Magnetization Transfer BOOST Noncontrast Angiography Improves Pulmonary Vein Imaging in Adults With Congenital Heart Disease.

BACKGROUND: Cardiac MRI plays an important role in the diagnosis and follow-up of patients with congenital heart disease (CHD). Gadolinium-based contrast agents are often needed to overcome flow-related and off-resonance artifacts that can impair the quality of conventional noncontrast 3D imaging. As serial imaging is often required in CHD, the development of robust noncontrast 3D MRI techniques is desirable. PURPOSE: To assess the clinical utility of noncontrast enhanced magnetization transfer and inversion recovery prepared 3D free-breathing sequence (MTC-BOOST) compared to conventional 3D whole heart imaging in patients with CHD. STUDY TYPE: Prospective, image quality. POPULATION: A total of 27 adult patients (44% female, mean age 30.9 ± 14.8 years) with CHD. FIELD STRENGTH/SEQUENCE: A 1.5 T; free-breathing 3D MTC-BOOST sequence. ASSESSMENT: MTC-BOOST was compared to diaphragmatic navigator-gated, noncontrast T2 prepared 3D whole-heart imaging sequence (T2prep-3DWH) for comparison of vessel dimensions, lumen-to-myocardium contrast ratio (CR), and image quality (vessel wall sharpness and presence and type of artifacts) assessed by two experienced cardiologists on a 5-point scale. STATISTICAL TESTS: Mann-Whitney test, paired Wilcoxon signed-rank test, Bland-Altman plots. P < 0.05 was considered statistically significant. RESULTS: MTC-BOOST significantly improved image quality and CR of the right-sided pulmonary veins (PV): (CR: right upper PV 1.06 ± 0.50 vs. 0.58 ± 0.74; right lower PV 1.32 ± 0.38 vs. 0.81 ± 0.73) compared to conventional T2prep-3DWH imaging where the PVs were not visualized in some cases due to off-resonance effects. MTC-BOOST demonstrated resistance to degradation of luminal signal (assessed by CR) secondary to accelerated or turbulent flow conditions. T2prep-3DWH had higher image quality scores than MTC-BOOST for the aorta and coronary arteries; however, great vessel dimensions derived from MTC-BOOST showed excellent agreement with standard T2prep-3DWH imaging. DATA CONCLUSION: MTC-BOOST allows for improved contrast-free imaging of pulmonary veins and regions characterized by accelerated or turbulent blood flow compared to standard T2prep-3DWH imaging, with excellent agreement of great vessel dimensions. EVIDENCE LEVEL: 1 TECHNICAL EFFICACY: Stage 2.

Hybrid trans-apical atrioventricular valve-in-valve implantation in Fontan circulation.

Trans-apical approach has been proved successful in failing surgical bio-prosthesis in both mitral and aortic position in adult patients. Recently, valve-in-valve treatments have been applied even in patients with complex congenital heart disease. Here, we report the case of a 32 years old lady with left atrial isomerism, complete AV septal defect, interrupted inferior vena cava with azygos continuation who underwent Kawashima procedure with atrial Fontan. Severe systemic atrioventricular valve regurgitation necessitated a 33 mm Perimount valve implantation and conversion to lateral tunnel Fontan. After only 4 years there was severe valve stenosis and the patient underwent successful trans-apical transcatheter implantation of a 29 mm Sapien valve.

Dynamic heart rate response to multi-day unsupported ultra-endurance cycle racing: a case report.

