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ZusammenfassungDie pulmonale Hypertension (PH) ist eine progressive Gefäßerkrankung und führt über eine Widerstands- und/oder Druckerhöhung im kleinen Kreislauf zu einem fortschreitenden Rechtsherzversagen. Auch wenn mithilfe aktueller medikamentöser Therapien eine deutliche Verbesserung der Lebensqualität und des Überlebens der Betroffenen erreicht werden konnte, bleibt die PH eine zumeist nichtheilbare Erkrankung, die im fortgeschrittenen Stadium eine Lungentransplantation notwendig macht. Interventionelle Verfahren, wie die Anlage eines interatrialen Shunts (z. B. durch atriale Septostomie oder den Atrial Flow Regulator) oder eines Reversed Potts Shunt, verbessern die RV-Funktion und die ventrikuläre Interaktion durch Schaffung einer prä- oder posttrikuspidalen „Eisenmenger-Physiologie“ und stellen eine Überbrückung oder sogar Alternative zur Lungentransplantation dar. Bei Patienten mit segmentaler PH oder chronisch thrombembolischer pulmonaler Hypertension (CTEPH) stellt die Ballonangioplastie eine bereits etablierte Intervention zur Verbesserung der pulmonalen Perfusion und damit rechtsventrikulären (RV-)Funktion dar. Dagegen ist die pulmonalarterielle Denervation ein neuartiges Verfahren, mit dem Ziel, die neurohumorale Dysregulation bei PH positiv zu beeinflussen. Der individuelle Einsatz solcher Interventionen, additiv zu den bereits etablierten medikamentösen Therapien, erweitert die Behandlungsmöglichkeiten und kann die Prognose betroffener Patienten noch weiter verbessern.

Background Prenatally diagnosed pericardial teratoma present a rare finding with an unfavourable prognosis due to frequently associated Foetal hydrops and limited treatment options. We report a successful surgical resection of a prenatally diagnosed cardiac teratoma in a 1160 g neonate with severe Foetal hydrops and cardiac deterioration. Case summary The patient was transferred in utero to our institution due to prior diagnosed pericardial mass and severe foetal hydrops, which necessitated caesarean section one day after arrival at a gestational age of 28 + 0 weeks. After intubation, the patient was stabilized by surgical drainage of 60 mL of pericardial effusion. Further clinical worsening of the patient on the day of life 12 demanded urgent intervention, so that in toto resection of the tumour was performed at a bodyweight of 1160 g. Histopathological analysis revealed a teratoma and the patient is in excellent clinical condition one year after surgery. Discussion This case report demonstrates that an interdisciplinary, two-staged approach can be a feasible and promising treatment option in patients with prenatally diagnosed teratoma and severe Foetal hydrops in a critical circulatory state. Furthermore, it illustrated that resection of pericardial masses can be successfully performed at a bodyweight as low as 1160 g.

Aberrant origin of the subclavian artery (SCA) is a well-known vascular anomaly as part of congenital heart diseases with the left subclavian artery (LSCA) being more frequently affected than the right subclavian artery (RSCA). Complete isolation of the SCA is an even more infrequent aortic arch anomaly, occurring in less than 1% for the LSCA and even less for the RSCA. Isolation of the RSCA in patients with d-transposition of the great arteries (D-TGA) is even scanter with only a hand full of cases being reported in the literature. However, isolation of the RSCA has important implications on hemodynamics and surgical strategies. In this case report, we present a newborn patient with D-TGA which presented with distinct differential cyanosis. While the right upper extremity appeared pink with an oxygen saturation of 100%, the rest of the body was cyanotic. At first, this appearance was interpreted as the Harlequin phenomenon during primary care. However, detailed echocardiography revealed an aberrant origin of the RSCA from the right pulmonary artery, which led to the differential cyanosis. The patient underwent arterial switch operation on day of life two including dissection and reimplantation of the RSCA. The special hemodynamic situation of this is discussed in terms of pathophysiology and as well as its impact on perioperative and surgical management.

Background: The right ventricle serves as the subaortic systemic ventricle (sysRV) in patients with congenitally corrected transposition of the great arteries (ccTGA) and in patients with transposition of the great arteries (TGA) surgically repaired by an atrial switch. SysRV can lead to late complications, primarily heart failure, significant regurgitation of the systemic atrioventricular (AV) valve, and ventricular arrhythmias with sudden cardiac death. We sought to investigate the value of 2D- and 3D-echocardiographic parameters of sysRV function. Methods: Consecutive adult patients with sysRV who presented at the adult congenital heart disease outpatient clinic were prospectively enrolled. All patients received comprehensive transthoracic echocardiography, including 3D-echocardiography, cardiac magnetic-resonance-imaging (CMR), cardiopulmonary-exercise-testing, and blood analysis for NT-proBNP. Results. A total of 27 patients were included, 18 with TGA and nine with ccTGA. Median age was 37 years (Q1 = 31, Q3 = 44), 44% were male, median NT-proBNP was 189 pg/mL (Q1 = 155, Q3 = 467), sufficient 3D-echocardiography datasets were acquired in 78% of patients. All echocardiographic 2D and 3D volumetric function parameters correlated with CMR data, whereas a correlation was not seen with any of the longitudinal function parameters. NT-proBNP correlated with tricuspid annular plane systolic excursion (r = −0.43, p = 0.02) and CMR ejection fraction (EF) (r = −0.62, p = 0.003). Conclusion: Systematic evaluation of sysRV is complex and should include not only volumetric parameters but also parameters of longitudinal function in addition to measurement of NT-proBNP. In patients with good image quality, 3D-echocardiography can be used to assess volumes and EF.

Im Rahmen einer webbasierten Onlinebefragung wird die derzeitige Praxis der Diagnostik und Therapie der KMPA bei osterreichischen Padiatern erhoben. Erstmals gibt es damit fur Osterreich Daten zur Diagnostik und Therapie, sowie zur Verordnungs- und Bewilligungspraxis von Therapeutika der KMPA. Es finden sich deutliche Defizite und Diskrepanzen zu den derzeitig gultigen Empfehlungen, die zur Durchfuhrung von nicht notwendigen, nicht empfohlenen Laborbestimmungen fuhren. Die orale Provokation mit Kuhmilch nach erfolgreicher klinischer Symptomverbesserung und die Verordnung von nicht evidenzbasierten Nahrungen sind durchaus haufig zu finden. Die Bewilligungspraxis durch die Krankenkassen wird vonseiten vieler Arzte als unbefriedigend, uneinheitlich und nicht klar geregelt empfunden