Stephen A. Hart

Long-term outcome and impact of surgery on adults with coronary arteries originating from the opposite coronary cusp.

Background— An anomalous coronary artery from the opposite sinus of Valsalva may increase sudden death risk in children and young adults, and surgical intervention is often recommended. The impact of this lesion when recognized in the adult and its management are ill defined. Methods and Results— We reviewed 210 700 cardiac catheterizations performed over a 35-year period at a single institution and identified 301 adults with an anomalous coronary artery from the opposite sinus of Valsalva, either anomalous right coronary artery from the left cusp or anomalous left main coronary artery from the right cusp. Patients were stratified by the pathway of the anomalous artery and the chosen treatment. Among the 301 patients with anomalous coronary artery from the opposite sinus of Valsalva (0.14% of the cohort), 79% had anomalous right coronary artery from the left cusp, and 18% had an interarterial course (IAC). Patients with IAC were younger (52±13 versus 59±13 years; P=0.001) and more likely to undergo surgical intervention (52% versus 27%; P<0.001), but mortality was not increased with IAC. Among the 54 patients with IAC, 28 underwent surgical repair with no perioperative deaths. Patients evaluated since 2000 were significantly more likely to be referred for surgery (P=0.004). Surgical patients were more likely to have abnormal stress tests (90% versus 43%; P=0.01) and had more extensive atherosclerosis but less diabetes mellitus (0% versus 23%; P=0.01). Long-term survival at 10 years appeared similar in both groups. Conclusions— In this single-center cohort study of patients with an anomalous coronary artery from the opposite sinus of Valsalva, surgical management appears to have been favored recently. Despite no perioperative mortality, a positive impact on long-term survival was not observed. The impact of surgery in older adults with anomalous coronary arteries arising from the opposite coronary sinus with IAC deserves further study.

Prevalence and repair of intraoperatively diagnosed patent foramen ovale and association with perioperative outcomes and long-term survival

CONTEXT A recent survey suggested that cardiothoracic surgeons may alter planned procedures to repair incidentally discovered patent foramen ovale (PFO). How frequently this occurs and the impact on outcomes remain unknown. OBJECTIVE To measure the frequency of incidentally discovered PFO closure during cardiothoracic surgery and determine its perioperative and long-term impact. DESIGN, SETTING, AND PATIENTS We reviewed the intraoperative transesophageal echocardiograms of 13,092 patients without prior diagnosis of PFO or atrial septal defect undergoing surgery at the Cleveland Clinic, Cleveland, Ohio, from 1995 through 2006. Postoperative outcomes were prospectively collected until discharge. MAIN OUTCOME MEASURES All-cause hospital mortality and stroke were predetermined primary outcomes; length of hospital stay, length of intensive care unit stay, and time on cardiopulmonary bypass were secondary outcomes. RESULTS Intraoperative PFO was diagnosed in 2277 patients in the study population (17%), and risk factors for stroke were similar in patients with and without PFO. After propensity matching was performed with the comparator groups, patients with PFO demonstrated similar rates of in-hospital death (3.4% vs 2.6%, P = .11) and postoperative stroke (2.3% vs 2.3%, P = .84). Surgical closure was performed in 639 PFO patients (28%), and surgeons were more likely to close defects in patients who were younger (mean [SD] age, 61.1 [14] vs 64.4 [13] years; P < .001), were undergoing mitral or tricuspid valve surgery (51% vs 32%, P < .001), or had history of transient ischemic attack or stroke (16% vs 10%, P < .001). Patients with repaired PFO demonstrated a 2.47-times greater odds (95% confidence interval, 1.02-6.00) of having a postoperative stroke compared with those with unrepaired PFO (2.8% vs 1.2%, P = .04). Long-term analysis demonstrated that PFO repair was associated with no survival difference (P = .12). CONCLUSIONS Incidental PFO is common in patients undergoing cardiothoracic surgery but is not associated with increased perioperative morbidity or mortality. Surgical closure appears unrelated to long-term survival and may increase postoperative stroke risk.

