71 research outputs found
Current recommendations for the pharmacologic therapy in Kawasaki syndrome and management of its cardiovascular complications
No full textKawasaki syndrome is a potentially life-threatening disease of early childhood that untreated holds a risk of severe coronary involvement. Its diagnosis is made via a list of clinical signs because etiology and pathophysiology are still unknown and no specific laboratory tool is available. Appropriate therapy with intravenous immunoglobulins and aspirin reduces the incidence of coronary abnormalities to less than 5%. Immunoglobulins have been shown to be highly effective in reducing disease symptoms or their severity and chiefly in reducing the rate of coronary artery aneurysm development. Aspirin is firstly used in high dose for its anti-inflammatory properties and then in low dose for its anti-thrombotic effects. Timely diagnosis and precociously administered treatment are two crucial points in the definition of prognosis for Kawasaki syndrome. In this review heart complications are discussed and therapeutic options stratified according to both severity of coronary involvement and grading of cardiovascular risk
What do parents know about the malformations afflicting the hearts of their children?
No full textTraditionally, medical professionals have entrusted the parents of children with chronic illness with the task of imparting knowledge about the illness to the children. This practice assumes that parents understand the illness, and that they pass on their knowledge in a manner appropriate for the individual child. The aim of our study was to assess the knowledge that parents of children with a cardiac defect have about the malformation in the heart of their child. We sent a modified version of the Leuven questionnaire to 350 families. The questionnaire was filled in and sent back to our centre by 148 families. Parents showed a good knowledge of the name and anatomical characteristics of the cardiac disease suffered by their child. Parents with a child taking drugs were not very knowledgeable about the regime, side effects, and interaction with other drugs or food. Only one-quarter of the parents knew the definition of endocarditis, although almost two-fifths were aware that unexplained fever for more than 5 days was the most typical symptom. Less than half of the parents knew that endocarditis could recur. About two-fifths of parents knew the real possibility of their child being involved in competitive sports; but almost half of parents were unable to answer this question. The poor knowledge about particular aspects of the disease, treatment, and preventive measures revealed by our parents may have major consequences. The results of our study are relevant to general daily clinical practice
Illness understanding in adults with congenital heart disease
No full textBACKGROUND: Adult patients with congenital heart disease need information regarding their clinical diagnosis, medications and side effects, endocarditis prophylaxis, reproductive issues, employment, future surveillance, treatments, and possible reoperations. Accurate understanding of chronic illness in these patients is associated with less distress, less confusion, improved satisfaction with medical care, better compliance with treatment, and a better emotional status, all key factors for good health-related quality of life. The aim of the present study was to assess the level of knowledge that adult patients with congenital heart disease followed in our Center have about their heart condition.
METHODS: A questionnaire on knowledge about congenital heart disease was sent by mail to 200 adults affected by a cardiac congenital disease chosen randomly from all patients regularly followed in our department.
RESULTS: Patients had good knowledge about the treatment received, the importance of follow-up, the prognosis of their condition, and the possibility of taking part in physical activities. The patients receiving drug treatment were moderately knowledgeable about their treatment. The anatomy of the heart defect, factors contributing to the onset of endocarditis, the impact of smoking and alcohol, and the possible inheritance of the heart condition were poorly understood by the patients. Most of female patients knew that the oral contraceptive pill was the most appropriate method of birth control; most of them were aware that pregnancy would cause additional risks to their health. Multiple logistic analysis showed that four correct answers were related to the age of the patient.
CONCLUSIONS: Overall the results indicated that the educational efforts of all the staff (physicians and nurses) have given encouraging results, but there are still significant gaps in knowledge that need more educational work
Outcome of newborns with asymptomatic monomorphic ventricular arrhythmia
No full textBackground: Frequent premature ventricular contractions (PVCs), couplets (CPLTs) and episodes of ventricular tachycardia are extremely rare in the neonatal population. Limited information is available with regard to clinical relevance and outcome.
Objectives: To evaluate the clinical characteristics and outcomes of a group of newborns with ventricular arrhythmias without heart disease.
Patients and design: Between January 2000 and January 2003, 16 newborns with ventricular arrhythmias in the absence of heart disease were studied. The newborns were divided into three groups: PVC group (n = 8), CPLT group (n = 4) and ventricular tachycardia group (n = 4). All patients underwent physical examination, electrocardiography, Holter monitoring and echocardiography at diagnosis and at follow-up (1, 3, 6 and 12 months, and yearly thereafter).
Results: Mean (standard deviation, SD) age of the patients was 3 (1.19) days in the PVC group, 3.25 (0.95) days in the CPLT group and 6.5 (9.1) days in the ventricular tachycardia group. Median follow-up was 36 months (range 24 48 months). PVCs disappeared during follow-up in all the neonates, in the PVC group, at a mean (SD) age of 2.1 (1.24) months; in the CPLT group, couplets disappeared at a mean (SD) age of 6.5 (1) months. All patients with ventricular tachycardia were treated; ventricular tachycardia disappeared at a mean (SD) age of 1.7 (0.9) months. Neither death nor complications occurred.
