Gemella sanguinis Infective Endocarditis-Challenging Management of an 8-Year-Old with Duchenne Dystrophy and Undiagnosed Congenital Heart Disease: A Case Report.

Congenital heart disease (CHD) remains a predisposing cardiac condition for infective endocarditis (IE). Case report: We present the case of 8-year-old boy with no known pre-existing cardiac disease diagnosed with infective endocarditis (IE) with Gemella sanguinis. After admission, he underwent transthoracic echocardiography (TTE), which revealed the presence of Shone syndrome with a bicuspid valve, mitral parachute valve and severe aortic coarctation. He developed a paravalvular aortic abscess with severe aortic regurgitation and left ventricle (LV) systolic dysfunction for which he required a complex surgical intervention after six weeks of antibiotic treatment, consisting of Ross operation and coarctectomy, with a complicated postoperative course, cardiac arrest and ECMO support for five days. The evolution was slow and favorable, with no significant residual valvular lesions. However, persistent LV systolic dysfunction and increased muscle enzymes required further investigation to establish a genetic diagnosis of Duchenne disease. As Gemella is not considered a frequent pathogen of IE, no current guidelines refer specifically to it. Additionally, the predisposing cardiac condition of our patient is not currently classified as "high-risk" for IE; this is not considered an indication for IE prophylaxis in the current guidelines. Conclusion: This case illustrates the importance of accurate bacteriological diagnosis in infective endocarditis and poses concerns regarding the necessity of IE prophylaxis in "moderate risk" cardiac conditions such as congenital valvular heart disease, especially aortic valve malformations.

Ductus Arteriosus Stenting in Newborns - Transcatheter Approach as a Bridge Therapy for Corrective Surgery.

Duct-dependent congenital heart disease requires attentive therapeutic management since the only source of pulmonary blood flow in newborns is provided by the patent ductus arteriosus. The patency of the duct is the main objective in the first hours of life and it is guaranteed by prostaglandin E1 infusion, but it is not a long-term solution for this type of cardiac malformation. In order to augment pulmonary blood, there are two types of interventions that can be performed: a classical surgical shunt or stenting of the ductus arteriosus, a fairly new alternative to cardiac palliative surgery. Case selection for this type of procedure is essential regarding the patients' outcome. We present the management of a newborn diagnosed with (pseudo)atretic pulmonary valve, large ventricular septal defect and patent ductus arteriosus, who underwent an interventional procedure to secure pulmonary blood flow by placing a drug-eluting stent in the ductus arteriosus. The patient's evolution was not uneventful, several complications appeared, but after three months of neonatal intensive care we were able to discharge him in good clinical condition.

Cardiovascular Complications of Hemolytic Uremic Syndrome in Children.

Introduction: Hemolytic uremic syndrome is the most frequent cause of acute renal failure in children, commonly after gastrointestinal infections with E. coli or Salmonella, and it is characterized by progressive renal failure associated with microangiopathic hemolytic anemia and thrombocytopenia. Cardiac involvement is frequently encountered and can be potentially fatal in hemolytic uremic syndrome. It is usually determined by overhydration, hypertension, anemia, diselectrolytemia, acid-base disorders and tendency to form thrombi, and it consists in the following conditions: pericarditis, myocardial infarction, dilated cardiomyopathy, cardiac failure, and arrythmias. Objective: The aim of this study is to observe the incidence of cardiovascular complications in children with acute hemolytic uremic syndrome, underline which are the most useful tools in establishing an accurate diagnosis, and discover the treatment protocol that has the most powerful impact upon the cardiovascular manifestations. Materials and methods: We studied a number of 50 children who checked in the Nephrology Department of "M. S. Curie" Emergency Clinical Hospital in Bucharest, Romania, between January 2016 and August 2020. We performed the clinical examination of all patients as well as several paraclinical tests such as electrocardiogram, transthoracic echocardiography, arterial blood pressure monitorization, and vascular Doppler ultrasound. Patients included in the study were aged between five and 40 months. Discussion and results: The majority of these children were diagnosed with arterial hypertension and some of them with cardiac failure and profound venous thrombosis. Transthoracic echocardiography revealed pathological aspects such as left ventricular hypertrophy, diastolic dysfunction, systolic dysfunction of the left ventricle, mitral regurgitation, aortic regurgitation, and pericarditis. Cardiac ultrasound findings were reversible in the majority of patients, most of them being treated with ACE inhibitors (eventually in association with other antihypertensive drugs).

