Rare survival of left ventricular free wall rupture following myocardial infarction.

Myocardial free wall rupture is a rare but usually fatal complication of acute myocardial infarction (MI) especially if it occurs out of hospital and occurs in 2-4% of patients who suffer from acute MI. Rapid diagnosis is essential but not always easy as diagnostic tests may be inconclusive. In this case report authors examine a rare and unique patient survival after left ventricular free wall rupture following MI. The patient developed chest pain and hypotension in the hospital and was taken directly to the catheterization laboratory where a diagnostic angiogram showed a high-grade occlusion of a very small marginal branch, fluoroscopy demonstrated a large pericardial effusion, which was drained then auto transfused back to the patient using a femoral vein sheath. Rapid diagnostic testing including transesophageal echocardiography with Definity, transthoracic echocardiography, aortography and left ventriculography were all negative for dissection and rupture. Despite the negative diagnostic test, a high index of suspicion for rupture led to urgent surgical exploration where a large 4-cm hole was found in the lateral wall. Repair was successful and the patient left the hospital about several weeks later.

QT Adaptation and Intrinsic QT Variability in Congenital Long QT Syndrome.

BACKGROUND: Increased variability of QT interval (QTV) has been linked to arrhythmias in animal experiments and multiple clinical situations. Congenital long QT syndrome (LQTS), a pure repolarization disease, may provide important information on the relationship between delayed repolarization and QTV. METHODS AND RESULTS: Twenty-four-hour Holter monitor tracings from 78 genotyped congenital LQTS patients (52 females; 51 LQT1, 23 LQT2, 2 LQT5, 2 JLN, 27 symptomatic; age, 35.2±12.3 years) were evaluated with computer-assisted annotation of RR and QT intervals. Several models of RR-QT relationship were tested in all patients. A model assuming exponential decrease of past RR interval contributions to QT duration with 60-second time constant provided the best data fit. This model was used to calculate QTc and residual "intrinsic" QTV, which cannot be explained by heart rate change. The intrinsic QTV was higher in patients with long QTc (r=0.68; P<10(-4)), and in LQT2 than in LQT1/5 patients (5.65±1.28 vs 4.46±0.82; P<0.0002). Both QTc and intrinsic QTV were similar in symptomatic and asymptomatic patients (467±52 vs 459±53 ms and 5.10±1.19 vs 4.74±1.09, respectively). CONCLUSIONS: In LQTS patients, QT interval adaptation to heart rate changes occurs with time constant ≈60 seconds, similar to results reported in control subjects. Intrinsic QTV correlates with the degree of repolarization delay and might reflect action potential instability observed in animal models of LQTS.

A Novel Solution for the High Defibrillation Threshold in Patients with a DF-4 Lead: Adding a High Voltage Adaptor/Splitter.

A high defibrillation threshold occurs in approximately 6% of implants. The defibrillation threshold can be improved by addition of a defibrillation lead. However, the DF-4 high energy ICD header precludes the addition of a defibrillation lead. Here we report on use of a new high voltage adaptor/splitter that enables the addition of an extra defibrillation lead.

Left Atrial Diastolic Dysfunction And Pulmonary Venous Hypertension In Atrial Fibrillation: Clinical, Hemodynamic And Echocardiographic Characteristics.

BACKGROUND: Left ventricular diastolic dysfunction has been well described; diastolic abnormalities of the LA are less frequently recognized and poorly understood. OBJECTIVE: The purpose of this study was to investigate the clinical, hemodynamic and echocardiographic features of left atrial (LA) diastolic dysfunction. METHODS: Patients with atrial fibrillation (AF), severe LA enlargement, and pulmonary venous hypertension (PVH, Group 1) were compared to patients with pulmonary arterial hypertension (PAH), normal LA size and sinus rhythm (Group 2). All underwent right heart catheterization and transthoracic echo to evaluate hemodynamics and LA function. Mitral regurgitation was evaluated by transesophageal echocardiography. LA diastolic function was measured by comparing filling fraction, pulmonary venous flow and compliance. RESULTS: Right atrial, pulmonary artery systolic and mean pressures were similar. Mean wedge pressure were increased in Group 1, 20.8±2.6 versus 9.7±2.8 mm of Hg (p<0.0001). The most striking hemodynamic difference was large V wave in Group 1 without significant mitral regurgitation. LA filling fraction was abnormal in Group 1, 11.4%±8.5 compared to Group 2, 111.5%±44 (p<0.0001). LA compliance was 0.39±0.27 ml/m2/mmHg in Group 1 versus 6.8±4.54 ml/m2/mmHg in Group 2 (p=0.001). There was a strong negative correlation between the V wave and LA filling fraction (r= 0.756, p<0.001). The ratio of the height of the transmitral E wave divided by the S/D ratio (the LA diastolic dysfunction index) correlated very strongly with the V wave (r=0.907, p<0.001). CONCLUSION: LA diastolic dysfunction is present in some patients with long standing AF and PVH. LA diastolic dysfunction, in addition to left ventricular diastolic dysfunction, may contribute to the syndrome of heart failure with preserved left ventricular systolic function.