Association of anemia and long-term survival in patients with pulmonary hypertension

BACKGROUND Anemia is a marker of worsened clinical outcome in patients with heart failure from left ventricular dysfunction. Pulmonary hypertension often results in right ventricular dysfunction. Accordingly we sought to examine the association of hemoglobin levels and long-term all-cause mortality in a cohort of patients with pulmonary hypertension. METHODS Baseline demographic information, clinical characteristics and fasting blood work were obtained in a cohort of 145 patients with pulmonary hypertension referred for pulmonary vasodilator testing. Data was retrospectively analyzed with Cox-proportional hazards analysis. RESULTS Baseline characteristics of the cohort included age (mean±SD) 55.8±14.6 years, 75% women, 50% with idiopathic pulmonary hypertension, mean pulmonary artery pressure 46.1±14.2 mm Hg and arterial O(2) saturation 91±6 %. The most commonly utilized pulmonary hypertension specific therapeutic agents in descending order of frequency were epoprostenol (27%), sildenafil (21%), bosentan (17%), and treprostinil (6%). Over a median follow-up of 2.1 years, there were 39 deaths (26.9%). Patients who died had significantly lower hemoglobin levels than those survived (12.2±2.3 vs. 13.7±2.0, p<0.001). After adjustment for known predictors of death and pulmonary hypertension etiology, anemic patients were 3.3 times more likely to die than non-anemic patients (95% CI [1.43-7.51], p=0.005). CONCLUSIONS Hemoglobin levels closely parallel survival in pulmonary hypertension. Modification of anemia in this disorder could alter the clinical course and calls for further research in this area.

Impact of diabetes in patients with pulmonary hypertension

Diabetes complicates management in a number of disease states and adversely impacts survival; how diabetes affects patients with pulmonary hypertension (PH) has not been well characterized. With insulin resistance having recently been demonstrated in PH, we sought to examine the impact of diabetes in these patients. Demographic characteristics, echo data, and invasive hemodynamic data were prospectively collected for 261 patients with PH referred for initial hemodynamic assessment. Diabetes was defined as documented insulin resistance or treatment with antidiabetic medications. Fifty-five patients (21%) had diabetes, and compared with nondiabetic patients, they were older (mean years ± SD, 61 ± 13 vs. 56 ± 16; P = 0.02), more likely to be black (29% vs. 14%; P = 0.02) and hypertensive (71% vs. 30%; P < 0.001), and had higher mean (±SD) serum creatinine levels (1.1 ± 0.5 vs. 1.0 ± 0.4; P = 0.03). Diabetic patients had similar World Health Organization functional class at presentation but were more likely to have pulmonary venous etiology of PH (24% vs. 10%; P = 0.01). Echo findings, including biventricular function, tricuspid regurgitation, and pressure estimates were similar. Invasive pulmonary pressures and cardiac output were similar, but right atrial pressure was appreciably higher (14 ± 8 mmHg vs. 10 ± 5 mmHg; P < 0.001). Despite similar management, survival was markedly worse and remained so after statistical adjustment. In summary, diabetic patients referred for assessment of PH were more likely to have pulmonary venous disease than nondiabetic patients with PH, with hemodynamics suggesting greater right-sided diastolic dysfunction. The markedly worse survival in these patients merits further study.

When 'blue babies' grow up: What you need to know about tetralogy of Fallot.

Most babies born with tetralogy of Fallot undergo corrective surgery and survive to adulthood. However, as they get older they are prone to a number of long-term problems, and they often do not receive expert-level follow-up care. This review of the adult complications of tetralogy of Fallot should help primary care practitioners identify these patients, make appropriate and timely referrals, and educate patients and their families. Children born with tetralogy of Fallot and other congenital heart defects are living longer—long enough, eventually, to present to your clinic.

Impact of fibrinogen levels on angiographic progression and 12-year survival in the armed forces regression study.

We assessed the role of fibrinogen levels on angiographic progression and long-term survival among 111 patients with coronary disease enrolled in the Armed Forces Regression Study (AFREGS). Baseline fibrinogen levels and quantitative coronary angiography were performed initially and at 30 months. Progression or nonregression of coronary disease was more prevalent in patients with high fibrinogen than patients with normal fibrinogen (66.1% vs 45.5%; P = .022). Twelve-year cardiovascular (CV) mortality was substantially higher if fibrinogen was elevated (17.9% vs 3.6%, P = .016). Among patients with elevated fibrinogen and angiographic progression or nonregression, there were 10 deaths and all were CV. Elevated levels of fibrinogen predict the angiographic progression of existing coronary disease and likelihood of CV death. Among patients with elevated levels of fibrinogen, angiographic progression identifies a significantly increased likelihood of a fatal CV event.

