-
Journal of the American College of Cardiology 04/2013; · 14.16 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Objectives: Evaluation of the use of a dual wire technique to deliver stents. Background: Placement of a stent in lesions close to bifurcations or without an anatomic obstruction can be problematic. Patients and Methods: Stents were implanted in twelve patients between August 2010 and August 2012. Indications were: complex anatomic obstruction in 9, external compression of pulmonary veins in 2 and exclusion of an aortic aneurysm in 1 patient. Results: Median age and body weight of the group were 11.6 years (range 1.6 - 34.8) and weight 36 kg (range: 10 - 78), respectively. All stents were safely delivered and patency of all side vessels was maintained; the distal end of the stent was flared to a median ratio of 1.4 (range: 1.2 - 1.8). The clinical objective was met in all patients: in lesions with stenosis, diameter increased from 5.9 mm (range: 1 - 13) to 9.9 mm (range: 8 - 17) (p < 0.01, 95% CI: 2.0 - 7.2); saturations improved in both patients with external pulmonary venous compression and in another an aortic aneurysm was excluded. One patient developed a hemothorax and one required blood transfusion for bleeding from the valve of the sheath. Conclusions: The dual wire stenting technique is effective to accurately deliver and anchor stents into lesions close to side branches and bifurcations. Side branch patency is maintained; the clinical objective (gradient relief, aneurysm exclusion, relief of external compression) can safely be reached. Technique and balloon selection should be based on the underlying anatomical substrate. © 2013 Wiley Periodicals, Inc.
Catheterization and Cardiovascular Interventions 04/2013; · 2.29 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVE: To report on the feasibility of assessing cardiac structures on post-mortem 3-tesla MRI (pmMRI) and to provide morphometric data in fetuses without cardiac abnormalities. METHODS: Retrospective single center study on 3T pmMRI of 39 consecutive fetuses without cardiac abnormalities (13-38 weeks of gestation). Fetal cardiac anatomy was assessed and measurements of cardiac structures were performed on T2-weighted 3D multiplanar reconstructed images. Linear regression analysis was performed to examine changes of cardiac dimensions during gestation. RESULTS: The four-chamber view of the fetal heart could be obtained and the measurements of cardiac chambers and ventricular walls could be performed in all 39 cases. The aorta and the pulmonary artery were visualized and their diameters were measured in 38 (97.4%) fetuses, ductus arteriosus in 32 (82%). All measurements showed strong linear correlation with gestational age. The relationship of the diameters of the pulmonary artery, aorta, and ductus arteriosus remained constant over pregnancy. All these observations are consistent with what is known from prenatal ultrasound. CONCLUSIONS: The present study proves the feasibility of visualizing normal cardiac structures on 3-tesla pmMRI in fetuses beyond 14 weeks. We provide morphometric data that may enable diagnostic evaluation of cardiac abnormalities on pmMRI. © 2013 John Wiley & Sons, Ltd.
Prenatal Diagnosis 03/2013; · 2.11 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Background:Since survival rates of patients with congenital heart disease (CHD) have improved, issues beyond the quantity of life have become more important. Quality of life (QOL) has mainly been investigated in adults with CHD. Hence, research about QOL among adolescents with CHD is much needed.Aim:To compare the QOL of adolescents with CHD with that of control subjects from the general population and to explore whether sense of coherence (SOC) explains differences in QOL between patients and controls.Methods:In this cross-sectional, comparative study, we included 429 patients (229 boys; 200 girls) aged 14-18 years, who were matched to control subjects for age and sex. QOL was measured with a Linear Analogue Scale; SOC was measured using the SOC-13.Results:Median QOL score in patients was 82 (Q1=75; Q3=90). The QOL of patients was significantly (Z=-5.888; p<0.001) better than that of controls (median=80; Q1=70; Q3=85.5). Adjusted for other potentially confounding factors, linear mixed modelling showed that the better QOL in patients was explained by a higher SOC (mean=61.4±12.0 vs. 53.6±10.4) and better perceived physical health (mean=87.0±13.8 vs. 85.3±13.2).Conclusions:This study found that adolescents with CHD have a good QOL, one that is better than that of control subjects from the general population. A stronger SOC and better perceived physical health are potential resources for better QOL in patients.
