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Background: In Fontan patients, attrition of ventricular function is well recognized, but early detection of ventricular dysfunction is difficult. The aim of this study is to longitudinally assess ventricular strain in Fontan patients using a new method for cardiac magnetic resonance (CMR) feature tracking, and to investigate the relationship between ventricular strain and cardiac systolic function., Methods and Results: In this prospective, standardized follow-up study in 51 Fontan patients, age ≥ 10 years, CMR and concomitant clinical assessment was done at the start of the study and after 2 years. CMR feature tracking was done combining the dominant and hypoplastic ventricles. Global longitudinal strain (GLS) (-17.3% versus -15.9%, P = 0.041) and global circumferential strain (GCS) (-17.7 versus -16.1, P = 0.047) decreased over 2 years' time. Ejection fraction (EF) (57%), cardiac index (CI) (2.7 l/min/m 2 ) and NYHA functional class (97% in class I/II) were preserved. The strain values of the combined dominant and hypoplastic ventricles were significantly worse compared to those of the dominant ventricle only (GLS -16.8 (-19.5 to -14.0) versus -18.8 (-21.3 to -15.3) respectively, P = 0.001, GCS -18.3 (-22.1 to -14.8) versus -22.5 (-26.3 to -19.4) respectively, P < 0.001)., Conclusions: This study showed a decrease in cardiac strain over 2 years in Fontan patients without clinical signs of Fontan failure, where EF, CI and clinical status were still preserved. Cardiac strain might be a sensitive early indicator of systolic ventricular decline. Furthermore, combined strain of the hypoplastic and dominant ventricles seems a more accurate representation of cardiac strain in functionally univentricular hearts., (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: We evaluate pregnancy outcome and anticoagulation regimes in women with mechanical and biological prosthetic heart valves (PHV) for congenital heart disease., Methods: Retrospective multicenter cohort studying pregnancy outcomes in an existing cohort of patients with PHV., Results: 52 women had 102 pregnancies of which 78 pregnancies (46 women) ≥20 weeks duration (59 biological, 19 mechanical PHV). Miscarriages (n = 19, ≤20 weeks) occurred more frequently in women using anticoagulation (P < .05). During 42% of pregnancies of women with mechanical PHV a combined low molecular weight heparin (LMWH) vitamin-K-antagonist anticoagulation regime was used (n = 8). Overall, cardiovascular, obstetric and fetal/neonatal complications occurred in 17% (n = 13), 68% (n = 42) and 42% (n = 27) of the pregnancies. Women with mechanical PHV had significantly higher cardiovascular (12% vs 32%, P < .05), obstetric (59% vs 85%, P = .02) and fetal/neonatal (34% vs 61%, P < .05) complication rates than women with biological PHV. This was related to PHV thrombosis (n = 3, P < .02), post-partum hemorrhage (P < .02), cesarean section (P < .02), low birth weight and small for gestational age (both P < .05). PHV thrombosis occurred in 3 pregnancies, including 2/5 pregnancies with pulmonary mechanical PHV. PHV thrombosis was related to necessary cessation of anticoagulation therapy or insufficient monitoring of LMWH. Other cardiovascular complications occurred equally frequent in both groups., Conclusion: Complications occur more often in pregnancies of women with a mechanical PHV than in women with a biological PHV, mainly caused by PHV thrombosis and bleeding complications. Meticulous monitoring of anticoagulation in pregnant women is necessary. Women with a pulmonary mechanical PHV are at high risk of complications., (Copyright © 2018. Published by Elsevier B.V.)