Conclusions: Ventricular arrhythmias in newborns without heart disease have a good long-term prognosis. Frequent PVCs and CPLTs do not require treatment. Sustained ventricular tachycardia or high-rate ventricular tachycardia must be treated, but the prognosis is generally favourable
Exercise -unrelated sudden death as the first event of anomalous origin of the left coronary artery from the right aortic sinus
No full textCongenital anomalous origins of the coronary arteries represents a rare but well-described cause of myocardial ischemia and sudden death. Left coronary artery (LCA) arising from the right sinus of Valsalva is a rare congenital coronary anomaly that seems to be commonly associated with sudden death in young trained athletes. The possibility of a coronary artery anomaly should always be considered in young individuals with a history of chest pain or syncope, particularly if the episodes are triggered by exercise. We describe a case of congenital LCA anomaly in an asymptomatic 10-year-old girl with no family history of sudden death; no previous unexplained syncopal episodes or exercise-induced symptoms were reported. She experienced a cardiac arrest while she was resting at school and was not recoverable despite early emergency department admission and intensive prolonged cardiopulmonary resuscitation attempts. Post-mortem pathological findings revealed a single origin from the right sinus of Valsalva for both right and left coronary arteries. The LCA was compressed between the aorta and the pulmonary trunk. Histologic features suggested recent ischemia. Although sudden death can be the first manifestation of this condition, it is important to be particularly aware of prodromic symptoms: exertional dyspnea, chest pain, syncope or dizziness. Recognition during life of this coronary anomaly is mandatory to prevent the risk of sudden death and to plan surgical correction if clinically indicated
Large pericardial effusion requiring pericardiocentesis as cardinal sign of macrophage activation syndrome in systemic onset-juvenile idiopathic arthritis
No full textWe report a case of large pericardial effusion which has been managed with pericardiocentesis as the main presentation feature of a dramatic clinical picture, only retrospectively framed as referred to macrophage activation syndrome in a child with juvenile idiopathic arthritis at its onset. The risk of developing this rare and severe complication should be recognized in various pathological settings of childhood, above all in children displaying systemic signs of juvenile idiopathic arthritis
Is serum Troponin T a useful marker of myocardial damage in newborn infants with perinatal asphyxia?
No full textAIM: To assess the correlation of echocardiographic signs of myocardial damage to serum cardiac troponin T (cTnT) concentrations in newborn infants with perinatal asphyxia.
METHODS: Electocardiograms (ECG) and echocardiograms (Echo) were obtained during the first 24 h of life from 29 asphyxiated and 30 control infants and correlated with cTnT concentrations. The echocardiographic parameters included systolic ventricular performance, preload, afterload, diastolic function, stroke volume (SV), left ventricular output (LVO), hyperechogenity of the papillary muscles and insufficiency of the atrioventricular valves.
RESULTS: LVO and SV were lower but CTnT were significantly higher in asphyxiated than in control infants: 0.15 (010-0.23) vs. 0.05 (0.02-0.13), p < 0.001). Asphyxiated infants with signs of myocardial damage were associated with significantly higher cTnT than those without, 0.20 (0.11-0.28) and 0.11 (0.05-0.14 ug/L), p = 0.04.
CONCLUSION: Cardiac troponin may prove to be valuable in evaluating myocardial damage in birth asphyxia. However, the degree of prematurity may complicate the assessment
Dealing with Chronic Non-Bacterial Osteomyelitis: A practical approach
No full textBACKGROUND: Chronic Non-Bacterial Osteomyelitis (CNO) is an inflammatory disorder that primarily affects children. Although underestimated, its incidence is rare. For these reasons, no diagnostic and no therapeutic guidelines exist. The manuscript wants to give some suggestions on how to deal with these patients in the every-day clinical practice. MAIN BODY: CNO is characterized by insidious onset of bone pain with local swelling. Systemic symptoms such as fever, skin involvement and arthritis may be sometimes present. Radiological findings are suggestive for osteomyelitis, in particular if multiple sites are involved. CNO predominantly affects metaphyses of long bones, but clavicle and mandible, even if rare localizations of the disease, are very consistent with CNO diagnosis. CNO pathogenesis is still unknown, but recent findings highlighted the crucial role of cytokines such as IL-1β and IL-10 in disease pathogenesis. Moreover, the presence of non-bacterial osteomyelitis among autoinflammatory syndromes suggests that CNO could be considered an autoinflammatory disease itself. Differential diagnosis includes infections, malignancies, benign bone tumors, metabolic disorders and other autoinflammatory disorders. Radiologic findings, either with Magnetic Resonance or with Computer Scan, may be very suggestive. For this reason in patients in good clinical conditions, with multifocal localization and very consistent radiological findings bone biopsy could be avoided. Non-Steroidal Anti-Inflammatory Drugs are the first-choice treatment. Corticosteroids, methotrexate, bisphosphonates, TNFα-inhibitors and IL-1 blockers have also been used with some benefit; but the choice of the second line treatment depends on bone lesions localizations, presence of systemic features and patients' clinical conditions. CONCLUSION:
CNO may be difficult to identify and no consensus exist on diagnosis and treatment. Multifocal bone lesions with characteristic radiological findings are very suggestive of CNO. No data exist on best treatment option after Non-Steroidal Anti-Inflammatory Drugs failure
Isolated myocardial non-compaction in an infant with distal 4q trisomy and distal 1q monosomy
No full textLevosimendan in two neonates with ischemic heart failure and pulmonary hypertension
No full textWe report the successful and safe use of levosimendan, a new calcium-sensitizing agent with positive inotropic and vasodilatory action, in 2 critically ill term newborns with acute heart failure and pulmonary hypertension in the absence of any underlying heart malformation and/or previous cardiosurgical procedures. During the neonatal period, levosimendan may represent an ideal drug for immature myocardium characterized by a higher calcium-dependent contractility than in adults
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