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Rodica Togănel

Abstract

Infective endocarditis (IE) is an infection of the endocardium and/or heart valves with the formation of a thrombus and secondary damage of the involved tissue, with significant mortality and severe complications. The prevention of bacterial endocarditis is of great controversy. Antimicrobial prophylaxis is usable in the prevention of endocarditis by killing bacteria before or after their extension to the damaged endocardium. No human studies offer strong evidence to support the efficacy of antibiotic prophylaxis so far, thus it could be potentially dangerous. Therefore, the European Society of Cardiology (ESC) may need to reconsider and update the previous guidelines with the proposal of reducing the prophylactic approach of IE. The 2015 Task Force recommends prophylaxis for highest risk patients undergoing highest risk procedures, focused on prevention rather than prophylaxis of IE, especially in nosocomial endocarditis.

Open access

Rodica Togănel

Abstract

Critical congenital heart disease (CHD) represents a special type of cardiovascular emergency due to the complexity of the associated pathology. In many cases, urgent surgery or catheter-based intervention is required as the condition might be life-threatening. In patients with ductal-dependent lesions, closure of the patent ductus arteriosus (PDA) within the first few days postpartum, can cause sudden clinical deterioration with potentially life-threatening consequences. The diagnostic challenges, clinical presentation and particularities related to the closure of PDA in life-threatening critical CHD are presented.

Open access

Rodica Togănel

Abstract

Congenital heart defects are among the most frequent anomalies present at birth, representing a heterogeneous group of malformations, both in terms of pathogenesis and clinical significance of the lesion. Failure to grow is well documented in infants with complex congenital heart defects; the presence of associated chromosomal abnormalities, cyanosis, and cardiac failure adds to the complexity and challenge. Malnutrition etiology can be grouped into the following three categories: inadequate intake, inefficient absorption and utilization, and/or increased energy needs. The consequences of malnutrition are both short and long term, timely nutritional intervention being necessary in order to maintain an adequate nutritional state. Because there are several types of congenital heart defects and multiple mechanisms by which they produce failure to thrive, no single strategy will be adequate to treat all cases. Medical complications such as chylotorax, necrotizing enterocolitis, laryngeal and neurological dysfunction play a major role in the requisite nutrition therapy in infants with congenital heart defect; limited access to human milk and parenteral concerns, as well as stress about feeding are also factors that can contribute to poor outcomes concerning nutrition and growth. Protocols are being considered and designed, and a systematic approach is always needed. The quality of life for patient and family, as well as getting the child back on track for age-appropriate development are always at the fore-front of each care plan.

Open access

Liliana Gozar, Cristina Blesneac and Rodica Toganel

Abstract

Background Cor triatriatum dexter is an extremely rare congenital anomaly, and in most cases, without hemodynamic significance. Congenital cytomegalovirus infection is the most common viral infection, the majority of infected infants being asymptomatic and only 5-15% being symptomatic in the neonatal period.

Case report We present the case of a female infant, aged 3 months. Clinical examination reveals cranial and facial anomalies. The karyotype was normal. Laboratory tests revealed the presence of anti-CMV antibodies in urine. Imaging studies showed cerebral and renal anomalies. Echocardiographic exam revealed a congenital anatomical feature without hemodynamic significance - cor triatriatum dexter.

Conclusions although cytomegalovirus infection is mostly asymptomatic, it can present as a complex pathological association, in which a congenital cardiac anatomical feature without hemodynamic significance, considered by some authors as an anatomical variant, can appear

Open access

Iolanda Muntean, Carmen Șuteu and Rodica Togănel

Abstract

Background: Pulmonary arterial hypertension is associated with right ventricular dilation and failure. As a result, left ventricular geometry is affected by shifting of the interventricular septum towards the left ventricle.

Aim of the study: The aim of the study was to assess the effect of chronic right ventricular pressure overload on left ventricular longitudinal function and synchronicity in idiopathic pulmonary arterial hypertension children, using speckle-tracking echocardiography.

Material and methods: We prospectively evaluated 13 children (5 with idiopathic pulmonary arterial hypertension and 8 sex- and age-matched controls) using conventional and speckle-tracking echocardiography and clinical status (WHO functional class). Left ventricular longitudinal strain curve was generated for 17 segments and global left ventricular longitudinal peak systolic strain was calculated. Dyssynchrony index of the left ventricle was determined calculating the standard deviation of time to peak-systolic strain for 12 left ventricular, 6 basal and 6 midventricular segments, from short axis views.

