Unique presentation of cutis laxa with Leigh-like syndrome due to ECHS1 deficiency.

Clinical finding of cutis laxa, characterized by wrinkled, redundant, sagging, nonelastic skin, is of growing significance due to its occurrence in several different inborn errors of metabolism (IEM). Metabolic cutis laxa results from Menkes syndrome, caused by a defect in the ATPase copper transporting alpha (ATP7A) gene; congenital disorders of glycosylation due to mutations in subunit 7 of the component of oligomeric Golgi (COG7)-congenital disorders of glycosylation (CDG) complex; combined disorder of N- and O-linked glycosylation, due to mutations in ATPase H+ transporting V0 subunit a2 (ATP6VOA2) gene; pyrroline-5-carboxylate reductase 1 deficiency; pyrroline-5-carboxylate synthase deficiency; macrocephaly, alopecia, cutis laxa, and scoliosis (MACS) syndrome, due to Ras and Rab interactor 2 (RIN2) mutations; transaldolase deficiency caused by mutations in the transaldolase 1 (TALDO1) gene; Gerodermia osteodysplastica due to mutations in the golgin, RAB6-interacting (GORAB or SCYL1BP1) gene; and mitogen-activated pathway (MAP) kinase defects, caused by mutations in several genes [protein tyrosine phosphatase, non-receptor-type 11 (PTPN11), RAF, NF, HRas proto-oncogene, GTPase (HRAS), B-Raf proto-oncogene, serine/threonine kinase (BRAF), MEK1/2, KRAS proto-oncogene, GTPase (KRAS), SOS Ras/Rho guanine nucleotide exchange factor 2 (SOS2), leucine rich repeat scaffold protein (SHOC2), NRAS proto-oncogene, GTPase (NRAS), and Raf-1 proto-oncogene, serine/threonine kinase (RAF1)], which regulate the Ras-MAPK cascade. Here, we further expand the list of inborn errors of metabolism associated with cutis laxa by describing the clinical presentation of a 17-month-old girl with Leigh-like syndrome due to enoyl coenzyme A hydratase, short chain, 1, mitochondria (ECHS1) deficiency, a mitochondrial matrix enzyme that catalyzes the second step of the beta-oxidation spiral of fatty acids and plays an important role in amino acid catabolism, particularly valine.

Iron deficiency anemia and cardiac mortality in patients with left ventricular systolic dysfunction undergoing coronary stenting.

AIM: The aim of this study was to assess cardiac mortality in patients with reduced ejection fraction (EF< or =45%) and anemia (Hb< or =12 g/dL) undergoing coronary stenting and to investigate whether iron-deficiency anemia influenced outcome when compared to non-anemic patients or patients with other types of anemia. METHODS: One hundred twenty consecutive patients undergoing percutaneous coronary intervention (PCI) between April 2003 and December 2005 were identified and followed for a median of 30 months. Patients were divided into 2 groups, anemic (Hb< or =12 g/dL) and non-anemic. Anemic patients were then divided into 3 sub-groups based on laboratory analysis and anemia work-up: iron-deficiency, malignancy-associated, and anemia of chronic disease (including chronic kidney disease). Mortality rates and cause of death were retrieved using both the Social Security database and the hospital records. RESULTS: Thirty-one percent of patients had iron deficiency, 24% had a malignancy-associated anemia and 45% had anemia of chronic disease. Overall mortality was 12% of which 29% was cardiac death. All-cause and cardiac mortality were significantly higher in anemic vs. non-anemic patients, (31% vs. 6%, P<0.001, and 10% vs. 1%, P=0.016, respectively). Iron-deficiency anemia strongly predicted cardiac mortality (33% vs. 1% in non-anemic patients, P<0.001), while malignancy-associated anemia was the strongest predictor of non-cardiac death (57% vs. 4% in non-anemic patients, P<0.001). Anemia of chronic disease neither predicted cardiac nor non-cardiac death. CONCLUSIONS: To the authors' knowledge, this is the first study to show that iron-deficiency anemia is a strong predictor of cardiac death when compared to patients with other types of anemia or to non-anemic patients.

