Fernando Baraona

Galectin‐3 Is Elevated and Associated With Adverse Outcomes in Patients With Single‐Ventricle Fontan Circulation

Background Galectin‐3 may play a role in cardiac and noncardiac fibrosis, and elevated circulating levels of this protein predict adverse outcomes in patients with heart failure who do not have congenital heart disease. We investigated galectin‐3 in adults with single‐ventricle Fontan circulation, patients who are prone to premature clinical deterioration in the context of extensive multiorgan fibrosis. Methods and Results We measured plasma galectin‐3 concentrations in 70 ambulatory adult Fontan patients and 21 age‐ and sex‐matched control participants. Galectin‐3 level was significantly higher in the Fontan group (11.85 ng/mL, interquartile range 9.9 to 15.0 ng/mL) versus the control group (9.4 ng/mL, interquartile range 8.2 to 10.8 ng/mL; P<0.001). Among Fontan patients, galectin‐3 was positively correlated with age, uric acid, and high‐sensitivity C‐reactive protein and negatively correlated with estimated glomerular filtration rate. There was no significant relationship between galectin‐3 and oxygen saturation, Fontan type, or ventricular morphology. Over a median follow‐up of 461 days, 15 events occurred among the Fontan patients: 12 nonelective hospitalizations (with 2 subsequent deaths) and 3 deaths without prior hospitalization. Patients with elevated galectin‐3 (n=19, defined as >2 SD above the control group mean value) had a higher risk of nonelective hospitalization or death (hazard ratio 6.0, 95% CI 2.1 to 16.8, P<0.001). This relationship persisted after individual adjustment for covariates including age, New York Heart Association functional class, C‐reactive protein, and estimated glomerular filtration rate and after multivariable adjustment for independently predictive covariates (hazard ratio 9.2, 95% CI 2.4 to 35.2, P=0.001). Conclusions Galectin‐3 concentrations are elevated among adults with a Fontan circulation, and elevated galectin‐3 is associated with an increased risk of nonelective cardiovascular hospitalization or death.

Estimated glomerular filtration rate and urine biomarkers in patients with single-ventricle Fontan circulation

Objectives To define whether adults with a Fontan circulation, who have lifelong venous congestion and limited cardiac output, have impaired glomerular filtration rate (GFR) or elevated urinary biomarkers of kidney injury. Methods We measured circulating cystatin C and creatinine (n=70) and urinary creatinine, albumin, kidney injury molecule-1 (KIM-1), neutrophil gelatinase-associated lipocalin (NGAL) and N-acetyl glucosaminidase (NAG) (n=59) in ambulatory adult Fontan patients and 20 age-matched and sex-matched controls. Urinary biomarkers were normalised to urine creatinine concentration. Survival free from non-elective cardiovascular hospitalisation was compared by estimated GFR and urinary biomarker levels using survival analysis. Results Cystatin C GFR was lower in the Fontan group compared with controls (114.2±22.8 vs 136.3±12.8 mL/min/1.73 m2, p<0.0001); GFR<90 mL/min/1.73 m2 in 14.3% vs 0% of controls. Albumin-to-creatinine ratio (ACR), KIM-1 and NAG were elevated compared with controls; ACR=23.2 (7.6–38.3) vs 3.6 (2.5–5.7) mg/g, p<0.0001; NAG=1.8 (1.1–2.6) vs 1.1 (0.9–1.6) U/g, p=0.02; KIM-1=0.91 (0.52–1.45) vs 0.33 (0.24–0.74) ng/mg, p=0.001. Microalbuminuria, ACR>30 mg/g, was present in 33.9% of the Fontan patients but in none of the controls. Over median 707 (IQR 371–942)-day follow-up, 31.4% of patients had a clinical event. Higher KIM-1 and NAG were associated with higher risk of non-elective hospitalisation or death (HR/+1 SD=2.1, 95% CI 1.3 to 3.3, p=0.002; HR/+1 SD=1.6, 95% CI 1.05 to 2.4, p=0.03, respectively); cystatin C GFR was associated with risk of the outcome (HR/+1 SD=0.66, 95% CI 0.48 to 0.90, p=0.009) but creatinine-based GFR was not (HR/+1 SD=0.91, 95% CI 0.61 to 1.38, p=0.66). Neither ACR nor NGAL was associated with events. Conclusions The Fontan circulation is commonly associated with reduced estimated GFR and evidence for glomerular and tubular injury. Those with lower cystatin C GFR and tubular injury are at increased risk of adverse outcomes.

Coronary Arteries in Childhood Heart Disease: Implications for Management of Young Adults.

