TIPS stent migration causing sinus of Valsalva rupture.

A man in his 40s presented with haematemesis and melaena and was found to have a massive variceal bleed. Endoscopic procedures were ineffective at controlling the bleed; thus, an emergent transjugular intrahepatic portosystemic shunt (TIPS) procedure was performed. There were no noted complications from the procedure and the patient was eventually discharged home. A month later, a murmur was auscultated on routine physical examination. This prompted an outpatient transthoracic echocardiogram which revealed a TIPS stent in the inferior vena cava (IVC) extending into the right atrium along with a ruptured sinus of Valsalva with left to right shunt.The patient declined surgical intervention. He is currently being followed in the outpatient setting with serial echocardiograms and medical management.

Pulmonary homograft failure presents as abdominal ascites.

Ascites is a condition typically attributed to liver disease but can be cardiac in aetiology as well. We report of an atypical case of pulmonary homograft failure status-post the Ross-Yacoub procedure as an underlying cause of anasarca. A man in his mid-30s with a history of congenital heart disease presented with abdominal distension and exercise intolerance. His prior history was relevant for congenital bicuspid aortic valve with critical aortic stenosis. Abdomen and pelvis CT revealed a grossly dilated inferior vena cava while transthoracic echocardiogram showed severe homograft pulmonary stenosis and regurgitation. Further investigation with cardiac MRI demonstrated severe pulmonary valve stenosis and regurgitation with significant tricuspid regurgitation and right ventricular dilatation. The transcatheter stent in the right ventricular to pulmonary artery conduit followed by transcatheter valve replacement, which resulted in full resolution of his presenting symptoms.

Comparison of electrocardiogram quality and clinical interpretations using prepositioned ECG electrodes and conventional individual electrodes.

BACKGROUND: Efforts have been made to simplify and reduce technical errors, such as limb leads reversal and inaccurate chest leads placement, for the 12-lead ECG tests. We compared standard ECG using individual electrodes with a novel pre-positioned electrode system to determine equivalency. METHODS: Subjects were recruited from the Emergency Department and cardiac lab of an acute care hospital in Los Angeles. First, subjects underwent a conventional 12-lead ECG using Philips PageWriter (clinical ECG). A second ECG was then performed using a novel system containing pre-positioned electrodes and a compact recorder (study ECG). All ECGs were reviewed by 3 blinded, board-certified adult cardiologists using 14 pre-specified ECG diagnostic categories to determine if the interpretations of clinical ECG and study ECG of the same patient were "equivalent". Majority rule was applied when there were discrepant interpretations among the 3 cardiologists. RESULTS: One hundred subjects, ages 18 to 74 completed the study. With pre-positioned electrodes, the rate of "electrode fit" as judged by the research associates at the time of lead placement was 96.2%. We found that the study ECG system was equivalent (in clinical interpretation) to the clinical ECG system, with equivalency rate of 96% (95% confidence interval 92% to 100%) in "overall interpretation". The equivalence rate for the 14 ECG diagnostic categories ranged from 96% to 100%, with mean 99.2 ± 1.1%. CONCLUSIONS: 12-lead ECGs performed using single-piece, pre-positioned electrodes are clinically equivalent to those performed using 10 individually placed conventional electrodes. With 4 sizes for adults, the single-piece electrodes can fit 96% of the study patients.

Cardiovascular and Neonatal Outcomes in Pregnant Women With High-Risk Congenital Heart Disease.

Congenital heart disease (CHD) increases the risk of adverse maternal and neonatal outcomes. However, previous studies have included mainly women with low-risk features. A single-center, retrospective analysis of pregnant women with CHD was performed. Inclusion criteria were the following high-risk congenital lesions and co-morbidities: maternal cyanosis; New York Heart Association (NHYA) functional class >II; severe ventricular dysfunction; maternal arrhythmia, single ventricle (SV) physiology, severe left-sided heart obstruction and severe pulmonary arterial hypertension. Multivariate analyses for predictors of adverse maternal cardiovascular and neonatal outcomes were performed. Forty-three women reported 61 pregnancies. There were no maternal or neonatal deaths. Maternal cardiac (31%) and neonatal (54%) complications were frequent. The most frequent cardiac events were pulmonary edema, arrhythmia, and reduced NYHA class. Previous arrhythmia conferred a 12-fold increase in the odds of experiencing at least one major cardiac complication. Maternal SV physiology was an independent risk factor for low birth weight, risk of neonatal intensive care unit admission and lower gestational age. Maternal cyanosis and severe pulmonary arterial hypertension also predicted adverse neonatal outcomes. In conclusion, mothers without antepartum arrhythmia or functional incapacity are unlikely to experience arrhythmias or a decrease in NYHA class during pregnancy. In addition, SV physiology is a robust predictor of neonatal complications. Antepartum counseling and assessment of maternal fitness are crucial for the woman with CHD.

Superior vena cava stenting guided by alternative imaging modalities in a patient with severe contrast allergy: a case report and brief literature review.