European journal of cardiovascular nursing: journal of the Working Group on Cardiovascular Nursing of the European Society of Cardiology 02/2013;
-
[show abstract]
[hide abstract]
ABSTRACT: Background:To optimize long-term outcomes, patients with congenital heart disease (CHD) should adopt health-promoting behaviors. Studies on health behavior in afflicted patients are scarce and comparability of study results is limited. To enlarge the body of evidence, we have developed the Health Behavior Scale-Congenital Heart Disease (HBS-CHD).Aims:We examined the psychometric properties of the HBS-CHD by providing evidence for (a) the content validity; (b) validity based on the relationships with other variables; (c) reliability in terms of stability; and (d) responsiveness.Methods:Ten experts rated the relevance of the HBS-CHD items. The item content validity index (I-CVI) and the averaged scale content validity index (S-CVI/Ave); the modified multi-rater Kappa and proportion of missing values for each question were calculated. Relationships with other variables were evaluated using six hypotheses that were tested in 429 adolescents with CHD. Stability of the instrument was assessed using Heise's method; and responsiveness was tested by calculating the Guyatt's Responsiveness Index (GRI).Results:Overall, 86.3% of the items had a good to excellent content validity; the S-CVI/Ave (0.81) and multi-rater Kappa (0.78) were adequate. The average proportion of missing values was low (1.2%). Because five out of six hypotheses were confirmed, evidence for the validity of the HBS-CHD based on relationships with other variables was provided. The stability of the instrument could not be confirmed based on our data. The GRI showed good to excellent capacity of the HBS-CHD to detect clinical changes in the health behavior over time.Conclusion:We found that the HBS-CHD is a valid and responsive questionnaire to assess health behaviors in patients with CHD.
European journal of cardiovascular nursing: journal of the Working Group on Cardiovascular Nursing of the European Society of Cardiology 01/2013;
-
[show abstract]
[hide abstract]
ABSTRACT: BACKGROUND: -Accurate measures are critical when attempting to distinguish normal from pathological changes in cardiac function during exercise, yet imaging modalities have seldom been assessed against invasive exercise standards. We sought to validate a novel method of biventricular volume quantification by cardiac magnetic resonance imaging (CMR) during maximal exercise. METHODS AND RESULTS: -CMR was performed on 34 subjects during exercise and free-breathing using an ungated real-time CMR ("RT-ungated") sequence. ECG and respiratory movements were retrospectively synchronized enabling compensation for cardiac cycle and respiratory phase. Feasibility of RT-ungated imaging was compared with standard exercise CMR imaging with ECG gating ("gated"), Accuracy of RT-ungated CMR was assessed against an invasive standard (direct Fick) and reproducibility was determined following a second bout of maximal exercise. Ventricular volumes were able to be analyzed more frequently during high-intensity exercise using RT-ungated as compared with gated CMR (100% vs. 47%, p<0.0001) and with better inter-observer variability for RT-ungated (coefficient of variation CV=1.9% and 2.0% for left and right ventricular stroke volumes, respectively) than gated (CV=15.2% and 13.6%), p <0.01. Cardiac output determined by RT-ungated CMR proved accurate against the direct Fick method with excellent agreement (intraclass correlation coefficient R=0.96) which was highly reproducible during a second bout of maximal exercise (R=0.98). CONCLUSIONS: -By combining real-time ungated CMR with post-hoc analysis incorporating compensation for respiratory motion, highly reproducible and accurate biventricular volumes can be measured during maximal exercise.
Circulation Cardiovascular Imaging 12/2012; · 5.94 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The ideal treatment strategy for pulmonary sequestration whether resection or embolization in childhood is not clearly defined. Our institution has no clear policy, therefore both therapies are performed.
The aim of this study was to assess local management strategies of children presenting with pulmonary sequestrations.
This is a retrospective, single-institutional review. The main inclusion criterion was the established diagnosis of a pulmonary sequestration. Forty-eight patients were divided into three groups based on treatment received: conservative management (n = 5), surgery (n = 22) and embolization (n = 21).
The median age at treatment was 8.0 months (range 1.2- 166.0) in the surgical, 4.0 months (range 0.2 - 166.0) in the embolization and 8 months (range 0.3 - 197.0) in the conservatively managed groups, respectively. Age at treatment was similar in the surgical and embolization groups (P = 0.9). Recurrent chest infections were the most common clinical presentation in the surgical group, whilst cardiac failure was the most frequent symptom in the embolization group (P < 0.01; 95% CI: 0.3 to 0.9). There were six complications in the surgical group and one in the embolization group (P = 0.1). In one patient embolization was not possible. Outcomes in both groups were comparable with good results on follow-up.
