Cardiac rupture as a life-threatening outcome of Takotsubo syndrome: A systematic review.

BACKGROUND: Takotsubo syndrome (TS) is a reversible cause of heart failure; however, a minority of patients can develop serious complications, including cardiac rupture (CR). OBJECTIVES: Analyze case reports of CR related to TS, detailing patient characteristics to uncover risk factors and prognosis for this severe complication. METHODS: We conducted a systematic search of MEDLINE and Embase databases to identify case reports of patients with TS complicated by CR, from inception to October 2023. RESULTS: We included 44 subjects (40 females; 4 males) with a median age of 75 (IQR 71-82) years, of White/Caucasian (61%) or East Asian/Japanese (39%) ethnicity. An emotional trigger was present in 15 (34%) subjects and an apical ballooning pattern was observed in all cases (100%). ST-segment elevation was reported in 39 (93%) of 42 cases, with the anterior myocardial segments (37 [88%]) being the most compromised, followed by lateral (26 [62%]) and inferior (14 [33%]) segments. The median time to cardiac rupture was 48 (5-120) hours since admission, with the left ventricular free wall (25 [57%]) being the most frequent site of perforation. Surgery was attempted in 16 (36%) cases, and 28 (64%) patients did not survive. CONCLUSIONS: CR related to TS is a rare complication associated with high mortality and affecting elderly females, specially from White/Caucasian or East Asian/Japanese descent, presenting with anterior or lateral ST-segment elevation, and an apical ballooning pattern. Although data is limited and additional prospective studies are needed, the awareness of this life-threatening complication is crucial to early identify high-risk patients. CONDENSED ABSTRACT: Cardiac rupture is a rare complication of Takotsubo syndrome. We conducted a systematic review of cases complicated by cardiac rupture, and we identified 44 subjects (40 females and 4 males) with a median age of 75 (IQR 71-82) years, of White/Caucasian (61%) or East Asian/Japanese (39%) ethnicity, all with an apical ballooning pattern (100%). The median time to cardiac rupture was 48 (5-120) hours since admission, with the left ventricular free wall (25 [57%]) being the most frequent site of perforation. Surgery treatment was attempted in 16 (36%) cases, and 28 (64%) patients did not survive.

Pheochromocytoma-induced cardiogenic shock: A multicentre analysis of clinical profiles, management and outcomes.

OBJECTIVE: There is still uncertainty about the management of patients with pheochromocytoma-induced cardiogenic shock (PICS). This study aims to investigate the clinical presentation, management, and outcome of patients with PICS. METHODS: We collected, retrospectively, the data of 18 patients without previously known pheochromocytoma admitted to 8 European hospitals with a diagnosis of PICS. RESULTS: Among the 18 patients with a median age of 50 years (Q1-Q3: 40-61), 50% were men. The main clinical features at presentation were pulmonary congestion (83%) and cyclic fluctuation of hypertension peaks and hypotension (72%). Echocardiography showed a median left ventricular ejection fraction (LVEF) of 25% (Q1-Q3: 15-33.5) with an atypical- Takotsubo (TTS) pattern in 50%. Inotropes/vasopressors were started in all patients and temporary mechanical circulatory support (t-MCS) was required in 11 (61%) patients. All patients underwent surgical removal of the pheochromocytoma; 4 patients (22%) were operated on while under t-MCS. The median LVEF was estimated at 55% at discharge. Only one patient required heart transplantation (5.5%), and all patients were alive at a median follow-up of 679 days. CONCLUSIONS: PICS should be suspected in case of a CS with severe cyclic blood pressure fluctuation and rapid hemodynamic deterioration, associated with increased inflammatory markers or in case of TTS progressing to CS, particularly if an atypical TTS echocardiographic pattern is revealed. T-MCS should be considered in the most severe cases. The main challenge is to stabilize the patient, with medical therapy or with t-MCS, since it remains a reversible cause of CS with a low mortality rate.

Postoperative pericardial effusion, pericardiotomy, and atrial fibrillation: An explanatory analysis of the PALACS trial.

