Spontaneous Coronary Artery Dissection and Papillary Muscle Rupture in Patient With Undiagnosed Vascular Ehler-Danlos Syndrome.

We present the case of a woman with acute coronary syndrome on the basis of spontaneous coronary artery dissection causing a papillary muscle rupture with severe mitral regurgitation and acute heart failure. The patient subsequently underwent successful emergent surgery of both the mitral and tricuspid valves. Postoperatively, the patient was diagnosed with vascular Ehlers-Danlos syndrome. (Level of Difficulty: Advanced.).

A 33-year follow-up after valvular surgery for carcinoid heart disease.

AIMS: Valvular surgery has improved long-term prognosis in severe carcinoid heart disease (CaHD). Experience is limited and uncertainty remains about predictors for survival and strategy regarding single vs. double-valve surgery. The aim was to review survival and echocardiographic findings after valvular surgery for CaHD at our institution. METHODS AND RESULTS: Between 1986 and 2019, 60 consecutive patients, median age 64 years, underwent valve surgery for severe CaHD. Operations involved combined tricuspid valve replacement (TVR) and pulmonary valve replacement (PVR) in 42 cases, and TVR-only or TVR with pulmonary valvotomy (no PVR) in 18 patients. All implanted valves were bioprosthetic. Preoperative echocardiography, creatinine, NT-pro-brain natriuretic peptide (NT-pro-BNP), and 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) were obtained. 30-Day mortality was 12% (n=7), and 8% for the most recent decade 2010-2019. Median survival was 2.2 years and maximum survival 21 years. Patients undergoing combined TVR and PVR had significantly higher survival compared with operations without PVR (median 3.0 vs. 0.9 years, P = 0.02). Preoperative levels of NT-pro-BNP and 5-HIAA in the top quartile predicted poor survival. On preoperative echocardiograms, pulmonary regurgitation was severe in 51% and indeterminate in 17%. Postoperative echocardiography confirmed relatively good durability of bioprostheses, relative to the patients' limited oncological life expectancy. CONCLUSION: Valvular surgery in CaHD has an acceptable perioperative risk. Survival for combined TVR and PVR was significantly higher compared with operations without PVR. Bioprosthetic valve replacement appears to have adequate durability. Preoperative echocardiography may underestimate pulmonary pathology. Combined TVR and PVR should be considered in most patients.

Cardiac Imaging in Carcinoid Heart Disease.

Carcinoid disease is caused by neuroendocrine tumors, most often located in the gut, and leads in approximately 20% of cases to specific, severe heart disease, most prominently affecting right-sided valves. If cardiac disease occurs, it determines the patient's prognosis more than local growth of the tumor. Surgical treatment of carcinoid-induced valve disease has been found to improve survival in observational studies. Cardiac imaging is crucial for both diagnosis and management of carcinoid heart disease; in the past, imaging was accomplished largely by echocardiography, but more recently, imaging for carcinoid heart disease has increasingly become multimodal and warrants awareness of the particular diagnostic challenges of this disease. This paper reviews the pathophysiology and manifestations of carcinoid heart disease in light of the different imaging modalities.

Surgical aspects of valve replacement in carcinoid heart disease.

Tricuspid and pulmonary valve replacement in patients with advanced carcinoid heart disease (CaHD) reduces right heart failure and improves prognosis. The surgical literature is limited concerning description of technical aspects of valve replacement in CaHD. Although a dedicated multidisciplinary care is required for these frail patients, optimization of surgical technique is important and may lead to better postoperative outcomes.

Quadruple Bioprosthetic Valve Replacement in a Patient With Severe Carcinoid Heart Disease.

Carcinoid heart disease typically affects the tricuspid and pulmonary valves, causing severe regurgitation and/or stenosis. Valve surgery has been shown to reduce right heart failure and improve long-term prognosis in these patients. We report a severe case of a patient with all 4 heart valves involved who underwent successful quadruple bioprosthetic valve replacement. (Level of Difficulty: Intermediate.).

Long-Term Risk of Ischemic Stroke After the Cox-Maze III Procedure for Atrial Fibrillation.

