Extracardiac Causes Research Articles

Plasma Biomarkers for Prediction of Sudden Cardiac Death

Sudden cardiac death (SCD) accounts for most cardiovascular deaths in the United States, claiming an estimated 300 000 lives per year.1 Despite intense efforts to develop resuscitation techniques, survival rates are still low, at ≈5%.2 This has led to a renewed focus in this field directed toward optimizing risk prediction to identify the candidate that would qualify for prophylactic or preventive interventions.3 At present, the clinical risk stratification is largely limited to the use of left ventricular ejection fraction (LVEF) for identifying primary prophylaxis candidates, but specificity of this predictor is modest.4 Moreover, the absolute numbers of these high-risk patients in the general population are low. Instead, the highest prevalence of SCD is likely observed among previously asymptomatic individuals, even though it is well-known that coronary artery disease (CAD) is present in most SCD cases.5 This underscores the need for the development of risk stratification models that can be applied to the general population where the largest health benefit is likely to be realized. The underpinnings of SCD are complex, and it is reasonable to believe that the risk stratification instrument of the future is likely to involve other components beyond clinical risk markers. Novel genomic variants show promise but are still in early phases of development as risk stratification targets. However, the “unbiased” approaches made possible through genomewide association studies have led to the recent identification of specific genetic variants that could predict SCD.6,7 This has reignited the interest in plasma biomarkers as a means for risk stratification. This article will review the emerging evidence and role of plasma biomarkers in predicting SCD in the general population and suggest a blueprint for future studies of potential utility. ### Uniform Definition of SCD Given the complexity and dynamic nature of the condition, there has been some variability …

An Unusual Case of Suspected Microvascular Angina in a Newborn

Myocardial ischemia in pediatric population is uncommon and usually due to congenital heart disease or extracardiac conditions leading to poor coronary perfusion. A 6-day-old newborn presented with respiratory distress and signs of heart failure. ECG, echocardiography, and laboratory results were consistent with myocardial ischemia. Coronary angiography was performed to exclude anomalous origin of coronary arteries, showing normal coronary artery origin and course. Thrombophilia and extra-cardiac causes were ruled out. Clinical conditions improved with mechanical ventilation and diuretics, enzyme levels lowered, repolarisation and systolic function abnormalities regressed, but ischemic electrocardiographic and echocardiographic signs still presented during intense crying. Becaues of suspicion of microvascular angina, therapy with ASA and beta-blocker was started. At 5 month followup, the baby was in good clinical condition and no more episodes were recorded. We believe it is an interesting case, as no similar cases have been recorded till now.

Home-based telemonitoring of simple vital signs to reduce hospitalization in heart failure patients: real-world data from a community-based hospital

Whether home telemonitoring after acute episodes of heart failure (HF) may reduce de-novo cardiac decompensation is disputed. We tested home telemonitoring of blood pressure (BP), heart rate (HR), and blood oxygen saturation (SO2) to reduce rehospitalization in patients with recent admission for acute HF. METHODS; We screened patients hospitalized in Cardiology due to prominent cardiac cause of acute dyspnea, and pulmonary/peripheral congestion, and with one admission or more for similar symptoms/signs in the previous year. Patients with acute coronary syndrome, poor prognosis due to extracardiac causes, and reduced self-sufficiency and cognitive ability were excluded. Of the selected patients, 63% accepted and received a device for BP, HR and SO2 measurement connected to an analogical modem for data transmission to a hospital server. Patients were educated to measure vital signs 3 times/week. A dedicated doctor-nurse unit monitored the patients' data twice weekly to manage therapeutic adjustments of diuretic dosage or in-hospital visits if necessary. HF treatment was standardized based on current guidelines. Unplanned hospitalizations for HF or all-cause death were primary endpoints; unplanned hospitalizations (total) for any cause, and all-cause death were the composite endpoints. Twenty-three patients (mean age 70 years, range 44-80 years) were recruited: 26% were women, 61% had coronary heart disease, 52% chronic lung disease, 57% renal insufficiency, 30% anemia; 17% had moderate or severe mitral regurgitation. At a mean follow-up of 302 days (range 55-622 days), 12 patients experienced the composite endpoints (52%, p=0.1), with the primary endpoint occurring in 8 patients (35%, 1 sudden death, p=0.058), the secondary endpoints occurring in 2 patients, and hospitalization not for HF occurring in 2 patients. The total number of hospitalizations/patient/year decreased from 2.2 ± 1.3 in the previous year to 0.9 ± 1.2 during the study period (p<0.01). On average, systolic BP tended to decrease, but BP, HR and SO 2 values prior to the index event (1-7 days) did not significantly differ from those recorded at the beginning of telemonitoring. In HF, home telemonitoring of simple variables had no significant impact on all-cause hospitalization/mortality, but was associated with a higher patient compliance and achievement of therapeutic targets, which may translate into a reduction in hospitalization rates for HF.

