Hospitalization Rates, Prevalence of Cardiovascular Manifestations and Outcomes Associated With Amyloidosis in the United States.

Background Amyloidosis is a multisystem disease characterized by the deposition of misfolded insoluble precursor protein fibrils in several organs including the heart. Cardiac amyloidosis can result in a wide range of complications that may lead to significant morbidity and mortality. However, contemporary data in the United States (US) on cardiac amyloidosis is scarce despite these negative consequences. In view of this lack of contemporary data, we sort to assess the prevalence, trends of hospitalization, and outcomes of cardiovascular manifestations in amyloidosis. We also explored this retrospective data for factors that may be associated with in-hospital mortality of amyloidosis hospitalization. Methods We used the national (nationwide) inpatient sample (NIS) database from January 1, 2007 - December 31, 2014, of adult hospitalizations. We studied the prevalence and trends of hospitalizations of amyloidosis among patient with or without cardiovascular co-morbidities. Results We identified 137,797 amyloidosis hospitalizations from 2007 to 2014 of which 87,164 (63.2%) had cardiovascular manifestations. The overall mean age was 70.3±12 years. There were more males (54.5%) overall. The trend of amyloidosis hospitalizations increased significantly from 2007 through 2014 (34 to 73 per 100,000, Ptrend <0.001) and in-hospital mortality decreased from 8.4 to 6.8 per 100 amyloidosis hospitalizations, Ptrend <0.001). Conclusion Our study showed that hospitalizations of amyloidosis have increased considerably over the past decades with a concurrent decline in in-hospital mortality. Despite this decline and after adjusting for other factors, amyloidosis hospitalization with cardiovascular manifestations was still associated with higher in-hospital mortality. Screening of patients with amyloidosis for cardiovascular manifestations should be more accessible to prevent undesired outcomes.

Closure of a Prosthetic Mitral Valve Paravalvular Leak Using a Ventricular Septal Defect (VSD) Amplatzer Occluder Device.

BACKGROUND Paravalvular leaks (PVL) are becoming more commonly experienced in clinical practice due to the increasing number of mitral valve replacements performed. There are about 182 000 valve replacements performed annually, with a 5-15% prevalence rate of paravalvular leaks. Due to increased mortality associated with surgical repair, percutaneous transcatheter closure procedures are increasingly being performed as an alternative to repeat surgery. CASE REPORT We present the case of a 52-year-old woman with past medical history of mitral valve endocarditis who developed worsening acute heart failure 1 month after surgical bioprosthetic mitral valve replacement. Transesophageal echocardiography at the time revealed dehiscence of the bioprosthetic mitral valve and severe mitral regurgitation. She subsequently had emergent surgical bioprosthetic mitral valve replacement and annular reconstruction. The post-operative course was complicated by increasing dyspnea and lower-extremity edema, with recurrent pericardial tamponade requiring placement of a pericardial window. Based on her multiple comorbidities, most notably the concomitant right ventricular failure with severe pulmonary hypertension and prior pericardial patch repair with compromise of her mitral valve annulus, she was deemed inoperable for re-do surgery and eventually underwent a successful percutaneous closure of the mitral paravalvular leak with a ventricular septal defect (VSD) Amplatzer occluder device. The patient made good recovery and was discharged home a few days after the procedure. CONCLUSIONS Although use of the Amplatzer VSD occluder device for this indication currently remains off-label, our report supports the use of the VSD occluder device in this subset of patients considering the high mortality rates associated with repeat surgical procedure.

Sustained Ventricular Tachycardia as a Harbinger of Cardiac Amyloidosis.