BACKGROUND: The long-term risk of stroke after surgical treatment of atrial fibrillation is not well known. We performed an observational cohort study with long follow-up after the "cut-and-sew" Cox-maze III procedure (CM-III), including left atrial appendage excision. The aim was to analyze the incidence of stroke/transient ischemic attack (TIA) and the association to preoperative CHA2DS2-VASc (age in years, sex, congestive heart failure history, hypertension history, stroke/TIA, thromboembolism history, vascular disease history, diabetes mellitus) score. METHODS: Preoperative and perioperative data were collected in 526 CM-III patients operated in four centers 1994 to 2009, 412 men, mean age of 57.1 ± 8.3 years. The incidence of any stroke/TIA was identified through analyses of the Swedish National Patient and Cause-of-Death Registers and from review of individual patient records. The cumulative incidence of stroke/TIA and association with CHA2DS2-VASc score was estimated using methods accounting for the competing risk of death. RESULTS: Mean follow-up was 10.1 years. There were 29 patients with any stroke/TIA, including 6 with intracerebral bleedings (2 fatal) and 4 with perioperative strokes (0.76%). The remaining 13 ischemic strokes and six TIAs occurred at a mean of 7.1 ± 4.0 years postoperatively, with an incidence of 0.36% per year (19 events per 5,231 patient-years). In all CHA2DS2-VASc groups, observed ischemic stroke/TIA rate was lower than predicted. A higher risk of ischemic stroke/TIA was seen in patients with CHA2DS2-VASc score 2 or greater compared with score 0 or 1 (hazards ratio 2.15, 95% confidence interval: 0.87 to 5.32) but no difference by sex or stand-alone versus concomitant operation. No patient had ischemic stroke as cause of death. CONCLUSIONS: This multicenter study showed a low incidence of perioperative and long-term postoperative ischemic stroke/TIA after CM-III. Although general risk of ischemic stroke/TIA was reduced, patients with CHA2DS2-VASc score 2 or greater had a higher risk compared with score 0 or 1. Complete left atrial appendage excision may be an important reason for the low ischemic stroke rate.

Long-Term Follow-Up of Cardiac Rhythm in 320 Patients After the Cox-Maze III Procedure for Atrial Fibrillation.

BACKGROUND: The Cox-maze III (CM-III) procedure is the gold standard for surgical treatment of atrial fibrillation (AF). Excellent short-term results have been reported, but long-term outcomes are lesser known. The aim was to evaluate current cardiac rhythm in a nationwide cohort of CM-III patients with very long follow-up. METHODS: Perioperative characteristics were retrospectively analyzed in 536 "cut-and-sew" CM-III patients operated on from 1994 to 2009 in 4 centers. Of these, 54 patients had died and 20 were unavailable at follow-up. The remaining 462 patients received a survey concerning arrhythmia symptoms, rhythm, and medication; of these, 320 patients (69%), comprising 252 men, with a mean age of 67 years (range, 47 to 87 years), and 83% with stand-alone CM-III, returned a current 12-lead electrocardiogram. Long-term monitoring was evaluated in 40 sinus rhythm patients. Postoperative stroke/transient ischemic attack was evaluated by register analysis. RESULTS: Mean follow-up was 111 ± 44 months (range, 36-223 months). Electrocardiogram analysis showed sinus rhythm in 219 of 320 patients (68%), and regular supraventricular rhythm (sinus, nodal, or atrial pacing) in 262 (82%), with 75% off class I/III antiarrhythmic medication. This group had lower arrhythmia symptom scores and medication use. Rhythm outcome did not differ by gender, age, type of AF, or stand-alone vs concomitant operation. Patients with more than 10 years of follow-up had a lower rate of regular supraventricular rhythm (69% vs 91%, p = 0.02). Long-term monitoring showed freedom from AF/atrial flutter in 38 of 40 patients (95%). The incidence of stroke/transient ischemic attack was 0.37% per year (11 patients). CONCLUSIONS: In a single-moment electrocardiogram evaluation 9 years after the cut-and-sew CM-III, 82% of patients were in sinus rhythm or other regular supraventricular rhythm. These findings support a long-lasting positive effect of the CM-III procedure, which is relevant when evaluating current nonpharmacologic therapies for AF.