SerpinB2 regulates stromal remodelling and local invasion in pancreatic cancer.

Pancreatic cancer has a devastating prognosis, with an overall 5-year survival rate of ~8%, restricted treatment options and characteristic molecular heterogeneity. SerpinB2 expression, particularly in the stromal compartment, is associated with reduced metastasis and prolonged survival in pancreatic ductal adenocarcinoma (PDAC) and our genomic analysis revealed that SERPINB2 is frequently deleted in PDAC. We show that SerpinB2 is required by stromal cells for normal collagen remodelling in vitro, regulating fibroblast interaction and engagement with collagen in the contracting matrix. In a pancreatic cancer allograft model, co-injection of PDAC cancer cells and SerpinB2-/- mouse embryonic fibroblasts (MEFs) resulted in increased tumour growth, aberrant remodelling of the extracellular matrix (ECM) and increased local invasion from the primary tumour. These tumours also displayed elevated proteolytic activity of the primary biochemical target of SerpinB2-urokinase plasminogen activator (uPA). In a large cohort of patients with resected PDAC, we show that increasing uPA mRNA expression was significantly associated with poorer survival following pancreatectomy. This study establishes a novel role for SerpinB2 in the stromal compartment in PDAC invasion through regulation of stromal remodelling and highlights the SerpinB2/uPA axis for further investigation as a potential therapeutic target in pancreatic cancer.

JRK is a positive regulator of ß-catenin transcriptional activity commonly overexpressed in colon, breast and ovarian cancer.

The loss of ß-catenin inhibitory components is a well-established mechanism of carcinogenesis but ß-catenin hyperactivity can also be enhanced through its coactivators. Here we first interrogated a highly validated genomic screen and the largest repository of cancer genomics data and identified JRK as a potential new oncogene and therapeutic target of the ß-catenin pathway. We proceeded to validate the oncogenic role of JRK in colon cancer cells and primary tumors. Consistent with a ß-catenin activator function, depletion of JRK in several cancer cell lines repressed ß-catenin transcriptional activity and reduced cell proliferation. Importantly, JRK expression was aberrantly elevated in 21% of colorectal cancers, 15% of breast and ovarian cancers and was associated with increased expression of ß-catenin target genes and increased cell proliferation. This study shows that JRK is required for ß-catenin hyperactivity regardless of the adenomatous polyposis coli/ß-catenin mutation status and targeting JRK presents new opportunities for therapeutic intervention in cancer.

Acute changes in global and regional rest left ventricular function after successful coronary angioplasty: comparative results in stable and unstable angina.

The immediate effects of successful percutaneous transluminal coronary angioplasty on global and regional left ventricular function were assessed by comparing 30 degrees right anterior oblique left ventricular angiograms performed immediately before and after angioplasty on 39 patients undergoing 42 successful procedures. Mean (+/- SD) lesion stenosis decreased from 88 +/- 10% to 35 +/- 11% (p less than or equal to 0.001), whereas left ventricular ejection fraction increased from 57 +/- 11% to 64 +/- 10% (p less than or equal to 0.001) for the entire group. Left ventricular functional changes were further subgrouped according to stability of angina. Eighteen procedures were performed on 17 patients with stable angina: 24 procedures were performed on 22 patients with unstable angina defined as angina at rest or on minimal activity or recently accelerated angina. There were no significant subgroup differences in mean age, gender ratio, vessel anatomy, drug therapy or extent of coronary stenosis before or after angioplasty. Global ejection fraction increased significantly for the unstable group (from 54 +/- 11% to 66 +/- 9%, p less than or equal to 0.001) but was unchanged for the stable group (from 61 +/- 9% to 61 +/- 11%, p = NS). In unstable angina, regional ejection fraction (segmental area method) increased for both jeopardized (from 37 +/- 11% to 52 +/- 9%, p less than or equal to 0.001) and nonjeopardized myocardial segments (from 43 +/- 13% to 51 +/- 13%, p less than or equal to 0.001), but improvement was significantly (p less than or equal to 0.02) greater in jeopardized segments.(ABSTRACT TRUNCATED AT 250 WORDS)

Angiographic diagnosis of posterior mitral valve leaflet prolapse.