Survival of patients with congenital heart defects has improved dramatically. Many will undergo interventional catheter or surgical procedures later in life. Others will develop atherosclerotic or post-surgical coronary heart disease. The coronary artery anatomy in patients with congenital heart disease differs substantially from that seen in the structurally normal heart. This has implications for diagnostic procedures as well as interventions. The unique epicardial course seen in some defects could impair interpretation of coronary angiograms. Interventional procedures, especially at the base of the heart, risk injuring unusually placed coronary arteries so that coronary artery anatomy must be delineated thoroughly prior to the procedure. In this review, we will describe the variants of coronary artery anatomy and their implications for interventional and surgical treatment and for sudden death during late follow-up in several types of congenital heart defects including: tetralogy of Fallot, truncus arteriosus, transposition of the great arteries, double outlet right ventricle, congenitally corrected transposition of the great arteries and defects with functionally one ventricle. We will also discuss the coronary abnormalities seen in Kawasaki disease.

Acute effect of iloprost inhalation on right atrial function and ventricular dyssynchrony in patients with pulmonary artery hypertension.

Right atrium function and ventricular function have significant prognostic value in pulmonary arterial hypertension patients. Acute changes in right ventricular synchrony and right atrium function postiloprost inhalation have not been evaluated.

Sobrevida a mediano plazo en los pacientes con hipertensión arterial pulmonar en la era de terapias vasodilatadoras específicas del territorio vascular pulmonar

Background: Pulmonary arterial hypertension (PAH) is a rare and progressive disease. Long-term survival remains poor despite of advances in specific vasodilator therapy. Aim: To describe the survival rate in a cohort of PAH patients in two referral centers in Chile. Patients and methods: One hundred fifteen patients aged 43 ± 15.6 years (85 % females) with PAH qualified for this study. Their median pulmonary artery pressure was 55.4 ±14 mm Hg and their six minutes walking capacity was 368 ± 119 m. They were followed for 58 ± 0.4 months and their actual survival rates were compared with the estimated survival using the equation proposed by the French registry of PAH. Results: One, two and three year survival rates were 97, 94 and 89%, respectively. The observed survival rates were greater than the estimated survival. Conclusions: The improvement in survival rates observed in this cohort of patients is similar to what has been described in literature.

Spontaneous rupture of tricuspid valve papillary muscle in pulmonary hypertension secondary to HIV infection. Report of one case

Acute primary tricuspid regurgitation (TR) secondary to papillary muscle rupture is an extremely rare clinical situation. We report a 42-year-old male with pulmonary artery hypertension (PAH) secondary to HIV infection, who presented with an acute TR due to spontaneous papillary muscle rupture. He remained in cardiogenic shock despite therapy with inotropic drugs and pulmonary vasodilator therapy. He was subjected to a tricuspid valve replacement. In the postoperative period the patient had severe PAH, which was successfully controlled with inhaled nitric oxide. Tricuspid valve replacement and adjunctive use of pulmonary vasodilator therapy can be a life saving and useful approach in this condition.Acute primary tricuspid regurgitation (TR) secondary to papillary muscle rupture is an extremely rare clinical situation. We report a 42 years old male with pulmonary artery hypertension (PAH) secondary to HIV infection, who developed an acute TR due to spontaneous papillary muscle rupture. He remained in cardiogenic shock despite therapy with inotropic drugs and pulmonary vasodilators. He was subjected to a tricuspid valve replacement. In the postoperative period the patient had severe PAH, which was successfully managed with inhaled nitric oxide. Tricuspid valve replacement and adjunctive use of pulmonary vasodilator therapy can be a live saving and useful approach in this condition.

Rotura espontánea de válvula tricúspide en un paciente con hipertensión pulmonar secundaria a VIH

Acute primary tricuspid regurgitation (TR) secondary to papillary muscle rupture is an extremely rare clinical situation. We report a 42-year-old male with pulmonary artery hypertension (PAH) secondary to HIV infection, who presented with an acute TR due to spontaneous papillary muscle rupture. He remained in cardiogenic shock despite therapy with inotropic drugs and pulmonary vasodilator therapy. He was subjected to a tricuspid valve replacement. In the postoperative period the patient had severe PAH, which was successfully controlled with inhaled nitric oxide. Tricuspid valve replacement and adjunctive use of pulmonary vasodilator therapy can be a life saving and useful approach in this condition.Acute primary tricuspid regurgitation (TR) secondary to papillary muscle rupture is an extremely rare clinical situation. We report a 42 years old male with pulmonary artery hypertension (PAH) secondary to HIV infection, who developed an acute TR due to spontaneous papillary muscle rupture. He remained in cardiogenic shock despite therapy with inotropic drugs and pulmonary vasodilators. He was subjected to a tricuspid valve replacement. In the postoperative period the patient had severe PAH, which was successfully managed with inhaled nitric oxide. Tricuspid valve replacement and adjunctive use of pulmonary vasodilator therapy can be a live saving and useful approach in this condition.

