Abnormal glucose metabolism in patients with Fontan circulation: Unique characteristics and associations with Fontan pathophysiology.

BACKGROUND: Fontan patients exhibit a high prevalence of abnormal glucose metabolism (AGM). We aimed to characterize AGM and clarify its association with Fontan pathophysiology. METHODS: We prospectively evaluated AGM with plasma glucose dynamics [mg/dL; fasting glucose (FPG), and maximum glucose increase (PG-spike)] during oral glucose tolerance test and hemoglobin A1c (HbA1c) in 276 consecutive Fontan patients (aged 19 ±â€¯7 years). Of these, 176 patients had serial AGM assessments with a mean interval of 6.5 years. RESULTS: Initial analysis revealed a high prevalence of impaired glucose tolerance (38.4%) and diabetes mellitus (DM) (4.7%), and positive family history, high HbA1c, and high central venous pressure independently predicted presence of DM. HbA1c was independently determined by hypersplenism and presence of DM (P < .05). Serial assessments revealed an increased PG-spike and a decreased HbA1c (P < .001 for both). Prevalence of DM increased (6.3% to 10.3%), and positive family history, high liver enzymes, and AGM predicted new onset of DM (P < .05 for all). Twenty-one patients died during 7.1-year follow-up. FPG (P < .01) and PG-spike (P < .05) independently predicted all-cause mortality. Particularly, patients with FPG ≤ 74 and/or PG-spike ≥85 had a mortality rate 8.7 times higher than those without (P = .0129). CONCLUSIONS: AGM progressed even in young adult Fontan patients, and HbA1c showed limited predictive value for progression. Oral glucose tolerance test plays important roles in uncovering unique Fontan AGM as well as predicting all-cause mortality.

Peripartum Management of Pregnant Women With Congenital Heart Disease.

BACKGROUND: The average maternal age at delivery, and thus the associated maternal risk are increasing including in women with congenital heart disease (CHD). A comprehensive management approach is therefore required for pregnant women with CHD. The present study aimed to investigate the factors determining peripartum safety in women with CHD.Methods and Results:We retrospectively collected multicenter data for 217 pregnant women with CHD (age at delivery: 31.4±5.6 years; NYHA classifications I and II: 88.9% and 7.4%, respectively). CHD severity was classified according to the American College of Cardiology/American Heart Association guidelines as simple (n=116), moderate complexity (n=69), or great complexity (n=32). Cardiovascular (CV) events (heart failure: n=24, arrhythmia: n=9) occurred in 30 women during the peripartum period. Moderate or great complexity CHD was associated with more CV events during gestation than simple CHD. CV events occurred earlier in women with moderate or great complexity compared with simple CHD. Number of deliveries (multiparity), NYHA functional class, and severity of CHD were predictors of CV events. CONCLUSIONS: This study identified not only the severity of CHD according to the ACC/AHA and NYHA classifications, but also the number of deliveries, as important predictive factors of CV events in women with CHD. This information should be made available to women with CHD and medical personnel to promote safe deliveries.

Beneficial Effect of Endovascular Treatment on Villalta Score in Japanese Patients With Chronic Iliofemoral Venous Thrombosis and Post-Thrombotic Syndrome.

BACKGROUND: Post-thrombotic syndrome (PTS), the most common complication of deep venous thrombosis (DVT), develops in ≥50% of patients with iliofemoral DVT. However, the benefit of endovascular treatment in Japanese patients with chronic DVT and PTS remains unclear. Methods and Results: Between June 2014 and May 2016, endovascular treatment was performed in 11 consecutive Japanese patients with chronic iliofemoral DVT and PTS refractory to anticoagulant therapy and elastic compression stockings. We evaluated the technical success rate, complications, patency, Villalta score, calf circumference, and popliteal vein reflux in both the acute stage (the day following endovascular treatment) and chronic stage (after 6 months). Imaging follow-up included venous duplex scanning and/or magnetic resonance venography. The technical success rate was 81.8%, without complications. In patients with successful intervention, the Villalta score improved significantly, from 9.0±3.7 preoperatively to 3.6±2.5 in the acute phase (P<0.01) and 2.9±2.1 in the chronic phase (P<0.001). The bilateral difference in lower thigh circumference also improved significantly, from 2.6±1.0 cm preoperatively to 1.4±1.0 cm in the chronic phase (P<0.001). However, popliteal vein reflux did not improve. In patients with successful intervention, venous patency rate was 100% at 6 months post-intervention. CONCLUSIONS: Endovascular treatment is safe and effective in Japanese patients with chronic iliofemoral DVT and PTS.

Successful Transcatheter Atrial Septal Defect Closure Prior to Coronary Artery Bypass Grafting Using Anti-Congestive Therapies and Intraaortic Balloon Pumping in a Patient with Severe Ischemic Cardiomyopathy and Triple-Vessel Coronary Artery Disease.

