitral valve replacement. Histologic and cytochemical evaluation confirmed that the tumor was a myxofibrosarcoma. Despite chemotherapy, the tumor recurred and the patient died 3 months after surgery.

Esmolol facilitated extubation in a patient with severe systolic dysfunction following myocardial infarction.

We describe the case of a 59-year-old-man with acute myocardial infarction and severely impaired left ventricular systolic function who was intubated because of recurrent ventricular fibrillation in the setting of coronary angioplasty. Repeated ventilator weaning attempts and extubation initially failed, as severe tachycardia and hypertension occurred each time the patient began to awaken. Pre-treatment with esmolol infusion prevented the above haemodynamic changes, allowing successful extubation. Esmolol administration at ventilator weaning seems to be a safe and effective option, even in selected patients with impaired left ventricular contractility.

Congenitally corrected transposition of the great arteries in a seventy-year-old woman.

Corrected transposition of the great arteries is a rare condition, and few patients with this abnormality survive past 50 years of age because of associated defects, or the subsequent development of atrioventricular valvular insufficiency or heart block or both. The case of our patient is of interest not only because she reached old age, but also because she lived a normal life, presenting with minor cardiac impairment and palpitations at the age of 70 years.

Primary pericardial fibrosarcoma presenting as "near" cardiac tamponade.

A 19-year-old male presented with fever, substernal pain, dyspnea, and distended neck veins. Diagnostic investigations, such as echocardiography and magnetic resonance imaging, provided evidence of a large mass within the pericardial sac, attached by a broad base to the parietal pericardium and lying along the right ventricular free wall. A partial pericardiectomy was performed to relieve the patient's symptoms, and histologic examination of a biopsy specimen showed features of a malignant, spindle cell, mesenchymal neoplasm. The patient underwent surgical treatment during which the tumor was found to infiltrate the anterior surface of the right ventricle. Histologically, the tumor was identified as a high-grade fibrosarcoma, and additional chemotherapy was given.

Fistulous Connection of a Greatly Dilated Tortuous Right Coronary Artery to the Coronary Sinus.

Congenital coronary artery fistulas (CAFs) constitute an unusual cardiovascular anomaly. The aneurysmal appearance of CAFs is not uncommon and depends on the shunt size. However, few cases of ectatic coronary arteries (type III according to Markis et al. classification-diffuse ectasia in one vessel) supplying the fistulas have been reported. Below, we report the case of a 65-year-old woman, who referred to our department because of worsening exertional dyspnea. Echocardiographic evaluation, both transthoracic and transesophageal, performed after admission disclosed a giant tortuous ectatic right coronary artery with a fistulous connection to the coronary sinus. The presence of the CAF was confirmed by cardiac catheterization. In addition, we discuss the pathophysiology of the above congenital anomaly, as well as its management.