NEW FINDINGS: What is the main observation in this case? Ultra-endurance cycle racing is known to lead to suppressed heart rates as a product of time spent racing. This case report identifies a racer who experienced this phenomenon initially, but then uniquely experienced an overall increase in heart rate late in the race. What insight does it reveal? In this case, unique chronotropic disturbances to heart rate occurred as a result of the many extreme demands of ultra-endurance racing. Work should now focus on identifying the frequency of this response in other racers and whether the main causes are physiological, environmental or genetic in nature. ABSTRACT: Participation in ultra-endurance cycling events, such as the Transcontinental Race, is increasing. These extremely demanding races provide a unique opportunity for field observation of the limits of human endurance physiology and, importantly, when these limits might be exceeded and cross over into pathology. The heart is of special interest in this field, and previous data suggest that 'reverse drift' of heart rate occurs as a product of time and load in races of 24-48 h, whereas transient structural abnormalities have been observed upon completion of running ultramarathons. Here, we report a unique case of a male cyclist racing in the Transcontinental Race over an extended period of 14 days characterized by extreme workloads and a low quantity and quality of sleep. The heart rate response was dynamic over the course of the race and defined by a U-shaped quadratic relationship. A larger scale study is required to determine the relevance of this information to the ultra-endurance cycling community.

Physiological and phenotypic characteristics of late survivors of tetralogy of fallot repair who are free from pulmonary valve replacement.

BACKGROUND: Pulmonary valve replacement (PVR) after repair of tetralogy of Fallot is commonly required and is burdensome. Detailed anatomic and physiologic characteristics of survivors free from late PVR and with good exercise capacity are not well described in a literature focusing on the indications for PVR. METHODS AND RESULTS: Survival and freedom from PVR were tracked in 1085 consecutive patients receiving standard tetralogy of Fallot repair in a single institution from 1964 to 2009. Of 152 total deaths, 100 occurred within the first postoperative year. Surviving patients between 10 and 50 years of age had an annual risk of death of 4 (confidence limit, 2.8-5.4) times that of normal contemporaries. To date, 189 patients have undergone secondary PVR at mean age of 20±13 years (36% of those alive at 40 years of age). A random sample of 50 survivors (age, 4-57 years) free from PVR underwent cardiovascular magnetic resonance, echocardiography, and exercise testing. These patients had mildly dilated right ventricles (right ventricular end-diastolic volume=101±26 mL/m(2)) with good systolic function (right ventricular ejection fraction=59±7%). Most had exercise capacity within normal range (z peak o2=-0.91±1.3; z e/ co2=0.20±1.5). In patients >35 years of age with normal exercise capacity, there was mild residual right ventricular outflow tract obstruction (mean gradient, 24±13 mm Hg), pulmonary annulus diameters <0.5z, and unobstructed branch pulmonary arteries. CONCLUSIONS: An important proportion of patients require PVR late after tetralogy of Fallot repair. Patients surviving to 35 years of age without PVR and with a normal exercise capacity may have had a definitive primary repair; their right ventricular outflow tracts are characterized by mild residual obstruction and pulmonary annulus diameter <0.5z.

Exercise capacity, quality of life, and resilience after repair of tetralogy of Fallot: a cross-sectional study of patients operated between 1964 and 2009.

INTRODUCTION: Patients with repaired tetralogy of Fallot have good long-term survival but less is known about the subjectively assessed quality of life or objectively measured functional status of those who have not required subsequent pulmonary valve replacement. We assessed these parameters in a group of children and adults free from pulmonary valve replacement after tetralogy of Fallot repair. METHODS AND RESULTS: A random sample of 50 subjects--16 children and 34 adults, aged 4.1-56.7 years---who had undergone tetralogy of Fallot repair and were free from subsequent pulmonary valve replacement underwent cardiopulmonary exercise testing and completed standardised questionnaires assessing health-related quality of life and resilience. Patients were generally asymptomatic (median New York Heart Association class = I). Exercise capacity was within two standard deviations of normal for most children and adults (mean z VO2max: 0.20 ± 1.5; mean z VE/VCO2: -0.9 ± 1.3). Children reported a total health-related quality of life score similar to healthy norms (78 ± 10 versus 84 ± 1, p = 0.73). Adult survivors also reported quality of life scores comparable to healthy norms. Resilience was highly correlated with all domains of health-related quality of life (r = 0.713, p < 0.0001). CONCLUSIONS: Patients who have undergone tetralogy of Fallot repair in childhood and have not required pulmonary valve replacement have a good long-term health-related quality of life. The finding that patients with greater resilience had better health-related quality of life suggests that it may be beneficial to implement interventions to foster resilience.