The left ventricular cineangiograms of 22 asymptomatic, apparently healthy male aviators without noninvasive (echocardiographic or auscultatory) evidence of mitral valve prolapse were reviewed and compared with those of 12 men with noninvasive evidence of mild mitral valve prolapse. The maximal protrusion of the posterior mitral valve leaflet superior and posterior to a line perpendicular to the long axis of the left ventricle at end-systole was measured from the right anterior oblique left ventricular cineangiogram by repeated observation of left ventricular inflow. The values were 7.5 +/- 1.6 mm in patients without mitral valve prolapse and 11.2 +/- 3.4 mm in patients with mitral valve prolapse (mean +/- 1 standard deviation). This measurement did not exceed 11 mm in any patient without prolapse. It is concluded that: 1) with meticulous attention to angiographic landmarks of the left ventricular inflow area, the limits of normal systolic posterior mitral leaflet motion can be defined; and 2) systolic motion outside these limits constitutes a quantitative criterion for the angiographic diagnosis of mitral valve prolapse.

Triple vessel coronary angioplasty: acute outcome and long-term results.

Triple vessel coronary angioplasty, defined as angioplasty of one or more lesions in each of the three major coronary arteries (left anterior descending, left circumflex, right coronary artery) was performed in 50 (11%) of 469 patients who had angioplasty of multiple vessels. There were 32 men and 18 women with a mean age of 56 years. All 50 patients had severe three vessel coronary disease and represent approximately 5% of patients with three vessel disease who had revascularization in this institution; 8 (16%) had previous coronary bypass surgery, and 23 (46%) had previous myocardial infarction. Unstable angina was present in 33 patients (66%) and 96% had Canadian Heart Association class III or IV angina; mean left ventricular ejection fraction was 57 +/- 11%. Angioplasty was performed in 176 vessels (3.5 vessels per patient, range 3 to 6) and in 250 lesions (5 lesions per patient, range 3 to 9); angiographic success was achieved in 240 lesions (96%) and 166 vessels (94%). Success in all vessels attempted was achieved in 40 (80%) of the 50 patients. Clinical success (angiographic success associated with clinical improvement) was obtained in all 50 patients in whom triple vessel angioplasty was performed; none of them required urgent bypass surgery and 5 patients (10%) had a non-Q wave myocardial infarction. In four other patients triple vessel angioplasty was planned but not performed because of failure to dilate the primary vessel; urgent bypass surgery was required in one of these, who developed a Q wave infarction. Thus, overall clinical success in 54 patients was 93%; the incidence rate of myocardial infarction was 11%, and that of urgent surgery 1.8%.(ABSTRACT TRUNCATED AT 250 WORDS)

Multivessel coronary angioplasty early after acute myocardial infarction.

Coronary angioplasty has been applied in patients with recent myocardial infarction, but results of angioplasty of multiple vessels early after myocardial infarction in patients with severe multivessel disease have not been reported. Coronary angioplasty of multiple vessels was performed in 105 patients 0 to 15 days (mean 5 +/- 4) after recent myocardial infarction. There were 77 men (73%) and 28 women (27%), with a mean age of 57 years. All patients had severe multivessel disease, 68% with two vessel and 32% with three vessel disease. Twenty-eight patients (27%) had successful thrombolysis before angioplasty and 70 (67%) had postinfarction angina. Mean left ventricular ejection fraction was 58 +/- 10% and was less than 45% in 13 patients (12%). Angioplasty was attempted in 319 lesions (mean 3 lesions per patient, range 2 to 9) and 252 vessels (mean 2.4 vessels per patient, range 2 to 4), with success in 302 lesions (95%) and 237 vessels (94%); angioplasty was done in two stages in 59 patients (56%). Clinical success was achieved in 102 patients (97%). Complications included myocardial infarction in six patients (5.7%) (one Q wave, five non-Q wave), urgent bypass surgery in two (1.9%) and death in one (0.9%); overall, seven patients (7%) had a major complication. All patients had a follow-up duration greater than 1 year (mean 31 months, range 12 to 73). Clinical recurrence developed in 24 patients (23%), of whom 21 had repeat angioplasty, 1 had bypass surgery and 2 were managed medically. Ten patients (9.8%) had a late infarction and 5 (4.9%) died of cardiac death during the follow-up period.(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary angioplasty in patients with severe left ventricular dysfunction.

