Search

Objectives We aimed to investigate tricuspid valve function and adverse events after conventional repair and valve replacement for Ebstein's anomaly and compare them with cone repair. Methods The medical records of 151 patients (mean age, 25 years; 62% were female) who underwent operation in a single center from 1985 to 2018 were retrospectively analyzed. To determine tricuspid valve regurgitation during follow-up, serial echocardiographic examination was used (n = 2397, tricuspid regurgitation grades were graphed for every patient). Results Thirty-nine patients underwent cone repair, 107 patients underwent other repair techniques, and 5 patients underwent valve replacement. The operative mortality was 1.3% (n = 2). Failed valve repair (defined as in-hospital death, conversion to replacement, or in-hospital reoperation) was less frequent after cone repair than after other repair techniques (5%, n = 2 vs 20%, n = 21, P = .039). Mean follow-up was 12.3 years (cone repair: 3.7 years). The 5-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation was lower after cone repair than after other repair techniques (8% vs 32%, P = .03). Among the patients undergoing other repair techniques, the 15-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation, severe tricuspid regurgitation, and reoperation was 58%, 37%, and 31%, respectively. During follow-up, 18 patients died (13 of cardiac and 5 of noncardiac causes). Among patients who died of cardiac causes, 10 of 13 had all 3 characteristics—moderate or greater tricuspid regurgitation, atrial fibrillation, and New York Heart Association classification III and IV—at their last medical evaluation. Conclusions Before cone repair, recurrent tricuspid regurgitation was considerable. Cone repair provided a higher rate of successful repair and a lower incidence of moderate or greater recurrent tricuspid regurgitation at the midterm follow-up.

Background Direct oral anticoagulants (DOACs) have been proven to be safe and effective in patients with acquired heart disease. However, data regarding their use in adults with congenital heart disease (ACHD) is scarce. Methods All ACHD under DOAC were retrospectively identified. Bleeding and thromboembolic events were registered. CHADS2, CHA2DS2-VASc and HASBLED scores were calculated. Risk factors for bleeding and thromboembolic events were identified. Results 215 ACHD patients (111 female, 48.4 ± 15.5 years) were included. CHD was severe in 44.2%, moderate in 23.7%, and simple in 32.1%. Indications for anticoagulation were: atrial arrhythmias (66.8%), cerebrovascular accidents (32.7%), pulmonary thromboembolism (3.7%), deep vein thrombosis (11.2%), atrial thrombi (6.5%), and Fontan circulation/TCPC (5.6%). Mean follow-up was 15.8 ± 15.8 months. Nine patients suffered a major and eight a minor bleeding. Thromboembolic events occurred in two patients. The annual risk for bleeding was 3.1%/patient/year and for thromboembolic events 0.7%/patient/year. A CHADS2-Score >2, HASBLED >3, and renal disease were identified as risk factors for bleeding (minor and major bleeding) in a univariate analysis. In the multivariate analysis, renal disease remained as an independent predictor for bleeding (HR 6.13, CI [1.04–36.27], p Conclusions A low rate of thromboembolic events was observed in ACHD patients under DOAC therapy. Bleeding complications were not negligible. Special attention has to be paid to those patients with advanced renal failure.

Background Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex multisystemic disorder. Due to increased systemic venous pressure and the inability to ensure systemic perfusion and metabolic requirements, the liver may develop congestion, fibrosis or cirrhosis. This study aimed to assess hepatic abnormalities in Eisenmenger patients non-invasively. Methods and results 10 adults with Eisenmenger syndrome (six female; median age 44.2years; range 23–62years) were enrolled and hepatic involvement was assessed - using clinical assessment, laboratory analysis, hepatic fibrotic markers, abdominal sonography and liver stiffness measurements (transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)). Using imaging and laboratory analysis, 60% (6/10) of the Eisenmenger patients had signs of liver fibrosis (5/10) or cirrhosis (1/10). While TE, however, showed no relevant liver abnormalities in any Eisenmenger patient, ARFI detected liver fibrosis in 5/10 and cirrhosis and 1/10 patients. Conclusions Adult Eisenmenger patients are at increased risk of hepatic impairment. Non-invasive screening could be helpful in detecting liver alterations. In our small series, however, TE could not detect fibrosis or cirrhosis in any affected patient, while ARFI was very reliable. Patients should be transferred to centres, where a multidisciplinary expert knowledge is available and a close collaboration between cardiologists and hepatologists exists.