First report from the European registry for anomalous aortic origin of coronary artery (EURO-AAOCA).

OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is a group of rare congenital heart defects with various clinical presentations. The lifetime-risk of an individual living with AAOCA is unknown, and data from multicentre registries are urgently needed to adapt current recommendations and guide optimal patient management. The European AAOCA Registry (EURO-AAOCA) aims to assess differences with regard to AAOCA management between centres. METHODS: EURO-AAOCA is a prospective, multicentre registry including 13 European centres. Herein, we evaluated differences in clinical presentations and management, treatment decisions and surgical outcomes across centres from January 2019 to June 2023. RESULTS: A total of 262 AAOCA patients were included, with a median age of 33 years (12-53) with a bimodal distribution. One hundred thirty-nine (53.1%) were symptomatic, whereas chest pain (n = 74, 53.2%) was the most common complaint, followed by syncope (n = 21, 15.1%). Seven (5%) patients presented with a myocardial infarction, 2 (1.4%) with aborted sudden cardiac death. Right-AAOCA was most frequent (150, 57.5%), followed by left-AAOCA in 51 (19.5%), and circumflex AAOCA in 20 (7.7%). There were significant differences regarding diagnostics between age groups and across centres. Seventy-four (28.2%) patients underwent surgery with no operative deaths; minor postoperative complications occurred in 10 (3.8%) cases. CONCLUSIONS: Currently, no uniform agreement exists among European centres with regard to diagnostic protocols and clinical management for AAOCA variants. Although surgery is a safe procedure in AAOCA, future longitudinal outcome data will hopefully shed light on how to best decide towards optimal selection of patients undergoing revascularization versus conservative treatment.

Free-breathing, Contrast Agent-free Whole-Heart MTC-BOOST Imaging: Single-Center Validation Study in Adult Congenital Heart Disease.

Purpose: To assess the clinical performance of the three-dimensional, free-breathing, Magnetization Transfer Contrast Bright-and-black blOOd phase-SensiTive (MTC-BOOST) sequence in adult congenital heart disease (ACHD). Materials and Methods: In this prospective study, participants with ACHD undergoing cardiac MRI between July 2020 and March 2021 were scanned with the clinical T2-prepared balanced steady-state free precession sequence and proposed MTC-BOOST sequence. Four cardiologists scored their diagnostic confidence on a four-point Likert scale for sequential segmental analysis on images acquired with each sequence. Scan times and diagnostic confidence were compared using the Mann-Whitney test. Coaxial vascular dimensions at three anatomic landmarks were measured, and agreement between the research sequence and the corresponding clinical sequence was assessed with Bland-Altman analysis. Results: The study included 120 participants (mean age, 33 years ± 13 [SD]; 65 men). The mean acquisition time of the MTC-BOOST sequence was significantly lower compared with that of the conventional clinical sequence (9 minutes ± 2 vs 14 minutes ± 5; P < .001). Diagnostic confidence was higher for the MTC-BOOST sequence compared with the clinical sequence (mean, 3.9 ± 0.3 vs 3.4 ± 0.7; P < .001). Narrow limits of agreement and mean bias less than 0.08 cm were found between the research and clinical vascular measurements. Conclusion: The MTC-BOOST sequence provided efficient, high-quality, and contrast agent-free three-dimensional whole-heart imaging in ACHD, with shorter, more predictable acquisition time and improved diagnostic confidence compared with the reference standard clinical sequence.Keywords: MR Angiography, Cardiac Supplemental material is available for this article. Published under a CC BY 4.0 license.

Provocation and localization of atrial ectopy in patients with atrial septal defects.