Results: Strain imaging showed significantly decreased global left ventricular longitudinal strain and increased dyssynchrony index in idiopathic pulmonary arterial hypertension patients as compared with controls (−16.80 ± 2.94 vs. −21.50 ± 1.60, p = 0.003, and 53.80 ± 16.72 vs. 22.25 ± 6.18, p=0.0001, respectively). There was a significant correlation between left ventricular longitudinal strain, dyssynchrony index and right ventricular fractional area changes (r = −0.66, p = 0.013, and r = −0.72, p = 0.005, respectively), right ventricular myocardial performance index (r = 0.86, p = 0.0001, and r = 0.93, p = 0.000, respectively), and LV eccentricity index (r = 0.82, p=0.001, and r = 0.93, p = 0.000, respectively) in the study population as a whole.

Conclusions: Left ventricular longitudinal systolic strain and synchronicity are impaired in idiopathic pulmonary arterial hypertension children with normal left ventricular ejection fraction.

Open access

Blesneac Cristina, Benedek Theodora, Togănel Rodica and Benedek I

Abstract

Background: Hypertrophic cardiomyopathy, one of the most common inherited cardiomyopathies, is a heterogeneous disease resulting from sarcomeric protein mutations, with an incidence in the adult population of 1:500. Current information on the epidemiology and outcomes of this disease in children is limited. Methods: Thirty-four children diagnosed with hypertrophic cardiomyopathy in the Pediatric Cardiology Department from Tîrgu Mureș were evaluated concerning familial and personal history, clinical, paraclinical and therapeutic aspects. Hypertrophic cardiomyopathy was defined by the presence of a hypertrophied, non-dilated ventricle, in the absence of a cardiac or systemic disease that could produce ventricular hypertrophy. Results: The youngest diagnosed child was a neonate, a total of 10 patients being diagnosed until 1 year of age. In 6 cases a positive familial history was found. Noonan syndrome was found in 2 cases. Only 21 patients were symptomatic, the predominant symptoms being shortness of breath on exertion with exercise limitations. Left ventricular outflow tract obstruction was present in 21 cases (61.7%). Twenty-four patients were on β-blocking therapy, while 4 patients underwent septal myectomy. Conclusions: Hypertrophic cardiomyopathy is a heterogeneous disorder in terms of evolution, age of onset, type and extent of hypertrophy, and the risk of sudden death. It can affect children of any age. There is a need for a complex evaluation, including familial and personal anamnesis, clinical examination, electrocardiogram and echocardiography of all patients. It is highly important to develop screening strategies, including genetic testing, for an early diagnosis, especially in asymptomatic patients with a positive familial background

Open access

Iolanda Muntean, Daniela Toma and Rodica Togănel

Abstract

Background: Simple transposition of the great arteries (TGA) is a cyanotic critical congenital heart defect representing a neonatal cardiovascular emergency, which requires surgery early after birth. In case of inadequate mixing between systemic and pulmonary circulations, creating an unrestrictive interatrial communication is mandatory. The aim of the present study was to identify the most important echocardiographic parameters that can predict the need for balloon atrial septostomy (BSA) in TGA neonates.

Material and methods: We retrospectively reviewed the echocardiographic recordings of newborns with TGA referred to our emergency room during a 6-year period. We measured the following echocardiographic parameters: atrial septal defect (ASD) size, interatrial septum (IAS) length, peak/mean inter-atrial pressure gradient, transverse diameter of the left atrium (LA), and transverse diameter of the right atrium (RA). Also, the ratio between ASD/IAS, the ratio between LA/RA, the ratio between mitral/tricuspid annulus, the ASD peak gradient/ASD diameter and the ASD diameter/(LA:RA ratio) were calculated.

Results: There were 37 neonates with simple TGA presented to the emergency room, split into two groups: those with BAS (n = 21) and those without BAS (n = 16). Besides significant differences between the two groups in ASD size, peak/mean interatrial pressure gradient, and LA:RA ratio, we found that ASD peak gradient/ASD diameter was significantly higher, but ASD diameter/(LA:RA ratio) was significantly lower in the group that required BAS compared with the group without BAS. Multivariate analysis showed that ASD diameter/(LA:RA ratio) was an independent predictor of septostomy requirement, with a cut-off value of 2.58.

Conclusion: ASD diameter/(LA/RA ratio) is a useful echocardiographic parameter that can provide supplementary information regarding inadequate mixing and the need for BAS in neonates with TGA.

Open access

Cristina Blesneac, Carmen-Corina Șuteu, Claudia Bănescu, Theodora Benedek, Imre Benedek and Rodica Togănel

Abstract

Background: LEOPARD syndrome is a complex dysmorphogenetic disorder of inconstant penetrance and various morphologic expressions. The syndrome is an autosomal dominant disease that features multiple lentigines, electrocardiographic changes, eye hypertelorism, pulmonary valve stenosis or hypertrophic cardiomyopathy, genital malformations, and a delayed constitutional growth hearing loss, which can be associated with rapidly progressive severe biventricular obstructive hypertrophic cardiomyopathy. No epidemiologic data are available on the real incidence of LEOPARD syndrome; however, this seems to be a rare disease, being often underdiagnosed, as many of its features are mild.