Postcardiac arrest syndrome: from immediate resuscitation to long-term outcome

The prognosis for postcardiac arrest patients remains very bleak, not only because of anoxic-ischemic neurological damage, but also because of the "postcardiac arrest syndrome," a phenomenon often severe enough to cause death before any neurological evaluation. This syndrome includes all clinical and biological manifestations related to the phenomenon of global ischemia-reperfusion triggered by cardiac arrest and return of spontaneous circulation. The main component of the postcardiac arrest syndrome is an early but severe cardiocirculatory dysfunction that may lead to multiple organ failure and death.Cardiovascular support relies on conventional medical and mechanical treatment of circulatory failure. Hemodynamic stabilization is a major objective to limit secondary brain insult. When the cause of cardiac arrest is related to myocardial infarction, percutaneous coronary revascularization is associated with improved prognosis; early angiographic exploration should then be discussed when there is no obvious extracardiac cause. Therapeutic hypothermia is now the cornerstone of postanoxic cerebral protection. Its widespread use is clearly recommended, with a favorable risk-benefit ratio in selected population. Neuroprotection also is based on the prevention of secondary cerebral damages, pending the results of ongoing therapeutic evaluations regarding the potential efficiency of new therapeutic drugs.

Efficiency of Renal Replacement Therapy for Cardiogenic Shock Complicated by Multiorgan Dysfunction

Objective: to analyze the efficiency of renal replacement therapy (RRT) for acute myocardial infarction complicated by cardiogenic shock and multiorgan dysfunction. Subjects and methods. Sixty-six patients with acute myocardial infarction complicated by cardiogenic shock were enrolled in the study. The postoperative period after coronary angioplasty was complicated by multiorgan dysfunction in all the patients. Results. The efficiency of postoperative RRT in abolishing systemic inflammatory response was evaluated. Conclusion. RRT incorporated within the first 12 hours following coronary angioplasty into an intensive care package for acute myocardial infarction complicated by cardiogenic shock has an effect in preventing multiorgan dysfunction and assists in reducing deaths due to extracardiac causes inflammatory response. Key words: acute myocardial infarction, cardiogenic shock, multiorgan dysfunction, renal replacement therapy.

Epicardial Ablation for Ventricular Tachycardia

Background— The purpose of this study was to describe the epicardial percutaneous ablation experience of 6 European high-volume ventricular tachycardia (VT) ablation centers. Methods and Results— Data from 218 patients with coronary artery disease (CAD, n=85 [39.0%]), idiopathic dilated of patients with idiopathic VT cardiomyopathy (IDCM, n=67 [30.7%]), arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARCD/C, n=13 [6%]), hypertrophic cardiomyopathy (HCM, n=5 [2.3%]), and absence of structural heart disease (n=48 [22%]) undergoing epicardial subxyphoid access for VT ablation were collected. The epicardial approach was attempted as first-line treatment in 78 patients (35.8%). Acute prevention of VT inducibility was obtained in 156 patients (71.6%). There were no procedure-related deaths. Cardiac tamponade occurred in 8 patients, and abdominal hemorrhage in 1 patient. Six patients died of electrical storm recurrence within 48 hours from the procedure. After a mean follow-up of 17.3±18.2 months, 60 patients (31.4%) presented with VT recurrence (39.3% of IDCM patients; 34.7% of CAD patients; 30.8% of ARVD/C patients; 25% of HCM patients; 17.1% of patients with idiopathic VT). Twenty patients (10.4%) died during follow-up (12 of heart failure, 2 of cardiac arrest, and 6 of extracardiac causes). Conclusions— In experienced centers, epicardial ablation of VT has an acceptable risk and favorable outcome. In selected patients, it is reasonable to consider as a first-line ablation approach.

