Persistent Valvular Regurgitation After Acute Rheumatic Fever: Early Predictors of Long Term Outcomes in a Pediatric Retrospective Cohort.

Describe the echocardiographic evolution of valvular regurgitation in patients with rheumatic carditis (RC) and to establish which features may predict long-term outcome, in the absence of acute rheumatic fever (ARF) relapse. Retrospective cohort study. 123 patients with confirmed RC, diagnosed at Turin Children's Hospital between 2010 and 2019. We reviewed the echocardiographic images recorded at diagnosis, after 6-8 weeks, after 6 months, then yearly, to assess which predictors at diagnosis are associated with the degree of improvement at 6 months. Secondly, we tested which variables predict the regression of pathological regurgitation of mitral (MV) or aortic valve (AV) during follow-up. At onset, 90.2% patients had MV regurgitation while 42.3% had AV involvement. 115 (93.5%) patients were treated with steroids and 70.8% experienced a downgrading of RC after 6 months. Steroids were associated with better outcomes at six months (p = 0.01). During follow-up (median 56.1 months), MV improved in 58.6% patients, AV in 46.2%. At multivariate analysis, erythrocyte sedimentation rate (ESR) was positively associated with regression of MV regurgitation (OR 1.02, p = 0.02), while higher degree of carditis at onset was negatively associated (OR 0.04, p < 0.01). Conversely, regression of AV regurgitation was more frequent in patients with bi-valvular involvement (OR 20.5, p = 0.03) and in absence of murmur at onset (OR 0.04, p = 0.01). This study indicates that valvular regurgitation improves overtime if there are no ARF recurrences during follow-up, especially when the MV is involved and in patients treated with steroids.

Borderline Ventricles: From Evaluation to Treatment.

A heart with a borderline ventricle refers to a situation where there is uncertainty about whether the left or right underdeveloped ventricle can effectively support the systemic or pulmonary circulation with appropriate filling pressures and sufficient physiological reserve. Pediatric cardiologists often deal with congenital heart diseases (CHDs) associated with various degrees of hypoplasia of the left or right ventricles. To date, no specific guidelines exist, and surgical management may be extremely variable in different centers and sometimes even in the same center at different times. Thus, the choice between the single-ventricle or biventricular approach is always controversial. The aim of this review is to better define when "small is too small and large is large enough" in order to help clinicians make the decision that could potentially affect the patient's entire life.

Successful treatment of pulmonary arterial hypertension in a 2-month-old female infant with incontinentia pigmenti: A case report.

Incontinentia pigmenti (IP) is a rare X-linked dominant neuroectodermal dysplasia affecting almost exclusively females. It is caused by loss-of-function mutations in the inhibitor of kappa light polypeptide gene enhancer in B cells, kinase gamma gene, formerly known as NF-κB essential modulator. The disorder is typically identified by peculiar skin findings that develop throughout the 1st year of life. Approximately one-third of patients has ocular and neurologic abnormalities causing severe disability. Defects of hair, nails, and teeth can also occur. Among systemic complications, pulmonary arterial hypertension (PAH) is uncommon but potentially life-threatening. Only six cases have been described in the literature so far, and four of them died before reaching 1 year of age. Herein, we report the case of a 2-month-old girl with IP and severe PAH, successfully treated with pulmonary antihypertensive and anti-inflammatory therapy.

The pathophysiology and complications of Fontan circulation.

The Fontan operation has been the final palliation for patients born with congenital heart defects with a functional single ventricle for more than 4 decades. The "normal" Fontan physiology is characterized by the loss of the sub-pulmonary ventricle with consequent elevated pressure in the caval system, non-pulsatile blood flow in the pulmonary circulation and at least mild reduction of the systemic output. When successful, this procedure is associated with a range of benefits including improved arterial saturation and abolishment of chronic volume overload, allowing a fairly normal life to the majority of patients through early adulthood. As we enter the 5th decade of caring for patients palliated with the Fontan procedure, it is evident that adult survivors face significant morbidity due to multiorgan dysfunction, early mortality and need for heart transplantation. Several late complications may occur: ventricular dysfunction, arrhythmia, cyanosis, exercise intolerance, elevated pulmonary vascular resistance, protein-losing enteropathy, plastic bronchitis, hepatic and renal complications. The mechanism of late Fontan failure is multifactorial and not completely understood, it depends on interactions between the ventricle, the pulmonary vascular bed, the venous and lymphatic compartments. Conclusions: the aim of this review is to describe the pathophysiology of Fontan circulation and the clinical and hemodynamic characteristics of early and late failing Fontan survivors, their association with morbidity and mortality, and the strategies for their management.

