A coronary right fistula canalized in a small accessory right atrial chamber.

The coronary artery fistulas are rare congenital anomalies with a very low incidence. These can be symptomatic or asymptomatic because the hemodynamic consequences of the fistula vary and depend on the shunt dimensions. Discordant opinions instead are present in the literature for the defect closing in asymptomatic patients. Here, we describe a patient affected by a coronary right fistula canalized in a small accessory right atrial chamber. During follow-up, we observed a progressive dilatation of the right coronary artery (maximum diameter 10.3 mm) with hemodynamic overload of the right sections.

Chronic pharmacological treatment in takotsubo cardiomyopathy.

Abstract Background Takotsubo cardiomyopathy is a disorder that has been appreciated only recently. In most of reported cases, this syndrome mimes an acute myocardial infarction. Till this moment no data are available from literature about the treatment in the acute phase of this disease. Aim of the study In our multicentric experience we have retrospectively looked at the benefits of a treatment with ACE-inhibitors, beta-blockers, Aspirin and calcium channels blockers, started until the early phases of the disease and continued for 30 days, in 36 patients affected by Takotsubo cardiomyopathy. We chose as endpoint of the study the efficacy of the used drug in improving left ventricular myoc…

Heart rate turbulence for guiding electric therapy in patients with cardiac failure

It is well-known that a reduction of the cardiac frequency variability, measurable with the Heart Rate Variability (HRV) system, is an indirect expression of the sympathetic-autonomic tone. Another index, Heart Rate Turbulence (HRT), has been recently suggested as a possible unit of measurement for the sympathetic-autonomic tone: this system allows to estimate the baro-reflex response of the carotid arteries to an early ventricular extra-systole by analysing heart rate variations induced by a premature beat.In our research we have analyzed this phenomenon in patients affected by moderate or severe cardiac failure. In particular, we divided 110 patients into two arms: subjects with or withou…

Reduced regional systolic function evolved compacted segments in noncompaction

Abstract In a recent report about noncompaction Nemes et al. reported that systolic left ventricular (LV) dysfunction assessed using the wall motion score during 2D echocardiography in noncompaction is not confined to noncompacted LV segments. These results confirmed some published reports of our group. Recently we analyzed a population of 238 patients affected by noncompaction, and demonstrated that the number of noncompacted segment is uncorrelated with the degree of systolic dysfunction. It is an attractive hypothesis that the systolic dysfunction could be depend on the fibrosis of the left ventricle, itself a consequence of microcirculatory dysfunction, which has been confirmed by repor…

Cardiovascular magnetic resonance characterization of a hamartoma in an asyntomatic child

Abstract Background The prevalence of primary cardiac neoplasms is approximately 0.3% and these masses should be distinguished from many of other primary and secondary processes that can occur in the heart. Further assessment of the left ventricular mass presents important clinical implications. Cardiovascular magnetic resonance was used. Clinical case An asyntomatic 12-year-old child was referred for cardiovascular magnetic resonance imaging to further assess a left ventricular mass found after an echocardiography, executed for assessment for sport activity. His past medical history was absolutely negative. The patient was in optimal state of health. The EKG showed an aspecific ST elevatio…

SYMPTOMATIC ACUTE MYOCARDIAL INFARCTION IN A PATIENT BEARER OF HEART TRANSPLANTATION FOLLOWING ISCHEMIC HEART DISEASE

In 2005 Syeda et al. reported that the major factor limiting the long term of cardiac transplantation is the development of accelerated arteriosclerosis that occurs in the coronary arteries of the cardiac allograft. Transplant arteriosclerosis is characterized by diffuse, uniform, concentric narrowing of the artery by a fibrous proliferation of sub-intima cells. This atherosclerosis was estimate to occur in approximately 50% of patients by 5 years after transplantation. Unfortunately, as a consequence of cardiac denervation, symptoms are often atypical or completely absent. When these are present, the symptoms are those typical of effort angina. Very uncommon is the acute coronary syndrome.…

Ventricular dysfunction and number of non compacted segments in non compaction: Non-independent predictors.

Abstract Background Isolated ventricular noncompaction (IVNC) is characterized by multiple prominent trabeculations and deep intertrabecular recesses. Some reports prove that the chronic heart failure may occur in approximately half of the patients. In this report we investigate the correlation between the number of non compacted segments and entity of systolic dysfunction from the registry and subregistries of the SIEC. Method To identify the correlation between ventricular dysfunction and number of segments involved in non compaction we evaluated a consecutive series of 238 patients affected by non compaction, from the SIEC (Societa Italiana di Ecografia Cardiovascolare) registry. The ave…

Left ventricular non-compaction cardiomyopathy in children: Is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction?

