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Pulmonary function is reduced in children after preterm birth. The variety of subgroups ranges from early to late preterm births. Limitations in pulmonary function can be observed even after late preterm birth without signs of bronchopulmonary dysplasia and/or history of mechanical ventilation. Whether this reduction in lung function is reflected in the cardiopulmonary capacity of these children is unclear. This study aims to investigate the impact of moderate to late premature birth on cardiopulmonary function. Cardiopulmonary exercise testing on a treadmill was performed by 33 former preterm infants between 8 and 10 years of age who were born between 32 + 0 and 36 + 6 weeks of gestation and compared with a control group of 19 children born in term of comparable age and sex. The former preterm children achieved comparable results to the term-born controls with respect to most of the cardiopulmonary exercise parameters $$(\dot{V}O_2peak 43.9\pm6.6\frac{\frac{ml}{kg}}{min}\min vs.41.9\pm8.8\frac{ml}{kg}/\min)$$ ( V ˙ O 2 p e a k 43.9 ± 6.6 ml kg min min v s . 41.9 ± 8.8 ml kg / min ) . The only differences were in a slightly higher oxygen uptake efficiency slope $$(OUES\;of\;1.6\;\pm0.4\;vs\;1.4\;\pm\;0.4)$$ ( O U E S o f 1.6 ± 0.4 v s 1.4 ± 0.4 ) and higher peak minute ventilation $$\dot VEpeak\;of\;55.2\pm 11.3 ml/min\;vs.\;49.1\;ml\;\pm\;8.8/min)$$ V ˙ E p e a k o f 55.2 ± 11.3 m l / m i n v s . 49.1 m l ± 8.8 / m i n ) in the group of children born preterm. With respect to heart rate recovery $$(-35.3\;\pm\;13.8\;bpm\;vs.\;-37.2\;bpm\;\pm\;14.0\;after\;1\;min)$$ ( - 35.3 ± 13.8 b p m v s . - 37.2 b p m ± 14.0 a f t e r 1 m i n ) and breathing efficiency $$\dot{(V}E/\dot VCO_2\;of\;35.9\;\pm\;4.1\;vs\;34.0\;\pm4.6)$$ ( V ˙ E / V ˙ C O 2 o f 35.9 ± 4.1 v s 34.0 ± 4.6 ) , there were no significant differences.Conclusion: Children born preterm did not show limitations in cardiopulmonary function in comparison with matched controls. What is Known:• Preterm birth is associated with reduced pulmonary function in later life, this is also true for former late preterms.• As a consequence of being born premature, the lungs have not finished their important embryological development. Cardiopulmonary fitness is an important parameter for overall mortality and morbidity in children and adults and a good pulmonary function is therefore paramount. What is New:• Children born prematurely were comparable to an age- and sex-matched control group with regards to almost all cardiopulmonary exercise variables.• A significantly higher OUES, a surrogate parameter for VO2peak was found for the group of former preterm children, most likely reflecting on more physical exercise in this group. Importantly, there were no signs of impaired cardiopulmonary function in the group of former preterm children.

Purpose Cardiopulmonary exercise testing (CPET) in preschoolers (4–6 years) represents a challenge. Most studies investigating CPET have been limited to older children (> 8 year). However, knowledge of the performance of small children is essential for evaluating their cardiorespiratory fitness. This study strives to compare a modified Bruce protocol with a new age-appropriate incremental CPET during natural movement running outdoors, using a mobile device. Methods A group of 22 4–6-year-old healthy children was tested indoor on a treadmill (TM) using the modified Bruce protocol. The results were compared with a self-paced incremental running test, using a mobile CPET device in an outdoor park. The speeds were described as (1) slow walking, (2) slow running, (3) regular running, and (4) running with full speed as long as possible. Results Mean exercise time outdoors (6,57 min) was significantly shorter than on the treadmill (11,20 min), $$\dot{V}{O}_{2peak}$$ V ˙ O 2 p e a k (51.1 ml/min/kg vs. 40.1 ml/min/kg), RER (1.1 vs. 0.98) and important CPET parameters such as $$\dot{V}E$$ V ˙ E max, O2pulse, heart rate and breath rate were significantly higher outdoors. The submaximal parameter OUES was comparable between both the tests. Conclusions Testing very young children with a mobile device is a new alternative to treadmill testing. With a significantly shorter test duration, significantly higher values for almost all cardiopulmonary variables can be achieved without losing the ability to determine VT1 and VT2. It avoids common treadmill problems and allows for individualized exercise testing. The aim is to standardize exercise times with individual protocols instead of standardizing protocols with individual exercise times, allowing for better comparability.

