Abstract: A rare case of prosthetic valve endocarditis from Acinetobacter spp occurring 9 months postoperatively is described. The patient initially received empirical therapy against pathogens commonly associated with prosthetic valve endocarditis, but his condition did not improve. Identification of bacteremia due to Acinetobacter spp was not attributed to any of the classic nosocomial factors such as presence of a catheter or a recent invasive procedure. The patient did well with an intravenous regimen of meropenem and tobramycin instituted according to susceptibility testing. Physicians should be aware of this rare association of a nosocomial pathogen such as Acinetobacter spp with prosthetic valve endocarditis occurring long after the initial cardiothoracic procedure.
Abstract: We evaluated the hypothesis that a relationship exists between inflammation and the outcome of pharmaceutical cardioversion with amiodarone in recent onset atrial fibrillation. We studied 86 patients with symptomatic recent onset AF and coexisting hypertension and/or chronic stable coronary artery disease. All study participants underwent evaluation with a standardized protocol including echocardiography, cytokine level measurement [interleukin-2 (IL-2), interleukin-6 (IL-6) and high sensitivity C reactive protein (hsCRP)] on admission and at 48h, and administration of intravenous amiodarone. By 48h, 70 patients cardioverted to sinus rhythm. Median serum IL-2 levels on admission were higher in non-cardioverted compared to cardioverted patients (P=0.002). At 48h, non-cardioverted had significantly higher IL-6 (P=0.005) and hsCRP values (P=0.001) compared to cardioverted. Multivariate logistic regression analysis showed that lower IL-2 admission levels were a powerful independent predictor for successful cardioversion (OR: 0.154, 95% CI: 0.043-0.552, P=0.004). In patients with hypertension and/or chronic stable coronary artery disease and symptomatic recent onset AF, low serum IL-2 levels on admission are associated with successful cardioversion with amiodarone. This observation highlights the role of inflammation in AF and might have further prognostic and therapeutic implications.
Abstract: A middle-aged male patient with progressive right ventricular heart failure due to pulmonary thromboembolism causing pulmonary hypertension was referred for evaluation of a mobile right ventricular mass. The mass originated from the pulmonary valve and was removed in surgery. Pathological assessment showed that the mass was a thrombus located on the pulmonary valve. Echocardiographic images before surgery as well as images of the removed mass and its histology, are presented to describe this very rare cause of pulmonary thromboembolism.
Abstract: OBJECTIVES : The aim of this study was to evaluate the longterm follow-up results of percutaneous transluminal septal myocardial ablation (PTSMA) in a large patient cohort. BACKGROUND : PTSMA by alcohol injection into septal branches has shown good acute and short-term results in symptomatic patients with hypertrophic obstructive cardiomyopathy. METHODS : A total of 100 consecutive symptomatic (NYHA class 2.8 +/- 0.6) patients underwent PTSMA. All patients had clinical and non-invasive follow-up at 3 months, 1 year, and annually up to 8 years. RESULTS : One patient died at day 2 after intervention due to fulminant pulmonary embolism following deep venous thrombosis, and eight patients required a permanent DDD-pacemaker due to post-interventional complete heart block. Acute reduction of the left ventricular outflow tract gradient was achieved from 76 +/- 37 to 19 +/- 21 mmHg at rest, from 104 +/- 34 to 43 +/- 31 mmHg during Valsalva maneuver, and from 146 +/- 45 to 59 +/- 42 mmHg post extrasystole (p < 0.0001, each). During follow-up (mean follow-up time: 58 +/- 14 months), three additional patients died (sudden death at 48 months, non-cardiac death at 49 months and stroke-related death at 60 months after the index procedure). All living patients showed clinical improvement to NYHA-class 1.4 +/- 0.6 (after 3 months, n = 99), 1.5 +/- 0.6 (after 1 year, n = 99), and 1.6 +/- 0.7 at final follow-up (n = 96; p < 0.0001, each). Non-invasive follow-up studies documented ongoing outflow tract gradient reduction, decrease of septal and left ventricular posterior wall thickness, and improvement of exercise capacity. CONCLUSIONS : PTSMA is an effective treatment for symptomatic patients with hypertrophic obstructive cardiomyopathy. Follow-up showed ongoing hemodynamic and clinical improvement without increased mortality and morbidity.