Both surgery and endovascular embolization are effective and safe treatments for pulmonary sequestration. The presenting symptoms dictate therapy: surgery if there is infection and embolization if a shunt needs to be abolished. Our institutional policy remains unchanged.
Acta cardiologica 12/2012; 67(6):629-34. · 0.61 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: BACKGROUND: It is unclear how autografts grow and dilate after the Ross operation in children. We analysed autograft growth and dilatation in children who underwent the Ross operation and examined the relationship of these factors to autograft failure. METHODS: From our institutional database, we retrospectively identified 33 children who underwent the Ross operation without aortic root reinforcement (mean age 9.9 years) and had normal body measurements and echocardiographic data throughout follow-up. RESULTS: Autograft insufficiency developed in 10 patients 5.1 years after the Ross operation. The average Z score at the development of autograft insufficiency was -0.1 (range from -2.0 to 6.1). The proportions of patients who remained free of autograft insufficiency at 5 and 10 years were 87.2% and 55.7%, respectively. A consistent trend in the time course of Z score was not found in any age group studied. CONCLUSIONS: Autograft growth and dilation after the Ross operation varied widely among patients, and the incidence of autograft insufficiency was independent of annulus size.
Cardiology in the Young 10/2012; · 0.76 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVES: Patients with pulmonary atresia and intact ventricular septum (PA-IVS) may have coronary sinusoids connected to a hypertensive right ventricle. Coronary perfusion may then completely depend on the right ventricular systolic pressure wave; decompression of the right ventricle can be deleterious in these patients. This study was set to investigate the treatment strategies and outcomes of patients with coronary sinusoids. METHODS: National multicentre retrospective analysis over 15 years (1985-2010) in a population of ∼10.2 million people. All patients with PA-IVS and coronary sinusoids were identified from local databases. All angiograms, echocardiograms, surgical reports and outcome data were reviewed. RESULTS: Thirty patients were identified. Right ventricular-dependent coronary circulation was present in nine patients (30%). A systemic-to-pulmonary artery shunt was created in 23 patients (77%) at a median age of 13.0 days (range: 1-479). A bidirectional Glenn was performed in 20 children at a median age of 7.8 months (range: 2.1-112.9) and 11 children proceeded to a Fontan repair at a median age of 3.6 years (range: 2.1-19.6). Pulmonary valve perforation and angioplasty were performed in six children and in three a simultaneous decompression procedure was also done. Ten patients died (33%). Four died at the time of systemic-to-pulmonary artery shunt and three during the bidirectional Glenn shunt. In all these patients myocardial ischaemia was reported. CONCLUSIONS: Mortality in PA-IVS with sinusoids is high. Right ventricular coronary dependence may lead to early death if the right ventricular systolic pressure wave is interrupted. Adequate understanding of the underlying pathology and pathophysiology is essential before attempting any procedure in this subgroup of children.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 10/2012; · 2.40 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: BACKGROUND: Patients with Senning repair for complete transposition of the great arteries (d-TGA) show an impaired exercise tolerance. Our aim was to investigate changes in exercise capacity in children, adolescents and adults with Senning operation. METHODS: Peak oxygen uptake (peak VO2), oxygen pulse and heart rate were assessed by cardiopulmonary exercise tests (CPET) and compared to normal values. Rates of change were calculated by linear regression analysis. Right ventricular (RV) function was assessed by echocardiography. RESULTS: Thirty-four patients (22 male) performed 3.5 (range 3--6) CPET with an interval of >= 6 months. Mean age at first assessment was 16.4 +/- 4.27 years. Follow-up period averaged 6.8 +/- 2 years. Exercise capacity was reduced (p<0.0005) and the decline of peak VO2 (-1.3 +/- 3.7 %/year; p=0.015) and peak oxygen pulse (-1.4 +/- 3.0 %/year; p=0.011) was larger than normal, especially before adulthood and in female patients (p<0.01). During adulthood, RV contractility changes were significantly correlated with the decline of peak oxygen pulse (r= -0.504; p=0.047). CONCLUSIONS: In patients with Senning operation for d-TGA, peak VO2 and peak oxygen pulse decrease faster with age compared to healthy controls. This decline is most obvious during childhood and adolescence, and suggests inability to increase stroke volume to the same extent as healthy peers during growth. Peak VO2 and peak oxygen pulse remain relatively stable during early adulthood. However, when RV contractility decreases, a faster decline in peak oxygen pulse is observed.