BACKGROUND: In the Posterior left pericardiotomy for the prevention of atrial fibrillation after cardiac surgery (PALACS) trial, posterior pericardiotomy was associated with a significant reduction in postoperative atrial fibrillation (POAF) after cardiac surgery. We aimed to investigate the mechanisms underlying this effect. METHODS: We included PALACS patients with available echocardiographic data (n = 387/420, 92%). We tested the hypotheses that the reduction in POAF with the intervention was associated with 1) a reduction in postoperative pericardial effusion and/or 2) an effect on left atrial size and function. Spline and multivariable logistic regression analyses were used. RESULTS: Most patients (n = 307, 79%) had postoperative pericardial effusions (anterior 68%, postero-lateral 51.9%). The incidence of postero-lateral effusion was significantly lower in patients undergoing pericardiotomy (37% vs 67%; P < .001). The median size of anterior effusion was comparable between patients with and without POAF (5.0 [IQR 3.0-7.0] vs 5.0 [IQR 3.0-7.5] mm; P = .42), but there was a nonsignificant trend towards larger postero-lateral effusion in the POAF group (5.0 [IQR 3.0-9.0] vs 4.0 [IQR 3.0-6.4] mm; P = .06). There was a non-linear association between postero-lateral effusion and POAF at a cut-off at 10 mm (OR 2.70; 95% CI 1.13, 6.47; P = .03) that was confirmed in multivariable analysis (OR 3.5, 95% CI 1.17, 10.58; P = 0.02). Left atrial dimension and function did not change significantly after posterior pericardiotomy. CONCLUSIONS: Reduction in postero-lateral pericardial effusion is a plausible mechanism for the effect of posterior pericardiotomy in reducing POAF. Measures to reduce postoperative pericardial effusion are a promising approach to prevent POAF.

Characteristics of Postoperative Atrial Fibrillation and the Effect of Posterior Pericardiotomy.

BACKGROUND: Postoperative atrial fibrillation (POAF) is a frequent complication of cardiac surgery. However, only a few detailed descriptions of the arrhythmia have been reported. We aim to describe the characteristics, outcomes, and variables associated with POAF and to evaluate how posterior pericardiotomy (PP) affects POAF characteristics. METHODS: In this post hoc analysis of the Posterior left pericardiotomy for the prevention of AtriaL fibrillation After Cardiac Surgery (PALACS) trial, we describe POAF characteristics based on continuous in-hospital telemetry data. RESULTS: Of 420 patients, 103 (24.5%) developed POAF. Median time to onset was 50.3 hours; 70.9% of events occurred within 3 days. Hemodynamic instability and rapid ventricular response occurred in 8.7% and 51.5% of cases, respectively. Most POAF patients received antiarrhythmics (97.1%), 22.3% electrical cardioversion, and 40.8% systemic anticoagulation. Median POAF duration was 24.0 hours; 70.9% of cases resolved within 36 hours. Median POAF burden was 15.9%. All patients were in sinus rhythm at follow-up. POAF was associated with longer hospitalization (7 vs 6 days; P < .001), but not increased mortality or morbidity. PP reduced POAF incidence (17.7% vs 31.3%; P = .001), especially after postoperative day 2 (time to POAF onset 41.9 vs 57.1 hours; P = .01). Age was associated with POAF. Female sex, coronary artery bypass grafting, beta blockers, and PP were inversely associated. CONCLUSIONS: POAF remains frequent after cardiac surgery. Hemodynamic instability is rare, although rapid ventricular response and need for electrical cardioversion are frequent. POAF burden is significant, and the arrhythmias resolve within 30 days. PP reduces POAF especially after postoperative day 2.

Multicolor flow cytometry on pericardial effusion for a prompt diagnosis and treatment of hematological malignancies with heart involvement.

