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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

Cristina Blesneac, Carmen Corina Şuteu, Marian Pop and Rodica Togănel

Abstract

Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH), that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.

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

Iolanda Muntean, Carmen Şuteu, Rodica Togănel and Claudia Bănescu

Abstract

Pulmonary arterial hypertension (PAH) is a progressive disease with a complex pathogenesis. The polymorphism of the gene of multidrug resistance-1 (MDR1) has been associated with many diseases including PAH.

Objective. In this study we aimed to investigate the relevance of the MDR1 polymorphism to pediatric PAH clinical course.

Methods. A total of 40 pediatric patients with PAH (secondary to congenital heart defects or idiopathic) and 40 control subjects were enrolled. Patients with PAH were divided into 2 groups, according to their evolution: 28 patients who remained clinically stable at 12-months (non-worsening group) and 12 patients who presented clinical worsening at 12-months (worsening group). Genomic DNA was genotyped for MDR1 gene polymorphisms as follows: C1236T, G2677T and C3435T.

Results. There were no significant differences between PAH children groups (clinical worsening and non-worsening) nor between PAH children and controls in terms of frequency distribution of the three studied genotypes or alleles.

Conclusions. The MDR1 polymorphism could not be correlated with the clinical evolution of pediatric PAH patients in our study.

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

Blesneac Cristina, Şuteu Carmen, Togănel Rodica, Benedek Theodora and I Benedek

Abstract

Background: Hypertrophic cardiomyopathy is a rather common hereditary disease with an autozomal dominant character, caused by mutations of genes that code for proteins of the cardiac sarcomere. The observed prevalence of this disease is much lower in pediatric patients compared to adults, because it’s late gene expression. Hypertrophic cardiomyopathy presenting in infancy has been shown to have a very high mortality.

Methods: Thirty-nine patients diagnosed with hypertrophic cardiomyopathy in the IIIrd Pediatric Cardiology Department from Tîrgu Mureş were included in this study. Patients were divided into two groups: group 1 – patients diagnosed during infancy, group 2 – patients diagnosed after 1 year of age. Data regarding familial and personal history, and echocardiographic findings were compared between these two groups.

Results: Group 1 included 17 patients and group 2 - 22 patients. Positive familial history was found in both groups (group 1 – 6 cases, group 2 – 3 cases), all of them in obstructive forms. Syncope was found in four cases, all of them in group 1 (p=0.02; odds ratio 15; 95% CI, 0.7473 to 301.1). While in group 1, asymmetric septal hypertrophy was predominant (64.7%), in group 2 – concentric left ventricular hypertrophy predominated (54.5%). Obstructive hypertrophic cardiomyopathy was found in 14 patients in group 1 (82.4%) compared to 13 patients in group 2 (59.1%). Diastolic function was impaired more predominantly in group 1 (p=0.0274; odds ratio 11.67; 95% CI, 1.526 to 89.17).

Conclusions: hypertrophic cardiomyopathy has an extensive clinical variability with regard to age of onset, severity and progression of disease.

Open access

Carmen Corina Şuteu, Theodora Benedek and Rodica Togănel

Abstract

Introduction: Pulmonary arterial hypertension (PAH) is a complex disease with poor prognosis. Serum uric acid has been proposed as a potentially non-invasive and objective parameter for prognosis and response to therapy.

Objectives: To investigate the potent relationship between serum uric acid levels and functional and echocardiographic parameters in children with PAH.

Methods: Serum uric acid levels were measured in 34 children with PAH and were correlated with the functional class, 6-minute walk test, and echocardiographic parameters at baseline and at 12 months follow-up.

Results: In pediatric PAH patients serum uric acid levels were higher compared with the control subjects (p = 0.001). In the high uric acid group serum uric acid levels were correlated with 6-minute walk test (p = 0.008), and with several echocardiographic parameters, such as pulmonary vascular resistance (p = 0.04), fractional area change (p = 0.05), left ventricle eccentricity index (p = 0.04), right atrial area (p = 0.03), right ventricle myocardial index (p = 0.01), and pericardial effusion (p = 0.001), markers of right ventricular overload and dysfunction.

Conclusions: Serum uric acid levels are easy to collect and measure, and correlate with both functional and echocardiographic parameters that reflect right ventricular dysfunction.

Open access

Carmen Corina Șuteu, Iolanda Muntean, Cristina Blesneac, Brîndușa Căpîlna, Theodora Benedek and Rodica Togănel

Abstract

Background: Alteration in lung function is present in children with pulmonary arterial hypertension (PAH). We aimed to study the pulmonary function indices obtained by spirometry in pediatric patients with PAH, and to correlate them with B-type natriuretic peptide (BNP) and echocardiographic parameters.

Methods: Thirty-one children with PAH were enrolled in this study, of those 26 patients (83.87%) associated shunt defect and 5 patients (16.12%) were diagnosed with idiopathic PAH. Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), FEV1/FVC, peak expiratory flow rate (PEFR), forced expiratory flow at 25–75% of the pulmonary volume (FEF 25–75) were evaluated by spirometry and correlated with BNP and echocardiographic parameters that reflect right ventricle function.

Results: Restrictive pattern of pulmonary function was present in 51.6% (n = 16) of all PAH children. There were significant correlations between BNP with FVC (p = 0.001), FEV1 (p = 0.001), and FEV1/FVC (p = 0.001). Serum BNP level was significantly increased in the group of patients without shunt. Of those echocardiographic parameters that reflect right ventricle function, we found that TAPSE significantly correlated with PEFR (r = −0.47, p <0.01), and with FEF 25–75 (r = −0.39, p <0.01).

Conclusions: Deterioration of the pulmonary function indices are correlated with BNP and echocardiographic parameters, markers of RV dysfunction. Being easy and reliable tests, pulmonary functions can be introduced among the follow-up tools in children with PAH.