Case presentation: We report the case of a 10-year-old female pediatric patient, diagnosed with obstructive hypertrophic cardiomyopathy at the age of 3 months, and recently diagnosed with LEOPARD syndrome. The patient first presented for a cardiologic examination at the age of 3 months, due to a murmur. She presented failure to thrive and psychomotor retardation, and was diagnosed with biventricular obstructive hypertrophic cardiomyopathy for which she had received high-dose beta-blocker therapy. At the age of 7 years she underwent a biventricular myectomy for relief of outflow tract obstruction, completed with another myectomy after 2 years due to progressive increase of pressure gradient in the left ventricular outflow tract. Prior to the second surgical intervention, multiple lentigines appeared on her skin, and genetic testing revealed the presence of LEOPARD syndrome.

Conclusion: LEOPARD syndrome is a rare disease, which can be very difficult to diagnose, especially based on features other than lentigines. Cardiac involvement in LEOPARD syndrome can be progressive and requires multiple medical and surgical interventions.

Open access

Şuteu Carmen, Blesneac Cristina, Togănel Rodica and Benedek Theodora

Abstract

Introduction: Pulmonary arterial hypertension (PAH) is a rare disease associated with significant morbidity and mortality. Pediatric patients often present with mixted aetiologies.

Objectives: To characterize the epidemiology, management and outcome of pediatric PAH.

Methods: Children with PAH were included and followed prospectively for six months. WHO functional class, 6-minute walk test, biomarkers, electrocardiogram, spirometers and echocardiographic parameters were evaluated in progressive PAH group.

Results: Two hundred and four children were included in the study from July 2012 until July 2013, with a mean age of 6.13 years. Transient PAH patients (n=170, 83.33%) included newborns with persistent pulmonary hypertension (n=8, 3.92%) and children with congenital heart defects with systemic-to-pulmonary shuntflow PAH (n=162, 79.41%) in whom PAH resolved after successful surgery correction. Progressive PAH (n=34, 16.66%) included patients with idiopathic PAH (n=5, 2.45%), Eisenmenger syndrome (n=17, 8.33%) and post-operative PAH (n= 6, 2.94%). Patients with progressive PAH remained stable in regards to clinical status, WHO functional class, 6-minute walk distance, biomarkers, spirometers parameters and echocardiographic parameters with prognostic value.

Conclusions: Pediatric PAH is characterized by various age-specific diagnoses, the majority of which comprise transient forms of PAH. Pediatric PAH associated with congenital heart defects represents a heterogeneous group with highly variable clinical courses. PAH specific therapies may have contributed to disease stability and favorable outcomes.

Open access

Daniela Toma, Simina-Elena Rusu, Cristina Blesneac, Marian Pop and Rodica Togănel

Abstract

Background: Critical aortic coarctation is defined as the severe narrowing of the isthmic aortic lumen, representing a neonatal cardiac emergency, part of the congenital heart diseases with duct-dependent systemic circulation.

Aim of the study: To assess the correlation between transthoracic echocardiography and computed tomography angiography (CTA) in the measurement of aortic diameters in a group of newborns diagnosed with duct-dependent aortic coarctation and/or associated hypoplastic aortic arch.

Material and method: We performed a retrospective study on neonates diagnosed with duct-dependent aortic coarctation and/or associated hypoplastic aortic arch between January 1, 2015 and March 1, 2017. The studied parameters were diameters of the aorta at the level of the aortic annulus, coronary sinuses, sinotubular junction, ascending aorta, proximal and distal aortic arch, and the aortic isthmus. Measurements were obtained by transthoracic echocardiography and thoracic CTA.

Results: Fifteen newborns diagnosed with duct-dependent aortic coarctation and/or associated hypoplastic aortic arch were included in this study. There was no statistically significant difference between the two imaging methods, the T test highlighting differences only between the measurements of the aortic annulus (p <0.016) and coronary sinuses (p <0.008). The patients included in the study associated other cardiovascular abnormalities: persistent ductus arteriosus (100%), atrial septal defect (100%), aortic arch hypoplasia (80%), bicuspid aortic valve (73.3%).

Conclusions: These methods reveal important information on the anatomy of the cardiovascular malformation and its impact on the clinical and paraclinical status of the patient, being fundamental for establishing an optimal therapeutic approach.