In patients with an atrial septal defect (ASD) and left ventricular (LV) dysfunction associated with coronary artery disease (CAD), to avoid the development of acute left heart failure (HF) and an increase in myocardial oxygen consumption following ASD closure, it is conceivable that coronary artery revascularization should be performed prior to ASD closure. We report the case of a 67-year-old man with a large secundum ASD and LV ejection fraction of 15.6% resulting from severe ischemic cardiomyopathy and triple-vessel CAD, both of which contributed to biventricular HF characterized by high left-to-right shunt (Qp:Qs of 7.1:1) and low systemic cardiac output. After evaluating his hemodynamics and biventricular function with cardiac catheterization and cardiovascular magnetic resonance imaging, we successfully conducted an inverse, stepwise strategy of transcatheter ASD closure using anti-congestive therapies, intraaortic balloon pumping, and subsequent balloon occlusion testing, followed by on-pump beating-heart coronary artery bypass grafting.

Report of the American Heart Association (AHA) Scientific Sessions 2016, New Orleans.

The American Heart Association (AHA) Scientific Sessions 2016 were held on November 12-16 at the Ernest N. Morial Convention Center, New Orleans, LA. This 5-day event featured cardiovascular clinical practice covering all aspects of basic, clinical, population, and translational content. One of the hot topics at AHA 2016 was precision medicine. The key presentations and highlights from the AHA Scientific Sessions 2016, including "precision medicine" as one of the hot topics, are herein reported.

In-hospital predictors for primary prevention of sudden death after acute myocardial infarction with cardiac dysfunction.

BACKGROUND: Current guidelines recommend prophylactic defibrillator implantation in patients with acute myocardial infarction (AMI) and left ventricular ejection fraction (LVEF) ≤40 % or LVEF ≤35 % plus heart failure symptoms or inducible ventricular tachyarrhythmias during an electrophysiology study at 40 days after AMI or 90 days after revascularization. In-hospital predictors of sudden cardiac death (SCD) after AMI during the index hospitalization remain unsettled. We sought to examine in-hospital predictors of SCD in patients with AMI and LVEF ≤40 % evaluated during the index hospitalization. METHODS: We retrospectively evaluated 441 consecutive patients with AMI and LVEF ≤40 % admitted to our hospital between 2001 and 2014 (77 % male gender; median age: 70 years; median hospitalization length: 23 days). The primary endpoint was a composite of SCD or aborted SCD at ≥30 days after AMI onset (composite arrhythmic event). LVEF and QRS duration (QRSd) on electrocardiography were measured at a median of 12 days and 18 days, respectively. RESULTS: During a median follow-up of 7.6 years, the incidence of composite arrhythmic events was 7.3 % (32 of 441 patients). In multivariable analysis, QRSd ≥100 msec (beta-coefficient = 1.54, p = 0.003), LVEF ≤23 % (beta-coefficient = 1.14, p = 0.007), and onset-reperfusion time > 5.5 h (beta-coefficient = 1.16, p = 0.035) were independent predictors of composite arrhythmic events. The combination of these 3 factors was associated with the highest rate of composite arrhythmic events compared with 0-2 factors (p < 0.001). CONCLUSIONS: The combination of QRSd ≥100 msec, LVEF ≤23 %, and onset-reperfusion time > 5.5 h during the index hospitalization provides precise risk stratification for SCD in patients early after AMI.

Prognostic value of von Willebrand factor in adult patients with congenital heart disease.

OBJECTIVES: von Willebrand factor (vWF) has prognostic value in patients with heart failure (HF) and in those with liver disease. Liver congestion, due to right-sided HF (RHF), is one of the major clinical pathophysiologic manifestations in adults with congenital heart disease (ACHD). The present study's purpose was to clarify the prognostic value of plasma levels of vWF antigen (vWF:Ag) in ACHD. METHODS: We measured vWF:Ag (%) in 382 consecutive patients (20 unrepaired cyanotic ACHD, 172 Fontan patients and 190 ACHD after biventricular repair) and compared the results with the clinical profiles and prognosis. RESULTS: The plasma vWF:Ag level was 130±53 (normal range: 55%-190%), and 48 patients (13%) showed high levels of vWF:Ag (≥190%). Older age, Fontan circulation, higher central venous pressure, lower arterial oxygen saturation and lower plasma levels of albumin were independently associated with high log (vWF:Ag) (p<0.05-0.0001). During the follow-up of 2.4±1.4 years, 15 patients died. High log (vWF:Ag) predicted the all-cause mortality (HR 1.63 per 0.1, 95% CI 1.40 to 1.96, p<0.0001). Specifically, patients with high vWF:Ag (≥165%) had a substantially higher risk of all-cause mortality (HR 56.4, 95% CI 11.4 to 1020, p<0.0001), and this prognostic value was independent of plasma levels of brain-type natriuretic peptide. CONCLUSIONS: High vWF:Ag may reflect RHF severity and related liver dysfunction with a strong prognostic value of all-cause mortality in ACHD. Thus, vWF:Ag might be an excellent biomarker for monitoring ACHD with RHF.