The applications for coronary angioplasty have greatly expanded and the procedure is now increasingly used in complex and potentially high risk conditions. This report describes the short- and long-term effects of coronary angioplasty in 61 patients with severely depressed left ventricular function (ejection fraction less than or equal to 35%) with unstable or refractory anginal symptoms, or both, in whom revascularization was necessary despite increased risk. In a retrospective analysis of 1,260 patients undergoing angioplasty between January 1985 through December 1987, 61 had an ejection fraction less than or equal to 35%. The common clinical presentation was unstable angina (70%) with or without recent myocardial infarction. Mean left ventricular ejection fraction was 27 +/- 6%. Forty-five patients (74%) had multivessel disease. Clinical success after angioplasty was achieved in 55 patients (90%). Major complications (death, infarction and emergency bypass surgery) occurred in five patients (8.2%), with death in two (3.2%). During long-term (mean 21 +/- 11 months) follow-up study of the 55 patients with successful angioplasty, 13 (23%) died, including 3 of noncardiac causes, and 11 (20%) had clinically symptomatic recurrence. Continued clinical success was present in 39 patients (71%), of whom 28 (51%) were event-free patients and 11 (20%) had clinical recurrence; a successful second angioplasty procedure was performed in 9 because of restenosis. Thus, in patients with depressed left ventricular function, coronary angioplasty can be performed with a short-term success rate comparable to that of routine angioplasty or surgical procedures. However, acute complications are more frequent and the late mortality rate is higher than in patients with less depressed function.

The M-Heart percutaneous balloon mitral Valvuloplasty Registry: initial results and early follow-up. The M-Heart Group.

The initial results, complications and early follow-up of 74 patients undergoing percutaneous balloon mitral valvuloplasty in seven hospitals participating in a multicenter registry are reported. Seventy-four patients with a mean age of 53 years had 75 valvuloplasty procedures performed over a 2.5 year period. Eighty-nine percent of the attempted procedures were completed and resulted in an increase in mean mitral valve area from 1.0 +/- 0.04 to 2.0 +/- 0.1 cm2 (p less than 0.0001); the valve area increased greater than or equal to 50% of the baseline valve area in 73% of the patients. Major complications included procedure-related death (2.7%), cardiac tamponade (6.7%), systemic embolism (2.7%) and emergency surgery (6.7%). At a mean follow-up period of 14.6 months, the condition of the majority of patients had improved, and 89% of 55 patients treated only with valvuloplasty were in New York Heart Association functional class I or II. Thus, hemodynamic and clinical improvement can be obtained in the majority of patients with mitral stenosis treated with balloon valvuloplasty in multiple centers. However, suboptimal results and major complications occurred in a significant number of patients and may limit this procedure to use by experienced operators in hospitals with facilities for cardiac surgery.

Reduction of ischemia with a new flow-adjustable hemoperfusion pump during coronary angioplasty. The Coronary Hemoperfusion Ischemia Prevention Study (CHIPS) Investigators.

A new flow-adjustable pump for coronary hemoperfusion to prevent ischemia during routine coronary angioplasty was evaluated in a multicenter prospective study of 110 patients. The protocol included patients who had angina or ST segment elevation during a control balloon inflation of less than or equal to 3 min. Hemoperfusion was performed by means of a new large lumen angioplasty catheter utilizing the patient's renal vein or femoral artery blood. Vessels perfused were the left anterior descending coronary artery (n = 74), right coronary artery (n = 39), left circumflex artery (n = 9) and coronary vein grafts (n = 15). Mean (+/- SD) perfusion flow was 41 +/- 9 ml/min (range 17 to 70); mean perfusion time was 9.3 +/- 4 min (median 8.5, range 2 to 30). Chest pain score (0 to 4) decreased from 2.9 +/- 1 to 1.4 +/- 1 during hemoperfusion (p less than 0.001); ST segment elevation score (0 to 4) decreased from 2.6 +/- 1 to 0.7 +/- 1 (p less than 0.005) and inflation time increased from 1.3 +/- 0.9 to 7 +/- 4 min, (p less than 0.001). At least a 50% increase in tolerated inflation time was obtained in 104 patients (95%). Free plasma hemoglobin and creatine kinase levels did not increase significantly over baseline values. Angioplasty was successful in 107 patients (97%), with mean stenosis reduced from 87 +/- 11% to 20 +/- 17%; 3 patients had urgent bypass surgery, 2 (1.8%) had a myocardial infarction (1 Q wave, 1 non-Q wave) and 2 (1.8%) died later in the hospital of probable noncoronary causes.(ABSTRACT TRUNCATED AT 250 WORDS)

