Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry Ann-Sophie Kaemmerer, Matthias Gorenflo, Dörte Huscher, David Pittrow, Peter Ewert, Christine Pausch, Marion Delcroix, Hossein A. Ghofrani, Marius M. Hoeper, Rainer Kozlik-Feldmann, Andris Skride, Gerd Stähler, Carmine Dario Vizza, Elena Jureviciene, Dovile Jancauskaite, Lina Gumbiene, Ralf Ewert, Ingo Dähnert, Matthias Held, Michael Halank, Dirk Skowasch, Hans Klose, Heinrike Wilkens, Katrin Milger, Christian Jux, Martin Koestenberger, Laura Scelsi, Eva Brunnemer, Michael Hofbeck, Silvia Ulrich, Anton Vonk Noordegraaf, Tobias J. Lange, Leonhard Bruch, Stavros Konstantinides, Martin Claussen, Judith Löffler-Ragg, Hubert Wirtz, Christian Apitz, Rhoia Neidenbach, Sebastian Freilinger, Attila Nemes, Christian Opitz, Ekkehard Grünig, Stephan Rosenkranz

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19–58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning–Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning–Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

(1) Background: Systemic mastocytosis is a rare, non-curable disease with potential life-threatening complications in patients receiving cardiac surgery. (2) Methods: This systematic review of the literature was prompted by the case of a life-threatening anaphylactic reaction during cardiac surgery related to systemic mastocytosis. The search of all types of studies, using several databases (Pubmed, Scopus and Web of Science), was conducted through September 2022 to identify the relevant studies. (3) Results: Twelve studies were included describing cases of patients undergoing cardiac surgery who were diagnosed with systemic mastocytosis. An adverse effect, namely anaphylaxis, has happened in three cases. Different strategies of premedication, intraoperative and postoperative management were used. In our case, the patient was admitted for elective biological aortic valve replacement due to severe aortic stenosis. Intraoperatively, the patient developed an anaphylactic shock during the administration of protamine after separation from the cardiopulmonary bypass. This anaphylaxis reaction was a complication of the pre-existing systemic mastocytosis and could be successfully managed by the administration of epinephrine, antihistamines and corticosteroids. (4) Conclusions: This systematic literature search and case report highlight the importance of careful preoperative planning, as well as coordination between cardiac surgeons, anesthesiologists and hemato-oncological specialists, in patients with rare but complication-prone diseases such as systemic mastocytosis.

Concomitant LAA occlusion has been shown to be an effective and safe treatment for patients with atrial fibrillation during cardiac surgery to prevent embolic stroke. Minimally invasive procedures are challenging due to restricted access to and visibility of the surgical site. Also, aortic endoclamping has been developed as an alternative surgical approach to exoclamping. The aim of this article is to demonstrate the method of beating heart LAA occlusion with the Atriclip^® (AtriCure, Mason, OH, USA) device during minimally invasive mitral valve surgery while using the endoclamping alternative for aortic cross-clamping.