Cardiac amyloidosis secondary to multiple myeloma.

Amyloidosis is a multisystemic disease caused by extracellular deposition of pathologic beta fibrillar proteins in multiple organs. Deposited fibrils can be either innunoglobulin light chanis or amyloid-A protein. The incidence of anyloidosis derived from anyloid-A protein, usually associated to an underlying disease, has been dimisishing over the decades in the United States producing clinical evidence of cardiac involvement in less than 5% of all cases. The extent of cardiac involvement is the determining prognostic factor. Early diagnosis and therapy aimed at the underlying disease may halt progression of cardiac dysfuction and improve prognosis. We report a case of a 63-year-old man who was diagnosed with cardiac amyloidosis secondary to multiple myeloma.

Aborted sudden cardiac death as a presentation of isolated non-compaction cardiomyopathy.

Left ventricular non-compaction cardiomyopathy (LVNC) is a rare disorder characterized by a thick myocardial wall with two distinct layers consisting of compacted and noncompacted myocardium. The major clinical manifestations of LVNC have been described as heart failure, cardiac arrhythmias, and cardioembolic events. In this report we present a case of a young woman with LVNC who presented with aborted sudden cardiac death. This is the second case of LVNC reported in Puerto Rico, but the first presenting with this complication. Recent advances in the field of cardiology allow the identification and diagnosis of this disease; thus, preventive and treatment strategies could be established for this potentially life-threatening condition. LVNC has to be considered in young patients presenting with sudden cardiac death.

Spontaneous hemothorax: first manifestation of pulmonary arteriovenous malformation in a patient with hereditary hemorrhagic telangiectasia.

Hereditary Hemorrhagio Telangiectasla (HHT) is a rare inherited autosomal dominant disorder characterized by systemic fibrovascular dysplasia making vessel walls more liable to spontaneous ruptures and injuries. Epistaxis is the first and the most common symptom; however patients may have a variety of serious complications due to vascular involvement of internal organs. In this report we present a case of a 39 year-old woman with recurrent epistaxis and family history of HHT who presented with right spontaneous hemothorax. Pulmonary angiography disclosed multiple pulmonary artery aneurysms with subsequent coil embolization. Considering that pulmonary arteriovenous malformations are associated with considerable morbidity and mortality, patients and family members with suspected HHT should be screened for these vascular malformations.

Ventricular tachycardia as a presentation of congenital left ventricular aneurysm.

Left ventricular aneurysm (LVA) describes a region of ventricular wall that is thinner than the adjacent myocardial segments, balloons outward and exhibits either akinesis or dyskinesis. Congenital LVA is diagnosed by exclusion with a very low incidence, especially in adult patients with very few cases reported. Clinically, most congenital LVA are asymptomatic. The most common complaints are typical angina or atypical chest pain followed by syncope and heart rhythm disturbances. We describe the case of a 26-year-old-woman that presented with monomorphic sustained ventricular tachycardia as a first manifestation of congenital LVA. Successful endoventricular circular patch placement, or Dor procedure was performed. Successful substrate modification for arrhythmia development was performed and confirmed by an electrophysiological study. This would constitute the first case of this nature reported in Puerto Rico.

Ventricular non-compaction cardiomyopathy.

Non-compaction of the left ventricle is an extremely rare cardiomyopathy resulting from a defective morphogenesis of the endomyocardium. It results in an architecturally aberrant ventricular wall consisting of two layers: a compacted layer and a loose interwoven meshwork with prominent trabeculae and deep intertrabecular recesses that communicate with the left ventricular cavity. This report describes the case of a 25-year-old man with dilated cardiomyopathy, due to non-compaction of the left ventricle, presenting with a transient ischemic attack.

Percutaneous coronary intervention versus medical therapy for coronary allograft vasculopathy. One center's experience.

BACKGROUND: Coronary allograft vasculopathy, a rapidly progressive form of atherosclerosis, remains the limiting factor in the long-term survival of heart transplant recipients. Some centers have attempted percutaneous coronary intervention to slow the disease process and thereby reduce mortality in these patients, but long-term follow-up data are scarce. We compared clinical outcomes in heart transplant recipients with coronary allograft vasculopathy who were treated either with percutaneous coronary intervention or with aggressive medical therapy alone. METHODS: A retrospective analysis of all heart transplant recipients at our institution who underwent surveillance coronary angiography for coronary allograft vasculopathy between 1995 and 2000 was performed. Patients with coronary allograft vasculopathy were stratified according to whether they received medical therapy or percutaneous coronary intervention. Baseline demographics, results of re-vascularization procedures and outcomes were analyzed. RESULTS: From 1995 to 2000, 301 patients underwent 602 coronary angiograms. Of the 79 patients who had angiographic evidence of coronary allograft vasculopathy, 53 were treated with aggressive medical therapy, while 26 underwent percutaneous coronary intervention in addition to aggressive medical therapy. At baseline, patients treated with aggressive medical therapy tended to be younger (54.6 +/- 13.8 years) than patients treated with percutaneous coronary intervention (62.6 +/- 7.6 years; p = 0.0079). Ejection fraction at time of diagnosis of coronary allograft vasculopathy was similar for both groups (medical therapy group, 44.4 +/- 13.4% vs percutaneous coronary intervention group, 47.2 +/- 12.7%; p = 0.38). In our cohort, heart transplant recipients with coronary allograft vasculopathy demonstrated greater mortality than heart transplant recipients without coronary allograft vasculopathy (p = 0.016). Patients who underwent percutaneous coronary intervention had a 60% re-stenosis rate at 6 months if they were treated with coronary angioplasty and an 18% re-stenosis rate if they received a coronary stent. Kaplan-Meier analysis showed no significant difference in survival in either treatment group at 1 year (80% for medical therapy group vs 95% for percutaneous coronary intervention group) or 3 years (68% for medical therapy group vs 79% for percutaneous coronary intervention group) after the angiographic diagnosis of coronary allograft vasculopathy. CONCLUSION: In this non-randomized trial, heart transplant recipients with coronary allograft vasculopathy were less likely to survive than patients without it. In addition, we found no statistical difference in mortality in heart transplant recipients with coronary allograft vasculopathy, regardless of whether they received percutaneous coronary intervention or aggressive medical therapy alone.

Incidencia de intoxicaciones por organofosforados: enero 1988-enero 1990 / Organophosphates intoxication incidence : January 1988- January 1990

Presentamos 38 casos de intoxicación por órganofosforados. Encontramos que el sexo masculino estuvo más afectado (63 por ciento de los casos). La mayor incidencia ocurrió en el grupo de 2-4 años de edad, (34.2 por ciento de los casos). El 84 por ciento de los casos ocurrió de forma accidental