Status of the myocardium and infarct-related coronary artery in 19 necropsy patients with acute recanalization using pharmacologic (streptokinase, r-tissue plasminogen activator), mechanical (percutaneous transluminal coronary angioplasty) or combined types of reperfusion therapy.

In acute myocardial infarction, myocardial salvage is dependent on rapid restoration of blood flow. Pharmacologic (streptokinase, recombinant tissue-type plasminogen activator), mechanical (percutaneous transluminal coronary angioplasty, guide wire perforation) or combined forms of reperfusion therapy can accomplish this goal, but their effects on infarcted myocardium and vessel occlusion site have not been compared at necropsy. The heart of 19 necropsy patients who had received various forms of acute reperfusion therapy was studied: 14 had pharmacologic or combined forms of reperfusion therapy (13 streptokinase and 1 tissue-type plasminogen activator, including 4 with combined balloon angioplasty) and 5 had had purely mechanical (balloon angioplasty) reperfusion therapy. Reperfusion was initially clinically successful in all 19 patients with the average time from onset of symptoms to reperfusion being 3.7 hours. Necropsy observations separated the 19 patients into distinct subgroups based on changes in the myocardium and infarct-related coronary arteries. Of the 19 patients, 14 (74%) had hemorrhagic myocardial infarction and they all received pharmacologic or combined forms of reperfusion therapy. The remaining five patients (26%) had nonhemorrhagic (anemic) infarction and were treated with balloon angioplasty therapy alone. Increased luminal cross-sectional area was present in 8 of 9 patients with acute balloon angioplasty but severe coronary atherosclerotic plaque remained in 9 of 10 patients without acute balloon angioplasty. Severe hemorrhage surrounded angioplasty sites in all four patients who also received streptokinase or tissue-type plasminogen activator. Severe bleeding at the angioplasty site compromised the dilated coronary lumen in one patient. No patient with angioplasty alone had intraplaque bleeding. Thus, acute coronary balloon angioplasty reperfusion therapy alone appears to avoid the potentially adverse effects of myocardial and intraplaque hemorrhage while simultaneously increasing luminal cross-sectional area at the site of acute occlusion.

High speed rotational atherectomy: outcome in calcified and noncalcified coronary artery lesions.

