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).
(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.
Background: Pulmonary arterial hypertension (PAH), a common complication in adults with congenital heart disease (CHD), leads to significant morbidity and mortality. Targeted PAH medication is available, but PAH-CHD patient data are limited. Several questions regarding indication, treatment escalation, and combination therapy remain unanswered. The aim of this study was therefore to evaluate PAH-specific treatment in adults with PAH-CHD to better understand PAH-specific therapy management. Methods:In this cross-sectional study we retrospectively examined clinical, demographic, and cardiaccatheterization data and medical management for PAH-CHD, and analyzed clinical course and midterm outcome.Results: Over up to 14 years (median, 6.2 years), 103 PAH-CHD patients (66% female) receiving targeted PAH-therapy for pre-tricuspid-shunt (15.5%), post-tricuspid-shunt (32.0%), and complex CHD (52.4%) were followed. Based on modified clinical European Society of Cardiology (ESC) classification, patients were assigned to the following subgroups: Eisenmenger syndrome (ES) (45.6%), severe pulmonary vascular disease (PVD) in complex CHD (20.4%), post-repair patients (19.4%), prevalent systemic-to-pulmonary shunt (3.9%), coincidental/small defects (0%), and Fontan circulation (10.7%). Changes in targeted PAH therapy were observed 249 times, with up to 6 (median, 2) therapy changes over a median period of 1.3 years. Over the study course, the medical treatment strategy changed towards combination therapy (baseline, 13.6%; study-end, 41%), resulting mostly in stabilized functional class or even improvement in cases of prevalent systemic-to-pulmonary shunt, ES, and patients with repaired CHD. Functional class deterioration, however, was seen in patients with severe PVD due to complex CHD, and Fontan patients.Of the 103 patients in the study, 25 died (24.3%). Patients with repaired CHD and patients with systemicto-pulmonary shunt or ES showed the best survival rates. Mortality was remarkably higher in patients with severe PVD in complex CHD and Fontan patients.Conclusions: Many patients with PAH-CHD benefited from targeted PAH therapy over a median period of 6.2 years. Treatment decisions after targeted PAH-medication initiation were based mainly on clinical assessment. To counteract disease progression, an escalation towards combination therapy was observed during the study course. We consider survival rates under targeted PAH medication to be favorable, particularly in the ES subgroup. Nevertheless, further research is needed to optimize the use of PAH medication, especially in patients with complex CHD.
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