BMC Cardiovascular Disorders 10/2012; 12(1):88. · 1.52 Impact Factor
-
08/2012; 5(8):e25-6. · 1.07 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A 74-year-old woman was treated by balloon pulmonary valvuloplasty for a symptomatic restenosis 30 years after surgical commissurotomy. The valve dilatation was complicated by a rupture of the right ventricular outflow tract, treated in emergency by a covered stent implantation. The hemodynamic situation was improved, but the free pulmonary regurgitation was leading to a symptomatic right ventricular enlargement. Because surgery was not a good option in this patient at high risk, restoration of pulmonary valve competence was performed by a transcatheter Sapien valve implantation. Transcatheter approach allowed to treat the severe complication of pulmonary valvuloplasty without risk associated with an emergent and redo surgery in a high-risk patient.
Congenital Heart Disease 07/2012; · 0.90 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Human homografts are frequently used to establish an anatomic continuity between the right ventricular outflow tract (RVOT) and the pulmonary artery. Their limited availability, especially in small sizes, has encouraged the use of alternative strategies, such as size-reduced bicuspid homografts. The study aim was to analyze the follow up of patients who had received a standard tricuspid or size-reduced bicuspid homograft in the RVOT position, and to investigate modifications of the patients' Z-scores over the years.
A consecutive series of 107 patients aged < or = 16 years, who underwent RVOT repair between 1989 and 2010 to treat tetralogy of Fallot (ToF), was retrospectively reviewed. Of these patients, 17 received a size-reduced bicuspid pulmonary homograft, while 90 received a standard tricuspid homograft. The mean follow up periods were 10.5 years (range: 0.02-21.4 years) for the whole study population, and 11.8 years and 3.4 years, respectively, for the tricuspid and size-reduced bicuspid homograft groups.
Freedom from mortality at 10 years was 95 +/- 3%. During the observation period, 27 patients (31%) in the tricuspid homograft group and two (125) in the size-reduced bicuspid group presented with graft failure. According to the multivariable analysis, the only independent predictor of graft failure was patient age (hazards ratio 0.86). The 17 patients who had received a size-reduced bicuspid homograft were then age-matched to an equal-sized population of tricuspid homograft patients. A comparative analysis of the time-weighted average of the Z-scores for these tricuspid and size-reduced bicuspid homograft subgroups during the follow up period failed to identify any statistical difference (p = 0.5).
In terms of Z-score evolution, size-reduced bicuspid homografts offer results which are comparable to those achieved with tricuspid homografts.
The Journal of heart valve disease 07/2012; 21(4):521-6. · 0.81 Impact Factor
-
American Journal of Medical Genetics Part A 06/2012; 158A(8):2053-4. · 2.39 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVE: Postsurgical atrioventricular block may complicate surgery for congenital heart defects and is generally considered permanent when persisting longer than 14 days after surgery. In this study, we evaluate the occurrence of spontaneous late recovery of atrioventricular conduction in postsurgical chronic atrioventricular block and discuss its clinical implications. METHODS: We retrospectively reviewed all cardiac surgical procedures on cardiopulmonary bypass between January 1993 and November 2010 in subjects younger than 18 years. Patients with postsurgical advanced second- or third-degree atrioventricular block persisting longer than 14 days after surgery were included. RESULTS: Of a total of 2850 cardiac surgical procedures on cardiopulmonary bypass, 59 (2.1%) were immediately complicated by chronic postsurgical atrioventricular block of advanced second (n = 4) or third degree (n = 55). In another 6 patients (0.2%), late occurrence of chronic advanced second- (n = 3) or third-degree (n = 3) atrioventricular block, without signs of any etiology other than previous surgery, was seen 0.4 to 10 years after surgery (median, 5.7 years). Late (>2 weeks) regression to either completely normal atrioventricular conduction or asymptomatic first-degree atrioventricular block occurred 3 weeks to 7 years (median, 3.1 years) after surgery in 7 (12%) patients with immediate postsurgical chronic atrioventricular block. CONCLUSIONS: Complete recovery of atrioventricular conduction or regression to asymptomatic first-degree atrioventricular block occurred in 12% of patients with postsurgical chronic second- or third-degree atrioventricular block. To prevent unnecessary adverse side effects of chronic ventricular pacing and to prolong battery longevity, ventricular pacing should be minimized in patients with recovered normal atrioventricular conduction.