OBJECTIVES: This study sought to determine the success and complication rates of high speed rotational coronary atherectomy in calcified and noncalcified lesions. BACKGROUND: Percutaneous transluminal coronary angioplasty and directional coronary atherectomy of calcified lesions are associated with reduced procedural success and increased complications. Rotational atherectomy using the Rotablator catheter abrades noncompliant plaque and may improve outcome in calcified lesions. METHODS: Data from the completed Multicenter Rotablator Registry of 2,161 rotational atherectomy procedures in single lesions were analyzed to determine the relative efficacy of rotational atherectomy for 1,078 calcified and 1,083 noncalcified lesions. The power of the study was 0.86 to detect a significant difference in outcome, if the true success rates in the noncalcified and calcified lesions were 96% and 93%, respectively. RESULTS: Patients with calcified lesions were older (mean [+/- SD] age 66.2 +/- 10.3 vs. 60.5 +/- 11.0 years, p = 0.0001) than those with noncalcified lesions. Calcified lesions were more frequently new (75% vs. 64%, p = 0.0001), angulated (27% vs. 22%, p = 0.02), eccentric (75% vs. 64%, p = 0.0001) and long (32% vs. 27%, > 10 mm in length, p = 0.01). They were also more often complex (57% vs. 46%, p = 0.001) and located in the left anterior descending coronary artery (51% vs. 44%, p = 0.001). Adjunctive coronary angioplasty was used in 82.9% of calcified and 66.9% of noncalcified lesions. Procedural success, defined as < 50% residual stenosis without major complications, was achieved in 94.3% of calcified and 95.2% of noncalcified lesions (p = 0.32). Major complication rates were 4.1% in calcified and 3.1% in noncalcified lesions (p = 0.24). Non-Q wave myocardial infarction was documented in 10.0% of calcified and 7.7% of noncalcified lesions (p = 0.054). Mean postprocedural residual stenosis was 21.6 +/- 13.9% in calcified and 23.3 +/- 15% in noncalcified lesions (p = 0.39). CONCLUSIONS: In this review of data from a large multicenter registry, the success rate of rotational atherectomy was not reduced by calcification despite the more frequent complex nature of the calcified lesions. The Rotablator catheter is likely to be the device of choice for percutaneous intervention in calcified lesions, but definitive conclusions await the results of randomized trials.

Is traditionally defined complete revascularization needed for patients with multivessel disease treated by elective coronary angioplasty? Multivessel Angioplasty Prognosis Study (MAPS) Group.

OBJECTIVES: The purpose of this study was to determine the effect of incomplete revascularization by percutaneous transluminal coronary angioplasty in patients with multivessel disease on adverse long-term cardiac events (death, coronary artery bypass surgery or myocardial infarction) and to develop an optimal definition of adequate revascularization based on clinical outcome. BACKGROUND: The effect of incomplete coronary revascularization by coronary angioplasty on long-term adverse clinical events remains controversial. METHODS: Three hundred seventy well characterized patients were followed-up for 27 +/- 16 months after angioplasty. Mean patient age was 58 +/- 11 years; 72% were male; 70% had two-vessel disease (> or = 50% diameter stenosis by caliper measurement); and the mean left ventricular ejection fraction was 58 +/- 11% (range 20% to 85%). Angioplasty was successfully accomplished in 339 patients (91.6%), but complete revascularization by the standard definition (no residual > or = 50% stenosis in a coronary artery > or = 1.5 mm in diameter) was achieved in only 91 patients (25%). RESULTS: Three-year event-free survival (i.e., freedom from death, myocardial infarction, coronary artery bypass surgery) in the entire cohort was 76.5%. By the standard definition, complete revascularization was strongly and negatively associated (p = 0.003) with long-term cardiac events, even after correction for the effects of other independent correlates of events, using Cox proportional hazard regression analysis. Seventeen other definitions, evaluating the severity and extent of residual stenoses and whether they were associated with contractile myocardium, were tested to find that which best stratified late event-free survival and had an outcome with complete revascularization no worse than that associated with the standard definition. The best definition for the entire cohort, having more predictive value than the standard definition, allowed < 10% of estimated left ventricular mass to be served by vessels with mild stenoses (< 60%) without being considered "incomplete." CONCLUSIONS: Mild stenoses in coronary arteries > or = 1.5 mm in diameter serving modest amounts of myocardium do not appear to need to be revascularized to achieve good long-term outcome with coronary angioplasty. Hence, angioplasty in such lesions may not be justified except when they are documented to cause life-style-limiting angina, and the standard definition of complete revascularization by angioplasty appears to be suboptimal. The importance of optimally defined adequate revascularization should be considered in the interpretation of the results of randomized trials assessing the clinical efficacy of coronary angioplasty compared with that of other modalities of therapy.

Prospective case-control comparison of percutaneous transluminal coronary revascularization in patients with multivessel disease treated in 1986-1987 versus 1991: improved in-hospital and 12-month results. Multivessel Angioplasty Prognosis Study (MAPS) Group.