Early Outcomes of Primary Sutureless Repair of the Pulmonary Veins

The "sutureless" repair technique has improved outcomes for post-repair pulmonary vein (PV) stenosis. The purpose of this study is to determine the early outcomes of primary sutureless repair of pulmonary venoocclusive disease in infants with congenital PV stenosis-hypoplasia or PVs at high risk for progressive stenosis. This is a retrospective review of infants who had primary sutureless repair of the PVs from October 2002 to April 2010. Twenty-five infants had primary sutureless repair of the PVs. Eighteen infants had total anomalous pulmonary venous return; 14 with obstruction, 10 with heterotaxy syndrome, and 9 with univentricular anatomy. Seven infants had congenital PV stenosis. There were 24 perioperative survivors (96%; 95% confidence interval [CI], 75% to 99%) and 2 late deaths from extracardiac causes. Follow-up was available on 21 out of 22 survivors at a median duration of 34 months (range, 9 to 100 months). Persistence-recurrence of PV stenosis occurred in 3 veins (3%) of 2 infants (8%). On follow-up echocardiography, right ventricular systolic pressure was normal in 13 out of 14 infants with a biventricular heart and 60% of systemic blood pressure in 1 infant. Kaplan-Meier 1-year cumulative survival was 88% (95% CI, 66% to 96%). Kaplan-Meier cumulative disease-free survival was 96% (95% CI, 75% to 99%) at 30 days and 84% (95% CI, 58% to 95%) at 1 year. By Cox proportional hazards, age, univentricular anatomy, and atrial isomerism-heterotaxy syndrome were not associated with an increased risk of death or persistence-recurrence. One-year disease-free survival was lower in infants with prematurity (p=0.0055) and low birth weight (p=0.0011). Primary sutureless repair is a feasible, safe, and relatively effective method of addressing congenital PV stenosis and (or) high-risk PVs, particularly in infants with single ventricle anatomy and (or) heterotaxy syndrome.

040 Non ST-elevation myocardial infarction with a normal coronary angiogram in women: is it serious?

The incidence and long term prognosis of women presenting troponin positive NSTE-ACS and a normal angiogram is unknown. To investigate the incidence, characteristics and long term prognosis of women presenting with a NSTEMI and normal coronary angiogram compared to women with NSTEMI and significant coronary artery disease (CAD) on the angiogram. Between 1999 and 2008, we screened patients presenting with an acute coronary syndrome (ACS) to identify those with a positive troponin and a normal angiogram. Extra-cardiac causes of troponin elevation were excluded. Women were matched by age and presence or absence of ST-elevation with a 1:2 ratio, to women with significant CAD. We compared characteristics and outcomes of the two groups of women. Over 4085 ACS patients, 1581 had a significant elevation of troponin, with 343 women (21.7%) and 1238 men (78.3%). One hundred and twelve patients (7.1%) had a normal angiogram with a 3-fold higher proportion of women (14.5%; n = 50) compared to men (5.0%, n = 52); p<0.001. When these women with a normal angiogram were compared with 100 women with significant CAD, they were less likely to have a high peak of troponin (> 5 times the upper limit of normal: 46% vs. 80%, p<0.0001). No other significant difference was found between the two groups. Mean follow-up was 4.3 +/− 3.1 years. Women with a normal angiogram had an excellent survival rate of 91.3% compared to 71.2% in women with CAD (p = 0.03), they had also a lower readmission rate for cardiovascular reason, (14% vs. 54%, p = 0.01). Women presenting with NSTEMI who have a normal angiogram, do not differ from women with NSTEMI and angiographic CAD. However, they have a smaller rise of troponin and a much better long-term prognosis.