Radiofrequency ablation of post-incisional atrial flutter and high-output heart failure in a patient with interrupted inferior vena cava and hereditary hemorrhagic telangiectasia.

A 61-year-old female with a history of secundum atrial septal defect repair and hereditary hemorrhagic telangiectasia presented with epistaxis. She was found to have atypical atrial flutter with 2:1 atrioventricular conduction. Radiofrequency ablation was planned, but inferior vena cava interruption precluded right atrial (RA) access. The RA was then accessed through both subclavian veins, and activation mapping revealed a dense atriotomy scar in the posterolateral inferior RA. Wavefront propagation proceeded caudally through an area of slow conduction confined by the atriotomy scar. Atypical atrial flutter terminated during a second radiofrequency application to an isthmus confined by 2 regions of dense scar. The arrhythmia did not recur, although the patient later experienced typical atrial flutter and atrial fibrillation. High-output heart failure due to systemic arteriovenous shunt was confirmed by cardiac catheterization and improved markedly with bevacizumab therapy.

An Unusual Etiology for Elevation of Activated Partial Thromboplastin Time (aPTT) in SLE: Acquired Hemophilia and Lupus Anticoagulant.

A 60-year-old female who has a history significant for diabetes, depression, and rheumatoid arthritis presented with a progressively enlarging hematoma of the left upper extremity. She was found to have an enlarging hematoma and an isolated elevation of activated partial thromboplastin time (aPTT). Lab work-up revealed low factor VIII activity levels and inhibitor titers at 13.38 Bethesda units (BU). Dilute Russell's viper venom time (dRVVT) revealed a lupus anticoagulant. Hemostasis was achieved with factor VIII inhibitor bypassing activity (FEIBA) and inhibitor eradication with-rituxan after the failure of first-line treatment with cyclophosphamide and prednisone.

Left ventricular myxoma: Missed vs metastatic.

Left ventricular myxomas account for 2.5% of all cardiac myxoma cases. There are very few case reports on left ventricular myxoma (LVM) presented after complete surgical resection of left atrial myxoma. Here we report a case of a 58-year-old male presented to the hospital for transient limb weakness, numbness and dysarthria. Magnetic resonance image of the brain revealed multiple thromboembolic cerebrovascular accidents. Transthoracic echocardiogram (TTE) revealed a left atrial myxoma. It was resected completely with good surgical margins. After one and half year he started having dizziness, and transient right sided weakness. Computer tomography scan of the head revealed a progression of thromboembolic disease. TTE revealed a LVM that was confirmed by transesophageal echocardiogram. It was resected with good surgical margins 3 wk after recurrent cerebrovascular accident.

Postpartum acquired hemophilia: a rare cause of postpartum hemorrhage.

A 36-year-old female started having postpartum vaginal bleeding after normal vaginal delivery. She underwent hysterectomy for persistent bleeding and was referred to our institution. An elevation of PTT and normal PT made us suspect postpartum acquired hemophilia (PAH), and it was confirmed by low factor VIII activity levels and an elevated factor VIII inhibitor. Hemostasis was achieved with recombinant factor VII concentrates and desmopressin, and factor eradication was achieved with cytoxan, methylprednisolone, and plasmapheresis.

Acquired Factor VIII Inhibitor: A Rare Etiology for Bruisability and Anemia.

Acquired factor VIII inhibitor is a very rare cause of easy bruisability, muscular hematomas, and subsequent anemia in the elderly patient population. The incidence of acquired factor VIII inhibitor is one in one million. Here we report an elderly patient who presented to the hospital for bruising easily fatigue and was found to have acquired factor VIII inhibitor on further investigation. He responded to the treatment appropriately, and hemostasis was achieved successfully with recombinant VII concentrates and eradication of the inhibitor with Imuran and steroid combination.

Effect of ß-adrenergic stimulation on QT interval accommodation.

