Subclinical Burden of Coronary Artery Calcium in Patients With Coarctation of the Aorta.

Coronary computed tomography (CT) angiography is often performed in adults with coarctation of the aorta (CoA) for anatomic assessment. As this population ages, assessment of atherosclerotic cardiovascular disease burden is important. Thus, quantitative and qualitative coronary artery calcium (CAC) scores were assessed for patients with CoA ≥16 years of age, who were seen at a referral center. CoA patients had either coronary CT angiography or chest CT with interpretable coronary information performed for clinical indications (follow-up, preoperative, or for symptoms) from 2004 to 2017. Qualitative CAC was determined based on low-dose CT and lung cancer screening protocols. Quantitative CAC scores were compared with an age- and gender-matched control cohort of patients chosen from an emergency department database of patients who received coronary CT angiography for chest pain evaluation. Atherosclerotic cardiovascular disease 10-year predicted risk scores were calculated for both cohorts. Out of 131 patients with CoA (mean age 46.1 ± 15.3 years), 22 patients (17%) had multivessel atherosclerotic disease on qualitative assessment. In the subgroup of patients ≥40 years, those with CoA were more likely to have a quantitative CAC score ≥400 compared with those without CoA (14% vs 4%, p = 0.02). Median atherosclerotic cardiovascular disease risk score was 8% (interquartile range 2% to 12%) for CoA patients ≥40 years, and 5% (interquartile range 2% to 9%) for patient without CoA ≥40 years. In conclusion, we determined that CoA patients have subclinical atherosclerosis identifiable on CT in high rates when compared with patients without CoA. Atherosclerotic cardiovascular disease should be assessed in these patients for prevention and treatment.

Familial Anomalous Origin of Right Coronary Artery from the Left Coronary Sinus.

Anomalous origin of a coronary artery from the opposite sinus of Valsalva is a rare congenital anomaly. The prevalence of familial clustering of coronary artery anomalies is unknown. Here we describe the case of a father and son, both of whom presented with major adverse cardiac events due to Anomalous origin of a coronary artery from the opposite sinus of Valsalva and both had right coronary artery arising from the left coronary cusp with an interarterial course.

The Clinical Significance of Relative Bradycardia.

INTRODUCTION: Relative bradycardia is a poorly understood paradoxical phenomenon that refers to a clinical sign whereby the pulse rate is lower than expected for a given body temperature. OBJECTIVE: To provide an overview and describe infectious and noninfectious causes of relative bradycardia. METHODS: PubMed and Medline databases were searched using individual and Medical Subject Headings terms including relative bradycardia, fever, pulse-temperature dissociation and pulsetemperature deficit in human studies published from inception to October 2, 2016. The causes and incidence of relative bradycardia were reviewed. RESULTS: Relative bradycardia is found in a wide variety of infectious and noninfectious diseases. The pathogenesis remains poorly understood with proposed mechanisms including release of inflammatory cytokines, increased vagal tone, direct pathogenic effect on the myocardium, and electrolyte abnormalities. The incidence of this sign varies widely, which may be attributable to multiple factors, including population size, time course for measuring pulse and temperature, and lack of a consistent definition used. The fact that this sign is not consistently identified in case series suggests that relative bradycardia is caused by mechanisms presumably involving or influenced by pathogen and host factors. CONCLUSIONS: Relative bradycardia is a sensitive but nonspecific clinical sign that may be an important bedside tool for narrowing the differential diagnosis of potential infectious and noninfectious etiologies. Recognizing this relationship may assist the clinician by providing bedside clinical clues into potential etiologies of disease, particularly in the setting of infectious diseases and in circumstances when other stigma of disease is absent.

Impaired right ventricular reserve predicts adverse cardiac outcomes in adults with congenital right heart disease.

OBJECTIVE: The prevalence of heart failure (HF) among adult patients with congenital heart disease (ACHD) is rising. Right ventricle (RV) exercise reserve and its relationship to outcomes have not been characterised. We aim to evaluate the prognostic impact of impaired RV reserve in an ACHD population referred for cardiopulmonary exercise testing (CPET). METHODS: This retrospective study evaluates patients with ACHD who underwent CPET (n=147) with first-pass radionuclide ventriculography at a single tertiary care centre. RV reserve was categorised as normal, mild to moderately or severely impaired. The primary composite clinical outcome included clinical right HF, arrhythmia, transplantation or death. RESULTS: Patients were median age 41±13 years, 50% were female and median follow-up was 1.1 (IQR: 0.7-2.0) years. Exercise RV reserve was impaired in 103 patients (70%), of whom 32% were asymptomatic. Resting RV systolic function poorly predicted RV reserve, with 52% of patients with severe impairment having a qualitatively normal echocardiographic assessment. The severely impaired reserve group had lower peak oxygen consumption (VO2)(17.2 vs 22.5 mL/kg/min, p<0.0001) compared with the normal reserve group, and was more likely to develop the composite outcome (48% vs 9%, log-rank p<0.001). Severely impaired RV reserve predicted event-free survival after adjusting for peak VO2, age, sex, RV pathology, QRS duration, New York Heart Association class, resting RV ejection fraction and RV dilation by echocardiography or MRI (HR 3.7, 95% CI 1.1 to 13.0, p=0.039). CONCLUSION: Impaired RV reserve, occurred in asymptomatic patients, was not well predicted by resting systolic function assessment, and strongly predicted adverse cardiovascular outcomes.

Frequency of Guideline-Based Statin Therapy in Adults With Congenital Heart Disease.

We aimed to evaluate atherosclerotic cardiovascular disease (ASCVD) risk estimates and guideline-based statin use for primary prevention of ASCVD in adults with congenital heart disease (ACHD). This was a case-controlled, retrospective study of 248 cases and 744 age- and gender-matched controls at a tertiary care referral center. ASCVD risk scores were calculated and used to assess indication for statin treatment for primary prevention per the 2013 American College of Cardiology and American Heart Association guideline on assessment of cardiovascular risk. There were no differences in average 10-year ASCVD risk scores between ACHD cases (4.6% ± 6.6%) and matched controls (5.1% ± 6.7%, p = 0.32). ACHD cases had lower total cholesterol (183 ± 38 vs 192.6 ± 35.3 mg/dL, p < 0.001) and were less likely to smoke (8.1% vs 14.6%, p = 0.008), yet had lower high density lipoprotein (52.6 ± 17.2 vs 55.3 ± 17.1 mg/dL, p = 0.03) and higher hypertension rates (38.7% vs 28.5%, p = 0.003). However, only 42.3% ACHD cases with a primary prevention statin indication were appropriately prescribed therapy as compared with 59.0% of controls (p = 0.04). In conclusion, ACHD cases have a similar 10-year ASCVD risk score than age- and gender-matched peers, but ACHD cases are less likely than their peers to be prescribed statin therapy for primary prevention per guideline-based recommendations.

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.