Candidaemia in a European Paediatric University Hospital: a 10-year observational study.

In this retrospective observational study covering 1998 to 2008, 32 patients (mean age: 7.50 years) were identified that had 35 episodes of candidaemia (0.47 cases/1000 hospital discharges). Cancer/allogeneic haematopoietic stem cell transplantation (43%) and congenital malformations/syndromes (21%) were the predominant underlying conditions. Central venous catheterization (90%), a history of antibacterial therapy (69%) and previous bacteraemia (54%) were frequent comorbidities. Candida albicans (46%) was most common, followed by Candida parapsilosis (17%) and Candida glabrata (14%). Resistance was infrequent and limited to non-albicans Candida spp. The 30-day and 100-day mortality rates were 11.4%.

QT interval prolongation and risk for cardiac events in genotyped LQTS-index children.

Congenital long-QT syndrome (LQTS) is an inherited cardiac disorder with a disturbance in repolarization characterized by a prolonged QT interval on the surface electrocardiogram and life-threatening ventricular tachycardia. Publications from the International LQTS Registry have provided information that the cardiac risk may be influenced by gender, genotype, exposure to arrhythmia triggers, and previous cardiac events. In children, early-onset of disease, changes in life style, and medical treatment is a sensitive issue and significant, gender-related differences of a first life-threatening event were reported. Thus, we investigated the clinical features of a large genotyped population of LQTS-index children (age < or =16 years) upon a single-center experience and determined risk factors for symptoms. Of 83 children [mean corrected QT interval (QTc) 510 +/- 74 ms], 89% had LQT1, -2, or -3. Nine patients (11%) were identified as having Jervell and Lange-Nielsen syndrome. Among symptomatic children (n = 51, 61%), syncope was the most prevalent symptom at initial presentation (49%); however, aborted cardiac arrest (ACA) occurred in 33% and sudden cardiac death (SCD) in 18%, respectively, as the initial manifestation. During a mean follow-up period of 5.9 +/- 4.7 years, 31% of the children developed symptoms while on therapy (86% syncope, 9% ACA, 5% SCD). Statistical analyses of risk factors for cardiac events showed that the QTc >500 ms was a strong and significant predictor for cardiac events during follow-up (p = 0.02). Furthermore, a prior syncope [hazard ratio (HR), 4.05; 95% confidence interval (CI), 1.1 to 15.0; p = 0.03] or an ACA (HR, 11.7; 95% CI, 3.1 to 43.4; p = <0.001) identified children with an increased risk for recurrent cardiac events compared to asymptomatic LQT children. LQTS-index children manifest with a high percentage of severe symptoms. Among presently validated risk factors for LQTS, a QTc interval >500 ms and a history of prior syncope or ACA were strong predictors for recurrent cardiac events.

Regurgitation of the atrioventricular valves after corrective surgery for complete atrioventricular septal defects - comparison of different surgical techniques.

OBJECTIVE: Different surgical approaches have been used to repair complete atrioventricular septal defects (AVSD). Regurgitant atrioventricular valves (AV-valves) are common after surgery. We compared different surgical techniques with respect to long-term postoperative AV-valve regurgitation. METHODS: In 69 patients with complete AVSD, three different surgical techniques were applied: Single-patch, two-patch, and modified techniques. The left-sided AV-valve cleft was surgically closed in all patients. RESULTS: A comparison of the results of the different techniques showed no difference in the degree of AV-valve regurgitation on either the right or the left side. The average degree was mild on both sides. Only one patient needed reoperation for severe left-sided AV-valve regurgitation. CONCLUSION: The different surgical techniques used for the correction of AVSD do not have a major bearing on the degree of AV-valve regurgitation.

Heart transplantation for isolated noncompaction of the left ventricle in an infant.

Isolated noncompaction of the left ventricular myocardium is a rare cardiomyopathy typically showing a "spongy" myocardium on ultrasound. We report on the ultrasonic and pathomorphological characteristics of an infant who, at the age of 40 days, was treated by heart transplantation for isolated noncompaction. Noncompaction should be suspected in newborns with otherwise unexplained cardiomyopathy and a "spongy" left ventricle. However, ultrasonic and pathological findings may be much less pronounced at this age than later in life.

[Atrial flutter after orthotopic heart transplantation due to recipient-to-donor transatrial conduction]. / Vorhofflattern in der Frühphase nach orthotoper Herztransplantation.

A few weeks after orthotopic heart transplantation, a male adolescent developed atrial arrhythmias of the donor heart due to an atypical recipient atrial flutter with a recipient-to-donor transatrial conduction resulting in an absolute arrhythmia. Under medication with propafenone, the atrial flutter of the donor heart could be terminated with cardioversion.

Late coil displacement after interventional closure of a perimembranous ventricular septal defect: a case report.

Muscular and perimembranous ventricular septal defects can be closed with nitinol plugs or spiral coils. Displacement and embolization of the device are well known complications that usually occur during or early after the procedure. We present the case of a 12-year-old boy with asymptomatic coil displacement detected at a routine examination 5 months after closure of a perimembranous ventricular septal defect.

Cardiac transplantation in neonatal Marfan syndrome -- a life-saving approach.

Marfan syndrome is a connective tissue disease with typical clinical signs and cardiac involvement. Its appearance in the neonatal period has a bad prognosis due to incompetence of all cardiac valves with subsequent congestive heart failure. Conservative management usually fails, the children die during their first year of life. We report on a girl with neonatal Marfan syndrome who suffered from regurgitance of all cardiac valves, enlarged ventricles, and dilated great arteries. She was NYHA class IV. At the age of six months she underwent heart transplantation. To prevent aneurysm formation and dissection of the great vessels, the whole aortic arch and pulmonary trunk were replaced as well.

Ventricular aneurysm or diverticulum? Clinical differential diagnosis.

Intrathoracic ventricular aneurysms and diverticula can be differentiated by several criteria. Contractility is the only reliable parameter: aneurysms expand, whereas diverticula contract during ventricular systole.

Cardiac leiomyosarcoma of the right atrium in a teenager: unusual manifestation with a lifetime history of atrial ectopic tachycardia.

A 16-year-old girl presented with atrial fibrillation. Transesophageal echocardiography revealed a right atrial leiomyosarcoma. Her past medical history was remarkable for incessant atrial ectopic tachycardia (AET) beginning in early infancy and continuing throughout childhood and adolescence that was refractive to medical and nonpharmacological treatment. After combined surgical and medical therapy, normal sinus rhythm was restored and the patient is currently in complete remission with no recurrent symptoms or atrial arrhythmias at 31 months after surgery and 23 months after the discontinuation of chemotherapy. Atrial tachycardia may be the first, and for prolonged periods, the only manifestation of a cardiac tumor and should prompt thorough investigation of its underlying morphological substrate.

An epicardial cyst in a child.

An 8-year-old girl, without any previous medical history, presented with a first short syncope. Physical examination was unremarkable. Transthoracic echocardiography revealed a thin-walled, echo-free cystic structure adjacent to the posterior wall of the left ventricle, and compressing it moderately. Other echocardiographic findings were normal. Both computed tomography and magnetic resonance imaging suggested a simple pericardial cyst, but during surgery we found an epicardial cyst with partial involvement of the circumflex branch of the left coronary artery. Cardiopulmonary bypass was necessary for successful resection of the cyst, leaving behind only the small area in continuity with the coronary artery.