Objective: Women with repaired coarctation of the aorta (rCoA) are at risk of hypertensive disorders and other complications during pregnancy. Hypertensive disorders in pregnant women are associated with inadequate uteroplacental flow, which is related to adverse offspring outcome. The aim of this study was to investigate the relationship of maternal cardiac function, placental function and pregnancy complications in women with rCoA., Methods: We included 49 pregnant women with rCoA and 69 controls from the prospective ZAHARA-studies (Zwangerschap bij Aangeboren HARtAfwijkingen, pregnancy in congenital heart disease). Clinical evaluation, echocardiography and uteroplacental Doppler flow (UDF) measurements were performed at 20 and 32weeks gestation. Univariable regression analysis was performed., Results: Comparison of rCoA and healthy women. In women with rCoA, tricuspid annular plane systolic excursion (TAPSE) decreased during pregnancy (25.7mm to 22.8mm, P=0.006). UDF indices and pregnancy complication rates were similar in both groups. Offspring of rCoA women had lower birth weight (3233g versus 3578g, P=0.001), which was associated with β-blocker use during pregnancy (β=-418.0, P=0.01). Association of cardiac function and UDF. Right ventricular (RV) function before pregnancy (TAPSE) and at 20weeks gestation (TAPSE and RV fractional area change) were associated with impaired UDF indices (umbilical artery pulsatility index at 20weeks β=-0.02, P=0.01, resistance index at 20 and 32weeks β=-0.01, P=0.02 and β=-0.02, P=0.01 and uterine artery pulsatility and resistance index at 20weeks gestation β=-0.02, P=0.05 and β=-0.01, P=0.02)., Conclusions: Women with rCoA tolerate pregnancy well. However, RV function is altered and is associated with impaired placentation., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Patients with a Fontan circulation tend to develop liver fibrosis, liver cirrhosis and even hepatocellular carcinoma. The aim of this study is to use the magnetic resonance technique diffusing-weighted imaging (DWI) for detecting liver fibrosis/cirrhosis in Fontan patients and to establish whether DWI results are associated with functional aspects of the Fontan circulation., Methods: In a cross-sectional study, 59 Fontan patients were evaluated by liver DWI. The association between apparent diffusion coefficients (ADC) and patient characteristics, laboratory measurements and functional aspects of the Fontan circulation (NYHA class, maximum oxygen uptake during exercise and cardiac index) was assessed., Results: Liver ADC values were low (0.82×10(-3)±0.11×10(-3) mm2/s) compared with literature values for healthy volunteers and correlated negatively with calculated liver fibrosis/cirrhosis scores (Fib-4 score, p=0.019; AST/ALT ratio, p=0.009) and gamma-glutamyl transferase (p=0.001). Furthermore, ADC values correlated negatively with follow-up duration (p<0.001) and positively with cardiac index (p=0.019). No correlation between ADC values and exercise tests was found. In multivariable analysis, the ADC values were independently correlated with follow-up duration after Fontan completion., Conclusions: The results of the current study suggest that progressive liver damage due to chronic congestion and potential hypoperfusion is reflected in the liver ADC values in Fontan patients. This study highlights that liver damage in the context of the Fontan circulation might be far more common than previously thought, and that the implementation of liver assessment in the routine follow-up of Fontan patients is recommendable., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Background: Normally, the inside of the left atrial (LA) body and pulmonary veins (PVs) is lined by vessel wall tissue covered by myocardium. In total anomalous pulmonary venous connection (TAPVC), no connection of the PVs with the LA body exists. These veins have an increased incidence of PV stenosis. We describe the consequences of the absent connection for the histopathology of the wall of the LA body and the PVs, and hypothesize on a mechanism predisposing to PV stenosis., Methods and Results: In 10 human neonates with TAPVC, the wall of the LA body and PVs were studied using histological and immunohistochemical techniques. As controls, 2 normal neonatal and adult hearts and 5 neonatal hearts with partial anomalous venous connection (PAPVC) or situs inversus were studied. In hearts with TAPVC no vessel wall tissue was found in the LA body and its myocardial layer was hypoplastic. No myocardial sleeve was found around the abnormally draining PVs. In hearts with PAPVC, only the non-LA draining PV lacked myocardial covering, whereas in situs inversus PVs connecting to the right-sided LA, were normally myocardialized., Conclusion: An open connection of the PVs with the morphological LA is necessary for the presence of vessel wall tissue in the LA and myocardialization of the PVs. Absence of myocardium covering the PVs is hypothesized to enhance susceptibility to PV stenosis and prevent onset of PV originating arrhythmias. The embryonic posterior heart field may be responsible for the abnormal myocardialization and smooth muscle cell formation in TAPVC.

Background: Maze surgery is a final solution for intractable atrial fibrillation (AF), but an adverse effect on postoperative sinus node function has been reported. Whether this also applies to other types of cardiac surgery is unclear., Methods: We assessed postoperative rhythm by means of repeated exercise tolerance testing, ambulatory electrocardiography, and non-invasive testing of autonomic function between 1 and 12 months after four types of cardiac surgery. Fourteen patients without structural cardiac disease and medically refractory AF underwent the maze III procedure, 11 patients with mitral valve disease and preoperative AF underwent valvar surgery combined with a (simplified) maze III procedure, and 8 patients with mitral valve disease in sinus rhythm (SR) underwent isolated valvar surgery. The control group consisted of eight patients with sinus rhythm who underwent coronary artery bypass surgery (CABG)., Results: One month after surgery, the chronotropic response to exercise was depressed, mean heart rate was high, and heart rate variability (HRV) was low, especially after maze III, combined surgery, and isolated valvar surgery. Twelve months after surgery, moderate improvements were observed. After CABG, considerably fewer abnormalities were observed, and HRV parameters recovered to a large extent. Non-invasive testing of autonomic function indicated disturbed vagal modulation of heart rate in all three groups with atrial incision., Conclusion: Thus, attenuation of HRV and vagal modulation of sinus node function are not confined to maze surgery but also apply to isolated mitral valve surgery. Atrial incision therefore appears to be crucial and presumably produces autonomic nervous damage followed by partial reinnervation. Nevertheless, cardiac surgery in general seems initially to impair sinus node function with partial recovery in the consecutive 12 months.