Pulmonale und pulmonalarterielle Hypertonie bei Erwachsenen mit angeborenen Herzfehlern: Real-world-Daten aus dem internationalen COMPERA-AHF-Register

Die pulmonale Hypertonie (PH) ist ein häufiges Residuum oder eine Komplikation, die den natürlichen sowie den postoperativen oder postinterventionellen Verlauf bei Patienten mit angeborenen Herzfehlern (AHF) modifiziert oder kompliziert. Da der klinische Verlauf der verschiedenen AHF sich erheblich unterscheidet, sind differenzierte klinischen Studien und Beobachtungsstudien dringend erforderlich. Hierbei unzureichend abgebildete Situationen müssen durch Daten aus Registerstudien ergänzt werden. Das COMPERA-Register wurde im 2007 entwickelt, um die aktuellen Managementstrategien bei Erwachsenen mit allen Formen einer PH, einschließlich der PH bei AHF (P(A)H-AHF), basierend auf „real-world data“ darzustellen. In der vorliegenden Untersuchung werden die Daten einer Untergruppe von 769 Patienten mit P(A)H-AHF vorgestellt, die zwischen Januar 2007 und September 2020 an 62 Zentren für PH und/oder AHF aus 12 Ländern erhoben wurden. Die eingeschossenen Patienten hatten post-trikuspidale Shunts (n = 359; 46,7%), prä-trikuspidale Shunts (n = 249; 32,4%), komplexe AHF (n = 132; 17,2%), angeborene linksventrikuläre oder aortale Obstruktionen (n = 9; 1,2%) oder verschiedene andere AHF (n = 20; 2,6%). Sie waren 45,3 ± 16,8 Jahre alt und in 67% handelte es sich um Frauen. Zum Zeitpunkt des Registereinschlusses befanden sich die Patienten in Funktionsklasse I / II (28,2%), III (56,0%) oder IV (3,8%) und sie hatten eine mittlere Gehstrecke von 369 ± 121 Metern im 6-Minuten-Gehtest. Insgesamt 358 Patienten hatten ein Eisenmenger-Syndrom, von denen nur 79 Patienten nicht unter einer gezielten PAH-Therapie standen; 22 EMAH hatten eine pulmonal(arteriell)e Hypertonie, aber ohne Eisenmenger Syndrom; bei neun Patienten bestand eine Fontan-Zirkulation. Zum Zeitpunkt des Registereinschlusses bestand eine gezielte PAH-Therapie bei 69% in Form einer Monotherapie und bei 31% eine Kombinationstherapie. Zum Zeitpunkt der letzten Verlaufskontrolle ist der Anteil der Patienten unter Kombinationstherapie in allen Gruppen gestiegen (im Gesamtkollektiv von 31 auf 53%). Als Wirkstoffe fanden zum Zeitpunkt des Registereinschlusses vorzugsweise Endothelinrezeptorantagonisten (ERA) (n=454), PDE-5-Inhibitoren (n=416) sowie andere PAH-spezifische Medikamente (Prostacycline, n=39; Riociguat, n= 17) Verwendung. Insgesamt wurde als ERA Bosentan (n = 289) häufiger als Macitentan (n = 83), Ambrisentan (n = 56) oder Sitaxentan (n = 26) verwendet. Als PDE-5-I wurde Sildenafil (n = 315) häufiger als Tadalafil (n = 101) eingesetzt. Die Art und Häufigkeit einer oralen Antikoagulation oder Thrombozytenaggregationshemmung unterschied sich je nach zugrunde liegender Diagnose bzw. Komorbidität. Während der Beobachtung im COMPERA-Register sind von 647 eingeschlossenen Patienten 151 (22,4 %) verstorben. Von ihnen standen zum Todeszeitpunkt 65 (43,0 %) unter einer Monotherapie, 67 (44,4 %) unter einer Kombinationstherapie und 19 (12,6 %) waren ohne medikamentöse PAH-Therapie. Das internationale COMPERA-CHD-Register liefert als momentan weltweit größtes Register umfassende „real-world-Daten“ zum Spontanverlauf sowie über Art und Durchführung moderner Behandlungskonzepte bei PAH-AHF. Das Register wird auch weiterhin Ärzten wertvolle Daten für das Management von P(A)H-AHF und zukünftig detaillierte Überlebensanalysen liefern

