IntroductionThe aim of our prospective study was to evaluate heart rate variability (HRV) in children with aortic valve stenosis (AS) and its relationship with left ventricular mass and peak transaortic valve pressure gradient (PG).Material and methodsSixty children with AS divided into 3 groups according to their PG and 60 healthy controls were studied. Holter ECG monitoring with time domain HRV analysis was performed. Left ventricular mass was calculated by echocardiography.ResultsMean values of all HRV parameters were statistically significantly lower (p < 0.001) in children with AS than in controls (respectively: SDNN 127.8 ±28.2 ms; 162.6 ±38.0 ms, SDNN day 99.7 ±26.6 ms; 134.1 ±36.1 ms, SDNN night 99.9 ±32.8 ms; 123.4 ±45.7 ms, SDANN 112.2 ±27.7 ms; 142.4 ±34.6, SDNNi 62.2 ±16.2 ms; 75.9 ±21.6, RMSSD 39.6 ±12.1 ms; 50.3 ±16.7 ms, rMSSD day 33.6 ±10.9 ms; 43.1 ±14.7 ms, rMSSD night 49.8 ±18.1 ms; 64.4 ±24.9 ms, pNN50 16.4 ±9.5%; 23.5 ±11.7%, pNN50 day 12.0 ±8.5%; 18.4 ±10.7%, pNN50 night; 26.5 ±14.8%; 36.4 ±17.4%. No significant differences between the mean values of HRV parameters in children with different PG and with and without myocardial hypertrophy were found. In children with AS and ventricular arrhythmia SDNN day was significantly lower (p < 0.05) compared to patients without arrhythmia (94.9 ±22.1 ms vs. 109.3 ±22.5 ms).ConclusionsIn children with AS the balance of the autonomic nervous systemic disturbed which manifests in an increase in sympathetic and decrease in parasympathetic activity. Transaortic valve pressure gradient and myocardial hypertrophy do not influence the HRV. The SDNN reduction during the day period may indicate the risk of ventricular arrhythmia in children with AS.

Intracardiac tumors in children are relatively rare, but their clinical consequences may include severe outflow tract obstruction, embolism, cardiac insufficiency, or rhythm disturbances. In some cases, the tumor may constitute part of a genetic condition and prompt additional investigations, as well as a modification of therapeutic management. Herein, we present a molecularly confirmed familial case of Gorlin syndrome with an early cardiac tumor as a presenting sign. We provide detailed clinical characteristics of the affected individuals and a useful review of syndromic causes of pediatric cardiac tumors in clinical practice.

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Total anomalous pulmonary venous return (TAPVR) is a rare congenital cyanotic heart disease, in which the lack of connection between all four pulmonary veins and left atrium is present. The interatrial communication allows the oxygenated blood flow to systemic circulation through the left atrium. The authors present a 5-month-old girl with heart failure symptoms. Poor weight gain, feeding difficulties, tachypnoe with use of accessory breathing muscles, hepatomegaly and low oxygen saturation level were observed. Serum concentration of NT-proBNP was elevated. Electrocardiography revealed right atrial and right ventricular enlargement. Chest X-Ray showed cardiomegaly and increased pulmonary vascular markings. The echocardiographic evaluation revealed pulmonary venous drainage to superior vena cava, right-sided heart chambers enlargement, main pulmonary artery dilation and atrial septal defect. Computed tomographic angiography was performed in order to acquire more accurate image of pulmonary veins connection. Supracardiac type of total anomalous pulmonary venous return was diagnosed and cardiosurgical correction was performed. The authors pay attention to the importance of recognizing the clinical symptoms of heart failure in infants by primary care physicians.

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.