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Open access

Iolanda Muntean, Carmen Șuteu and Rodica Togănel


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

Rodica Togănel, Iolanda Muntean, Carmen Duicu, Amalia Făgărăşan, Liliana Gozar and Claudia Bănescu


Background: Pulmonary arterial hypertension (PAH) is an incapacitating disease even in childhood, associated with very poor prognosis. The disease is characterised by endothelial dysfunction. Two of the key endothelial mediators involved in the PAH pathogenesis are nitric oxide (NO) and angiotensinogen (AGT). Purpose of the study: to evaluate the following gene polymorphisms: endothelial nitric oxide synthase (eNOS) G894T, eNOS 4b/4a, and angiotensinogen (AGT) M235T, as well as allele frequency and their association with PAH in children. Material and methods This study included 32 children with pulmonary arterial hypertension secondary to congenital heart disease, 46 children with congenital heart disease without pulmonary arterial hypertension referred to the Pediatric Cardiology Clinic Tg.Mures and 40 healthy controls. All patients underwent a complete physical with NYHA class evaluation, echocardiographic exam and eNOS (G894T, 4b/4a) as well as AGT M235T polymorphisms determination. Results The frequency of eNOS 894T allele (p < 0.0001) was significantly higher in patients with pulmonary arterial hypertension. Conclusions Our results advocate that there is a correlation between eNOS 894T allele and pulmonary arterial hypertension in children

Open access

Iolanda Muntean, Daniela Toma and Rodica Togănel


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

Amalia Făgărășan, Iolanda Muntean, Liliana Gozar, Sorina Pasc and Rodica Togănel


Introduction: The aim of this study was to study the anatomical types of total anomalous pulmonary venous connection (TAPVC), the associated cardiac and extracardiac congenital malformations, clinical manifestations, and postoperative evolution.

Materials and methods: Twenty-four patients with a mean age of 125 days, admitted to the Clinic of Pediatric Cardiology III between January 1, 2009 and December 31, 2015 and diagnosed with TAPVC were included in the study. The patients were evaluated clinically, electrocardiographically and echocardiographically, both pre- and postoperatively. Postoperative evolution was monitored at 1, 3, 6, 12 and 24 months.

Results: The anatomical types of TAPVC were: supracardiac in 50% of cases (12 patients), cardiac in 37.5% (9 patients) and mixed type in 12.5% of cases (3 patients). The first clinical manifestation was cyanosis in 72.2% of cases. Surgical correction was performed at a mean age of 37 days in obstructive forms, and 254 days in non-obstructive forms. From the study lot, 8.4% of patients had associated extracardiac malformations (anorectal agenesis and Ivemark syndrome). Early postoperative complications included pulmonary hypertension crisis (60% of cases), supraventricular arrhythmias (35% of cases) and chylothorax (8.4% of cases). Late postoperative complications included: reintervention in 8% of patients with mixed type TAPVC.

Conclusions: The most frequently encountered type was supracardiac TAPVC, which had a favorable postoperative evolution. Mixed type TAPVC had the highest rate of reintervention.

Open access

Iolanda Muntean, Carmen Şuteu, Rodica Togănel and Claudia Bănescu


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

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


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.