Superioridad del nuevo puntaje de riesgo ACC/AHA 2013 por sobre el puntaje de Framingham, en la predicción de riesgo de mortalidad cardiovascular en Santiago

Background: Recently, the American College of Cardiology and American Heart Association (ACC/AHA) proposed a new cardiovascular risk (CV) score. Aim: To evaluate the new risk score (ACC/AHA 2013) in a Chilean population. Material and methods: Between 2002 and 2014, body mass index (BMI), waist circumference, blood pressure, lipid profile and fasting blood glucose levels were measured and a survey about CV risk factors was responded by 3284 subjects aged 40 to 79 years (38% females), living in urban Santiago. ACC/AHA 2013, FRAM and Chilean FRAM scores were calculated. All-cause mortality was determined in July 2014 by consulting the death Registry of the Chilean Identification Service, with an average follow up of 7 ± 3 years. Results: The prevalence of risk factors were 78% for dyslipidemia, 37% for hypertension, 20% for smoking, 7% for diabetes, 20% for obesity and 54% for physical inactivity. The mean FRAM, Chilean FRAM and ACC/AHA scores were 8, 3 and 9%, respectively. During follow-up, 94 participants died and 34 deaths were of cardiovascular cause. Participants who died had a higher prevalence of hypertension (p<0.01) and diabetes (p<0. 01) and tended to be older (p=0.06). The FRAM score for 10 years for deceased and surviving patients was 12 and 8%, respectively (p = NS). The figures for the Chilean FRAM were 5 and 2%, respectively (p=0. 09). The figures for the ACC/AHA 2013 score were 33 and 9%, respectively (p =0.04). According to receiver operating characteristic curves, ACC/AHA 2013 had a higher area under de curve for CV mortality than FRAM and Chilean FRAM. Conclusions: The new ACC / AHA 2013 score, is better than traditional FRAM and Chilean FRAM scores in predicting cardiovascular mortality in a low risk population.

Does Good Aerobic Capacity Attenuate the Effects of Aging on Cardiovascular Risk Factors? Results from a Cross-Sectional Study in a Latino Population

Background. High aerobic capacity is associated with low cardiovascular (CV) risk. The aim of this study was to determine the CV RF burden in subjects with aerobic capacity ≥10 METs and compare it with those having <10 METs. Methods. Cross-sectional study in 2646 subjects (mean age 48 ± 12 years). Demographics, medical history, physical activity, cardiovascular RFs, fasting lipids and blood glucose levels, blood pressure, and anthropometric measurements were collected. Aerobic capacity was determined by exercise stress test. The ACC/AHA 2013 pooled cohort equation was used to calculate CV risk. Logistic models were built to determine the probability of having ≥2 RFs versus 0‐1 RF, by age and sex, according to aerobic capacity. Results. 15% of subjects had aerobic capacity < 10 METs. The ACC/AHA scores were 15% in men and 6% in women with <10 METs and 5% and 2%, respectively, in those with ≥10 METs. The probability of having ≥2 RFs increased with age in both groups; however, it was significantly higher in subjects with <10 METs (odds ratio [OR]: 2.54; 95% CI: 1.92–3.35). Conclusions. Aerobic capacity ≥ 10 METs is associated with a better CV RF profile and lower CV risk score in all age groups, regardless of gender.

Remodelado auricular derecho y niveles plasmáticos de Galectina-3 se relacionan con la capacidad funcional de pacientes con hipertensión arterial pulmonar

Introduccion: En pacientes con hipertension arterial pulmonar (HAP) Galectina- 3, biomarcador de fibrosis miocardica, se ha asociado a marcadores ecocardiograficos de remodelado ventricular derecho. La relacion entre Galectina- 3, remodelado auricular derecho (AD) y capacidad funcional (CF) en pacientes con HAP no ha sido explorado. El objetivo fue medir niveles de Galectina-3 y su relacion con CF y remodelado AD en pacientes con HAP Metodos: Estudio prospectivo observacional en que se incluyeron 14 pacientes con HAP En todos los pacientes se midieron los niveles de Galectina-3, proBNP, se evaluo la CF mediante test de caminata 6 minutos (TC6M) y se evaluo remodelado AD. Se consideraron para el analisis dos grupos segun la distancia caminada en TC6M (> 200 m vs. ≤ 200 m). Resultados: La edad promedio fue 43 ± 10 anos, el 84% mujeres. Los niveles de Galectina-3 fueron 16,1 ± 7,4 ng/mL y el TC6M fue 371 ± 142 mts. Los pacientes con TC6M< 200 m presentaron mayores niveles de Galectina-3 (27,3 ± 4,6 vs 13,7 ± 3,8; p=0,006) y mayor volumen AD (151 ± 21 vs 94 ± 43; p=0,04). Ademas, se observo una correlacion inversa entre el area AD y TC6M (-0,71; p=0,03). Conclusion: Niveles elevados de Galectina-3 y parametros de remodelado adverso en AD se relacionan con una menor CF en pacientes con HAP. Estos hallazgos apuntan a una mejor caracterizacion de pacientes con HAP y eventualmente la busqueda de nuevos objetivos terapeuticos.