The Journal of thoracic and cardiovascular surgery 06/2012; · 3.41 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We present a neonate with a complex congenital cardiopathy and a right-sided diaphragmatic hernia complicated with hepatopulmonary fusion. Radiography, abdominal US and multidetector CT (MDCT) demonstrated right-sided lung hypoplasia and liver herniation. In addition, MDCT angiography showed abnormal pulmonary vascular anatomy. At surgery, a right-sided diaphragmatic hernia with a partially herniated liver and hepatopulmonary fusion was confirmed. There was no aberrant systemic vascular supply towards the lower lobe, as seen in extralobar sequestration. MDCT angiography of the chest and upper abdomen with optimal enhancement and reconstruction of the pulmonary and hepatic vasculature can demonstrate associated anomalies in cases of suspected primary or secondary right lung hypoplasia.
Pediatric Radiology 04/2012; 42(9):1138-41. · 1.67 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A morphological right ventricle (RV) is not ideally suited for the long-term maintenance of the systemic circulation. The aim of this analysis was to evaluate the intermediate results and outcome of pulmonary artery banding (PAB) in an open-ended strategy.
This is a retrospective review of patients with systemic RVs who had undergone PAB in our institution from April 1985 to January 2011. PAB was placed in 5 patients late after the Senning operation and in 15 patients with corrected transposition; of whom, 6 had a large ventricular septal defect.
PAB was performed at a median age of 4.3 years (range: 0.9-14.9), median follow-up of 86 months (range: 0.5-379). All 20 patients are alive and are being followed up. Tricuspid regurgitation (TR), RV function and dilation showed no deterioration after banding (P = 0.9). Ninety per cent (18/20) have adequate ongoing palliation with PAB. One patient underwent a double-switch operation and one received an additional bidirectional Glenn shunt. A dilatable band was redilated with improvement in percutaneous saturation and in another the procedure was abandoned due to development of transient atrioventricular block. Functional class remained either unchanged or improved.
PAB was performed with no mortality and low morbidity. PAB in these heterogeneous patients provides true 'open ended palliation' by allowing left ventricular training in those going for anatomical repair, stabilizing or improving RV function and TR in others, thereby delaying surgery. It can also be left in place as long-term palliation. The addition of a dilatable band allows manipulation of pulmonary flows, but longer follow-up is required to provide data on best management strategies for these complex patients.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 04/2012; 41(4):913-8. · 2.40 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: An infant developed severe right heart failure early after truncal repair with a pulmonary homograft. A mechanical obstruction by narrowing could not be identified at the homograft or pulmonary arteries. However, functional obstruction was caused by an extreme windkessel effect in a massively dilated homograft that absorbed rather than transmitted the pulse wave. Effective treatment consisted of replacing the dilated homograft by a rigid aortic homograft of equal size as the initial homograft. When confronted with circulatory failure after allograft placement, the clinician should not only look for obstruction by narrowing, but also consider the windkessel phenomenon.
Interactive cardiovascular and thoracic surgery 03/2012; 15(1):181-2.
-
[show abstract]
[hide abstract]
ABSTRACT: A typical scimitar vein was surgically corrected in a female patient of 35 years old. The postoperative course was uneventful and in the first months after the functional capacity normalized. Six months after the correction, the patient developed progressive dyspnea. Kinking and distal stenosis of the intracardiac baffle which connected the scimitar vein to the left atrium were diagnosed. Balloon dilatation and stenting could normalize the blood flow through the baffle. The patient remains symptom-less up to six months after the procedure.© 2012 Wiley Periodicals, Inc.
Catheterization and Cardiovascular Interventions 03/2012; · 2.29 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The proximal region of the long arm of chromosome 22 is rich in low copy repeats (LCR). Non-allelic homologous recombination (NAHR) between these substrates explains the high prevalence of recurrent rearrangements within this region. We have performed array comparative genomic hybridization in a normally developing girl with growth delay, microcephaly, and truncus arteriosus, and have identified a novel recurrent 22q11 deletion that spans LCR22-4 and partially affects the common 22q11.2 deletion syndrome and the distal 22q11 deletion syndrome. This deletion is atypical as it did not occur by NAHR between any of the major LCRs found on 22q11.2. However, the breakpoint containing regions coincide with highly homologous regions. An identical imbalance was reported previously in a patient with striking phenotypic similarity. Computational gene prioritization methods and biological evidence denote the genes CRKL and MAPK1 as the highest ranking candidates for causing congenital heart disease within the deleted region.
American Journal of Medical Genetics Part A 03/2012; 158A(3):574-80. · 2.39 Impact Factor