Development of Pulmonary Arterial Hypertension in a Patient Treated with Qing-Dai (Chinese Herbal Medicine).

Pulmonary arterial hypertension (PAH) is a rare, devastating disease, characterized by elevated pulmonary arterial pressure due to pulmonary microvascular obstruction, which can result in heart failure and death. PAH can be associated with exposure to certain drugs or toxins. We herein report a case in which PAH developed in a patient with refractory ulcerative colitis during treatment with "Qing-Dai," a Chinese herbal medicine. The patient's PAH improved after the discontinuation of Qing-Dai.

Successful multi-step catheter intervention for thrombotic inferior vena cava filter retrieval.

A 45-year-old man, who was implanted with an inferior vena cava (IVC) filter in his infrarenal IVC, had a complication of deep vein thrombosis (DVT) propagation from the IVC, beyond the IVC filter, to the right calf and left external iliac veins. The entire IVC filter was covered with a massive thrombus. We first decided to retrieve the IVC filter itself, which was suspected of causing metallic allergy. The thrombotic IVC filter was successfully retrieved using multi-step catheter intervention. To our knowledge, this is the first case report to describe successful multi-step catheter intervention for retrieval of an IVC filter covered with a massive thrombus. .

Prolonged QRS duration as a predictor of right ventricular dysfunction after balloon pulmonary angioplasty.

BACKGROUND: Balloon pulmonary angioplasty (BPA) has shown beneficial effects for chronic thromboembolic pulmonary hypertension (CTEPH). However, previous studies have shown less cardiac output improvement and symptoms remaining after BPA, implying poor right ventricular (RV) function recovery. Therefore, we investigated the residual RV dysfunction after BPA to reveal risk factors, clinical effects, and possible underlying histopathological mechanisms. METHODS AND RESULTS: We investigated 61 consecutive CTEPH patients who underwent cardiovascular magnetic resonance before and 3 and 12 months after BPA series. Residual dysfunction (RD) of RV was defined as RV end-diastolic volume index >100 ml/m2 or RV ejection fraction (EF) <45% at 12-month follow-up. Patients were divided into RD (44%) and normalized dysfunction (ND) (56%) groups. Compared with the ND group, the RD group had significantly worse World Health Organization (WHO) functional class at follow-up. No significant hemodynamic differences were observed between the groups. On multivariable logistic regression analysis, male sex (odds ratio [OR] 12.5, p = 0.004) and prolonged QRS duration (OR 1.08, p = 0.029) were independently associated with residual RV dysfunction. Additionally, RV histopathology in 11 CTEPH autopsy cases showed that QRS duration was correlated with RV fibrosis area. CONCLUSIONS: Relatively high percentage (44%) of residual RV dysfunction with worse WHO functional class was observed in CTEPH patients even after BPA. Prolonged QRS duration may predict poor recovery in RV function after BPA.

Efficacy and safety of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension guided by cone-beam computed tomography and electrocardiogram-gated area detector computed tomography.

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease characterized by chronic obstructive thrombus and pulmonary hypertension. Balloon pulmonary angioplasty (BPA), an emerging alternative catheter-based treatment for inoperable patients with CTEPH, has not yet been standardised, especially for lesion assessment in distal pulmonary arteries. Recent advancement in computed tomography enables distal CTEPH lesions to be visualized. METHODS: We retrospectively studied 80 consecutive patients with inoperable CTEPH who received BPA guided by cone-beam computed tomography (CT) (CBCT) or electrocardiogram (ECG)-gated area detector CT (ADCT) for target lesion assessment. We collected clinical and hemodynamic data, including procedural complications, before BPA and at 3 months and 1year after BPA. RESULTS: Three hundred eight-five BPA sessions (4.8 sessions/patient) were performed for the lesions of subsegmental arteries (1155 lesions), segmental arteries (738 lesions), and lobar arteries (4 lesions) identified by CBCT or ECG-gated ADCT. Significant improvements in the symptoms, 6-min walk distance, brain natriuretic peptide level, exercise capacity, and haemodynamics were observed 3 months and 1year after BPA. No cases of death or cardiogenic shock with a low rate of severe wire perforation (0.3%) and severe reperfusion oedema (0.3%) were observed. CONCLUSIONS: BPA guided by CBCT or ECG-gated ADCT is effective and remarkably safe in patients with CTEPH . These new advanced CT techniques may be useful in pre-BPA target lesion assessment.