OBJECTIVES: This study sought to ascertain whether early and 12-month clinical outcomes after percutaneous coronary revascularization have improved between 1986-1987 and 1991. BACKGROUND: Since the mid-1980s, when the results of percutaneous revascularization were considered to be somewhat static, justifying large-scale clinical trials of percutaneous transluminal coronary angioplasty versus other modes of therapy, balloon technology has improved, and several new percutaneous revascularization techniques have become available. The clinical results of the current integrated approach to revascularization compared with those for coronary angioplasty alone in the late 1980s are not known. METHODS: In this prospective case-control study, 200 consecutively treated patients with multivessel disease in 1991 were studied prospectively and compared with 400 consecutive patients from the same centers during 1986-1987. Patients from 1991 were matched with earlier patients on the basis of four previously described prognostic determinants (left ventricular ejection fraction, presence of unstable angina, diabetes and target lesion morphology score) and the treating institution and were assessed for treatment outcome (completeness of revascularization, procedural success and event-free survival [freedom from death, myocardial infarction and further revascularization]). RESULTS: The 1991 cohort of patients was older (mean [+/- SD] age 62 +/- 11 vs. 58 +/- 11 years, p < 0.001) and tended to have slightly worse left ventricular function (ejection fraction 56 +/- 10% vs. 58 +/- 11%, p = 0.009) than the 1986-1987 cohort. Overall lesion morphology risk scores were similar. New devices (other than coronary angioplasty) were used in 26% of patients. The 1991 patient cohort had more frequent total revascularization (35% vs. 21%, p = 0.003), fewer emergency bypass operations (1.0% vs. 5.5%, p = 0.006) and an improved overall procedural success rate (90% vs. 84%, p = 0.04). In addition, at 12 months the event-free survival rate was superior in the 1991 cohort (73.3% vs. 63.6%, p = 0.02), although there was no difference in infarct-free survival rate (94.6% vs. 93.2%, p = NS). CONCLUSIONS: Improved results with percutaneous revascularization in 1991 have important implications for patient care and interpretation of ongoing randomized trials enrolling patients in the late 1980s and intending to compare standard coronary angioplasty with other forms of therapy.

Comparison of complications during percutaneous transluminal coronary angioplasty from 1977 to 1981 and from 1985 to 1986: the National Heart, Lung, and Blood Institute Percutaneous Transluminal Coronary Angioplasty Registry.

Because the effects of changing technology in percutaneous transluminal coronary angioplasty, increased operator experience and use of the procedure in patients with extensive disease are unknown in regard to complication patterns, the initial 1977-1981 cohort and the recent 1985-1986 cohort of the National Heart, Lung, and Blood Institute Percutaneous Transluminal Coronary Angioplasty Registry were analyzed with respect to complications. Compared with the initial cohort of 1,155 patients, the 1,801 new cohort patients were older and had an increased prevalence of multivessel coronary artery disease, depressed left ventricular function and prior infarction. Overall complication rates in the recent cohort were either unchanged or decreased from the rates in the initial cohort despite a higher risk patient population. The most significant decreases were in the incidence of coronary spasm (p less than 0.001) and the need for emergency coronary bypass surgery (p less than 0.01). Overall in-hospital mortality was low but was dependent on the extent of vessel disease--0.2% for single vessel disease, 0.9% for double vessel disease and 2.2% for triple vessel disease (p less than 0.001 for linear trend). Acute coronary complications of branch occlusion, dissection or abrupt closure were associated with increased rates of death, nonfatal infarction or need for emergency surgery. Factors showing a multivariate association with increased mortality included a history of congestive heart failure (p less than 0.001), age greater than or equal to 65 years (p less than 0.01), triple vessel or left main coronary artery disease (p less than 0.05), female gender (p less than 0.05) and new onset angina.(ABSTRACT TRUNCATED AT 250 WORDS)

Balloon angioplasty versus new device intervention: clinical outcomes. A comparison of the NHLBI PTCA and NACI registries.