Background: Malignancies represent 15-50% of total causes of pericardial effusions (PE). Routine analyses recommended to be performed on pericardial fluid include general chemistry, cytology, polymerase chain reaction, and microbiological cultures. Multicolor flow cytometry (FC) is a laboratory test that already proved to be useful in the detection of lymphoproliferative and metastatic malignancies in pleural and peritoneal effusions, but current guidelines do not mention its use on PE to reach a diagnosis. Methods: Our institutional protocol foresees to routinely perform a multicolor FC analysis on pericardial fluid samples obtained by pericardiocentesis, in addition to other guidelines-recommended analyses. A sample of 15-30 ml is analyzed using a lyse and wash staining method using combination panels of antibodies, allowing to detect specific cellular subpopulations, analyzing tens to hundreds of thousands of cells in few seconds. The present manuscript aims to report our single-center experience with this diagnostic tool in patients presenting with PE requiring pericardiocentesis. Results: Routine use of multicolor FC on pericardial fluid samples in our institution allowed to reach a definite diagnosis of cardiac lymphomas in two patients presenting with otherwise unexplained severe PE. This resulted in immediate start of combined immunotherapy, with patients' clinical improvement. At 6 months follow-up both patients are alive and presented a complete disease regression. Conclusion: Preliminary evidence from routine use of multicolor FC on PE support that this is a promising tool to reach a rapid diagnosis of hematological malignancies with heart involvement, leading to a prompt initiation of targeted therapies.

'Here comes the story of the Hurricane': a case report of AL cardiac amyloidosis and myocardial bridging.

Background: Cardiac amyloidosis (CA) is a rapidly progressive infiltrative cardiomyopathy, whose role is emerging as a not-so-rare disorder leading to heart failure (HF). Myocardial bridge (MB) is the most common inborn coronary artery variant, and its clinical relevance is still matter of debate. The exceptional coexistence of these two conditions could accelerate disease progression and worsen the already compromised clinical conditions. Case summary: We present the case of a 76-year-old female patient experiencing relapsing HF decompensation and presenting to our centre with dyspnoea at rest and severe peripheral congestion. Echocardiogram showed severe concentric hypertrophy, severe biventricular contractile dysfunction, and third-degree diastolic dysfunction. Coronary angiography excluded epicardial atherosclerotic disease, though displaying a long intramyocardial course of left anterior descending artery. Physiological invasive test was achieved in terms of instantaneous wave-free ratio (iFR), both at baseline and after inotropic and chronotropic stimuli, and attested haemodynamic significance. Concurrently, the diagnostic flow chart for CA was accomplished, by means of both invasive (periumbilical fat biopsy, bone marrow aspiration) and non-invasive tests (99mTc-diphosphonate scintigraphy, serum-urine immunofixation) that confirmed the suspect of primary amyloidosis. Acute HF therapy was personalized according to the singularity of the case, avoiding both nitrates and beta-blockers, then first cycle of chemotherapy was started. Discussion: Our clinical case shows a unique interaction between infiltrative cardiomyopathy and coronary artery abnormality. Amyloidosis can contribute to the ischaemic burden of the MB and this may, in turn, abbreviate the path to HF decompensation.

Clinical Impact of Heart Team Decisions for Patients With Complex Valvular Heart Disease: A Large, Single-Center Experience.

Background A multidisciplinary approach might be pivotal for the management of patients with valvular heart disease (VHD), but clinical outcome data are lacking. Methods and Results At our institution, since 2014, internal guidelines recommended heart team consultations for patients with VHD. The clinical/echocardiographic characteristics, treatment recommendations, performed treatment, and early clinical outcomes of consecutive, hospitalized patients with VHD undergoing heart team evaluation were collected. Surgical risk was prospectively assessed by the EuroSCORE II and STS-PROM. The primary end point of the study was early mortality. A total of 1004 patients with VHD with high clinical complexity (mean age, 75 years; mean EuroSCORE II, 9.4%; mean STS-PROM, 5.6%; 48% ischemic heart disease; 29% chronic kidney disease, 9% oncologic/hematologic diseases) were enrolled. The heart team recommended an interventional treatment for 807 (80%) patients and conservative management for 197 (20%) patients. Management crossovers occurred in only 5% of patients. The recommended intervention was cardiac surgery for 230 (23%) patients, percutaneous treatment in 516 (51%) patients, and hybrid treatment in 61 (6%) patients. Early mortality occurred in 24 patients (2.4%) and was independently predicted by aortic stenosis, left ventricular ejection fraction, pulmonary artery systolic pressure, and conservative management recommendation. In patients referred to treatment, observed early mortality (1.7%) was significantly lower (P<0.001) than expected on the bases of both the STS-PROM (5.2%) and EuroSCORE II (9.7%). Conclusions Within the limitations of its single-center and observational design, the present study suggests that heart team-based management of patients with complex VHD is feasible and allows referral to a wide spectrum of interventions with promising early clinical results.

