Ada Stefanescu

The Challenge of Congenital Heart Disease Worldwide: Epidemiologic and Demographic Facts

Congenital heart disease (CHD) afflicts a large number of children every year. The incidence of CHD is generally considered to be 8 per 1,000 live births. However, this estimate is perhaps inaccurate and does not take into consideration regional differences. A large review of the literature was performed to establish the true incidence of CHD and geographical variations. Data on the incidence of specific lesions and their geographical variation, as well as on mortality from CHD, was also reviewed. Taking into consideration the available data on incidence, mortality, and access to care, the global challenge that CHD represents was analyzed. Insight into how to confront this challenge is given.

Prognostic value of plasma myeloperoxidase concentration in patients with stable coronary artery disease

BACKGROUND There are no studies yet on the usefulness of myeloperoxidase (MPO) as a prognostic tool in patients with stable coronary artery disease (CAD). METHODS The study included 382 patients with clinical and angiographic confirmation of stable CAD. Blood samples for MPO measurement were taken before angiography. Myeloperoxidase was determined using an enzyme immunoassay. The primary end point of the study was all-cause mortality. RESULTS Patients were categorized into 2 groups: the high-MPO group included patients in the third tertile of MPO levels (>75.0 microg/L; 127 patients), and the low-MPO group included patients in the first (<52.6 microg/L) and second tertiles (52.6-75.0 microg/L) of MPO levels (255 patients). The median follow-up was 3.5 [3.3-4.8] years. There were 35 deaths (9.2%) during the follow-up. The MPO concentration was 60.1 [47.0; 83.8] microg/L in survivors and 72.7 [54.8; 105.1] microg/L in nonsurvivors (P = .06). There were 17 deaths in the high-MPO level and 18 deaths in the low-MPO group: Kaplan-Meier estimates of mortality were 18.3% and 10.5% with an odds ratio of 1.96 (95% confidence interval [1.02-3.76], P = .04). The Cox proportional hazards model adjusting for correlates of mortality showed that plasma MPO was not an independent correlate of mortality (hazard ratio 1.06, 95% confidence interval [0.71-1.59], P = .77 for 1 SD increase in the log variable). CONCLUSION Although elevated plasma MPO concentration is associated with a more advanced cardiovascular risk profile, plasma MPO does not predict mortality independent of other cardiovascular risk factors in patients with stable CAD.

Anomalous Aortic Origin of a Coronary Artery: Surgical Repair With Anatomic- and Function-Based Follow-Up

BACKGROUND Anomalous aortic origin of the coronary artery (AAOCA) with an interarterial (IAC) course is an uncommon congenital anomaly. Surgical indications and repair techniques have evolved. We have managed 259 adult patients with AAOCA over 40 years. Our management strategy includes anatomic- and function-based surveillance to select surgical candidates. We reviewed our surgical cohort and analyzed anatomic and functional outcomes. METHODS We queried our heart center databases to obtain the names of all patients with AAOCA managed at our institution between 1974 and 2014. We performed a retrospective chart review. RESULTS Two hundred fifty-nine patients were managed for AAOCA. Sixty-one underwent surgical intervention. Twenty-six with associated coronary atherosclerosis were excluded. Thirty-one who underwent surgical repair were analyzed. Mean age was 42.5 ± 2.7 years. Twenty-four patients (77.4%) had right AAOCA. Six (19.4%) had left AAOCA. One (3.2%) had bilateral coronary anomalies. Repair techniques included 21 unroofing procedures (67.7%), 6 translocations (19.4%), and 4 coronary artery bypass grafting (CABG) procedures (12.9%). Mean follow-up was 3.8 ± 0.8 years. Thirteen patients underwent follow-up anatomic testing with computed tomography. Twelve of these patients had widely patent coronary arteries, and 1 patient had mild coronary artery stenosis. Seventeen patients underwent functional testing. Fifteen of these patients had no evidence of ischemia. One patient had reversible ischemia after CABG, and 1 had subclinical ischemia after unroofing. There was 1 late mortality from endocarditis. CONCLUSIONS Our multidisciplinary program uses a treatment algorithm to select patients with AAOCA for surgical intervention. Only a small subset requires an operation, and we favor unroofing and translocation techniques. With this paradigm, outcomes are excellent, as validated with anatomic- and function-based testing.