Rare Case Report: Left Atrial Sarcoma Obstructing the Left Ventricular Inflow

Malignant cardiac tumors of the heart are extremely rare and may present tremendous diagnostic and therapeutic challenges. These tumors are able to infiltrate the heart and metastasize systemically. Early detection is often elusive as the clinical presentation is highly variable, posing significant diagnostic and therapeutic difficulties. Despite a multidisciplinary approach, the prognosis for patients with malignant cardiac tumors remains guarded. Early diagnosis and a multidisciplinary approach involving cardiac surgeons, oncologists and critical care specialists are crucial in the management of this disease. Further research is needed to better understand the pathomechanisms of tumor-related complications and to develop effective treatment strategies to improve patient outcomes. The rare case of a 78-year-old woman with left atrial tumor requiring emergency surgery for acutely developing mitral valve obstruction is presented. Pathology confirmed an undifferentiated pleomorphic sarcoma. This patient tragically did not survive, highlighting the difficulties of managing such a rare and deceptive heart disease.</jats:p

Left Atrial Appendage Occlusion on a Beating Heart during Minimally Invasive Valve Surgery Using an Aortic Endoclamp: A Case Report

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.Deutsche Forschungsgemeinschaft and Friedrich-Alexander-Universität Erlangen-Nürnber

A Practical Approach to Systemic Mastocytosis Complications in Cardiac Surgery: A Case Report and Systematic Review of the Literature

(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

Pericardial tamponade, a diagnostic chameleon: from the historical perspectives to contemporary management

Background Pericardial tamponade (PT) early after cardiac surgery is a challenging clinical entity, not infrequently misrecognized and often only detected late in its course. Because the clinical signs of pericardial tamponade can be very unspecific, a high degree of initial suspicion is required to establish the diagnosis. In addition to clinical examination the deployment of imaging techniques is almost always mandatory in order to avoid delays in diagnosis and to initiate any necessary interventions, such as pericardiocentesis or direct cardiac surgical interventions. After a brief overview of how knowledge of PT has developed throughout history, we report on an atypical life-threatening cardiac tamponade after cardiac surgery. A 74-year-old woman was admitted for elective biological aortic valve replacement and aorto-coronary-bypass grafting (left internal mammary artery to left anterior descending artery, single vein graft to right coronary artery). On the 10th postoperative day, the patient unexpectedly deteriorated. She rapidly developed epigastric pain radiating to the left upper abdomen, and features of low peripheral perfusion and shock. There were no clear signs of pericardial tamponade either clinically or echocardiographically. Therefore, for further differential diagnosis, a contrast-enhanced computed tomography scan was performed under clinical suspicion of acute abdomen. Unexpectedly, active bleeding distally from the right coronary anastomosis was revealed. While the patient was prepared for operative revision, she needed cardiopulmonary resuscitation, which was successful. Intraoperatively, the source of bleeding was located and surgically relieved. The subsequent postoperative course was uneventful. Conclusions In the first days after cardiac surgery, the occurrence of life-threatening situations, such as cardiac tamponade, must be expected. Especially if the symptoms are atypical, the entire diagnostic armamentarium must be applied to identify the origin of the complaints, which may be cardiac, but also non-cardiac. Central message A high level of suspicion, immediate diagnostic confirmation, and rapid treatment are required to recognize and successfully treat such an emergency (Fig. 5). Perspective Pericardial tamponade should always be considered as a complication of cardiac surgery, even when symptoms are atypical. The full range of diagnostic tools must be used to identify the origin of the complaints, which may be cardiac, but also non-cardiac (Fig. 5)

Medical treatment of pulmonary hypertension in adults with congenital heart disease : updated and extended results from the International COMPERA-CHD Registry