Noncompaction of the ventricular myocardium (LVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. In 2002 Jenni et al. [Jenni R, Wyss CA, Oechslin EN, Kaufmann PA. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol 2002; 39:450-454.] reported a microvascular dysfunction in 12 patients affected by non compaction: areas of restricted myocardial perfusion have been documented by scintigraphy, suggesting a reduction of Coronary flow reserve. McMahon et al reported in a recent article a reduction of TD velocities in children with noncompaction of the left ventricle, compared with nor…

Diagnosis and definition of biventricular non-compaction associated to Ebstein's anomaly

Abstract Background Non-compaction of ventricular myocardium is a rare congenital cardiomyopathy characterized by the presence of an extremely thickened endocardial layer with prominent trabeculations and deep recesses in communication with ventricular chamber and determining the typical spongeous aspect. The diagnosis of non-compaction of ventricular myocardium is possible through the identification of morphological alterations by echocardiographic evaluation. Ebstein's anomaly is a rare congenital cardiac disease, defined as the significant apical displacement of the part of the tricuspid valve causing significant tricuspid regurgitation and reduction of the functional right ventricle, ri…

Neuromuscular disorders and non compaction

Abstract For neurological disorders we consider every possible affection of the central nervous system or neuro-muscular junction, in the presence of complex syndromes as well, such as Cornelia De Lange syndrome or any other. In our experience the incidence of neurological or neuromuscular envolment in patients affected by noncompaction is low. An incidence of comorbidities higher than 20% is not recorded in any centre, in published experiences. Data of the Vienna group could be likely influenced by selection bias (second level centre) or territorial genetic factors, or could depend on higher sensibility of used diagnostic techniques of neurological disease. But, if it was so, in our opinio…

Ventricular tachycardia in non-compaction of left ventricle: Is this a frequent complication?

Background: Isolated left ventricular non-compaction is the result of incomplete myocardial morphogenesis, leading to persistence of the embryonic myocardium. The condition is recognized by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricle. Whether these intertrabecular recesses are a favorable substrate for ventricular arrhythmias is unclear. Some reports have found that the fatal ventricular arrhythmias may occur in approximately half of the patients. In this report we investigated about this association. Methods and Results: In total we evaluated a continuous series of 238 patients affected by non-compaction. Periodic Holter monitoring w…

Anticoagulant drugs in noncompaction: A mandatory therapy?

BACKGROUND: Noncompaction of left ventricular myocardium is a rare congenital cardiomyopathy resulting from an incomplete myocardial morphogenesis that leads to the persistence of the embryonic myocardium. This condition is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. It is not clear, in noncompaction of myocardium, whether intertrabecular recesses could be responsible for thrombi formation and thromboembolic complications. METHODS: The prevalence of stroke and echocardiographic finding of thrombus was evaluated in a continuous series of 229 patients (men and women) affected by nonc…

THE NONCOMPACTION OF THE LEFT VENTRICULAR MYOCARDIUM: OUR PEDIATRIC EXPERIENCE

OBJECTIVES: The noncompaction of the left ventricular myocardium is a rare congenital heart disease, characterized by an excessive prominence of trabecular meshwork, spaced out by deep intertrabecular recesses, consequent to the arrest of the normal myocardium embryogenesis. Although there are numerous descriptions, the physiopathological effects of the structural alterations, just like the clinical spectrum and the evolution of the disease, are not totally clarified. In the present study, we have evaluated the natural history of the disease, the familial incidence and the alterations of the systolic and diastolic function. METHODS: We collected a series of 21 young patients who were affect…

AZITHROMYCIN IN AN OLDER WOMAN WITH DIABETIC GASTROPARESIS

Diabetic neuropathy is a common chronic complication of diabetes and cause of significant morbidity and mortality, because it may involve the autonomous and peripheral nervous systems. Autonomic diabetic neuropathy is a challenging chronic complication of long-standing diabetes manifested with hypotension, syncope, gastroparesis, diarrhea, constipation, bladder dysfunction, sexual dysfunction, cardiac arrest, and/or sudden death. We present a case of diabetic gastroparesis in an older woman. The patient was an 83-year-old woman with a 40-year history of type 2 diabetes who was admitted with hypoglycemia, malnutrition, persistent vomiting, and obstinate constipation. After several unsuccessf…

Treatment of Tako-tsubo cardiomyopathy

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Neuromuscular disorders and non compaction: How much is the strength of the association and how can it be suspected?

In many reports Finsterer and Stöllberger reported a strong association between non compaction of the left ventricle and neuromuscular disorders. In the same report the authors described a neurological involvement in more than 50%. Recently we published our personal experience, about 21 paediatric patients: only 4 patients (19%) showed a neuromuscular disorder, and only 1 of them showed an increased plasmatic level of CK, and in particular of MM isoform, with a normal level of CK-MB. None presented high levels of troponine. Through the experience of 3 centres we collected 61 patients affected by non compaction that performed a neurological control, and only 14 (21%) were affected by neuromu…

Supraventricular arrhythmias in noncompaction of left ventricle: Is this a frequent complication?