Abstract: To further examine the genetic and clinical features of hypertrophic cardiomyopathy caused by mutations in the cardiac troponin T (cTnT) gene, we screened 143 probands from our hypertrophic cardiomyopathy population for mutations in this gene. We report that the Arg278Cys missense mutation in the cTnT gene had a different clinical presentation in 2 different families and was associated with a clinical profile that deviates from what is currently expected for cTnT gene mutations.
Abstract: OBJECTIVES: Left main coronary artery dimensions were evaluated in patients with and without arterial hypertension, in the presence of coronary artery disease, and correlated to left ventricular mass. METHODS AND RESULTS: Intracoronary ultrasound was performed in 25 patients (pts) with coronary artery disease and hypertension (+HTN) and in 25 pts with coronary artery disease only (-HTN). Maximal left main dimensions and left ventricular mass index (LVMI) were measured. +HTN pts had greater LVMI compared to -HTN, (+HTN = 183 +/- 17 g/m2 vs. -HTN = 82 +/- 22 g/m2, p < 0.0001), while maximal left main vessel area was similar (25.99 +/- 5.01 mm2 vs. 25.62 +/- 3.3 mm2, p = 0.8). No correlation was found between LVMI and left main vessel area in + HTN. CONCLUSIONS: Pts with +HTN despite the increased LVMI do not manifest increased LM dimensions. This may affect mid-term clinical outcome.
Abstract: BACKGROUND: Some mutations of cardiac sarcomeric proteins causing hypertrophic cardiomyopathy (beta-myosin heavy chain) are associated with skeletal muscle fiber dysfunction, while subclinical skeletal myopathy can be diagnosed by electromyography (EMG) in a substantial proportion of hypertrophic cardiomyopathy patients. METHODS: In 49 consecutive, unrelated patients with hypertrophic cardiomyopathy, conventional EMG of deltoid, vastus lateralis, tibialis anterior and soleus muscles was performed. No patient had clinically detectable muscle weakness. We compared the clinical and echocardiographic characteristics between patients with normal and patients with myopathic EMG. RESULTS: Myopathic EMG findings were demonstrated in 13 patients (26.5%), 26 patients (53.1%) had normal findings and 10 patients (20.4%) had indeterminate recordings. There was no significant difference in mean age, maximum wall thickness, left ventricular fraction shortening, NYHA class, the existence of left ventricular outflow tract obstruction, syncope, or the occurrence of nonsustained ventricular tachycardia in the Holter recording among the three groups. Comparison between the myopathic and the normal group revealed that nine patients from the latter (34.6%) had a positive history of sudden death in the family, whereas no patient had such a history in the former group (P=0.015). CONCLUSION: The higher prevalence of a family history of sudden death in patients with normal EMG, although not thoroughly explained by our data, may reflect differences in the genetic substrate produced by the higher prevalence of high-risk mutations that are not expressed in skeletal muscle (e.g. troponin T). Further evaluation in genotyped patients is warranted.
Abstract: Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disease characterized by unexplained left ventricular hypertrophy, typically involving the interventricular septum. Hypertrophy may be present in infants, but commonly develops during childhood and adolescence. Management of children with HCM aims to provide symptomatic relief and prevention of sudden death, which is the primary cause of death. Unfortunately, no randomized comparative trials to date have assessed different treatment options in HCM. Medical treatment with negative inotropic agents (beta-adrenoceptor antagonists [beta-blockers], verapamil) is the first therapeutic choice in all symptomatic patients. Beta-blockers also appear to have prognostic merit in children. Surgical myectomy is effective in reducing symptoms in children with left ventricular (LV) obstruction who are unresponsive to medical treatment, although a repeat operation may be needed in a substantial proportion of patients due to relapse of LV obstruction. The recently introduced percutaneous septal ablation can also be regarded as a feasible alternative in this cohort. Technical limitations of both invasive therapeutic options should be carefully considered, preferably in experienced centers. Results of recent randomized trials indicate that dual chamber pacing, once considered a therapeutic option for patients with HCM, should only be used as treatment for conduction abnormalities. Regular clinical risk stratification for sudden death is of vital importance for the prevention of sudden death in young patients. Familial history of sudden death at a young age, LV hypertrophy >3 cm, unexplained syncope, nonsustained ventricular tachycardia in Holter monitoring, and abnormal blood pressure response during exercise are currently considered clinical risk factors for sudden death. Each factor has a low positive predictive accuracy, but patients having two or more of these risk factors are deemed as high risk. Secondary prevention of sudden death in patients successfully resuscitated from cardiac arrest and/or sustained ventricular tachycardia warrants treatment with an implantable cardioverter defibrillator (ICD). Primary prevention of sudden death in patients considered to be at high risk should aim at the management of obvious arrhythmogenic mechanisms (paroxysmal atrial fibrillation, sustained monomorphic ventricular tachycardia, conduction system disease, accessory pathway, myocardial ischemia), and the prevention and/or management of ventricular tachyarrhythmias with amiodarone and/or ICD implantation, respectively. The choice of treatment in children is greatly influenced by technical aspects, such as adverse effects of amiodarone, and ICD implantation difficulties or complications. Amiodarone could also be used as a bridge in children at high risk, until they reach adulthood, possibly achieving a lower risk status, or until their physical growth permits ICD implantation as long-term therapy.