Long-term follow-up of pseudoinfarction pattern in two children

Pseudoinfarction is defined as the presence of abnormal Q-waves with or without ST-segment changes which are not associated with myocardial ischemia. In adults, different causes of pseudoinfarction pattern have been described in cardiac diseases [1] associated with cardiomyopathy [2], hyperkalemia [3] or Wolf–Parkinson–White syndrome [4], and extra cardiac causes such as acute pancreatitis [5]. There is only one report about pseudoinfarction in infancy with symptomatic congestive heart failure, which was caused by the presence of an accessory atrioventricular conduction pathway and most likely intermittent supraventricular tachycardia [6]. We report about two children who were admitted in the outpatient clinic to clarify the heart murmur and found to have ECG abnormalities without any signs of clinical impairments. First patient was a 5-day-old female infant with a small membranous ventricular septal defect (VSD). Her ECG showed sinus rhythm, right axis deviation with deep Q-waves (up to -0.5 mV) in leads II, III, aVF and in pre cordial leads V4–V6. In addition, there was a discrete ST-elevation up to 0.2 mV in leads II and aVF. At the age of 6 weeks and 3.5 months, we observed left axis deviation with deepening of above mentioned Q-wave in ECG (Fig. 1a). Her clinical status was totally normal. The echocardiography confirmed normal size and function of the heart with hemodynamically not relevant left-to-rightshunt through the small membranous VSD. The second patient was a 2.5-year-old boy also with a small membranous VSD. Although in his first years of live, the ECG’s showed normal conduction, the ECG abnormalities were observed at the age of 2 years with extreme left axis deviation and deep Q-waves (up to -0.7 mV) in leads I, aVF and pre cordial leads V4–V6 without significant ST-elevation (Fig. 1b). His clinical status showed no impairments. Physical ability was not limited and there were no episodes of chest pain or dyspnoea in the past medical history. The echocardiography confirmed also in this case normal size and function of the heart with hemodynamically not relevant left-to-right-shunt through the small membranous VSD. The blood work, including CK and CK-MB was within normal range for both patients. The echocardiogram was not thought to have completely ruled out the possibility of a coronary artery abnormality, and angiographic visualization of the coronary arteries was considered appropriate to rule out anomalous left coronary artery from the pulmonary trunk (Bland–white–Garland syndrome). In both cases, the angiography showed a normal anatomy of the heart, great vessels and the coronary arteries. The hemodynamic evaluation was also entirely normal. The already by echocardiography described small membranous VSD was confirmed in both patients. A continuous follow-up investigation revealed in first patient (latest 6 years after initial investigation) a normal ECG (Fig. 1c) with spontaneous closure of VSD and in A. Wessel K. Norozi Department of Paediatric Cardiology, Medical School Hannover, Hannover, Germany

Fortuitous Discovery of Partial Uhl Anomaly in a Male Adult

We report the case of a 44-year-old man referred to our institution for suspicion of arrhythmogenic right ventricular (RV) cardiomyopathy. Both familial and personal medical histories were unremarkable, except the notion of a slight unexplored heart murmur during childhood. He decided to undergo a “40s” medical checkup. On presentation to the general practitioner, he had described a slight fatigue for 8 months without concomitant stress. Physical examination revealed no abnormality. ECG showed a type I atrioventricular block and a complete right bundle-branch block (Figure 1A). Transthoracic echocardiography showed an unusual left ventricle (LV) with a leftward curveted interventricular septum (IVS) and preserved global and segmental LV systolic function, suggestive of high systolic RV pressure or takotsubo cardiomyopathy (Figure 1B and Movie I in the online-only Data Supplement). However further analysis showed normal systolic pulmonary artery pressure and LV filling pressures. The apical 4-chamber view showed an abnormal RV with an aneurysm-like shape of the basal lateral free wall (Figure 1B and Movie II in the online-only Data Supplement) but with normal kinetics. A first cardiac magnetic resonance image showed biventricular abnormalities, particularly focused on RV wall motion, which justified additional morphological investigations. No abnormal pulmonary venous return was noticed. Invasive coronary angiogram was normal. The patient was later referred to our unit by his attending cardiologist and underwent invasive hemodynamics and biventricular contrast angiography. Cardiac index (pulmonary artery thermodilution) and biventricular volumes were normal. Blood gas content analysis disclosed an intracardiac shunt. RV angiography (Figure 2A and 2B) showed a truncated RV apex and the absence of trabeculations in the anteroapical and inferoapical zones, therefore characterized by a smooth …

Immediate Percutaneous Coronary Intervention Is Associated With Better Survival After Out-of-Hospital Cardiac Arrest