BACKGROUND: Atrial fibrillation (AF) is associated with atrial septal defects (ASDs), but the mechanism of arrhythmia in these patients is poorly understood. We hypothesised that right-sided atrial ectopy may predominate in this cohort. Here, we aimed to localise the origin of spontaneous and provoked atrial ectopy in ASD patients. METHODS: Following invasive calibration of P-wave axes, 24-h Holter monitoring was used to determine the chamber of origin of spontaneous atrial ectopy. Simultaneous electrogram recording from multiple intra-cardiac catheters was used to determine the chamber of origin of isoprenaline-provoked ectopy. Comparison was made to a group of non-congenital heart disease AF patients. RESULTS: Amongst ASD patients, a right-sided origin for spontaneous atrial ectopy was significantly more prevalent than a left-sided origin (24/30 patients with right-sided ectopy vs. 14/30 with left-sided ectopy, P = 0.015). Amongst AF patients, there was no difference in the prevalence of spontaneous right vs. left-sided ectopy. For isoprenaline-provoked ectopy, there was no significant difference in the proportions of patients with right-sided or left-sided ectopy in either group. CONCLUSIONS: When spontaneous atrial ectopy occurs in ASD patients, it is significantly more prevalent from a right-sided than left-sided origin. Isoprenaline infusion did not reveal the predilection for right-sided ectopy during electrophysiology study.

CArdiac MagnEtic resonance assessment of bi-Atrial fibrosis in secundum atrial septal defects patients: CAMERA-ASD study.

AIMS: Atrial septal defects (ASD) are associated with atrial arrhythmias, but the arrhythmia substrate in these patients is poorly defined. We hypothesized that bi-atrial fibrosis is present and that right atrial fibrosis is associated with atrial arrhythmias in ASD patients. We aimed to evaluate the extent of bi-atrial fibrosis in ASD patients and to investigate the relationships between bi-atrial fibrosis, atrial arrhythmias, shunt fraction, and age. METHODS AND RESULTS: Patients with uncorrected secundum ASDs (n = 36; 50.4 ± 13.6 years) underwent cardiac magnetic resonance imaging with atrial late gadolinium enhancement. Comparison was made to non-congenital heart disease patients (n = 36; 60.3 ± 10.5 years) with paroxysmal atrial fibrillation (AF). Cardiac magnetic resonance parameters associated with atrial arrhythmias were identified and the relationship between bi-atrial structure, age, and shunt fraction studied. Bi-atrial fibrosis burden was greater in ASD patients than paroxysmal AF patients (20.7 ± 14% vs. 10.1 ± 8.6% and 14.8 ± 8.5% vs. 8.6 ± 6.1% for right and left atria respectively, P = 0.001 for both). In ASD patients, right atrial fibrosis burden was greater in those with than without atrial arrhythmias (33.4 ± 18.7% vs. 16.8 ± 10.3%, P = 0.034). On receiver operating characteristic analysis, a right atrial fibrosis burden of 32% had a 92% specificity and 71% sensitivity for predicting the presence of atrial arrhythmias. Neither age nor shunt fraction was associated with bi-atrial fibrosis burden. CONCLUSION: Bi-atrial fibrosis burden is greater in ASD patients than non-congenital heart disease patients with paroxysmal AF. Right atrial fibrosis is associated with the presence of atrial arrhythmias in ASD patients. These findings highlight the importance of right atrial fibrosis to atrial arrhythmogenesis in ASD patients.

TBX3 and TBX5 duplication: A family with an atypical overlapping Holt-Oram/ulnar-mammary syndrome phenotype.

