Systemic Artery Vasoconstrictor Therapy in Fontan Patients with High Cardiac Output-Heart Failure: A Single-Center Experience.

Failed Fontan Patients with high cardiac output (CO) heart failure (HF) might have vasodilatory syndrome and markedly high mortality rates. The aim of this study was to review the clinical effects of vasoconstrictor therapy (VCT) for failed Fontan hemodynamics. We retrospectively reviewed 10 consecutive patients with Fontan failure (median age, 33 years) and high CO-HF who had received VCT. The hemodynamics were characterized by high central venous pressure (CVP: median, 16 mm Hg), low systolic blood pressure (median, 83 mm Hg), low systemic vascular resistance (median, 8.8 U·m2), high cardiac index (median, 4.6 L/min/m2), and low arterial oxygen saturation (median, 89%). VCT included intravenous noradrenaline infusion for five unstable patients, oral midodrine administration for nine stable patients, and both for four patients. After VCT introduction with a median interval of 1.7 months, the median systolic blood pressure (102 mm Hg, p = 0.004), arterial oxygen saturation (90%, p = 0.03), and systemic vascular resistance (12.1 U·m2, p = 0.13) increased without significant changes in CVP or cardiac index. After a median follow-up of 21 months, the number of readmissions per year decreased from 4 (1-11) to 1 (0-9) (p = 0.25), and there were no VCT-related complications; however, five patients (50%) developed hepatic encephalopathy, and six patients (60%) eventually died. VCT was safely introduced and could prevent the rapidly deteriorating Fontan hemodynamics. VCT could be an effective therapeutic strategy for failed Fontan patients with high CO-HF.

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.

Ruptured mitral valves chordae tendineae around a convalescent infant with acute Kawasaki disease.

Severe valvulitis owing to acute Kawasaki disease leading to severe mitral regurgitation is a rare event in infants. Further, there is less information about underlying ruptured mitral chordae tendineae causing severe mitral regurgitation. We encountered ruptured mitral chordae tendineae in three female patients after Kawasaki disease. The age at the onset of Kawasaki disease ranged from 3 to 8 months, and detection of ruptured mitral chordae tendineae was from 24 to 90 days. Two patients had acute heart failure, and one was asymptomatic. One patient underwent mitral annuloplasty, and the others responded to medication. These ruptured mitral chordae tendineae occurred after the remission of the initial acute Kawasaki disease, in the early course and the convalescent of acute Kawasaki disease. Further, the recurrent fever was also detected in them. The ruptured mitral chordae tendineae in an infant within 6 months can be detected by systolic heart murmur around the convalescent stage of acute Kawasaki disease, although the prevalence is very low.

Septal Flash-like Motion of the Earlier Activated Ventricular Wall Represents the Pathophysiology of Mechanical Dyssynchrony in Single-Ventricle Anatomy.

BACKGROUND: In biventricular physiology, abnormal septal motion is a hallmark of mechanical dyssynchrony in the left bundle branch block. However, in single-ventricle (SV) physiology, morphologic variations in systemic ventricles pose a challenge in evaluating the negative impact of mechanical dyssynchrony. The present study aimed to characterize the pathologic dyssynchronous contraction patterns in patients with SV. METHODS: In this retrospective study, 70 consecutive postoperative patients with SV anatomy with prolonged QRS duration (25 female patients; median age, 14 years) were enrolled. We divided each SV into two regions and analyzed independent strains using two-dimensional speckle-tracking echocardiography. From an earlier activated ventricular wall, we calculated the strain ratio (Rstrains) of two values (%) during the QRS period and the ejection period: (100 + Strainejection)/(100 + StrainQRS). We reviewed the clinical profiles, B-type natriuretic peptide plasma levels, exercise capacity, and morbidity. Six patients who underwent cardiac resynchronization therapy (CRT) were analyzed regarding changes in strain patterns and ventricular volume. RESULTS: Higher Rstrains, indicating a preceding contraction and subsequent dyskinetic dilation of the earlier activated ventricular wall, was associated with increased B-type natriuretic peptide, reduced exercise capacity, and poor outcome. However, delayed contraction of the later activated ventricular wall was not associated with the effects. Decreases in Rstrains and ventricular volume reductions were observed in all patients after CRT. CONCLUSIONS: A specific strain pattern in an earlier activated ventricular wall indicates mechanical dyssynchrony in patients with SV. This pattern is very similar to the septal flash in adult patients with left bundle branch block. This strategy might be a promising approach for selecting appropriate candidates for CRT in patients with SV.

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.

Positive pediatric exercise capacity trajectory predicts better adult Fontan physiology rationale for early establishment of exercise habits.

OBJECTIVE: Exercise training is recommended for its possible favorable effects on Fontan pathophysiology. This study aimed to elucidate the impact of pediatric exercise capacity trajectory, which may mimic the effect of exercise training, on late adult Fontan pathophysiology. METHODS: Since 1990, 97 Fontan patients had consecutively undergone two serial cardiopulmonary exercise tests (CPX1 and CPX2) during childhood (ages 8 ±â€¯2 and 14 ±â€¯2 years) and one during adulthood (CPX3; age 23 ±â€¯5 years). The changes in peak oxygen uptake (PVO2: % of normal value) from CPX1 to CPX2 (1-dPVO2) and from CPX2 to CPX3 (2-dPVO2) were calculated, and then the patients were divided into four subgroups according the 1-dPVO2 and 2-dPVO2. RESULTS: In their adulthood, when compared with groups with negative 1-dPVO2, the central venous pressure, plasma brain natriuretic peptide level, and renal resistive index were lower, whereas liver synthetic function, body fat-free percentage, and PVO2 were higher in those with positive 1-dPVO2 (p < 0.05-0.0001). However, these favorable associations of 2-d-PVO2 with adult Fontan pathophysiology were not observed, except for the PVO2. After CPX3, 13 unexpected events occurred, and the risk was 76% lower in the groups having positive 1-dPVO2 than in those with negative 1-dPVO2 (hazard ratio, 0.24; 95% confidence interval, 0.09-0.62; p = 0.0035). CONCLUSIONS: A positive exercise capacity trajectory during childhood predicts better adult Fontan pathophysiology, including better prognosis. Thus, prescription of exercise could be a promising strategy in the management of pediatric Fontan patients.