Background: Isolated left ventricular noncompaction is the result of incomplete myocardial morphogenesis, leading to persistence of the embryonic myocardium. The condition is recognised by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricle. Whether these intertrabecular recesses are a favorable substrate for supraventricular arrhythmias is unclear, even if the incidence of chronic heart failure seems to be high. Results: We evaluated a continuous series of 238 patients affected by noncompaction. In 4 cases the patients reported palpitations and in 4 an episode of syncope. Periodic holter monitoring was performed every 6 months for 4 years. O…

A cardiac fibroma in a 7-year-old asymptomatic girl admitted for ECG anomalies

Helicobacter pylori and atrial fibrillation: absence of correlations

Segmental dyskinesia in Wolff–Parkinson–White syndrome: A possible cause of dilatative cardiomyopathy

Wolff-Parkinson-White (WPW) is a syndrome characterized by the presence of an accessory pathway that skipping A-V node may lead the electrical stimulus from the atrium directly to the ventricle. Some studies reported the finding of myocardial dyskinesia in the segments precociously activated by the accessory pathway, at echocardiogram and at nuclear cardiac study. Soria et al. reported, in 1985, an increased incidence of dilative cardiomyopathy in patients with WPW. The pathophysiological pathway that leads to ventricular dilation may be due to the increase of end-diastolic pressure secondary to a tachycardia-induced cardiomyopathy. Tachycardia-induced cardiomyopathy is usually secondary to…

Transient mid-ventricular dyskinesia: a variant of Takotsubo syndrome

Takotsubo Cardiomyopathy is characterized by a reversible systolic left ventricular apical ballooning. A new pattern of dyskinesia in the absence of angiographic evidence of coronary artery stenosis has been indicated like a variant of takotsubo cardiomiopathy: mid-ventricular akinesis with preservation of apical and basal contractilities revealed at echocardiograms and ventriculographies. We report the case of a 65 years old patient with this pattern, reverted in 4 weeks.

Evaluation of diastolic function by the Tissue doppler in children affected by non- compaction

The Tissue doppler analysis is a new echocardiographic approach to evaluate the diastolic function. In this report we performed a Tissue doppler analysis in a series of 15 children affected by non-compaction. The bidimensional echo-cardiogram showed a systolic function which was reduced only in 8 patients. Alterations of the diastolic function were founded in 7 patients: in 2 cases, a reduction of the E tissue wave was present in all segments. In 3 patients the diastolic dysfunction was limited to apical and lateral segments. In the last 2 children a reduction of the E wave interested only the apical segments. There was a strong correlation between systolic and diastolic dysfunction: in fac…

Sympathetic tone and ventricular tachycardia

Background The pathogenesis of idiopathic monomorphic ventricular tachycardia is not clear. We suppose that a lack of balance of the sympathetic system could be involved. Frequency domain analyses of the heart rate can be useful to understand autonomic system balance. Therefore we performed this evaluation on a sample of seven children affected by idiopathic monomorphic ventricular tachycardia. Methods and results We performed a Holter recording for palpitations on all the children, with an average age of 12 (range: 7-18 years). In all the patients many episodes of repeated sustained or nonsustained ventricular tachycardia were demonstrated, with an average heart rate of 170 bpm. We exclude…

Letters to the Editor

Clinical findings of Takotsubo cardiomyopathy: results from a multicenter international study

BACKGROUND: Takotsubo cardiomyopathy consists of reversible systolic left ventricular apical ballooning associated with chest pain. Electrocardiographic abnormalities and the minimal rise of serum cardiac markers are similar to those in acute myocardial infarction, but without evidence of myocardial ischemia or injury. To date, many reports concerning this kind of acute reversible heart failure have been published, but the information available about the management of affected patients is scarce and the clinical data are incomplete. AIMS: In the present study, we report a collection of 40 patients who were affected by Takotsubo cardiomyopathy obtained in a multicentric international study, …

Il polimorfismo C4887A del gene CYP1A1 influenza il metabolismo estrogenico e la massa ossea

Magnetic resonance in isolated noncompaction of the ventricular myocardium

Abstract Non-compaction of the ventricular myocardium (LCVM) is a rare disorder of myocardial morphogenesis usually diagnosed in paediatric age. The diagnosis was echocardiographically made on the basis of a reported spongeous/compacted ratio >2 in one or more segments of the left ventricle during the diastolic period. We aimed to test the diagnostic accuracy of cardiovascular magnetic resonance (CMR) imaging in distinguishing pathological left ventricular non-compaction. Methods We collected a consecutive series of 8 patients, 5 males and 3 females, with a mean age of 14.9 years with non-compaction of left ventricular myocardium. All patients were admitted in our divisions of cardiology. I…