Holt-Oram syndrome (HOS) is a rare, autosomal dominant heart-hand syndrome caused by mutations in the TBX5 gene. A wide spectrum of TBX5 mutations have been reported previously, most resulting in a null allele leading to haploinsufficiency. TBX5 gene duplications have been previously reported in association with typical and atypical HOS phenotypes. Ulnar-Mammary syndrome (UMS) is a distinct rare, autosomal dominant condition caused by mutations in the TBX3 gene. TBX5 and TBX3 are physically linked in cis on human chromosome 12 and contiguous chromosome 12q24 deletions comprising both TBX5 and TBX3 genes have been previously reported but to our knowledge, duplications have never been described. We report on a large German family with at least 17 affected individuals over 6 generations bearing a duplication at 12q24.21 identified on array-CGH comprising both TBX5 and TBX3 genes. Affected patients are presenting with HOS and UMS symptoms, consisting of variable limb anomalies involving the radial and the ulnar rays and cardiac findings such as congenital heart defects, persistent arterial duct or aortic stenosis, and non-classical symptoms, such as supernumerary nipples and cardiomyopathy. Fluorescence in situ hybridisation confirmed a tandem duplication at the 12q24.21 locus. This is the first report of a contiguous TBX3/TBX5 duplication associated with HOS/UMS phenotype.

Imaging the adult with simple shunt lesions: position paper from the EACVI and the ESC WG on ACHD. Endorsed by AEPC (Association for European Paediatric and Congenital Cardiology).

In 2018, the position paper 'Imaging the adult with congenital heart disease: a multimodality imaging approach' was published. The paper highlights, in the first part, the different imaging modalities applied in adult congenital heart disease patients. In the second part, these modalities are discussed more detailed for moderate to complex anatomical defects. Because of the length of the paper, simple lesions were not touched on. However, imaging modalities to use for simple shunt lesions are still poorly known. One is looking for structured recommendations on which they can rely when dealing with an (undiscovered) shunt lesion. This information is lacking for the initial diagnostic process, during repair and at follow-up. Therefore, this paper will focus on atrial septal defect, ventricular septal defect, and persistent arterial duct. Pre-, intra-, and post-procedural imaging techniques will be systematically discussed. This position paper will offer algorithms that might help at a glance. The document is prepared for general cardiologists, trainees, medical students, imagers/technicians to select the most appropriate imaging modality and to detect the requested information for each specific lesion. It might serve as reference to which researchers could refer when setting up a (imaging) study.

Quantitative assessment of homograft function 1 year after insertion into the pulmonary position: impact of in situ homograft geometry on valve competence.

AIMS: To prospectively evaluate homograft function with cardiac magnetic resonance (CMR) imaging 1 year after insertion into the pulmonary position, and to assess the impact of in situ homograft geometry, surgical factors, and 'intrinsic' homograft properties on early valve incompetence. METHODS AND RESULTS: A total of 60 patients (mean age 21 +/- 10 years; 35 females) with congenital heart disease underwent pulmonary valve replacement with homograft insertion and were prospectively enrolled into a study protocol that included serial echocardiography and CMR 1 year after surgery. None of the patients had homograft stenosis but 10 (17%) had significant homograft incompetence (i.e. pulmonary regurgitation fraction >20% on CMR). A higher incidence of 'eccentric' pulmonary forward flow pattern (P < 0.001, Fisher's exact test), more acute 'homograft distortion angle' (P < 0.001), larger relative 'annular' size (P < 0.01), and greater pre-homograft right ventricular outflow tract (RVOT) diameters (P = 0.01) at CMR was seen in those with worse homograft function. In a backward multivariate linear regression model, 'eccentric' pulmonary forward flow pattern (r(part) = 0.36, P < 0.001), 'homograft distortion angle' (r(part) = 0.31, P = 0.001), and pre-homograft RVOT diameter (r(part) = 0.19, P = 0.03) were independently associated with the degree of pulmonary regurgitation (in %) at 1 year. CONCLUSION: Using CMR, in this prospective cohort study, we have shown that significant valve incompetence is present in one-sixth of patients after homograft insertion into the pulmonary position, and that alterations in the in situ homograft geometry were associated with the likelihood of developing valve incompetence. These findings imply that mechanical factors may have an important impact on homograft performance.

Pulmonary Artery Aneurysm Mimicking a Patent Ductus Arteriosus.