Posterior left pericardiotomy for the prevention of atrial fibrillation after cardiac surgery: an adaptive, single-centre, single-blind, randomised, controlled trial.

BACKGROUND: Atrial fibrillation is the most common complication after cardiac surgery and is associated with extended in-hospital stay and increased adverse outcomes, including death and stroke. Pericardial effusion is common after cardiac surgery and can trigger atrial fibrillation. We tested the hypothesis that posterior left pericardiotomy, a surgical manoeuvre that drains the pericardial space into the left pleural cavity, might reduce the incidence of atrial fibrillation after cardiac surgery. METHODS: In this adaptive, randomised, controlled trial, we recruited adult patients (aged ≥18 years) undergoing elective interventions on the coronary arteries, aortic valve, or ascending aorta, or a combination of these, performed by members of the Department of Cardiothoracic Surgery from Weill Cornell Medicine at the New York Presbyterian Hospital in New York, NY, USA. Patients were eligible if they had no history of atrial fibrillation or other arrhythmias or contraindications to the experimental intervention. Eligible patients were randomly assigned (1:1), stratified by CHA2DS2-VASc score and using a mixed-block randomisation approach (block sizes of 4, 6, and 8), to posterior left pericardiotomy or no intervention. Patients and assessors were blinded to treatment assignment. Patients were followed up until 30 days after hospital discharge. The primary outcome was the incidence of atrial fibrillation during postoperative in-hospital stay, which was assessed in the intention-to-treat (ITT) population. Safety was assessed in the as-treated population. This study is registered with ClinicalTrials.gov, NCT02875405, and is now complete. FINDINGS: Between Sept 18, 2017, and Aug 2, 2021, 3601 patients were screened and 420 were included and randomly assigned to the posterior left pericardiotomy group (n=212) or the no intervention group (n=208; ITT population). The median age was 61·0 years (IQR 53·0-70·0), 102 (24%) patients were female, and 318 (76%) were male, with a median CHA2DS2-VASc score of 2·0 (IQR 1·0-3·0). The two groups were balanced with respect to clinical and surgical characteristics. No patients were lost to follow-up and data completeness was 100%. Three patients in the posterior left pericardiotomy group did not receive the intervention. In the ITT population, the incidence of postoperative atrial fibrillation was significantly lower in the posterior left pericardiotomy group than in the no intervention group (37 [17%] of 212 vs 66 [32%] of 208 [p=0·0007]; odds ratio adjusted for the stratification variable 0·44 [95% CI 0·27-0·70; p=0·0005]). Two (1%) of 209 patients in the posterior left pericardiotomy group and one (<1%) of 211 in the no intervention group died within 30 days after hospital discharge. The incidence of postoperative pericardial effusion was lower in the posterior left pericardiotomy group than in the no intervention group (26 [12%] of 209 vs 45 [21%] of 211; relative risk 0·58 [95% CI 0·37-0·91]). Postoperative major adverse events occurred in six (3%) patients in the posterior left pericardiotomy group and in four (2%) in the no intervention group. No posterior left pericardiotomy related complications were seen. INTERPRETATION: Posterior left pericardiotomy is highly effective in reducing the incidence of atrial fibrillation after surgery on the coronary arteries, aortic valve, or ascending aorta, or a combination of these without additional risk of postoperative complications. FUNDING: None.