Funding Information: The authors are indebted to the COMPERA investigators and their staff. We explicitly thank Dr. Claudia S. Copeland for the professional editing of the final draft of the manuscript. Funding: COMPERA is funded by unrestricted grants from Acceleron, Actelion Pharmaceuticals (Janssen), Bayer, OMT and GSK. These companies were not involved in data analysis or the writing of this manuscript. Funding Information: ICMJE uniform disclosure form (available at https:// dx.doi.org/10.21037/cdt-21-351). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. Dr. DH reports non-financial support from Actelion, Boehringer-Ingelheim, and Shire, outside the submitted work; Dr. DP reports personal fees from Actelion, Biogen, Aspen, Bayer, Boehringer Ingelheim, Daiichi Sankyo, and Sanofi, outside the submitted work; Dr. MD reports personal fees from Actelion, Bayer, GSK and MSD, outside the submitted work; Dr. HAG reports personal fees from Actelion, Bayer, Gilead, GSK, MSD, Pfizer and United Therapeutics, outside the submitted work; Dr. MG reports personal fees from Actelion, Bayer and GSK, outside the submitted work; Dr. MMH reports personal fees from Acceleron, Actelion, Bayer, MSD and Pfizer, outside the submitted work; Dr. CDV reports personal fees from Actelion, Bayer, GSK, MSD, Pfizer, and United Therapeutics, outside the submitted work; Dr. RE reports personal fees from Actelion, Boehringer Ingelheim, OMT, Bayer, and Berlin Chemie; grants from Actelion and Boehringer Ingelheim, outside the submitted work; Dr. MH reports grants and personal fees from Actelion, personal fees from Bayer, Berlin Chemie, Boehringer Ingelheim, GSK, Janssen, Novartis and MSD, outside the submitted work; Dr. MH reports personal fees from Acceleron, Actelion, AstraZeneca, Bayer, BERLIN CHEMIE, GSK, MSD, Novartis and OMT, outside the submitted work; Dr. HW reports personal fees from Action, Bayer, Biotest, Boehringer, GSK, Pfizer, and Roche, outside the submitted work; Dr. DS reports personal fees from Actelion, Bayer, and GSK, outside the submitted work; Dr. LS reports personal fees from Actelion, Bayer, and MSD, outside the submitted work; Dr. SU reports grants from Swiss National Science Foundation, Zurich Lung, Swiss Lung, and Orpha Swiss, grants and personal fees from Actelion SA/Johnson & Johnson, Switzerland, and MSD Switzerland, outside the submitted work; Dr. TJL reports personal fees from Actelion, Janssen-Cilag, BMS, MSD, and OMT GmbH, outside the submitted work; Dr. LB reports personal fees from Actelion, outside the submitted work; Dr. MC reports personal fees from Boehringer Ingelheim Pharma GmbH, Roche Pharma, and Boehringer Ingelheim, outside the submitted work; Dr. HW reports personal fees from Boehringer Ingelheim, and Roche, outside the submitted work. Dr. EG reports personal fees from Actelion, Janssen, Bayer, MSD, Bial, OrPha Swiss GmbH, OMT and Medscape, outside the submitted work; Dr. SR reports personal fees from Actelion, Bayer, GSK, Pfizer, Novartis, Gilead, MSD, and United Therapeutics, outside the submitted work. The authors have no other conflicts of interest to declare. Publisher Copyright: © Cardiovascular Diagnosis and Therapy. All rights reserved.Background: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. Methods: The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD Results: A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of followup, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of “Non-Eisenmenger PAH” patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described. Conclusions: Analyzing “real life data” from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the “Non-Eisenmenger PAH” group and to patients with complex CHD, including Fontan patients.publishersversionPeer reviewe

A Dual-Pathogen Mitral Valve Endocarditis Caused by <i>Coxiella burnetii</i> and <i>Streptococcus gordonii</i>—Which Came First?

Infective endocarditis (IE) is still a life-threatening disease with high morbidity and mortality. While usually caused by a single bacterium, poly-microbial infective endocarditis (IE) is rare. Here, we report a (blood-culture-negative) dual pathogen mitral valve IE caused by Coxiella burnetii and Streptococcus gordonii: A 53-year-old woman was presented to an internal medicine department with abdominal pain for further evaluation. Within the diagnostic work up, transthoracic echocardiography (TTE) revealed an irregularly shaped echogenic mass (5 × 13 mm) adherent to the edge of the posterior mitral valve leaflet and protruding into the left atrium. As infected endocarditis was suspected, blood cultures were initially obtained, but they remained negative. Chronic Q fever infection was diagnosed using serologic testing. After the occurrence of cerebral thromboembolic events, the patient was admitted for mitral valve surgery. Intraoperatively, a massively destructed mitral valve with adhering vegetations was noted. Examination of the mitral valve by broad-range bacterial polymerase chain reaction (PCR) and amplicon sequencing confirmed Coxiella burnetii infection and yielded Streptococcus gordonii as the second pathogen. Based on the detailed diagnosis, appropriate antibiotic therapy of both pathogens was initiated, and the patient could be discharged uneventfully on the 11th postoperative day after a successful minimal-invasive mitral valve replacement

Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair

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

Lessons from the short- and mid-term outcome of medical rehabilitation in adults with congenital heart disease

Background: The number of adults with congenital heart disease (ACHD) is steadily increasing. Over their life-time, many of the affected patients require medical rehabilitation after interventional or surgical treatment of residua, sequels or complications of their congenital heart defect (CHD). However, up to now only scarce data exist about indication, performance and outcomes of cardiac rehabilitation in ACHD. Methods: The course and outcome of rehabilitation after previous interventional or surgical treatment in ACHD was analyzed in a retrospective cohort study