Retrograde systolic flow in the main pulmonary artery (PA) is a hallmark echocardiographic sign of patent ductus arteriosus. We describe a case of PA aneurysm mimicking patent ductus arteriosus flow. Using 4-dimensional flow cardiac magnetic resonance imaging, this echocardiographic feature is explained and altered flow dynamics in the aneurysmal PA are visualized. (Level of Difficulty: Intermediate.).

Percutaneous secundum atrial septal defect closure for the treatment of atrial arrhythmia in the adult: A meta-analysis.

BACKGROUND: Atrial arrhythmias are common in patients with atrial septal defects (ASD) but the effects of percutaneous closure on atrial arrhythmia prevalence is unclear. We investigated the effects of ASD device closure and the impact of age at time of closure on prevalent atrial arrythmia. METHODS: Meta-analysis of studies reporting atrial arrhythmia prevalence in adult patients before and after percutaneous closure was performed. Primary outcomes were prevalence of 'all atrial arrhythmia' and atrial fibrillation alone post closure. Sub-group analysis examined the effects of closure according to age in patients; <40 years, ≥40 and ≥ 60 years. 25 studies were included. RESULTS: Meta-analysis of all studies demonstrated no reduction in all atrial arrhythmia or atrial fibrillation prevalence post-closure (OR 0.855, 95% CI 0.672 to 1.087, P = .201 and OR 0.818, 95% CI 0.645 to 1.038, P = .099, respectively). A weak reduction in all atrial arrhythmia and atrial fibrillation was seen in patients ≥40 years (OR 0.77, 95% CI 0.616 to 0.979, P = .032 and OR 0.760, 95% CI 0.6 to 0.964, P = .024, respectively) but not ≥60 years (OR 0.822, 95% CI 0.593 to 1.141, P = .242 and OR 0.83, 95% CI 0.598 to 1.152, P = .266, respectively). No data were available in patients <40 years. This, and other limitations, prevents conclusive assessment of the effect of age on arrhythmia prevalence. CONCLUSIONS: Overall, percutaneous ASD closure is not associated with a reduction in atrial arrhythmia prevalence in this meta-analysis. A weak benefit is seen in patients ≥40 years of age, not present in patients ≥60 years.

Right Ventricle Has Normal Myofilament Function But Shows Perturbations in the Expression of Extracellular Matrix Genes in Patients With Tetralogy of Fallot Undergoing Pulmonary Valve Replacement.

Background Patients with repair of tetralogy of Fallot (rToF) who are approaching adulthood often exhibit pulmonary valve regurgitation, leading to right ventricle (RV) dilatation and dysfunction. The regurgitation can be corrected by pulmonary valve replacement (PVR), but the optimal surgical timing remains under debate, mainly because of the poorly understood nature of RV remodeling in patients with rToF. The goal of this study was to probe for pathologic molecular, cellular, and tissue changes in the myocardium of patients with rToF at the time of PVR. Methods and Results We measured contractile function of permeabilized myocytes, collagen content of tissue samples, and the expression of mRNA and selected proteins in RV tissue samples from patients with rToF undergoing PVR for severe pulmonary valve regurgitation. The data were compared with nondiseased RV tissue from unused donor hearts. Contractile performance and passive stiffness of the myofilaments in permeabilized myocytes were similar in rToF-PVR and RV donor samples, as was collagen content and cross-linking. The patients with rToF undergoing PVR had enhanced mRNA expression of genes associated with connective tissue diseases and tissue remodeling, including the small leucine-rich proteoglycans ASPN (asporin), LUM (lumican), and OGN (osteoglycin), although their protein levels were not significantly increased. Conclusions RV myofilaments from patients with rToF undergoing PVR showed no functional impairment, but the changes in extracellular matrix gene expression may indicate the early stages of remodeling. Our study found no evidence of major damage at the cellular and tissue levels in the RV of patients with rToF who underwent PVR according to current clinical criteria.

Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome?