Posterior Left pericardiotomy for the prevention of postoperative Atrial fibrillation after Cardiac Surgery (PALACS): study protocol for a randomized controlled trial.

BACKGROUND: Postoperative atrial fibrillation (POAF) is a common complication following cardiac surgery. POAF is associated with increased morbidity and hospital costs. We herein describe the protocol for a randomized controlled trial to determine if performing a posterior left pericardiotomy prevents POAF after cardiac surgery. METHODS/DESIGN: All patients submitted to cardiac surgery at our institution will be screened for inclusion into the study. The study will consist of two parallel arms with random allocation between groups to either receive a posterior left pericardiotomy or serve as a control. Masking will be done in a single-blinded fashion to the patient. Patients will be continuously monitored postoperatively for the occurrence of atrial fibrillation until discharge. At the follow-up clinic visit (15-30 days after surgery), the primary endpoint (atrial fibrillation) and other secondary endpoints, such as pleural or pericardial effusion, will be assessed. A total sample size of 350 subjects will be recruited. DISCUSSION: POAF is associated with increased morbidity, prolonged hospital stay, and increased costs after cardiac surgery. Several strategies aimed at reducing the incidence of POAF have been investigated, including beta-blockers, amiodarone, and statins, all with suboptimal results. Posterior left pericardiotomy has been associated with a reduction of POAF in previous series. However, these studies had limited sample sizes and suboptimal methodology, so that the efficacy of posterior pericardiotomy in preventing POAF remains to be definitively proven. Our randomized trial aims to determine the effect of a posterior left pericardiotomy on the incidence of POAF. TRIAL REGISTRATION: ClinicalTrials.gov, ID: NCT02875405 , protocol record 1502015867. Registered on July 2016.

"Near-zero" fluoroscopic exposure in supraventricular arrhythmia ablation using the EnSite NavX™ mapping system: personal experience and review of the literature.

PURPOSE: Radiofrequency catheter ablation (RFCA) of supraventricular tachyarrhythmias carries a small but non-negligible radiation risk. Studies in children already showed the feasibility of using three-dimensional mapping systems as the primary guide for catheter visualization and positioning in these RFCAs. We aim to demonstrate the feasibility and safety of such an approach in young and middle-aged patients. METHODS: Fifty patients (age 34 ± 12) with supraventricular tachyarrhythmias underwent electrophysiological study; of these, 47 patients proceeded to RFCA guided by the EnSite NavX(TM) system (23 with atrioventricular nodal reentry tachycardia, 16 with an accessory pathway, six with typical atrial flutter, and two with right atrial tachycardia). RESULTS: In 38/50 cases (76%), electroanatomical mapping avoided fluoroscopy entirely, including four cases requiring access to the left heart chambers by a retrograde approach. In the remaining 12/50 cases (24%), fluoroscopy use was limited to 122 ± 80 s, with a correspondingly low radiation exposure (dose area product 1.3 ± 1.1 mGy × m(2)). All procedures were acutely successful, with a procedural time of 113 ± 37 minutes, and without incurring in any major complication. Over a mean follow-up of 12 ± 3 months, we observed one recurrence of pre-excitation and one relapse of atrial flutter. CONCLUSIONS: Our study shows that non-fluoroscopic RFCA of supraventricular tachyarrhythmias using the EnSite NavX(TM) system is feasible, safe, and effective in a population of relatively young adults. Our experience of a non-fluoroscopic approach in these procedures deserves consideration, particularly in the young or in other patients at higher radiation risk.

Rippling muscle disease and cardiomyopathy associated with a mutation in the CAV3 gene.

