Flavia Ventriglia

Human Parvovirus B19 Infection in Infancy Associated with Acute and Chronic Lymphocytic Myocarditis and High Cytokine Levels: Report of 3 Cases and Review

Human parvovirus B19 infection is occasionally associated with acute lymphocytic myocarditis (ALM). Three infants with B19 virus-associated ALM were followed up clinically, histologically, and immunovirologically. Each infant had B19 virus DNA in the blood or B19 virus-specific IgM antibodies. Two infants with postnatal infection recovered after immunosuppressive therapy. The third infant with possible prenatal infection developed chronic persistent myocarditis associated with persistent B19 virus DNA in the blood. All 3 infants had increased levels of interferon-gamma, tumor necrosis factor-alpha, and interleukins -6 and -8. Four newborns with congenital B19 virus infection and 4 infants and children who had postnatally acquired B19 virus infection without myocarditis all had normal levels of these cytokines. These observations suggest that B19 virus infection in infancy causes ALM in some infants and children.

Potential role of fetal cardiac evaluation with magnetic resonance imaging: preliminary experience

To report our experience with magnetic resonance imaging (MRI) in fetal heart evaluation.

Myocardial Scintigraphy with 99mTc-Sestamibi in Children with Kawasaki Disease

Kawasaki disease (KD) is a systemic vasculitis syndrome of early childhood. It involves particularly the coronary arteries and may cause aneurysms and thrombotic occlusions. Echocardiography is the most useful method of detecting coronary aneurysms. Nevertheless, obstructive lesions are difficult to evaluate and often need invasive coronary angiography. An important feature of this disease is the possibility of finding coronary pathology several years after the onset. This characteristic makes KD an important cause of coronary artery disease (CAD) in young adults. Thus patients with KD and previously diseased coronary arteries should be kept under long-term control. However, coronary angiography is invasive and cannot be performed repeatedly, espe cially in young patients. As an alternative, thallium 201 scintigraphy has been employed, but its low-energy photons are suboptimal for standard gamma-camera imaging, particularly in children aged less than three years. To verify the usefulness of a noninvasive assessment of myocardial perfusion, the authors used rest and dipyridamole 99mTc-Sestamibi scan in 15 children (ranging from one to six years of age) with Kawasakis cardiac involvement. Coronary aneurysms have been demonstrated by echocardiography in 12 patients; 8 patients were also submitted to cardiac catheterization. The sensitivity of 99mTc-Sestamibi imaging for detection of overall coronary lesions was 88% and the specificity was 93%. These data suggest that rest/dipyridamole 99mTc-Sestamibi scintigraphy is an accurate and noninvasive method for the detection and follow-up of Kawasakis cardiac damage even in patients aged one year.

Radioisotope assessment of heart damage in hypertransfused thalassaemic patients

Twenty-five thalassaemic patients treated with repeated blood transfusion (BT) and intensive iron removal therapy were studied by echocardiography and rest/stress equilibrium gated radionuclide angiocardiography (EGNA). Stress left ventricular ejection fraction (LVEF) showed an important negative correlation with number of BTs (r = −0.75). Abnormal values of stress LVEF were measured after 200 BTs: these data demonstrate the effectiveness of stress LVEF in the follow-up of patients who have undergone repeated BTs and the clinical importance of intensive chelation therapy. Peak filling rate did not show diagnostic value in the early detection of iron cardiotoxicity. However, its inverse correlation with BT (r = −60) indicates that iron overload depresses the diastolic parameters.

Changed outcomes of fetuses with congenital heart disease: new Italian Multicentre study.

Objectives To analyse the outcomes of fetuses with congenital heart disease between 2000 and 2005 in comparison to a previous multicentre study regarding the period 1983–1996. Participants and methods Data of seven centres were prospectively collected, the inclusion criteria being a confirmed fetal diagnosis after birth or at autopsy and a known follow-up, for at least 6 months after birth. Data of 649 fetuses, median age at diagnosis 24 weeks’ gestation (15–37), 340/649 (52.4%) diagnosed before 24 weeks, were analysed. Results Sixty seven and 59 cases had chromosomal or extracardiac anomalies (10.3 and 9.1%). Termination of pregnancy was chosen in 21.6% of cases versus 28.9% in the previous study, being significantly lower in cases with early diagnosis (P <0.001). Out of 509 fetuses continuing pregnancy, 23 died in utero (4.5%) and 110 (21.1%) postnatally, versus 43% in the previous study (P <0.0017). Total surgical/postprocedure death occurred in 20.6% (59/287 infants) versus 37% previously (P <0.003), 67 infants being premature and 35 with associated chromosomal or extracardiac anomalies. The current overall mortality rate was higher in cases with chromosomal or extracardiac anomalies (59.5 and 51.35%, respectively, whereas it was 20.1% in isolated congenital heart disease). Overall current survival was 376 of 509 (73.9%) versus 45% in the previous study (P <0.0001). Conclusion Our data show a reduced overall and surgical mortality, with respect to our previous study, resulting from resulting from an improved perinatal management and treatment of affected fetuses in the more recent era.