Background— Acute coronary occlusion is the leading cause of cardiac arrest. Because of limited data, the indications and timing of coronary angiography and angioplasty in patients with out-of-hospital cardiac arrest are controversial. Using data from the Parisian Region Out of hospital Cardiac ArresT prospective registry, we performed an analysis to assess the effect of an invasive strategy on hospital survival. Methods and Results— Between January 2003 and December 2008, 714 patients with out-of-hospital cardiac arrest were referred to a tertiary center in Paris, France. In 435 patients with no obvious extracardiac cause of arrest, an immediate coronary angiogram was performed at admission followed, if indicated, by coronary angioplasty. At least 1 significant coronary artery lesion was found in 304 (70%) patients, in 128 (96%) of 134 patients with ST-segment elevation on the ECG performed after the return of spontaneous circulation, and in 176 (58%) of 301 patients without ST-segment elevation. The hospital survival rate was 40%. Multivariable analysis showed successful coronary angioplasty to be an independent predictive factor of survival, regardless of the postresuscitation ECG pattern (odds ratio, 2.06; 95% CI, 1.16 to 3.66). Conclusions— Successful immediate coronary angioplasty is associated with improved hospital survival in patients with or without ST-segment elevation. Therefore, our findings support the use of immediate coronary angiography in patients with out-of-hospital cardiac arrest with no obvious noncardiac cause of arrest regardless of the ECG pattern.

New developments in atrial antiarrhythmic drug therapy

Atrial fibrillation (AF) is a growing clinical problem associated with increased morbidity and mortality. Currently available antiarrhythmic drugs (AADs), although highly effective in acute cardioversion of paroxysmal AF, are generally only moderately successful in long-term maintenance of sinus rhythm. The use of AADs is often associated with an increased risk of ventricular proarrhythmia, extracardiac toxicity, and exacerbation of concomitant diseases such as heart failure. AF is commonly associated with intracardiac and extracardiac disease, which can modulate the efficacy and safety of AAD therapy. In light of the multifactorial intracardiac and extracardiac causes of AF generation, current development of anti-AF agents is focused on modulation of ion channel activity as well as on upstream therapies that reduce structural substrates. The available data indicate that multiple ion channel blockers exhibiting potent inhibition of peak I(Na) with relatively rapid unbinding kinetics, as well as inhibition of late I(Na) and I(Kr), may be preferable for the management of AF when considering both safety and efficacy.

Esophageal Foreign Body Masquerading as Myocardial Infarction with Atrioventricular Block

To the Editor: Symptoms after esophageal foreign body impaction vary from dysphagia to retrosternal discomfort. We describe an unusual, hitherto unreported presentation of an esophageal impaction. A 25-yr-old man was scheduled for urgent esophagoscopy after esophageal impaction from a meat bolus. Complaints included dysphagia, odynophagia, vomiting, and a foreign body sensation along with retrosternal discomfort 2 days after ingestion of meat. The discomfort in the retrosternal region was dull and aching in nature, nonradiating, increased on swallowing, had not increased in severity since its onset, and was not associated with any palpitations, syncope, breathlessness, or dysphoria. The findings from physical examination and laboratory investigations were unremarkable except for partial collapse of the left lung, along with evidence of pulmonary tuberculosis on the chest radiograph. A 12-lead electrocardiogram (ECG) showed evidence of myocardial infarction in the lateral wall (ST elevation in lead I/aVL with reciprocal changes in inferior leads), T-wave inversion in leads V1–V4, and a recent onset 2:1 atrioventricular (AV) block (Fig. 1). The acquired nature of the AV block and its recent onset were obvious from its presence in precordial leads and concomitant absence in limb leads. Because the limb leads are recorded first during a 12-lead ECG, followed by the precordial leads, this ECG captured the very moment of the onset of AV block (Fig. 1). Considering the possibility of the meat bolus leading to cardiac compression and thus inducing ECG changes, an urgent computed tomography scan was advised. Simultaneously, troponin levels were measured. The computed tomography scan showed the meat bolus to be impacted intraluminally at the midesophageal level (T5-7), with a maximum transverse diameter of 1.9 cm, and was compressing the left atrium and AV sulcus posteriorly (Fig. 2). Meanwhile, the troponin concentration was normal (0.05 μg/L). Esophagoscopy was conducted uneventfully under general anesthesia, with resolution of all ECG changes in the immediate postoperative period. Normal cardiac troponin levels and resolution of ECG changes after removal of the meat bolus suggested mass effect on the heart by the meat bolus as the likely etiology of the cardiac alterations.Figure 1.: Perioperative 12-lead electrocardiogram showing ST elevation in lead I/aVL with reciprocal changes in inferior leads, T-wave inversion in leads V1–V4, 2:1 atrioventricular block in precordial leads, and right axis deviation.Figure 2.: Computed tomography scan showing left atrial and atrioventricular sulcus compression.Previous reports of reversible ECG changes resembling those of myocardial ischemia or infarction from cardiac compression by extracardiac causes include a gastric tube located retrosternally after esophageal reconstruction, causing marked reversible ST-T changes due to cardiac compression.1–4 An anterior mediastinal tumor has also been reported to cause reversible ST-T changes, which disappeared after tumor removal.5 In a slight variation of these case reports, the cardiac compression in our patient was caused by a pressure effect from the posterior rather than the anterior mediastinum. A previous case report of left atrial and ventricular compression from the posterior mediastinum followed pyloroplasty, wherein a dilated stomach conduit resulted in sensation of chest compression along with ST elevation in leads II, III, and avF.6 The symptoms and ST changes subsided after nasogastric suction and decrease in stomach conduit size. This case suggests that a large esophageal foreign body may alter the ECG simulating myocardial ischemia or infarction, whereas in reality reflecting a mass effect on the cardiac chambers. Asha Tyagi, MD, DNB, MNAMS Nitin Behl, DA Debyani Dey, MD Shuchi Bhatt, MD Ashok K. Sethi, DA, MD UCMS and GTB Hospital Delhi, India [email protected]

