Long-term outcome after surgery for congenital heart disease in infancy and childhood

Before the era of cardiac surgery about half of the patients born with a congenital heart defect died within the first year of life. Survival until adulthood was very rare for patients with transposition of the great arteries (20 years survival <1 %), rare with tetralogy of Fallot (20 years survival <10%) and although survival beyond two decades was more common for patients with atrial septal defect, ventricular septal defect and pulmonary stenosis, their life expectancy also was considerably reduced

Assessment of pulmonary valve and right ventricular outflow tract with real-time three-dimensional echocardiography

Aim: Assessment of pulmonary valve (PV) and right ventricular outflow tract (RVOT) using real-time 3-dimensional echocardiography (RT3DE). Methods: Two-dimensional echocardiography (2DE) and RT3DE were performed in 50 patients with congenital heart disease (mean age 32 ± 9.5 years, 60% female). Measurements were obtained at parasternal views: short axis (PSAX) at aortic valve level and long axis (PLAX) with superior tilting. RT3DE visualization was evaluated by 4-point score (1: not visualized, 2: inadequate, 3: sufficient, and 4: excellent). Diameters of PV annulus (PVAD), and RVOT (RVOTD) were measured by both 2DE and RT3DE, while areas (PVAA) and (RVOTA) by RT3DE only. Results: By RT3DE, PV was visualized sufficiently in 68% and RVOTexcellently in 40%. PVAD and PVAA were measured in 88%. RVOTD and PVAD by 2DE at PLAX were significantly higher than PSAX (P < 0.0001) and lower than that by RT3DE (P < 0.001). Conclusion: RT3DE helps inRVOT and PV assessment adding more details supplemental to 2DE

Delivery of care for adult patients with congenital heart disease in Europe: Results from the Euro Heart Survey

Aims: The increasing number of adults with congenital heart disease (CHD) has prompted the development of recommendations for the management of these patients and for the organization of their healthcare. The aim of this report is to describe the delivery of care in Europe for adults with congenital cardiac anomalies. Methods and results: As part of the Euro Heart Survey on Adult Congenital Heart Disease, we obtained data from 71 voluntarily participating centres that detailed their care practices for these patients. Forty-eight of these centres were specialist centres and 23 were non-specialist centres. We found that only 19% of the specialist centres complied with defined standards for optimal care structure. The criteria that appeared to be most difficult for all centres to achieve were performing 50 congenital heart operations or more per year and involving nurse specialists in the care of these patients. Conclusion: This survey indicated that the provision of care in Europe for adults with congenital

Cardiac status and health-related quality of life in the long term after surgical repair of tetralogy of Fallot in infancy and childhood

The long-term results of surgical repair of tetralogy of Fallot were assessed by means of extensive cardiologic examination of 77 nonselected patients 14.7 +/- 2.9 years after surgical repair of tetralogy of Fallot in infancy and childhood. Because of the frequent use of a transannular patch (56%) for the relief of right ventricular outflow tract obstruction, the prevalence of elevated right ventricular systolic pressure was low (8%), but the prevalence of substantial right ventricular dilation with severe pulmonary regurgitation was high (58%). The exercise capacity of patients with a substantially dilated right ventricle proved to be significantly decreased (83% +/- 19% of predicted) when compared with that of patients with a near normal sized right ventricle (96% +/- 13%). Eight out of 10 patients who had needed treatment for symptomatic arrhythmia had supraventricular arrhythmia, which makes supraventricular arrhythmia--in numbers--a more important sequela in the long-term survivors than ventricular arrhythmia. Older age at the time of the operation and longer duration of follow-up were not associated with an increase in prevalence or clinical significance of sequela

Aortic valve and aortic arch pathology after coarctation repair

OBJECTIVE: To investigate the incidence of clinical problems related to a bicuspid valve (aortic stenosis and regurgitation) and the incidence of ascending aorta and aortic arch pathology in combination with coarctation repair. PATIENTS: 124 adult patients after surgical correction of aortic coarctation were studied. The incidence of aortic valve, ascending aorta, and aortic arch pathology was determined using echocardiography and magnetic resonance imaging. The median age at coarctation repair was 9 years and at last follow up 28 years. RESULTS: Three patients died from aorta pathology. Aortic valve disease was found in 63% of the patients, requiring an intervention in 22%, at a median of 13 years after coarctation repair. Ascending aorta dilatation was observed in 28% and aortic arch abnormalities in 23%, among whom kinking of the aortic arch was found in 12%. Antihypertensive medication was used in 24%. In the patients with hypertension the age at operation and age at follow up were significantly higher (p = 0.0001 and p < 0.0001, respectively). CONCLUSION: In addition to the well known problems of hypertension and recoarctation, aortic valve and aortic arch pathology are commonly encountered in patients with previous coarctation repair. Aortic abnormalities may predispose to dilatation and dissection, thus necessitating careful lifelong attention in all patients with coarctation

Excellent survival and low incidence of arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age. A prospective follow-up study of 21-33 years

AIMS: Although studies have suggested good long-term results, arrhythmias, pulmonary hypertension and left ventricular dysfunction are mentioned as sequelae long-term after surgical atrial septal defect closure at young age. Most studies were performed only by questionnaire and in a retrospective manner. The long-term outcome is very important with regard to future employment and acceptance on insurance schemes. METHODS AND RESULTS: One hundred and thirty-five consecutive ASD-patients, operated on in childhood, were studied longitudinally with ECG, echocardiography, exercise testing and Holter-recording 15 (10-22) and 26 (21-33) years after surgery. During follow-up no cardiovascular mortality, stroke, heart failure and no pulmonary hypertension occurred. Symptomatic supraventricular tachyarrhythmias were present in 6% after 15 years, and an additional 2% occurred in the last decade; 5% needed pacemaker implantation. No relation was found between arrhythmias and type of ASD, baseline data, right ventricular dimensions, or age at operation. Left and right ventricular function and dimension remained unchanged. Slightly more patients had right atrial dilatation at last follow-up. Exercise capacit