BACKGROUND Cardiac amyloidosis is an infiltrative cardiomyopathy caused by the extracellular deposition of insoluble precursor protein amyloid fibrils. These depositions of protein amyloid fibrils are found on the atria and ventricles and can cause a wide array of arrhythmias; however, sustained ventricular arrhythmias are quite uncommon. CASE REPORT A 71-year-old man with a history of hypertension developed a sudden onset of shortness of breath, profuse diaphoresis, lightheadedness, and presyncope. Upon emergency medical services' arrival, an initial electrocardiogram revealed wide complex tachycardia with a heart rate of 220 to 230 beats per min. He was subsequently given, in succession, magnesium, adenosine, and amiodarone with no change in heart rate or rhythm. Due to ongoing symptoms of diaphoresis and the development of dyspnea, he underwent direct current cardioversion and was converted from ventricular tachycardia to atrial fibrillation at controlled rates. A transthoracic echocardiogram and cardiac magnetic resonance imaging showed features suspicious for cardiac amyloidosis. A subsequent 99m technetium pyrophosphate single-photon emission computerized tomography scan revealed a grade 3 visual uptake and a heart-to-contralateral lung ratio of 1.92, consistent with transthyretin amyloidosis. The patient was treated with tafamidis and an implantable cardioverter-defibrillator for secondary prevention of ventricular arrhythmia. CONCLUSIONS This case highlights the need to consider cardiac amyloidosis in the differential diagnoses of patients with persistent ventricular arrhythmia and no prior history of heart disease.

Spontaneous Coronary Artery Dissection Treated with Direct Coronary Stenting.

BACKGROUND Spontaneous coronary artery dissection (SCAD) is a rare medical emergency characterized by non-traumatic and non-iatrogenic tearing of the intima of a coronary artery, with an estimated incidence of 1-4%. CASE REPORT A 39-year-old woman with no known cardiac risk factors or recent trauma presented with acute chest pain, electrocardiographic (ECG) changes consistent with ST-elevation acute coronary syndrome, and elevated cardiac enzymes. Coronary angiography revealed near-complete stenosis of the distal left anterior descending (LAD) coronary artery with findings consistent with coronary artery dissection. Due to ongoing chest pain refractory to medical therapy, she underwent successful complex intervention on the distal LAD lesion with a 2.0×30 mm Onyx drug-eluting stent that was post-dilated to high pressure with a 2.5 noncompliant balloon, reducing the 99% stenosis to a 0% residual. She recovered fully and was discharged on aggressive risk factor modification with dual antiplatelet therapy (aspirin and clopidogrel) and high-intensity statin. CONCLUSIONS Spontaneous coronary artery dissection (SCAD) is a rare condition that can present with ECG changes and ischemic symptoms identical to ST-elevation transmural myocardial infarction secondary to plaque rupture. Coronary angiography is required to evaluate patients, and, depending on the catheterization findings, the patient's hemodynamic profile, and severity of ischemic symptoms, complex interventions such as direct coronary stenting can best treat patients such as ours, while medical management might be considered for others.

Persistent Exertional Chest Pain in a Marathon Runner: Exercise-induced, Painful, Left Bundle Branch Block Syndrome Treated With His-Bundle Pacing.

A 49-year-old woman presented with exercise-induced chest discomfort during long-distance running that was occasionally present during rest. Significant coronary artery disease was excluded and a diagnosis of "painful left bundle branch block (LBBB) syndrome" was made after correlation of LBBB aberrancy with symptoms during Holter monitoring. The patient underwent confirmatory testing consisting of rapid atrial pacing below and above 130 beats per minute, the rate cut-off for LBBB manifestation. His bundle pacing implantation was performed resulting in both non-selective and selective morphologies depending on output, both of which manifested with a painless narrow QRS regardless of rate. She was rendered completely pain free during long-distance running and remains so 6-months later. Her pain at rest, now thought to be due to severe anxiety secondary to her painful LBBB, has also subsided. Exercise-induced, painful LBBB is a rare phenomenon that manifests as chest discomfort when LBBB is present. This disease is frequently misdiagnosed as coronary angina, has limited medical treatment options, and can be disabling. HBP is an attractive treatment for this syndrome in an effort to avoid electromechanical dyssynchrony, the presumed mechanism of discomfort. This case report adds to the growing literature of painful LBBB syndrome and its effective treatment with HBP, with the added caveat that it can present with persistent symptoms at rest, in the setting of enhanced anxiety. HBP should be considered early on in the treatment of such patients.

Evaluating the etiology of mechanical valve obstruction: use of clinical parameters, fluoroscopy, and echocardiography.

Prosthetic valve obstruction is a life-threatening complication most commonly caused by thrombus, pannus, or both. We report a St. Jude tricuspid valve obstruction, initially treated with thrombolytic therapy, found to be caused by pannus on pathologic examination. Clinical evaluation and diagnostic evaluation with fluoroscopy and echocardiography in distinguishing pannus from thrombus are reviewed.