Iron Deficiency Anemia in Celiac Disease.

The iron absorption process developsmainly in the proximal duodenum. This portion of the intestine is typically destroyed in celiac disease (CD), resulting in a reduction in absorption of iron and subsequent iron deficiency anemia (IDA). In fact, the most frequent extra-intestinal manifestation (EIM) of CD is IDA, with a prevalence between 12 and 82% (in relation with the various reports) in patients with new CD diagnosis. The primary treatment of CD is the gluten-free diet (GFD), which is associated with adequate management of IDA, if present. Iron replacement treatment historically has been based on oral products containing ferrous sulphate (FS). However, the absorption of FS is limited in patients with active CD and unpredictable in patients on a GFD. Furthermore, a poor tolerability of this kind of ferrous is particularly frequent in patients with CD or with other inflammatory bowel diseases. Normalization from anemic state typically occurs after at least 6 months of GFD, but the process can take up to 2 years for iron stores to replenish.

Percutaneous treatment of anomalous systemic artery to pulmonary venous fistulas in children: Description of three cases and review of the literature.

In the normal lung, the only communications between the systemic and pulmonary arterial systems are the connections between the bronchial and pulmonary arteries that occur at the respiratory bronchioles, where pulmonary and bronchial capillaries freely anastomose. Rarely, anomalous connections can occur between normal or aberrant systemic arteries and pulmonary vessels. We performed a comprehensive literature review of all available manuscripts on PubMed and Google Scholar that included a case report or case series with diagnosis of systemic artery to pulmonary venous fistulas who underwent percutaneous treatment. Furthermore, we report three cases of children diagnosed and treated in our Pediatric Cardiology Center.

Feralgine™ a New Approach for Iron Deficiency Anemia in Celiac Patients.

BACKGROUND: Celiac disease (CD) is an immunologically-mediated disorder characterized by duodenal mucosa villi atrophy. Iron absorption is usually reduced in celiac patients making every kind of oral iron treatment unhelpful because of malasorption. Feralgine™ is a new product that has been demonstrated to be more bioavailable. As such, the aim of our study was to evaluate the absorption of Feralgine™ in adult patients with CD. METHODS: Twenty-six adults affected by Iron Deficiency Anemia (IDA), of which 14 were also affected by CD and 12 were not affected by CD, were enrolled. An oral iron absorption test (OIAT) was performed in each patient by administrating Feralgine™, and serum iron was evaluated at baseline (T0) and after 2 h (T1) from the oral iron ingestion. RESULTS: The OIAT was well tolerated in all patients, and, surprisingly, an equivalent statistically significant improvement in serum iron occurred in the two groups of patients (IDA plus CD: T0 = 28.21 µg/dL vs. T1 = 94.14 µg/dL p = 0.004 and IDA without CD: T0 = 34.91 µg/dL vs. T1 = 118.83 µg/dL, p = 0.0003). CONCLUSIONS: These results demonstrated the high absorption of Feralgine™ in celiac patients, confirming our previous data obtained with Ferrous Bysglicinate in children with CD.

A Double Lane for a Better Circulation: Percutaneous Duplication of the Thoracic Aorta in a Child.

Aortic arch interruption or atresia consists in a complete loss of anatomical and luminal continuity between the ascending and descending thoracic aorta. It is usually diagnosed in newborn babies but may also be present in young children, adolescents, adults and elderly patients. In these cases, it can be congenital or more frequently acquired. This latter type of aortic arch atresia is a challenge, and surgery is usually the treatment of choice. Percutaneous approaches are also possible today. We describe a case of percutaneous aortic arch reconstruction in a 9-year-old boy.

Chronic idiopathic neutrophilia in two twins.

Neutrophilia in adults refers to an alteration in the total number of blood neutrophils that is in excess of about 7500 cells/µL. This definition is restrictive in childhood as neutrophil count is age-dependent. Chronic Idiopathic Neutrophilia (CIN) refers to a condition that persists for many years in individuals who appear otherwise healthy. CIN is rarely mentioned in scientific literature and in academic books of hematology; only few words are dedicated to this topic. We report a case study of two twins with CIN followed from the first year of life to 24 years of age. To the best of our knowledge this is the first case report of two twins with CIN followed through a long period of time. We believe that our observation may contribute to better understand and characterize this hematologic abnormality.