Virtual reality 3D echocardiography in the assessment of tricuspid valve function after surgical closure of ventricular septal defect

Background. This study was done to investigate the potential additional role of virtual reality, using three-dimensional (3D) echocardiographic holograms, in the postoperative assessment of tricuspid valve function after surgical closure of ventricular septal defect (VSD). Methods. 12 data sets from intraoperative epicardial echocardiographic studies in 5 operations (patient age at operation 3 weeks to 4 years and bodyweight at operation 3.8 to 17.2 kg) after surgical closure of VSD were included in the study. The data sets were analysed as two-dimensional (2D) images on the screen of the ultrasound system as well as holograms in an I-space virtual reality (VR) system. The 2D images were assessed for tricuspid valve function. In the I-Space, a 6 degrees-of-freedom controller was used to create the necessary projectory positions and cutting planes in the hologram. The holograms were used for additional assessment of tricuspid valve leaflet mobility. Results. All data sets could be used for 2D as well as holographic analysis. In all data sets the area of interest could be identified. The 2D analysis showed no tricuspid valve stenosis or regurgitation. Leaflet mobility was considered normal. In the virtual reality of the I-Space, all data sets allowed to assess the tricuspid leaflet level in a single holographic representation. In 3 holograms the septal leaflet showed restricted mobility that was not appreciated in the 2D echocardiogram. In 4 data sets the posterior leaflet and the tricuspid papillary apparatus were not completely included. Conclusion. This report shows that dynamic holographic imaging of intraoperative postoperative echocardiographic data regarding tricuspid valve function after VSD closure is feasible. Holographic analysis allows for additional tricuspid valve leaflet mobility analysis. The large size of the probe, in relation to small size of the patient, may preclude a complete data set. At the moment the requirement of an I-Space VR system limits the applicability in virtual reality 3D echocardiography in clinical practice

Assessment of tricuspid valve annulus size, shape and function using real-time three-dimensional echocardiography

Tricuspid annulus (TA) evaluation continues to be a major problem in the surgical decision-making process. Obviously, 2-dimensional transthoracic echocardiography (2D TTE) is limited in TA visualization due to its complex 3D shape. The study aimed to determine TA morphology, size and function with real-time three-dimensional echocardiography (RT3DE) in 40 patients divided into two equal groups (I: normal TA and II: dilated). 2D TTE measurements included TA diameter (TAD) at apical 4-chamber (AP4CH) and parasternal short axis (PSAX) views. RT3DE measurements included TA area (TAA), major TAD and minor TAD. TA fractional shortening (TAFS), and TA fractional area change (TAFAC) were calculated from end-systolic and end-diastolic measurements. RT3DE allowed visualization and measurement of the entire oval-shaped TA in all patients irrespective of its size (normal or dilated). 2D TTE measurement of TAD at both AP4CH and at PSAX views was significantly smaller than the major TAD measured by RT3DE (P<0.0001) and nearly matched with the minor TAD in all patients. Calculation of TAFS was comparable with both techniques. TAFAC was significantly higher than TAFS (P<0.0001). So, RT3DE could be relied on more accurately than 2D TTE in the assessment of TA size and function which may aid in decision-making and selection of proper surgical procedure when indicated

The use of contrast echocardiography for the detection of cardiac shunts

Recently, debate has erupted about the clinical significance of cardiovascular shunts. Several major health problems such as stroke and migraine have been associated with patent foramen ovale (PFO) with right-to-left shunt (RLS). The nature of the relationship between these syndromes and PFO is not clearly understood. Technical advances have led to more therapeutic options including device closure of PFO, hence prevention of such a PFO-related stroke has become feasible. Therefore, optimal diagnosis of PFO has become of greater clinical importance. Contrast echocardiography with non-transpulmonary contrast agents has been the cornerstone in diagnosis of PFO with RLS for over four decades. Despite being a relatively invasive procedure, transesophageal echocardiography (TEE) is considered the gold standard for detection of RLS. Several other echocardiographic techniques such as transthoracic echocardiography (TTE) with second harmonic imaging and transcranial Doppler ultrasonography (TCD) have shown increased sensitivity and specificity compared to TEE for the detection of PFO with RLS. Moreover, improvement of skills and techniques used for detection of these shunts has led to greater detection of small and large sized RLS in the echocardiographic laboratory. This review gives and overview of the echocardiographic techniques, contrast agents and manoeuvres used for detection of the major cardiovascular shunts and their clinical relevance to major health problems

Rapid and accurate measurement of LV mass by biplane real-time 3D echocardiography in patients with concentric LV hypertrophy: Comparison to CMR

Aims: To evaluate the accuracy of real-time three-dimensional echocardiography (RT3DE) using a biplane and multiplane method in determining left ventricular (LV) mass compared to cardiac magnetic resonance imaging (CMR). Methods and results: LV mass was measured in 18 adult patients with congenital aortic stenosis using CMR and echocardiography (M-mode, two-dimensional echocardiography (2DE), and RT3DE). RT3DE data were analysed using a biplane and multiplane method. No geometric assumptions were necessary using the multiplane RT3DE method.With regard to biplane or multiplane RT3DE, no tendency of over- or underestim