Cocoon devices for transcatheter closure of atrial septal defect and patent ductus arteriosus in children: Single center experience.

Closure of the atrial septal defect (ASD) and patent ductus arteriosus (PDA) are among the most frequent cardiac interventional procedures. This was a prospective study, which started together with the implementation of a national program of pediatric interventional cardiology in Romania. We used Cocoon devices in 83 consecutive cases from 92 implantations for ASD and PDA. 27 cases were ASD closure and 56 cases PDA closure. Regarding the ASD closure, the median age was 8.5 years (range 3-25 years) and median weight 25 kg (range 11.5-63 kg). The mean follow-up was 17.4 ±â€Š6.7 months (range 3-26 months). The mean ASD diameter by transesophageal echocardiography was 15.2 ±â€Š4.1 mm (range 8-26 mm). The mean device diameter used was 17.3 ±â€Š5.6 mm (range 8-32 mm). Regarding the PDA closure, the median age was 36 months (range 4-192 months) and median weight 14 kg (range 5-58 kg). The mean follow-up was 15 ±â€Š8 months (range 3-28 months). The mean PDA minimum diameter was 2.5 ±â€Š0.8 mm. The success implantation rate for both groups was 97.6% (2 cases of withdrawn for ASD and PDA), while the complication rate was 2.3% (including 2 ASD device embolization). In the first 24 hours, the closure rates were 96.3% for ASD, 98.2% for PDA, and 100% at 1-month follow-up for both procedures. On short and intermediate follow-up (3-28 months), no device-related complications were noted.The Cocoon devices are safe for transcatheter closure of both ASD and PDA, and the initial experience with their use in our emerging center is encouraging.

Unroofed coronary sinus: update on diagnosis and treatment.

Unroofed coronary sinus (UCS) is among the rarest congenital heart malformations in pediatric practice. Often, they are associated with persistent left superior vena cava (PLSVC) draining into the left atrium, and in this situation, there is a double intracardiac shunt, right-to-left and left-to-right. They are asymptomatic in the majority of the cases. Each ordinary imagistic method (transthoracic echocardiography, contrast transesophageal echocardiography, angiography) can be helpful in finding the diagnosis, but enhanced computed tomography scan can give an irrefutable diagnosis. The surgical intervention may consist in the recreation of a roof for the coronary sinus, and anastomosis of the PLSVC to the right atrial appendage.

Complex atrial septal defect closure in children.

Atrial septal defect (ASD) is one of the most common congenital heart diseases (CHDs) diagnosed in children. Symptoms in ASD may be absent, but palpitations and dyspnea in children sometimes reveal a complex structural and CHD. Diagnosis is established usually by transthoracic echocardiography, but in more complex cases such as coronary sinus defect, enhanced computed tomography or cardiac magnetic resonance may be used. Indication for closure depends on the dimension and on the hemodynamical impact. There are two methods for closure: surgical and interventional. Surgery may be applied to all types of ASD, while transcatheter closure may be used only for ostium secundum ASD. The most important issue in the interventional closure is the anatomical aspect related to the position of the defect. Both methods may have complications. Complex cases in children usually are not recommended for interventional closure, surgery being the recommended method. Oversizing of the device to be implanted according to the sizing balloon and not to the initial defect diameter may give a solution for large ASDs. Interventional closure of large defects in small children with an aneurysmal, multi-fenestrated, malaligned septum, or with other CHD associated may be challenging. Complications are present for both types of closure, but they are relatively different.