215 Evaluation of Syncope in Brugada Syndrome: The Effect on Outcome

The Effect on Outcome Risk stratification in pts with Brugada syndrome (BS) is difficult. The report from the 2 nd Consensus Committee on BS suggests that all pts with syncope without a “clear extracardiac cause” should have an implantable cardioverter-defibrillator (ICD). However a clear cause for syncope may be difficult to prove. All pts diagnosed with BS at Bordeaux CHU between 1999 and 2009 have been enrolled in a prospective registry. All patients with syncope were enrolled in the present study (Group 1 without and Group 2 with a “clear extracardiac cause” of syncope). Patients were followed in the clinic or by phone annually unless there was an event. Of 185 pts with BS, 49 (39 male, 47 ±12yo) had at least one syncope. All 27 pts in group 1 received an ICD. Of the 22 pts in group 2, 4 received an implantable loop recorder [MJW1] (ILR) and 1 patient received an ICD after positive EP study. With a mean follow-up of 53 ±38 months, 19 pts were asymptomatic, 5 had appropriate ICD therapy, 2 patients had further syncopal events not associated with cardiac arrhythmia and 1 pt died of a non cardiac cause in group 1. In group 2, 14pts were asymptomatic, 5 had recurrent atypical syncopal events (without arrhythmia shown by ILR in 3 pts), 2 experienced atypical chest pain and 1 died of drug abuse. Diagnosis of syncope is difficult, and 7% of BS pts presenting with syncope without a clear cardiac cause will have further events without cardiac arrhythmia. Better evaluation of syncope is required to spare pts unnecessary ICDs. group 1 27 patients syncope without a clear extracardiac cause group 2 22 patients syncope with a clear extracardiac cause p gender (male) 21 (80.8%) 18 (78.3%) 0.83 age (years old) 48 (+/- 12.2) 45 (+/- 11) 0.39 follow-up (months) 53 (+/- 36.1) 52 (+/- 42.3) 0.90 spontaneous type 1 ECG 21 (80.8%) 13 (56.5%) 0.07 EP Study: – positive 8 (29.7%) 1 (4.5%) 0.06 – negative 13 (48%) 17 (77.3%) – not done 6 (22.3%) 4 (18.2%) circunstances of diagnosis: – unexpected finding 4 (14.8%) 7 (31.8%) 0.43 – familial assessment 2 (7.4%) 2 (9.1%) – syncope 21 (77.8%) 13 (59.1%)

Should embolectomy be performed in late acute lower extremity arterial occlusions?