Blocked Atrial Bi/Trigeminy In Utero Evolving in Supraventricular Tachycardia after Birth

Transient episodes of fetal bradycardia (heart rate less than 110 bpm) are usually benign and typically result from increased vagal stimulation in the fetus. Causes of sustained fetal bradycardia include sinus bradycardia, blocked atrial bigeminy/trigeminy, high-degree atrioventricular block, and long QT syndrome. We present the case of a 34-year-old Caucasian patient referred to our department for “blocked atrial bigeminy with pseudobradycardia” detected elsewhere at 33 weeks of gestation. A fetal echocardiography showed during all the examination a blocked atrial trigeminy with a mean fetal heart rate of 100 bpm. After birth three subsequent ECGs until day 3 showed no evidence of atrial extrasystoles, confirming the well-known frequent regression of this kind of fetal benign arrhythmia, but on day 11 recurrence of supraventricular trigeminy and development of episodes of paroxystic supraventricular tachycardia were observed. On the basis of this observation, we recommend that fetuses with complex atrial ectopic beats should be closely monitored before and after birth for evidence of new arrhythmias.

Double aortic arch: postnatal obliteration of the left aortic arch. Is arterial duct closure responsible?

We present a case of double aortic arch with a predominant right and a double arterial duct detected by echocardiogram in a 28-week gestation foetus. The first evaluation revealed that both arches were perfused; the 1-month postnatal echocardiogram showed the closure of both arterial ducts and the partial obliteration of the left aortic arch between the left subclavian artery and the dorsal aorta. In our case, the postnatal obliteration of the left arch in a double aortic arch was probably due to the closure of the left-sided arterial duct.

Peculiarities of Prevalence and Morphology of Congenital Heart Disease Detected In Utero

Intrauterine echocardiography is changing our knowledge of congenital heart disease; cardiac defects diagnosed in utero have distinctive features of both prevalence and morphology when compared with those observed just after birth. We reviewed a series of 171 fetal heart conditions: 148 were diagnosed at intrauterine echocardiography, the diagnosis being verified at autopsy in 41, and 23 were observed at the postmortem only. Peculiarities of prevalence consisted in an excess of various defects, such as hypoplastic left heart syndrome, atrial isomerism, pulmonary atresia, and atrioventricular and atrial septal defects, and in a reduced number of completely different conditions, such as transposition of great arteries and aortic coarctation. Differences in prevalence have been attributed to difficulties in diagnosing some particular anomalies in utero, to the selection of pregnancies undergoing screening, and to the special intrauterine evidence of some heart defects. Peculiarities in morphology result from the coexistence with extracardiac malformations, from the changes in shape conditioned by fetal hemodynamics, and from the intrauterine evolution of the morphology of some malformations. We concluded that the knowledge of these characteristic traits was helpful to cardiac pathologists, pediatric cardiologists, and obstetricians, and allowed the re-evaluation of the role of hemodynamic factors in remodeling the malformed cardiovascular appara-tus.

The Embryology of the Interatrial Septum

Knowledge of the embryological development of the interatrial septum can provide the basis for understanding the morphogenesis of some congenital cardiac malformations. The interatrial septum consists of several embryological components including the septum primum, the anterosuperior and the posteroinferior cushions of the atrioventricular canal, the septum secundum, the left sinus valve, and the “spina vestibuli.” Atrial septation starts with the formation of the septum primum and the space between the free edge of the septum primum, and the atrioventricular cushion is the ostium primum. Before the closure of the ostium primum, multiple perforations appear in the septum primum, and their coalescence forms the ostium secundum. The septum secundum grows to the right of the septum primum. The complex of the lower rim of the septum secundum and the ostium secundum is called foramen ovale. During the fetal period because of the higher pressure in the right atrium, the blood passes from the right to the left atrium. After birth, when lung circulation begins its function, the pressure in the left atrium rises, and the foramen ovale is closed by the septum primum.