Assessment of Nutritional Intakes in Individuals with Obesity under Medical Supervision. A Cross-Sectional Study.

People with obesity in Romania are often under medical supervision, which is aimed to decrease body weight and treat accompanying metabolic disorders and cardiovascular implications. However, there is limited information regarding the implementation of dietary recommendations in adults with obesity. We aimed to evaluate the prevalence of reaching the recommended intakes of macro- and micro-nutrients in adults with obesity under medical supervision. Individuals with obesity, recruited in the context of a study with a larger scope (NutriGen ClinicalTrials.gov NCT02837367), who were under medical supervision underwent four 24 h recalls in order to assess daily food intakes. Macro- and micro-nutrient intakes were computed, and the prevalence of reaching recommended dietary allowances (RDAs) for each nutrient was calculated. The majority of subjects did not meet the recommended intakes for most nutrients. Energy from fat exceeded the threshold of 35% recommended intake, even in the lowest quartile of energy intake. The micronutrients with less than 5% of individuals reaching the RDAs were vitamin D, vitamin E, fluoride, and omega-3 fatty acids for both males and females, and choline, magnesium, and potassium in females. The burden of inadequate nutrition in individuals with obesity should be acknowledged and properly addressed within efforts to reduce obesity rates and associated disorders.

Prenatal Diagnosis in Great Artery Trasposition and Implications in Postnatal Outcome.

Great artery transposition, one of the most frequent and severe cyanotic heart malformations, represented the subject of many studies and research up to this moment. Although postnatal period is critical in this pathology, with correct management patients can benefit from complete surgical correction - arterial switch operation with good long term evolution. Prenatal diagnosis of Great Artery Transposition has an important contribution in choosing the appropriate treatment at the right time in postnatal period so that specific complications resulting from delaying the initiation of specific therapies can be avoided. This article proposes a review of existing data at this moment related to the importance of prenatal diagnosis in Great Artery Transposition and underlines how an accurate fetal diagnosis influences the decision of establishing the appropriate treatment in postnatal life for the children with this type of malformation and the complications that can be avoided.

Pulmonary Hypertension in Children - a Practical Approach.

Pulmonary hypertension (PH) is mean pulmonary arterial pressure above 25 mmHg at rest. Although considered a rare disease, the prevalence of PH in certain risk groups is higher, from 0.5% in patients with HIV infection to 30% in congenital heart disease (CHD) associating PH. In pulmonary arterial hypertension (PAH) associated with CHD, early changes of the vascular bed are reversible after correction of the defect, but there is a point from where the disease becomes irreversible or progression continues despite correction. Among patients with "operable" and "inoperable" CHD, there is a "gray area" group in between, which is defined by pulmonary vascular resistance equal to 4-8 WU/m and ratio of pulmonary vascular resistance to systemic vascular resistance of 0.3-0.5, measured by cardiac catheterization. In this situation a pulmonary vasoreactivity test is indicated. Pulmonary hypertension is a severe disease with increased morbidity and mortality. Pulmonary hypertension can result in death by decreased cardiac output, and heart failure.

Giant Cardiac Mass Detected to an Infant with Normal Fetal Echography and No Systolic Murmur in Early Postnatal Evolution.

Infective endocarditis is rare in children and is rarer on a normal structural heart in an infant without any surgical intervention. Most cases are related to a pre-existing congenital lesion, the most frequent etiology are Gram-positive cocci and the most feared are fungal agents. This report presents a 7-monthold infant with fungal endocarditis on a normal structural heart. The diagnosis was suspected on clinical examination and was confirmed by echocardiography and positive blood cultures. His particular clinical evolution after medical and surgical treatment illustrates a severe disease with poor prognosis which may be a complication of neonatal intensive care procedures. Unusual cause of fungal endocarditis in a previously healthy infant: neonatal hospitalization in intensive care unit.