We analyzed the embolectomy results and complications of patients who were operated on after a diagnosis of late acute arterial occlusion of lower extremities. A total of 122 patients operated on in our clinic between 2004 and 2009 for late acute arterial occlusion were included in the study. Late arterial occlusion was defined as occlusion occuring 72 hours after initial manifestation of the patient complaints related to the affected lower extremity. Average age of the 122 patients (71 male, 51 female) was 54.2 +/- 16.8 years. In this cohort, 64.75% of patients had cardiac pathologies, while 28.68% had extracardiac causes; 1.64% patients had cathetherization, 0.81% patient had malignancy, and 2.46% patients had a history of trauma. In 1.64% of the cases, no reason for thromboembolysis could be found. Thirty-one patients (25.40%) had additional surgical operations, 14 (11.47%) had fasciotomy, and 9 (7.37%) had amputation. Re-embolectomy was performed on 37 patients (30.32%) who had ongoing ischemia after an operation. Additional surgical operations were performed on 31 patients (25.40%) with ongoing ischemia. In 14 of these cases (11.47%), patients were treated with fasciotomy due to development of compartment syndrome. Amputation was performed on a total of 9 patients. Early in the postoperative phase, mortality was observed in 11 patients (9.01%). We believe that late embolectomies of acute late leg ischemia increases blood flow in the extremity and reduces the number of amputations required.

Abstract 5934: Arrhythmogenic Right Ventricular Cardiomyopathy Due to Desmosomal Gene Mutations Is a Progressive Biventricular Disease

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by a gradual loss of myocytes with fibro-fatty replacement. Mutations of gene encoding desmosomal proteins have been demonstrated in up to 50% of probands. The aim of this study was to assess the morphologic spectrum and the relative role of viruses on genotyped ARVC hearts. Design: Fourteen ARVC hearts (10 M/4 F, age range 7–64, mean 30 yrs), coming from sudden death (8), cardiac transplantation (4), heart failure (1) and extracardiac death (1) were investigated. Familial recurrence of ARVC was ascertained in 12 (86%). Genetic screening identified pathogenetic mutations in plakophilin-2 (5), desmoplakin (4), desmoglein-2 (2) and plakoglobin (3). Five hearts from patients with in ryanodine receptor 2 (Ryr2) gene mutations were also investigated. Morphopathologic investigation consisted of gross examination, histology, immunohistochemistry and molecular pathology (PCR) for cardiotropic viruses. Result: Cardiac involvement was biventricular in all but 1 case, a 7 year old girl who died of extracardiac causes and presented a structurally normal heart. Ventricular myocardium abnormalities consisted of fibro-fatty replacement in 12 cases and of subepicardial myocyte necrosis with acute inflammation and granulation tissue in a 15 year old boy who died suddenly. Inflammatory infiltrates were evident in 93% of cases, either diffuse (23%) or focal (77%), and mostly consisted of T-lymphocytes and macrophages. Molecular investigation was negative in all but one with Hepatitis C virus (7%). Ryr2 gene hearts showed only mild fatty infiltration confined to the antero-apical wall of the right ventricle. Conclusion: ARVC hearts from patients with desmosomal gene mutations are characterized by biventricular myocardial atrophy and fibro-fatty replacement. In early adolescence, the phenotype can be either absent or consistent with acute myocyte damage without fibro-fatty replacement, thus confirming the progressive nature of the disease. While myocarditis is a usual feature, viral genome is exceptionally detected in the myocardium as to support the reactive nature of inflammation. The pathologic features of Ryr2 hearts are not in keeping with ARVC.

Silent myocardial infarction

By autopsies of patients expired from different diseases not rarely chronic myocardial infarction is found, that was known before neither to patient nor to medical attendant (silent myocardial infarction) and is interpreted as incidental finding. Study of frequency, role in expectation of life, diagnosis and prognosis of silent myocardial infarction in relation to localisation. Retrospective study and statistical analysis of 1568 autopsies performed during 10 years. Acute or chronic myocardial infarction was found in 470 cases (30%), acute infarction in 177 cases (37%), 90% of which was diagnosed in vivo and patients died of infarction and its direct complications. In 293 cases (63%) chronic myocardial infarction was found, 109 cases (37%) were known and 184 cases (63%) were silent myocardial necrosis, the ratio of female/male patients was nearly the same (90/94 persons). 97 patients (32%) with chronic myocardial infarction died of cardiac cause - mostly in cardiac failure -, 196 (68%) of extracardiac cause, most of them of stroke, predominantly the patients with inferior infarction. Considering the silent causes, the myocardial infarction is more frequent and has better prognosis, than it is known from epidemiological data without autopsies, because 42% of these patients dies of extracardiac diseases, and the continuity of life is not shorter, than by patients without myocardial infarction. Knowledge of silent infarction gives possibility to estimate the physical charge of patients, their treatment and to prevent recurrence.