Aufgrund von kinderkardiologischen und -herzchirurgischen Behandlungserfolgen erreichen heutzutage mehr als 90 % der Patienten mit angeborenem Herzfehler das Erwachsenenalter. Die sehr heterogene Gruppe von schatzungsweise ca. 250.000–350.000 Patienten in Deutschland ubersteigt mittlerweile die Zahl der padiatrischen Patienten mit angeborenem Herzfehler (ca. 120.000). In der stetig wachsenden Gruppe der Erwachsenen mit angeborenem Herzfehler (EMAH) ist die grundlegende Unterscheidung anhand des zugrunde liegenden Herzfehlers und der individuellen Krankengeschichte empfehlenswert. Mithilfe einer systematischen Kategorisierung konnen der Behandlungs- und Betreuungsaufwand sowie das individuelle Risiko des Patienten relativ gut eingeschatzt werden. Nichtkorrigierte Herzfehler, Residualprobleme nach Korrekturen oder Palliationen und eine klinisch relevante Herzinsuffizienz sind die wichtigsten chirurgischen Behandlungsindikationen fur erwachsene Patienten mit angeborenen Herzfehlern.

Cardiac MRI is an accurate and reproducible technique for the assessment of left ventricular volumes and function. The accuracy of automated segmentation and the effects of manual adjustments have not been determined in children. To evaluate automated segmentation and the effects of manual adjustments for left ventricular parameter quantification in pediatric cardiac MR images. Left ventricular parameters were evaluated in 45 children with suspected myocarditis (age 13.4 ± 3.5 years, range 4–17 years) who underwent cardiac MRI. Dedicated software was used to automatically segment and adjust the parameters. Results of end-diastolic volume, end-systolic volume, stroke volume, myocardial mass, and ejection fraction were documented before and after apex/base adjustment and after apex/base/myocardial contour adjustment. The software successfully detected the left ventricle in 42 of 45 (93.3%) children; failures occurred in the smallest and youngest children. Of those 42 children, automatically segmented end-diastolic volume (EDV) was 151 ± 47 ml, and after apex/base adjustment it was 146 ± 45 ml, after apex/base/myocardial contour adjustment 146 ± 45 ml. The corresponding results for end-systolic volume (ESV) were 66 ± 32 ml, 63 ± 29 ml and 64 ± 28 ml; for stroke volume (SV) they were 85 ± 25 ml, 83 ± 23 ml and 83 ± 23 ml; for ejection fracture (EF) they were 57 ± 10%, 58 ± 9% and 58 ± 9%, and for myocardial mass (MM) they were 104 ± 31 g, 95 ± 31 g and 94 ± 30 g. Statistically significant differences were found when comparing the EDV/ESV/MM results, the EF results after apex/base adjustment and after apex/base/myocardial contour adjustment and the SV results (except for comparing the SVs after apex/base adjustment and after apex/base/myocardial contour adjustment). Automated segmentation for the evaluation of left ventricular parameters in pediatric MR images proved to be feasible. Automated segmentation + apex/base adjustment provided clinically acceptable parameters for the majority of cases.

The objective was to identify abdominal lymphatic malformations in pediatric patients with protein-losing enteropathy after palliation of complex congenital heart disease with total cavo-pulmonary connection (TCPC). In 2006, we performed complete hemodynamic and laboratory workup and thoracic and abdominal MRT screens in three patients who newly presented with symptoms of protein-losing enteropathy. All three patients, aged 3, 5, and 7 years, showed excellent TCPC hemodynamics with central venous pressures of 10-13 mm Hg. None of the patients had right-to-left overflow. All three patients showed extensive thoracic and mesenterial lymphangiomatosis. One patient died after 18 months of therapy, which included long-term parenteral nutrition, somatostatin, subcutaneous heparin injections, and frequent albumin and immunoglobulin substitution. The other two patients are in stable condition. Lymphangiomatosis might play an unknown role in the pathogenesis of protein-losing enteropathy after TCPC. It remains unclear whether lymphangiomatosis is a primary congenital disease related to the cardiac disease or if it is triggered by repeated surgery or venous congestion. The presence of lymphangiomatosis should be given diagnostic and therapeutic consideration in TCPC patients in the future.

We assessed the cardiac findings in Duchenne muscular dystrophy (DMD) and Becker-Kiener muscular dystrophy (BMD) patients in the large outpatient group of our single-center institution. The analysis included the revision of patient records (retrospectively) and current echocardiography, electrocardiogram (ECG), and Holter ECG data (prospectively). Reduced left ventricular fraction shortening (FS)25% was found in 24% of all patients (50 DMD, 12.1 +/- 4.7 years: 20 BMD, 17.1 +/- 8.5 years). Median age of onset of FS25% was 16.8 +/- 1.0 in DMD and 30.4 +/- 3.4 in BMD; (p0.05). Holter ECG in DMD demonstrated sinus tachycardia in 26% deprivation of circadian rhythm in 31%, and reduced heart rate variability in 51%. For these reasons, we recommend carrying out echocardiography annually in DMD and BMD10 years. Because the rate of disturbances in the cardiac autonomic nervous system is high in DMD, we also recommend Holter ECG annually. Further investigation should be conducted to determine if treatment with ss-blockers and ACE inhibitors in combination reduces cardiac autonomic nervous imbalance, thus improving patient outcome in DMD.

Restrictive cardiomyopathy is a rare heart muscle disease in childhood. By presenting a case report of a 15 year old adolescent, the aim of this article is to describe diagnosis, pathogenesis and therapy of restrictive cardiomyopathy. A review of the literature comprising pediatric studies on restrictive cardiomyopathy serves as a basis to discuss recommendations for therapeutic strategies in pediatric patients with this rare disease.