Major adverse reactions to iodinated radiocontrast media are an uncommon but serious complication of cardiac catheterizations. We present a case of a 52-year-old woman with a sinus venosus atrial septal defect and partial anomalous pulmonary venous connection status post repair who presented with superior vena cava graft obstruction. Two overlapping stents were placed within the superior vena cava using gadolinium, CO2 angiography and transesophageal echocardiography for visualization of the graft stenoses. No iodinated media were used.

Coronary calcium scanning independently detects coronary artery disease in asymptomatic firefighters: a prospective study.

BACKGROUND: Sudden cardiac death is the leading cause of death among firefighters in the United States. Fire departments commonly maintain physical examination protocols, often with exercise stress testing, to detect risk of coronary heart disease. OBJECTIVE: We sought to determine whether coronary calcium detected by electron beam computed tomography (EBCT) adds incremental risk stratification beyond the traditional risk factors in asymptomatic community-based firefighters. METHODS: Three hundred ninety nine asymptomatic firefighters underwent a coronary calcium scan on a GE/Imatron C-150 Ultrafast EBCT scanner, using standardized imaging protocols. Framingham risk factor data were obtained on each patient by using a questionnaire. Agatston scores were derived and compared with national database of Agatston scores for asymptomatic populations on the basis of age and sex, allowing determination of a calcium percentile for each score. RESULTS: Coronary calcium was found only in men >34 years of age. Of the 53% who had positive scans (Agatston score > 0), 87% had higher than average Agatston scores compared with a national database (P < 0.01). Agatston score above the 75th percentile was found in 57% of firefighters. No correlation was observed between traditional risk factors and those with and without coronary calcium. CONCLUSIONS: Firefighters have a high burden of calcified coronary atherosclerosis, greater than anticipated on the basis of age and coronary risk factors.

Comparison of coronary calcium in firefighters with abnormal stress test findings and in asymptomatic nonfirefighters with abnormal stress test findings.

Firefighters are known to have an elevated rate of sudden cardiac death compared to the general population. It is unclear whether this finding is related to underlying cardiovascular risk factors or whether firefighting inherently carries additional risk. Our objective was to determine whether Los Angeles county firefighters have higher coronary artery calcium (CAC) scores and increased atherosclerosis as determined using 64-slice cardiac, multidetector computed tomography. A total of 647 asymptomatic firefighters evaluated as a part of a wellness protocol were referred for cardiac multidetector computed tomography to evaluate abnormal exercise treadmill test findings. They were matched by age and cardiovascular risk factors, with 2,533 asymptomatic subjects undergoing cardiac computed tomography because of abnormal electrocardiographic or exercise treadmill test findings. CAC and the prevalence of obstructive coronary artery disease by vessel were derived. Finally, the predictors of CAC were analyzed using regression analysis. Of the firefighters, 49% had detectable CAC compared to 43% of controls (p = 0.015). Although the lesions were most prevalent in the left anterior descending artery in both groups, more firefighters had any left anterior descending artery stenosis compared to the controls (p <0.0001). The firefighters also had more left main coronary artery lesions than did the controls (p <0.0001). The firefighters had significantly greater CAC scores than did with the controls (p <0.001). Furthermore, the firefighters had significantly greater mean CAC scores (66 ± 8 in firefighters vs 33 ± 4 for controls, p <0.001). Firefighter status was independently associated with a 41-point increase in the CAC score (p <0.001). In conclusion, asymptomatic firefighters had more atherosclerosis and CAC than the matched controls.

Quadricuspid aortic valve with sinus of Valsalva rupture.

A 22-year-old female with no medical history presented to the emergency room with 2 weeks of rapidly worsening dyspnea on exertion, orthopnea, and cough. On cardiac auscultation, she was noted to have to-and-fro murmurs and a continuous murmur with signs of right heart failure. Echocardiographic images obtained showed moderate to severe aortic regurgitation, severe tricuspid regurgitation, and a "windsock" originating in the right coronary sinus of Valsalva and terminating in the right atrium. The aortic valve had four leaflets, with the right leaflet function compromised by the ruptured sinus, causing aortic regurgitation. The patient underwent resection of the sinus aneurysm and aortic valve replacement with a bioprosthetic valve. Quadricuspid aortic valves are uncommon and are rarely associated with sinus of Valsalva aneurysm. The prevalence in the general population, clinical progression, and prognosis of this unique congenital abnormality are reviewed.

Pulmonary embolus caused by Suttonella indologenes prosthetic endocarditis in a pulmonary homograft.

A 28-year-old Cambodian man with a history of congenital heart disease presented with a 6-month history of increasing fatigue, night sweats, and weight loss. His surgical history included two Blalock-Taussig shunts, ventricular septal defect closure, and placement of a pulmonary valve conduit via a Rastelli procedure. Echocardiographic and cardiac computed tomographic studies revealed a vegetation in the pulmonary homograft. Blood cultures grew gram-negative rods that were eventually identified as Suttonella indologenes. The patient underwent a prolonged course of intravenous antibiotics, which was complicated by septic pulmonary embolism that clinically resolved. Bacterial endocarditis caused by aerobic gram-negative organisms is uncommon. The authors report the first case of S. indologenes endocarditis in a patient with complex congenital heart disease.