Atheromatous disease of the aorta and perioperative stroke

From the Division of Cardiovascular and Thoracic Surgery, Department of Surgery, and Division of Cardiology, Department of Medicine, Duke University Medical Center, Durham, NC; Cleveland Clinic Lerner College of Medicine of CaseWestern Reserve University, Cleveland, Ohio; Departments of Cardiovascular Medicine and Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio; and Department of Vascular Medicine, Vanderbilt Heart and Vascular Institute, Nashville, Tenn. Received for publication March 8, 2017; revisions received July 24, 2017; accepted for publication Aug 24, 2017. Address for reprints: Richard A. Krasuski, MD, Department of Cardiovascular Medicine, Duke University Health System, Durham, NC 27710 (E-mail: richard.krasuski@duke.edu). J Thorac Cardiovasc Surg 2017;-:1-9 0022-5223/

PINOT NOIR: Pulmonic INsufficiency imprOvemenT with Nitric Oxide Inhalational Response

36.00 Copyright 2017 by The American Association for Thoracic Surgery https://doi.org/10.1016/j.jtcvs.2017.08.132

Modified INOvent for delivery of inhaled nitric oxide during cardiac MRI.

BackgroundTetralogy of Fallot (TOF) repair and pulmonary valvotomy for pulmonary stenosis (PS) lead to progressive pulmonary insufficiency (PI), right ventricular enlargement and dysfunction. This study assessed whether pulmonary regurgitant fraction measured by cardiovascular magnetic resonance (CMR) could be reduced with inhaled nitric oxide (iNO).MethodsPatients with at least moderate PI by echocardiography undergoing clinically indicated CMR were prospectively enrolled. Patients with residual hemodynamic lesions were excluded. Ventricular volume and blood flow sequences were obtained at baseline and during administration of 40 ppm iNO.ResultsSixteen patients (11 with repaired TOF and 5 with repaired PS) completed the protocol with adequate data for analysis. The median age [range] was 35 [19–46] years, BMI was 26 ± 5 kg/m2 (mean ± SD), 50% were women and 75% were in NYHA class I. Right ventricular end diastolic volume index for the cohort was 157 ± 33 mL/m2, end systolic volume index was 93 ± 20 mL/m2 and right ventricular ejection fraction was 40 ± 6%. Baseline pulmonary regurgitant volume was 45 ± 25 mL/beat and regurgitant fraction was 35 ± 16%. During administration of iNO, regurgitant volume was reduced by an average of 6 ± 9% (p=0.01) and regurgitant fraction was reduced by an average of 5 ± 8% (p=0.02). No significant changes were observed in ventricular indices for either the left or right ventricle.ConclusioniNO was successfully administered during CMR acquisition and appears to reduce regurgitant fraction in patients with at least moderate PI suggesting a potential role for selective pulmonary vasodilator therapy in these patients.Trials registrationClinicalTrials.gov, NCT00543933

Response to Letter Regarding Article, “Long-Term Outcome and Impact of Surgery on Adults With Coronary Arteries Originating From the Opposite Coronary Cusp”

BACKGROUND The aim of this study was to assess the feasibility of delivering NO through a modified system to allow clearance of the magnetic field and thus compatibility with cardiac magnetic resonance (CMR). Nitric oxide (NO) is an inhalational, selective pulmonary vasodilator with a wide range of applications in a variety of disease states, including diseases that affect the right ventricle. Accurate assessment of dynamic changes in right ventricular function necessitates CMR; however, delivery of NO is only possible using equipment that is not magnetic resonance imaging (MRI) compatible (INOvent delivery system, Ohmeda, Inc., Madison, WI, USA). METHODS The INOvent delivery system was modified by using 35 ft. of standard oxygen tubing to allow NO delivery through an electrical conduit and into the MRI suite. The concentrations of oxygen (O(2)), nitrogen dioxide (a harmful byproduct, NO(2)) and NO were measured in triplicate using the built-in electrochemical analyzer on the INOvent. After confirmation of safety, the system was used to administer drug to a patient x, and dynamic MRI measurements were performed. RESULTS When the standard INOvent was set to administer 40 ppm of NO, the mean/standard deviation of gas delivered was as follows: NO: 42/0 ppm; NO(2): 0.3/0.1 ppm; and O(2): 93/0 ppm. In comparison, the gas delivery of the modified INOvent was follows: NO: 41/0 ppm; NO(2): 0.5/0 ppm; and O(2): 93.7/0.6 ppm. During administration to an index patient with severe pulmonic insufficiency (PI), a measurable reduction in PI was observed by CMR. CONCLUSIONS Nitric oxide can be administered through 35 ft. of standard oxygen tubing without significantly affecting dose delivery. This technique has potential application in patients with right-sided structural heart disease for determination of dynamic physiological changes.