Predicting Outcomes Using the Heart Failure Survival Score in Adults with Moderate or Complex Congenital Heart Disease

BACKGROUND Adults with congenital heart disease (CHD) face increased risk for morbidity and mortality with age, but few prognostic models exist. OBJECTIVE This study aims to assess whether the Heart Failure Survival Score (HFSS), which risk stratifies patients for heart transplantation, predicts outcomes in adults with moderate or complex CHD. METHODS This was a multicenter, retrospective study which identified 441 patients with moderate or complex CHD between 2005 and 2013, of whom 169 had all the HFSS parameters required to calculate the risk score. Because all study patients were deemed low risk by the HFSS, the score was dichotomized at the median (10.4). Outcomes included death, transplant or ventricular assist device (VAD), arrhythmia requiring treatment, nonelective cardiovascular (CV) hospitalizations, and the composite. Associations of mean HFSS and HFSS <10.4 with each outcome were assessed. RESULTS The cohort had mean ± standard deviation age of 33.6 ± 12.6 years, peak VO2 21.8 ± 7.5 mL/kg/min, HFSS of 10.45 ± 0.88, and median years follow-up of 2.7 (1.1, 5.2). There were five deaths (2.8%), no transplants or VADs, 25 arrhythmias (14.8%), 22 CV hospitalizations (13%), and 39 composites (23.1%). Lower mean HFSS was observed for patients who died (9.6 ± 0.83 vs. 10.5 ± 0.87, P = .02), arrhythmia requiring treatment (10.0 ± 0.70 vs. 10.5 ± 0.89, P = .005), CV hospitalizations (9.9 ± 0.73 vs. 10.5 ± 0.88, P = .002), and the composite (10.0 ± 0.70 vs. 10.6 ± 0.89, P < .001). The positive and negative predictive values of HFSS <10.4 for the composite were 34% and 88% respectively, with sensitivity and specificity 74% and 56%. CONCLUSIONS Although a low HFSS was significantly associated with outcomes, it did not adequately risk stratify adults with CHD, whose heterogeneous pathophysiology differs from that of the acquired heart failure population. Further studies are warranted to provide a more accurate prognosis.

Heart Failure in Adult Congenital Heart Disease

Opinion statementAdult congenital heart disease (ACHD) patients represent a special population in modern cardiology: though their numbers are growing, and they represent a high-resource utilization subgroup, a robust evidence-base of randomized trials is lacking. Much of the standard therapy is adapted from the treatment of ischemic and idiopathic left ventricle systolic failure, with a small, but growing body of evidence on medical therapy in select ACHD diagnoses. At our institution, for instance, there is a long tradition of using angiotensin antagonists in patients with a systemic right ventricle to prevent deleterious remodeling. The effects of beta-blockers on functional class in ACHD are yet unproven, but there is promising data on pulmonary vasodilators. Control of coronary risk factors and aerobic exercise should be considered for all. Prevention of arrhythmias is important, and multi-site pacing is an emerging therapy. New prognostic tools including natriuretic peptides and CPET are increasingly used to guide earlier initiation of these therapies.

HEART DISEASE ON HEARTBREAK HILL: ATYPICAL ACUTE CORONARY SYNDROME DURING THE BOSTON MARATHON

Acute complications of underlying coronary artery disease are the most common cause of sudden death during long-distance running events. Their incidence is rising as the participant pool becomes larger, older, and more medically diverse. A 57 year-old healthy woman developed nausea, mild chest and

MULTIORGAN INVOLVEMENT OF AMYLOID: HOW TO FLY UNDER THE RADAR

case: A 60 years-old gentleman was admitted for the second time in two months for dyspnea. He had a history of hypertension, idiopathic patchy motor neuropathic progress for 2 months, dysphagia and voice hoarseness for 6 months, and cryptomeningitis 10 years ago. Physical examination revealed a cachectic man with a jugular venous pressure of 8cm above the sternal angle, a normal S1 and S2, an S3, no murmurs, and decreased breath sounds to the mid right lung field. His EKG showed a normal sinus rhythm with low voltages. An echocardiogram showed significant biventricular hypertrophy and a depressed ejection fraction of 20%.

ARRHYTHMIAS AND CARDIOGENIC SHOCK AS A PRESENTATION OF HYPERTHERMIA

The incidence of exertional heat illness is rising in the United States. It is a rare cause of arrhythmias, direct myocardial injury and distributive shock, and carries a high rate of mortality if not treated promptly. A 45 years-old gentleman was admitted for altered mental status after he was

Use of the Labscale Tangential Flow Diafiltrator to Remove Tetrameric Hemoglobin from Polyhemoglobin, Purify Hemolysate, and Concentrate Polyhemoglobin

The Labscale tangential flow diafiltration system in conjunction with polyethersulfone Biomax filters (both purchased from Millipore Inc.) were used in the following studies: (1) A crucial step in the preparation of polyhemoglobin (polyHb) is the removal of tetrameric hemoglobin (Hb), which can cause adverse side-effects. The efficiency for this depends on the integrity of the 100kDa filters. Those with lower integrity were not effective whereas those with a higher integrity of 0.075ml/min were more effective. A filter with an integrity of 0.075ml/min can reduce the initial 11.2% tetrameric Hb concentration to 1.3% using a flow rate of 0.2ml/min. Higher flow rate of 2.2ml/min was not as effective. (2) Filtration with a 100kDa filter was quick and efficient in separating stroma-free hemoglobin solutions from cellular debris from hemolysate. (3) Both 5kDa and 100kDa filters are efficient in concentrating hemoglobin solutions.