OBJECTIVES: We sought to compare outcomes of patients treated in the National Heart, Lung, and Blood Institute (NHLBI) Percutaneous Transluminal Coronary Angioplasty (PTCA) and New Approaches to Coronary Intervention (NACI) registries. BACKGROUND: Coronary angioplasty has numerous shortcomings. New devices for performing coronary interventions have been introduced in an effort to improve clinical outcomes. METHODS: Under the sponsorship of the NHLBI, a registry of consecutive patients treated with PTCA during 1985 to 1986 was established. In 1990, the NHLBI funded a second registry, the NACI. The two registries used the same data coordinating center to collect detailed baseline and follow-up information. RESULTS: Patients enrolled in the NACI registry were older, had undergone more previous bypass surgery procedures and had more stenoses located in bypass grafts than patients in the NHLBI PTCA registry. Procedural success was achieved in 72.1% and 82.6% of patients in the PTCA and NACI registries, respectively; however, in-hospital and 1-year mortality rates were 1.0% versus 1.8% and 3.1% versus 5.9% for the PTCA versus NACI registries, respectively. After risk adjustment, there was no difference in 1-year mortality. Rates of target lesion revascularization (TLR) were 21.5% for the PTCA registry and 24.2% for the NACI registry. NACI registry patients had a higher risk for TLR and the composite end point of death, myocardial infarction or revascularization (relative risk 1.28 and 1.23, respectively). However, the NACI registry patients who received stents tended to have a lower adjusted TLR rate. CONCLUSIONS: This comparative study found no overall superiority of these newer devices in terms of patient survival or freedom from TLR after adjustment for baseline risk profiles. Although technologic improvements (especially improved stenting) continue, these observations highlight the importance of careful assessment of clinical results in the broad population of patients in whom interventions are used.

Impedance cardiograms reliably estimate beat-by-beat changes of left ventricular stroke volume in humans.

Linear regression was used to compare stroke volumes calculated from tetrapolar impedance cardiograms and simultaneous left ventriculograms in 14 patients undergoing diagnostic left heart catheterisation. We calculated three to five consecutive stroke volumes from each ventriculogram. Left ventricular stroke volumes estimated by the two methods correlated closely: the correlation coefficients from pairs of data obtained from individual patients ranged between 0.77 and 1.00 (average = 0.91), and the correlation coefficient for pooled data (all pairs from all subjects) was 0.79 (p less than 0.001). Changes in left ventricular stroke volume measured with the two methods also correlated well (r = 0.89 for pooled data, p less than 0.001). The results suggest that impedance cardiograms provide reliable estimates of changes of beat-by-beat left ventricular stroke volumes and reasonable estimates of absolute levels of beat-by-beat stroke volumes in humans.

Visualization of atrial myocardium with thallium-201: case report.

An adult patient evaluated for cyanotic congenital heart disease was found to have pulmonary atresia with intact ventricular septum, hypoplastic right ventricle, and right atrial enlargement. Thallium-201 myocardial imaging before surgical correction showed thallium activity in the right atrium. Following the establishment of a conduit from the right atrium to pulmonary artery, the right-atrial thallium uptake was even more prominent.

Use of blood-pool imaging in evaluation of diffuse activity patterns in technetium-99m pyrophosphate myocardial scintigraphy.

It has been suggested that diffuse Tc-99m pyrophosphate precordial activity may be due to persistent blood-pool activity in routine delayed views during myocardial imaging. To answer this question, we reviewed myocardial scintigrams recorded 60--90 min following the injection of 12--15 mCi of Tc-99m pyrophosphate for the presence of diffuse precordial activity, and compared these with early images of the blood pool in 265 patients. Diffuse activity in the delayed images was identified in 48 patients: in 20 with acute myocardial infarction and in 28 with no evidence of it. Comparison of these routine delayed images with early views of the blood pool revealed two types of patterns. In patients with acute infarction, 95% had delayed images that were distinguishable from blood pool either because the activity was smaller than the early blood pool, or by the presence of localized activity superimposed on diffuse activity identical to blood pool. In those without infarction, 93% had activity distribution in routine delayed views matching that in the early blood-pool images. The usefulness of the diffuse TcPPi precordial activity in myocardial infarction is improved when early blood-pool imaging is used to exclude persistence of blood-pool activity as its cause. Moreover, it does not require additional amounts of radioactivity nor complex computer processing, a feature that may be of value in the community hospital using the technique to "rule out" infarction 24--72 hr after onset of suggestive symptoms.