Caveolin-3, the myocyte-specific isoform of caveolins, is preferentially expressed in skeletal, cardiac and smooth muscles. Mutations in the CAV3 gene cause clinically heterogeneous neuromuscular disorders, including rippling muscle disease, or cardiopathies. The same mutation may lead to different phenotypes, but cardiac and muscle involvement rarely coexists suggesting that the molecular network acting with caveolin-3 in skeletal muscle and heart may differ. Here we describe an Italian family (a father and his two sons) with clinical and neurophysiological features of rippling muscle disease and heart involvement characterized by atrio-ventricular conduction defects and dilated cardiomyopathy. Muscle biopsy showed loss of caveolin-3 immunosignal. Molecular studies identified the p.A46V mutation in CAV3 previously reported in a German family with autosomal dominant rippling muscle disease and sudden death in few individuals. We suggest that cardiac dysfunction in myopathic patients with CAV3 mutations may be underestimated and recommend a more thorough evaluation for the presence of cardiomyopathy and potentially lethal arrhythmias.

Increased brain natriuretic peptide secretion is a marker of disease progression in nonobstructive hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is associated with increased plasma brain natriuretic peptide (BNP), but sequential plasma and myocardial BNP assessment in stable and dilated HCM has never been performed. METHODS AND RESULTS: Forty consecutive HCM patients (42 +/- 8 years, 25 males) underwent cardiac catheterization, angiography, and left ventricular (LV) endomyocardial biopsy. During follow-up (70.5 +/- 6.7 months), 30 patients (Group 1) remained stable whereas 10 patients (Group 2) progressed to dilated phase. Group 2 patients underwent a second invasive study with LV biopsy. BNP plasma levels were measured at baseline and at follow-up in all patients. All biopsies were processed for histology and immunohistochemistry with anti-BNP antibodies. BNP plasma levels remained unchanged in Group 1, whereas it significantly increased in all Group 2 patients who exhibited an elevation of LV and right ventricular end-diastolic pressure. Immunohistochemistry showed an increase of BNP-positive myocytes in follow-up biopsies when compared with baseline (75.0 +/- 15.0 % versus 29.8 +/- 10.0 %; P = .005) with a significant correlation with LV end-diastolic pressure (r = 0.78, P < .001) and plasma BNP (r = 0.83, P < .001). CONCLUSIONS: Progression to end-stage of HCM is characterized by further increase of myocardial and plasma BNP. Serial assessment of plasma BNP may provide noninvasive recognition of hemodynamic deterioration, allowing prompt institution of heart failure therapy.

Cardiac histological substrate in patients with clinical phenotype of Brugada syndrome.

BACKGROUND: The role of structural heart disease and sodium channel dysfunction in the induction of electrical instability in Brugada syndrome is still debated. METHODS AND RESULTS: We studied 18 consecutive patients (15 males, 3 females; mean age 42.0+/-12.4 years) with clinical phenotype of Brugada syndrome and normal cardiac structure and function on noninvasive examinations. Clinical presentation was ventricular fibrillation in 7 patients, sustained polymorphic ventricular tachycardia in 7, and syncope in 4. All patients underwent cardiac catheterization, coronary and ventricular angiography, biventricular endomyocardial biopsy, and DNA screening of the SCN5A gene. Biopsy samples were processed for histology, electron microscopy, and molecular screening for viral genomes. Microaneurysms were detected in the right ventricle in 7 patients and also in the left ventricle in 4 of them. Histology showed a prevalent or localized right ventricular myocarditis in 14 patients, with detectable viral genomes in 4; right ventricular cardiomyopathy in 1 patient; and cardiomyopathic changes in 3. Genetic studies identified 4 carriers of SCN5A gene mutations that cause in vitro abnormal function of mutant proteins. In these patients, myocyte cytoplasm degeneration was present at histology, whereas terminal dUTP nick end-labeling assay showed a significant increase of apoptotic myocytes in right and left ventricle versus normal controls (P=0.014 and P=0.013, respectively). CONCLUSIONS: Despite an apparently normal heart at noninvasive evaluation, endomyocardial biopsy detected structural alterations in all 18 patients with Brugada syndrome. Mutations in the SCN5A gene, identified in 4 of the 18 patients, may have induced concealed structural abnormalities of myocardiocytes that accounted for paroxysmal arrhythmic manifestations.