Mortality associated with adult congenital heart disease: Trends in the US population from 1979 to 2005.

BACKGROUND: Significant advances over the last 5 decades have allowed most patients with congenital heart disease to survive well past childhood and into adulthood. Population-based data from the United States are limited regarding mortality in adult survivors. METHODS: We used the Center for Disease Control Multiple Cause-of-Death registry to determine trends in mortality from 1979 to 2005 among individuals with congenital heart disease in the United States. RESULTS: There were significant reductions in death rates for adults with a number of congenital defects including ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, and Ebstein anomaly. Notably, when all ages were analyzed, there was a 71% decline in deaths associated with transposition of the great arteries (P = .001) and a 40% reduction in deaths associated with tetralogy of Fallot (P < .001). Mortality related to other lesions declined as well. Among adults with cyanotic lesions, the primary contributing cause of death was arrhythmia followed by heart failure. For adults with noncyanotic lesions, the major contributing cause before 1990 was arrhythmia; after 1990, myocardial infarction became the leading contributing cause of death. There was an overall decrease in the incidence of arrhythmia as the cause of death in all ages, particularly among children. CONCLUSIONS: Patients with congenital heart disease are living longer. Arrhythmia remains the primary contributing cause of death for those with cyanotic lesions. Myocardial infarction is now the leading contributing cause for adults with noncyanotic congenital heart disease consistent with late survival and an increasing impact of acquired heart disease.

Cardiac arrest in patients who smoke crack cocaine.

The aim of the study is to determine the clinical features and outcomes of cocaine users admitted to the hospital after cardiac arrest and compare them with nonusers. Cocaine is associated with cardiovascular complications, including ventricular arrhythmias; however, resuscitated cardiac arrest in relation to cocaine use is not a well-defined clinical entity. We reviewed available hospital charts at San Francisco General Hospital with the International Classification of Diseases, Ninth Revision diagnosis of cardiac arrest and cocaine use from 1994 to 2006. Clinical features and outcomes of cocaine users were compared with those of randomly selected control patients and age-matched controls with resuscitated cardiac arrest without cocaine use. We identified 22 patients with resuscitated cardiac arrest in the setting of cocaine use. Their average age was 42 +/- 10 years, >20 years younger than nonusers (68 +/- 16 years, p <0.01). After cardiac arrest, 12 of 22 patients (55%) who used cocaine had complete neurologic recovery in contrast to only 3 of 20 unmatched controls (15%, p <0.01) and 7 of 41 age-matched controls (17%, p <0.01). Only 10 of 22 cocaine users (46%) died compared with 15 of 20 unmatched controls (75%, p = 0.05) and 32 of 41 age-matched controls (78%, p <0.01). In a combined analysis of all patients, cocaine use was the only significant predictor of neurologic recovery (p <0.01) and survival (p <0.01). In conclusion, cocaine use is associated with cardiac arrest. In patients with cardiac arrest, cocaine users are younger than nonusers and more likely to survive with neurologic recovery, even compared with age-matched controls with cardiac arrest.

Perfusion of hearts with triglyceride-rich particles reproduces the metabolic abnormalities in lipotoxic cardiomyopathy.

Hearts with overexpression of anchored lipoprotein lipase (LpL) by cardiomyocytes (hLpL(GPI) mice) develop a lipotoxic cardiomyopathy. To characterize cardiac fatty acid (FA) and triglyceride (TG) metabolism in these mice and to determine whether changes in lipid metabolism precede cardiac dysfunction, hearts from young mice were perfused in Langendorff mode with [14C]palmitate. In hLpL(GPI) hearts, FA uptake and oxidation were decreased by 59 and 82%, respectively. This suggests reliance on an alternative energy source, such as TG. Indeed, these hearts oxidized 88% more TG. Hearts from young hLpL(GPI) mice also had greater uptake of intravenously injected cholesteryl ester-labeled Intralipid and VLDL. To determine whether perfusion of normal hearts would mimic the metabolic alterations found in hLpL(GPI) mouse hearts, wild-type hearts were perfused with [14C]palmitate and either human VLDL or Intralipid (0.4 mM TG). Both sources of TG reduced [14C]palmitate uptake (48% with VLDL and 45% with Intralipid) and FA oxidation (71% with VLDL and 65% with Intralipid). Addition of either heparin or LpL inhibitor P407 to Intralipid-containing perfusate restored [14C]palmitate uptake and confirmed that Intralipid inhibition requires local LpL. Our data demonstrate that reduced FA uptake and oxidation occur before mechanical dysfunction in hLpL(GPI) lipotoxicity. This physiology is reproduced with perfusion of hearts with TG-containing particles. Together, the results demonstrate that cardiac uptake of TG-derived FA reduces utilization of albumin-FA.