Background Although there is sufficient evidence that sex-/gender-based differences can influence treatment decisions and healthcare for adults with congenital heart disease (ACHD), there are major research shortfalls in this regard. The aim of this study was to assess these in a large ACHD cohort. Methods In this cross-sectional study, patient-reported outcome measures from a questionnaire completed by 3880 ACHD (53.6% female; 41.9 ​± ​17.1 years) were used to assess gender-related differences concerning underlying CHD condition, comorbidities, health care details, individual counselling needs, and quality of life (QoL). Results Significant differences observed included a female predominance in tetralogy of Fallot, atrial septal defects, and persistent ductus arteriosus, and a male predominance in aortic valve stenosis/insufficiency and transposition of the great arteries. The prevalence of coronary artery disease was significantly higher in men, while women reported a higher prevalence of pulmonary hypertension, arrhythmias, neurologic restrictions, and overall comorbidities. Men consulted their primary care physician more often if a CHD-related medical problem was suspected. A total of 2194 patients (54.4% female) stated that they had never been referred to an institution specialized in CHD. Both sexes had a high counselling demand, but there were significant differences between male and female participants. In terms of QoL, female participants reported greater impairments, especially in everyday activities, more pain/physical complaints, and more anxiety/depression. Conclusion Both specialists and primary care physicians (PCPs) require an understanding of the importance of gender-based differences in ACHD, since, for the majority of ACHD, PCPs are the first contact persons for medical issues.

The different biopsychosocial periods in a woman's life are all interactively associated with the cardiovascular system. The present study was designed to address questions related to sexuality and reproductive health in a large cohort of women with congenital heart disease. Overall, 536 women (median age 29 years, range 18 to 75) completed a questionnaire during their visit at 2 tertiary care centers for congenital heart disease. Patients were categorized according to their functional class and according to the degree of severity of the underlying heart defect. The median age at menarche was significantly delayed in patients with functional class III-IV and in women with complex or cyanotic anomalies. More than 1/4 of the women (29%) had at least once sought medical advice for menstrual discomforts, and the proportion was significantly increased for those in the worst functional class (49%, p

ObjectiveCardiac surgery with cardiopulmonary bypass for correction of congenital heart disease in neonates and small infants is associated with considerable neurologic sequelae. We assessed the extent to which mixed venous oxygen saturation as a measure for adequacy of perfusion, reflects the oxygenation status of upper and lower body compartments. Moreover, we evaluated potential benefits of near-infrared spectroscopic monitoring of regional tissue oxygenation.MethodsTwenty patients (body weight < 10 kg) undergoing open cardiac procedures with cardiopulmonary bypass were enrolled. Blood samples were obtained in parallel from inferior and superior caval vein cannulas and mixed venous line and assessed for venous oxygen saturation and lactate levels. Data were compared to simultaneously measured tissue oxygenation indices obtained by near-infrared spectroscopy from brain and lower limb.ResultsVenous oxygen saturation was lower and lactate concentration higher in blood from superior relative to inferior venous line. Mixed venous oxygen saturation correlated with venous oxygen saturation from inferior venous line and tissue oxygenation index of lower limb. No correlation was found between mixed venous oxygen saturation and venous oxygen saturation from superior venous line or cerebral tissue oxygenation index.ConclusionIn neonates and small infants undergoing cardiac surgery with cardiopulmonary bypass, considerable regional differences exist in venous oxygen saturation. Mixed venous oxygen saturation primarily represents lower-torso oxygen status but poorly reflects and systematically overestimates upper-body oxygenation. Near-infrared spectroscopy yields additional information on regional oxygenation and may be valuable in early and sensitive detection of regional malperfusion in critical organs such as the brain.

We determined the influence of head position on lateral ventricular cerebral volume in low-birth-weight infants by three-dimensional (3-D) ultrasound (US). Thirty-nine neonates were examined prospectively in a controlled and blinded study. We used a freehand 3-D US system to acquire data sets after head positioning for 3 h on left and right side in random order. The borders of the lateral ventricles were marked in stored cross-sections. Volumes were calculated as mean of duplicate measurements. Median volume of lateral cerebral ventricles was 1.03 (quartiles 0.78-1.36) mL. Median left ventricular volume was slightly larger than right one (p = 0.13). Down-side lateral ventricles showed smaller volumes than up-side positioned ventricles (p0.01). Freehand 3-D US allows quantification of small volumes as neonatal lateral cerebral ventricles. Head position influences the lateral cerebral ventricle volume in low-birth-weight infants.

Late-diastolic forward flow is a well-described phenomenon detectable by Doppler echocardiography in the pulmonary trunk. It is supported by a restrictive right ventricular diastolic function and by a low end-diastolic pulmonary artery pressure. A similar phenomenon for the left ventricle and the aorta has not been described. We report a case of a preterm infant with aortic stenosis and endocardial fibroelastosis, who underwent balloon valvuloplasty. Restrictive left ventricular diastolic filling led to high left atrial pressure (27 mm Hg) and a very pathologic ratio of early-to-late peak velocities (2.6) for an infant of 29 weeks' gestation. In combination with a low diastolic aortic pressure (24 mm Hg) caused by moderate aortic regurgitation after intervention, a late-diastolic forward flow was detectable in the aorta during left atrial contraction with pulsed Doppler echocardiography. (J Am Soc Echocardiogr 2000;13:866-8.)