BACKGROUND Percutaneous balloon aortic valvuloplasty (BAV) is an effective method of treatment for aortic stenosis in children. AIMS This study aimed to assess the long-term results of BAV in various age groups in the pediatric population. METHODS This retrospective study included 68 patients (newborns, infants, and children older than 1 year). We analyzed their demographic data and measurements performed with the use of cardiac catheterization, angiography, and echocardiography at 3 time points: before, directly after, and at late follow-up after BAV, including the invasive aortic valve peak-to-peak systolic pressure gradient (PG peak), maximum systolic pressure gradient (PG max) by Doppler echocardiography, and the severity of aortic regurgitation (AR). Long-term follow-up data were available for 57 patients. RESULTS The BAV procedure led to a significant reduction in the PG peak values. Late follow-up revealed a progression of AR. Aortic regurgitation of grade 2 or higher directly after BAV was the strongest risk factor for severe AR at late follow-up. After 5, 10, and 18 years after BAV, surgical treatment was not required in 90%, 77%, and 59.5% of patients, respectively. Severe AR and a residual PG less than 35 mm Hg at late follow-up were stronger risk factors for aortic valve surgery than moderate AR and PG higher than or equal to 35 mm Hg. CONCLUSIONS Balloon aortic valvuloplasty is an effective method of treatment for aortic stenosis in children, which delays the need for surgery. The progression of AR occurs at late follow-up. A greater reduction in PG max is a risk factor for at least moderate AR directly after BAV, which results in the progression of AR at late follow-up.

Aims The aim of the study was to evaluate left ventricular (LV) remodeling and systolic function using two-dimensional speckle tracking echocardiographic (2D STE) imaging in children at a long-term (more than 36 months, 107.5±57.8 months) after balloon valvuloplasty for aortic stenosis (BAV). Methods and results 40 patients (mean age 9,68 years, 75% male) after BAV and 62 control subjects matched to the age and heart rate were prospectively evaluated. The 2D STE assessment of LV longitudinal and circumferential strain and strain rate was performed. Left ventricular eccentric hypertrophy (LVEH) was diagnosed in 75% of patients in the study group. Left ventricular ejection fraction (LVEF) was normal in all patients. In study group, global longitudinal strain (GLS), global longitudinal strain rate (GLSr) were significantly lower compared with the controls: GLS (-19.7±2.22% vs. -22.3±1.5%, P< 0.001), GLSr (-0.89±0.15/s vs. -1.04 ±0.12/s, P < 0.001). Regional (basal, middle and apical segments) strain and strain rate were also lower compared with control group. Global circumferential strain (GCS), global circumferential strain rate (GCSr) as well as regional (basal, middle and apical segments) strain and strain rate were normal. Multivariable logistic regression analysis included: instantaneous peak systolic Doppler gradient across aortic valve (PGmax), grade of aortic regurgitation (AR), left ventricular mass index (LVMI), left ventricular relative wall thickness (LVRWT), left ventricular end-diastolic diameter (LVEDd), peak systolic mitral annular velocity of the septal and lateral corner (S’spt, S’lat), LVEF before BAV and time after BAV and showed that the only predictor of reduced GLS was LV eccentric hypertrophy [odds ratio 6.9; (95% CI: 1.37–12.5), P = 0.045]. Conclusion Patients at long-term observation after BAV present the subclinical LV systolic impairment, which is associated with the presence of its remodeling. Longitudinal deformation is the most sensitive marker of LV systolic impairment in this group of patients.

Aortic stenosis is a congenital heart disease which may present with different symptomatology and may lead to heart failure in newborn. A case of a 9-days old newborn with a severe aortic stenosis is presented. Due to impaired systolic function of the left ventricle and rapidly increasing symptoms of heart failure (tachypnea, dyspnoea, tachycardia) the neonate required pharmacological treatment (prostaglandin E1 infusion and phosphodiesterase inhibitor c-AMP) and balloon valvuloplasty as a matter of urgency. In chest X-ray the heart silhouette was enlarged, the electrocardiogram showed signs of overload of both ventricles. In echocardiography thickened aortic valves with a pin-hole opening, normal left ventricle size with signs of its hypertrophy and reduced contractility (ejection fraction 45%) and a moderate mitral regurgitation were found. Angiography and balloon valvuloplasty of the aortic valve was performed through puncture of the right femoral artery. After expansion of the balloon at the stenotic valve a very good result of the treatment was achieved – low invasive gradient (22 mm Hg) between the left ventricle and the aorta and a trivial aortic valve regurgitation. The good outcome of the procedure was confirmed and monitored in the next days. The general state of the child improved significantly, systolic function of the left ventricle returned to normal values. Because of a complication after the procedure – a thrombosis in the external iliac artery, a 3-month anti-clotting therapy was performed. No improvement in the patency of the vessel was observed; however, abundant collateral vessels provided the extremity with proper circulation.

Asymptomatic 17-years-old boy with a suspicion of the heart tumor was admitted to the Cardiology Department for further investigation. Due to the murmur over the heart echocardiography was performed revealing a bright mass near the left ventricular apex. He was in a good physical condition, no symptoms like chest pain, shortness of breath, syncope, palpitations were reported. On physical examination diminished heart sounds and systolic murmur 2-3/6 in the Levin’s scale over the heart were found. Laboratory data including troponin T, CK, CK-MB, NSE, urea acid, marker were within normal limits. On ECG abnormalities of the repolarization were recorded (ST segment depression and T waves inversion in the II, III, aVF leads). Transthoracic echocardiogram revealed a 4.7 x 5.8 cm tumor within the postero-lateral wall of the left ventricle, without left ventricular inflow nor outflow obstruction. Based on cardiac magnetic resonance hemangioma supplied from the right coronary artery was suspected. Cardiac catheterization was performed, the left ventricular mass with supply from vascular network of the right coronary artery was confirmed. No abnormalities of the central nervous system in magnetic resonance were found. Full-body positron emission tomography showed cardiac mass without any evidence of extra-cardiac fluorodeoxyglucose-avid disease. Cardiac hemangioma was recognized. Pharmacological treatment with propranolol was introduced without any impact on the tumor size during 6 months follow-up.

Scimitar syndrome is a rare, complex congenital anomaly characterized by partial anomalous pulmonary venous drainage into the inferior vena cava with hypoplasia of the right-sided lung, pulmonary artery and bronchial tree. Frequently aortopulmonary collaterals co-occur incrising pulmonary blood flow and hence the risk of pulmonary arterial hypertension. Surgical correction remains the gold-standard therapy. The autors present the case of a 23-days-old newborn after food aspiration, presenting respiratory symptoms: tachypnoe, tachycardia, respiratory effort with the use of accessory muscles, cough and crackles. The chest X-ray revealed the right lung consolidation. Laboratory markers of inflammatory process were negative. Aspiration pneumonia was diagnosed and the treatment was introduced. Despite the newborn presented respiratory distress with radiological changes. The echocardiography was performer and scimitar syndrome was suspected. The diagnosis was confirmed during cardiac catheterization in which aortopulmonary collateral artery running to the right lower lobe was demonstrated. The vessel was embolized with Amplatzer device.