The Berglin apical stitch: a simple technique to straighten things out in atrial fibrillation surgery.

In the Cox-Maze IV procedure, or in endocardial left atrial ablation, correct positioning of the surgical ablation probe within the left atrium might be difficult due to bulging or folds in the posterior left atrial wall. The Berglin apical stitch is a simple trick of the trade to create a smooth surface in the posterior left atrium that facilitates performing a safe transmural lesion and, consequently, may increase antiarrhythmic efficiency.

Early and long-term mortality in 536 patients after theCox-maze III procedure: a national registry-based study.

BACKGROUND: The cut-and-sew Cox-maze III procedure is the gold standard for surgical treatment of atrial fibrillation. The aim was to study early and long-term mortality based on registry analyses in Swedish Cox-maze III patients. METHODS: Preoperative and early postoperative data were analyzed in 536 patients (male/female (425/111), mean age 57 ± 8.6 years), operated from 1994 to 2009 in 4 centers; 422 (79%) underwent stand-alone Cox-maze III. Atrial fibrillation was paroxysmal in 38% and non-paroxysmal in 62%, mean duration was 7.8 ± 6.3 years. Patients were followed for survival or death in a validated national Cause-of-Death registry. Risk factors associated with observed survival were identified in univariable and multivariable analyses in a standard Cox proportional hazards model. RESULTS: Four early deaths (0.7%) occurred due to technical complications. At follow-up, 41 of 536 (7.6%) patients had died. Cause of death was cardiovascular in 19 of 536 (3.5%). No ischemic stroke-related death was registered. Univariable risk factors for all-cause mortality included hypertension (hazard ratio [HR] 2.8, confidence interval [CI] 1.5 to 5.3), heart failure (HR 2.4, CI 1.3 to 4.3), concomitant surgery (HR 2.2, CI 1.1 to 4.1), and postoperative complications (HR 2.5, CI 1.3 to 4.8). Gender, non-paroxysmal atrial fibrillation and long arrhythmia duration did not confer increased risk of death. Multivariable risk factors were hypertension (HR 2.9, CI 1.5 to 5.5) and postoperative complications (HR 2.4, CI 1.2 to 4.6). Survival for cardiovascular death at 5, 10, and 15 years was 98%, 96%, and 93%, respectively. CONCLUSIONS: Registry-based follow-up showed low early and long-term cardiovascular mortality and no stroke-related mortality. This is important baseline information when evaluating current surgical and nonsurgical treatment of atrial fibrillation.

A Swedish consensus on the surgical treatment of concomitant atrial fibrillation.

Atrial fibrillation (AF) is a common arrhythmia among patients scheduled for open heart surgery and is associated with increased morbidity and mortality. According to international guidelines, symptomatic and selected asymptomatic patients should be offered concomitant surgical AF ablation in conjunction with valvular or coronary surgery. The gold standard in AF surgery is the Cox Maze III ("cut-and-sew") procedure, with surgical incisions in both atria according to a specified pattern, in order to prevent AF reentry circuits from developing. Over 90% of patients treated with the Cox Maze III procedure are free of AF after 1 year. Recent developments in ablation technology have introduced several energy sources capable of creating nonconducting atrial wall lesions. In addition, simplified lesion patterns have been suggested, but results with these techniques have been unsatisfactory. There is a clear need for standardization in AF surgery. The Swedish Arrhythmia Surgery Group, represented by surgeons from all Swedish units for cardiothoracic surgery, has therefore reached a consensus on surgical treatment of concomitant AF. This consensus emphasizes adherence to the lesion pattern in the Cox Maze III procedure and the use of biatrial lesions in nonparoxysmal AF.

Learning what works in surgical cryoablation of atrial fibrillation: results of different application techniques and benefits of prospective follow-up.