BACKGROUND: QT interval correction formulas are based on instantaneous heart rate (HR), but QT interval adaptation to sudden HR change occurs gradually. In humans, the QT interval has been reported to reach a new steady-state value in about 2 minutes. OBJECTIVE: This study sought to assess ß-adrenergic stimulation effects on QT interval response to HR change. METHODS: Ten subjects (42.1 ± 15.3 years, 3 men) undergoing radiofrequency ablation of supraventricular tachycardia were studied. Atrial pacing for 5 minutes at an HR ranging from 60 to 140 beats/min was performed before and during dobutamine infusion (10 µg/kg/min). The QT response to sudden HR change was evaluated. RESULTS: The QT response to sudden HR change consists of an immediate response (IR), followed by a gradual monoexponential course to the new steady-state value. Linear function results in inferior fit of QT adaptation course (P < .05 compared with exponential). The time constant of the exponential is approximately 1 minute (50.9 ± 11.4 seconds). The IR magnitude is approximately 3% of the RR interval change (2.97% ± 2.01%). Dobutamine shortens steady-state QT at given HR (from 301.8 ± 11.2 ms to 290.6 ± 13.2 ms at 120 beats/min; P < .001) and increases IR magnitude (to 13.28% ± 8.99% of RR change; P < .01). During sinus rhythm, QT variability and QT variability index were significantly increased by dobutamine. CONCLUSION: QT adaptation has 2 distinct phases, which might have different mechanisms. The effect of ß-adrenergic stimulation on IR may increase QT variability by increasing the immediate response to HR variability. These results may be important in the assessment of drug effects on repolarization and for understanding of QT variability.

Exercise-induced atrial and ventricular tachycardias in a patient with left ventricular noncompaction and normal ejection fraction.

We report a patient with ventricular and atrial tachycardias reproducibly induced during exercise testing. Atrial tachycardia, but no sustained ventricular tachycardia, was induced during electrophysiological study. Catecholaminergic polymorphic ventricular tachycardia was considered because of normal echocardiogram, family history of sudden death, and polymorphic appearance of some of the nonsustained ventricular tachycardia episodes. However, most episodes of ventricular tachycardia were monomorphic. Cardiac magnetic resonance diagnosed isolated left ventricular noncompaction.

Focal monomorphic ventricular tachycardia as the first manifestation of amyloid cardiomyopathy.

52-year-old patient presented with palpitation and well tolerated monomorphic ventricular tachycardia. He had normal echocardiogram and coronary angiogram 3 months prior to presentation. Surface EKG revealed regular wide-complex tachycardia with right bundle branch block morphology and right inferior axis. In conjunction with recent negative cardiac evaluation, this suggested idiopathic focal ventricular tachycardia from anterolateral basal left ventricle. CARTO based activation mapping confirmed the presence of VT focus in that area. Radiofrequency ablation at the site of perfect pacemap resulted in a partial suppression of the focus. Echocardiogram was subsequently performed because of progressive dyspnea. It revealed asymmetrical thickening of posterolateral left ventricle, with delayed enhancement on contrast magnetic resonance imaging. Fine needle aspiration of abdominal fat stained with Congo red confirmed the diagnosis of systemic AL amyloidosis due to IgG lambda-light chain deposition. Consequently, the patient underwent placement of implantable defibrillator and hematopoetic stem cell transplantation. He remains in excellent functional status 18 months after presentation.

Sexual function in women receiving maintenance dialysis.

While substantial attention has been paid to the issue of sexual dysfunction in men on chronic dialysis, less is known about this problem in women with end-stage renal disease. We sought to assess sexual dysfunction in women on chronic dialysis and determine whether patients discuss this problem with their providers and receive treatment. We prospectively enrolled women receiving chronic hemodialysis or peritoneal dialysis in Pittsburgh, PA. We asked patients to complete the 19-item Female Sexual Function Index (FSFI) to assess sexual function and a 5-item survey that assessed whether patients had discussed sexual dysfunction with their providers and/or received treatment for this problem in the past. We enrolled 66 patients; 59 (89%) on hemodialysis and 7 (11%) on peritoneal dialysis. All patients completed the FSFI, of whom 53 (80%) had FSFI scores <26.55, consistent with the presence of sexual dysfunction. Of 37 patients who were married or residing with a significant other, 27 (73%) had sexual dysfunction. Among 24 participants who reported having been sexually active over the previous 4 weeks, 11 (46%) had sexual dysfunction. Only 21% of patients with sexual dysfunction had discussed this problem with their gynecologist, renal or primary provider, and 3 (6%) reported having received treatment. Sexual dysfunction is common in women on dialysis, even among patients who are married or residing with a significant other and those who are sexually active. However, few women discuss this issue with their providers or receive treatment.