Positive troponin-T in noncompaction is associated with neuromuscular disorders and poor outcome

Though cardiac troponin-T may be positive in hypertrophic and dilated cardiomyopathy, it is not known how often troponin-T is positive in left ventricular hypertrabeculation/ noncompaction (LVHT), an unclassified cardiomyopathy. This retrospective study aimed to assess how often troponin-T is positive in LVHT, is associated with elevated CK, is attributable to cardiac or extra-cardiac causes, in particular neuromuscular disorders (NMDs), or if it is a predictor of poor survival. Among 100 patients, detected over a period of 11 years, troponin-T was determined at least once in 71 (71%) of them. Troponin-T was determined once in 36 patients, twice in 8 cases, three times in 11 patients, and more than three times in 16 cases. Troponin- T was positive at least once in 12 patients (17%). Forty-five of the 71 patients suffered from a NMD (63%). Troponin-T positivity was associated with elevated CK in 6 cases. Troponin-T-positivity was attributable to acute myocardial ischemia in a single case, to chronic renal failure in 5 cases, to dilated cardiomyopathy in 4 cases, to atrial fibrillation in 3 cases, to heart failure in 4 cases, and to NMD in 10 cases. Troponin-T positivity in LVHT patients with NMD was assumed to be due to cardiac involvement in the disease. Among the 22 patients who died during the observational period troponin was determined in 16 and was positive in 4 (25%). Troponin-T is positive in 17% of the patients with LVHT. Most of these patients suffer from a NMD. Troponin-T positivity in LVHT predicts the presence of NMD and poor survival.

Impairment of mitochondrial respiratory chain enzyme activities in tetralogy of fallot

BackgroundDuring the last decade, disorders of the respiratory chain, so-called mitochondrial disorders, have emerged as a major clinical entity. Tetralogy of fallot (TOF) children >2 month of age are at risk for postoperative myocardial contractile failure. Myocardial ischemia is associated with a reduction in mitochondrial enzyme activity and have impaired metabolism resulting in decreased postoperative myocardial adenosine triphosphate (ATP) concentrations and increased lactate levels. With this in view, we measured the mitochondrial energy system (respiration and OXPHOS) and to study morphological changes from the right ventricular outflow tract (RVOT) muscle of patients with TOF. Methods30 infants with TOF were studied with age-matched control group consisted of 12 normal patients who died due to extracardiac causes. Mitochondrial respiratory chain complexes, OXPHOS, cytochrome content and ATPase activity were measured by documented standard procedure. Morphological changes examined with a transmission electron microscope. ResultsIn the presence of glutamate and succinate as substrates, the rate of mitochondrial oxygen consumption was significantly lower in RVOT muscles (p<0.001) by using with and without addition of ADP. The ADP/O ratio indices for glutamate and succinate were not significantly affected. The activities of rotenone-sensitive NADH cytochrome c reductase (complexes I+III), cytochrome c oxidase (complex IV) and the ratio of I and III to II and III complexes (complex I) were significantly lower in TOF (p<0.001). A significant reduction of total cytochrome content and ATPase activity (p<0.001) was noted in study group. Morphological changes were also seen in study group as compared with control. ConclusionsOXPHOS, mitochondrial respiratory chain complex I, I+III and IV, cytochrome content and ATPase activity are more impaired in RVOT muscles in patients with TOF.

Behandlungspfad „Akutes Koronarsyndrom“

The clinical pathway "acute coronary syndrome" of the university hospital Marburg describes the guideline-conform and consented management of patients with ST-segment elevation infarct (STEMI), non-ST-segment elevation infarct (NSTEMI) and Troponin negative unstable angina. A 12-lead ECG recording is made and read in all patients within 10 minutes. All patients with STEMI undergo immediate revascularisation using primary percutanuous catheter intervention (PCI) after administration of basic medical therapy. Primary PCI is also used in all patients with NSTEMI, persistent chest pain, rhythm or hemodynamic instability. Patients with unstable angina, who became free of symptoms after application of basic medication, but who have additional risk factors undergo cardiac catheterisation within 48 hours. Acute myocardial infarction can be ruled out in patients with twofold negative cardiac troponin levels during 6-12 hours. In the absence of further symptoms, these patiens undergo differential diagnostic evaluation of cardiac and extracardiac causes of chest pain. The introduction of this clinical pathway 2 years ago, which was consented before by the hospital board and the clinical directors, has lead to a remarkable improvement in the clinical decision-making at the emergency room of the hospital and reduced the door to intervention time considerably.