Atrial fibrillation (AF) in cardiac surgical patients is detrimental in the long perspective. Concomitant surgical ablation of AF is recommended in guidelines and performed in most centers. The article describes the experiences in a single institution with concomitant surgical argon-based cryoablation in 115 patients using three different application techniques (epicardial left atrium, endocardial left atrium, biatrial cryo-maze) and a structured local follow-up to one year postoperatively. Results showed cryoablation to be safe with few complications related to the ablation procedure and few thromboembolic events. In this study, a complete biatrial lesion set according to the classic Cox-maze III (CM III) lesion pattern yielded a higher success rate than left atrial procedures. At 12 months, patients in sinus or pacing rhythm, free of AF without antiarrhythmic drugs, were 27/39 (69%), 24/32 (74%) and 36/44 (82%) in the EpiLA, EndoLA and cryo-maze groups, respectively. A consistent prospective follow-up is essential not only for research purposes but also for assessing the local results of AF surgery in everyday practice. It may direct and develop the surgical ablation program, guide individual postoperative arrhythmia management and is needed to increase overall quality of surgical AF ablation.

Late cardiac tamponade by chylous pericardial effusion after coronary artery bypass surgery: case report.

Chylopericardium is an uncommon but serious complication after open heart surgery that often necessitates surgical treatment. We describe a case of continuous and severely symptomatic chylous pericardial effusion after coronary artery bypass grafting in which the diagnosis was established by lipid electrophoresis. Initial conservative management failed, and ligation of the thoracic duct and pericardial fenestration were finally required for a successful outcome.

The biatrial cryo-maze procedure for treatment of atrial fibrillation: a single-center experience.

OBJECTIVES: The Cox-maze III procedure is the benchmark for atrial fibrillation (AF) surgery but has been replaced by surgical ablation. We evaluated our experience with biatrial cryoablation using the full Cox-maze III lesion pattern, and adhering follow-up to current guidelines. DESIGN: Forty-three patients underwent the biatrial cryo-maze procedure as a concomitant (n = 37) or stand-alone procedure (n = 6). Mean age was 64.8 ± 9.5 years. Overall, AF was paroxysmal/persistent/permanent in 28/14/58%. Mean AF duration was 5.2 ± 6.5 years. Follow-up included prospective evaluation at 1, 3 and 12 months, long-term monitoring and transthoracic echocardiography. RESULTS: No mortality and no complications related to the ablation procedure occurred. One patient suffered a stroke at 12 months. In the concomitant group, rhythm was sinus/pacing without AF/AF in 65/16/19% at three months, and 59/22/19% at 12 months. Five patients received new pacemakers (12%). In the stand-alone group, 5/6 (83%) patients had sinus rhythm with no AF at three and 12 months. Overall, 35/43 patients (81%) had sinus/paced rhythm at 12 months with no AF and no anti-arrhythmic drugs. Echocardiography showed satisfactory results in all patients. CONCLUSIONS: The biatrial cryo-maze procedure is safe and effective in surgical patients with concomitant AF, and could be considered for selected patients with lone AF.

Long-term health-related quality of life after maze surgery for atrial fibrillation.

BACKGROUND: Atrial fibrillation (AF) significantly impairs health-related quality of life (QoL). As pharmacologic treatment may have intolerable side effects and is not always effective, other techniques for curing AF have evolved. The maze III procedure has a high long-term success rate in restoring and maintaining sinus rhythm, but the long-term impact on QoL has not been sufficiently demonstrated. METHODS: Thirty-four patients underwent the maze III procedure for paroxysmal (n = 9), persistent (n = 15) or permanent (n = 10) AF. Quality of life was assessed with the Swedish Short Form-36 survey. Mean follow-up time was 35 +/- 6 months. RESULTS: Sinus rhythm was maintained in 32 patients (94%). For all domains except bodily pain, all patients reported substantial worse QoL at baseline as compared with healthy controls. Postoperatively all scores improved significantly to the level of the general population, and for the majority of the scoring items this was observed after 12 months. Improvement was maintained during the remaining observation period. CONCLUSIONS: The maze III procedure significantly improves QoL in patients with AF. The results are consistent during an observation time of 35 months. Based on QoL effects in a long-term perspective, maze surgery should be considered in symptomatic patients with AF refractory to pharmacologic treatment or catheter ablation.