BACKGROUND: The timing of pulmonary valve replacement (PVR) for free pulmonary incompetence in patients with congenital heart disease remains a dilemma for clinicians. We wanted to assess the determinants of improvement after PVR for pulmonary regurgitation over a wide range of patient ages and to use any identified predictors to compare clinical outcomes between patient groups. METHODS AND RESULTS: Seventy-one patients (mean age 22+/-11 years; range, 8.5 to 64.9; 72% tetralogy of Fallot) underwent PVR for severe pulmonary regurgitation. New York Heart Association class improved after PVR (median of 2 to 1, P<0.0001). MRI and cardiopulmonary exercise testing were performed before and 1 year after intervention. After PVR, there was a significant reduction in right ventricular volumes (end diastolic volume 142+/-43 to 91+/-18, end systolic volume 73+/-33 to 43+/-14 mL/m(2), P<0.0001), whereas left ventricular end diastolic volume increased (66+/-12 to 73+/-13 mL/m(2), P<0.0001). Effective cardiac output significantly increased (right ventricular: 3.0+/-0.8 to 3.3+/-0.8 L/min, P=0.013 and left ventricular: 3.0+/-0.6 to 3.4+/-0.7 L/min, P<0.0001). On cardiopulmonary exercise testing, ventilatory response to carbon dioxide production at anaerobic threshold improved from 35.9+/-5.8 to 34.1+/-6.2 (P=0.008). Normalization of ventilatory response to carbon dioxide production was most likely to occur when PVR was performed at an age younger than 17.5 years (P=0.013). CONCLUSIONS: A relatively aggressive PVR policy (end diastolic volume <150 mL/m(2)) leads to normalization of right ventricular volumes, improvement in biventricular function, and submaximal exercise capacity. Normalization of ventilatory response to carbon dioxide production is most likely to occur when surgery is performed at an age

Rescue surgery following percutaneous pulmonary valve implantation.

OBJECTIVE: Percutaneous pulmonary valve insertion (PPVI) is an evolving alternative to surgical pulmonary valve insertion. The aim of this study is to review the acute complications of PPVI requiring emergency rescue surgery. PATIENTS AND METHODS: Between 09/2000 and 01/2007, 152 patients (pts), received a PPVI. Patient's charts were reviewed in retrospect. RESULTS: Emergency rescue surgery (ERS) took place in 6 pts (3.9%). Indications for ERS were: homograft rupture two pts, dislodgment of the stented valve in a dilated right ventricular outflow tract two pts, occlusion of the right pulmonary artery one pt and compression of the left main coronary artery one pt. Cardiopulmonary bypass was established through repeat sternotomy incision with femorofemoral cannulation in 2/6 pts. The stented valve was removed in five and replaced with a homograft in three and a valved conduit in two pts. One ruptured homograft was repaired leaving the stented valve in situ. All patients survived, one sustained mild neurological impairment. CONCLUSION: Although some of the acute complications of PPVI were probably related to a learning curve (4 among the first 50 pts and 2 among the last 102 patients) the need for ERS is unlikely to be completely abolished. This experience highlights the importance of close collaboration between cardiologists and surgeons in these evolving technologies. Highly skilled and responsive surgical back up is necessary to support the introduction and to sustain institutional programmes such as PPVI.

Delayed recurrent pleuropericardial effusions following atrial septal defect closure with Amplatzer septal occluder device: a case of postpericardiotomy syndrome?

The authors present the case of a 41-year-old woman, who presented with moderate pericardial effusion and bilateral pleural effusion 11 months following a secundum atrial septal defect closure with an Amplatzer septal occluder (ASO) device. The acute presentation responded well to diuretics and a short course of corticosteroid therapy. The patient, however, continued to experience pleuritic chest pain and a recurrence of pericardial effusion a month later. The patient showed significant symptomatic improvement with a 2-month course of down-titrating prednisolone. Six months later, the echocardiogram showed complete remission of pleural and pericardial effusion. The possibility of cardiac erosion following ASO implantation has been excluded. The aetiology of the reoccurrence of steroid-responsive pleuropericarditis remains unknown.