Abstract: Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy, which appears to represent an arrest in intrauterine endomyocardial morphogenesis. It is diagnosed both in children and adults. Its common presentation involves heart failure symptoms, ventricular tachyarrhythmias and thromboembolic events, but the age of onset varies widely. The diagnosis is made by the combined appearance of numerous, excessively prominent trabeculations and multiple deep intertrabecular recesses perfused from the ventricular cavity, commonly involving the apical and midventricular segments of the left ventricle. Although the peculiar echocardiographic picture may possibly lead to the correct diagnosis, this condition may be often misdiagnosed or unrecognized since it is not widely known.
Abstract: Hypertrophic cardiomyopathy (HCM) is due to a number of mutations of contractile protein genes such as beta-cardiac myosin, myosin binding protein-C, and troponin-T. Unlike troponin-T, beta-myosin is a constituent of slow skeletal muscle and its mutations generally have a better prognosis. In order to investigate the usefulness of electromyography in detecting skeletal muscle involvement in HCM, 46 patients were examined using both conventional electromyography (EMG) and quantitative electromyography (QEMG) methods. The QEMG involved motor unit potential (MUP) analysis, turns/amplitude (TAA) analysis, and power spectrum analysis of the interference pattern. Using conventional EMG, myopathic findings were demonstrated in 13 patients (28%). Receiver operating characteristic (ROC) analysis of the results of a discriminant function extracted using QEMG values, identified correctly 10 out of 11 normal controls and all 9 myopathic control patients, and displayed a 15% presence of myopathy (7 patients) among the cardiomyopathy group. The duration of MUPs was the most sensitive among the quantitative parameters in differentiating normal from myopathic subjects. Since skeletal muscle involvement may be due to distinct gene mutations, normal and myopathic EMG findings may reflect HCM subpopulations with a different genetic substrate.
Abstract: We showed previously that the handgrip apexcardiographic test (HAT) is a useful method for detecting left ventricular (LV) diastolic abnormalities in patients with coronary artery disease and systemic hypertension. This study evaluates the use of HAT for assessing the prevalence and types of exercise-induced diastolic abnormalities in patients with obstructive (n = 31) and nonobstructive (n = 35) hypertrophic cardiomyopathy (HC) as well as its potential value for separating healthy subjects and athletes from patients with HC. We obtained a HAT in 66 consecutive patients with HC and in 72 controls (52 healthy volunteers and 20 athletes). A positive HAT was defined by the presence of one of the following: (1) relative A wave to total height (A/H) during or after handgrip > 21% (compliance type), (2) total apexcardiographic relaxation time (TART) > 143 ms or the heart rate corrected TART (TARTI) during handgrip < 0.14, (relaxation type), (3) both types present (mixed type), and (4) diastolic amplitude time index (DATI = TARTI/[A/D]) during handgrip < 0.27. Of the controls, only 1 of 52 healthy subjects and 1 of 20 athletes showed a positive HAT, whereas of the total HC cohort 63 of 66 patients (95%) had a positive result. There was no significant difference in the distribution of these types between obstructive and nonobstructive HC. Further, no LV diastolic abnormalities were present in 10 of 35 patients (29%) with nonobstructive HC at rest and in 3 of 35 patients (9%) during handgrip, whereas of the patients with obstructive HC only 1 of 31 (3%) had no LV diastolic abnormalities at rest and none during handgrip. Based on HAT data, our study demonstrates that in HC (1) LV diastolic abnormalities are very frequent during handgrip; (2) patients with nonobstructive HC show significantly fewer LV diastolic abnormalities at rest than those with obstructive HC; and (3) no significant difference exists between obstructive and nonobstructive HC in the prevalence of types of handgrip-induced LV diastolic abnormalities. Consequently, HAT appears to be of clinical value as an additional tool for separating normal patients and athletes from patients with HC.
