Cavopulmonary window: case report of an unusual variant of a sinus venosus defect.

INTRODUCTION: Isolated partial anomalous pulmonary venous return (PAPVR) with intact atrial septum is a rare finding. A cavopulmonary window is a side-to-side veno-venous communication of the right upper pulmonary vein with the superior vena cava which in its course retains connection to the left atrium. CASE PRESENTATION: We present a case of this unusual variant of a sinus venosus defect far from the atrial roof. Haemodynamic significance of the shunt was confirmed by enlargement of right heart cavities, elevation of pulmonary artery pressure, and significant left-to-right shunting using multimodality cardiac imaging (transoesophageal echocardiography, cardiac magnetic resonance imaging, and right heart catheterization). The defect has been successfully repaired using minimally invasive axillary thoracotomy. DISCUSSION: Partial anomalous pulmonary venous return prevalence is low and about 0.4-0.7% in autopsy series of patients with congenital heart disease. This patient's unusual variant of a sinus venosus defect with a window between a pulmonary vein and the superior vena cava far from the atrial roof shows that a sinus venosus defect is not a true atrial septum defect. Left-to-right shunting generally increases with age. Usually, surgical treatment is considered in cases of significant left-to-right shunt (Q p:Q s > 1.5-2.0) and right heart dilatation.

Conventional versus 3-D Echocardiography to Predict Arrhythmia Recurrence After Atrial Fibrillation Ablation.

BACKGROUND: Arrhythmia recurrence after atrial fibrillation (AF) ablation remains high and requires repeat interventions in a substantial number of patients. We assessed the value of conventional and 3-D echocardiography to predict AF recurrence. METHODS AND RESULTS: Consecutive patients undergoing AF ablation by means of pulmonary vein isolation were included in a prospective registry. Echocardiograms were obtained prior to the ablation procedure, and analyzed offline in a standardized manner, including 3-D left atrial (LA) volumetry and determination of LA function and sphericity. The primary endpoint, AF recurrence (>30 seconds) between 3 to 12 months after AF ablation, was independently adjudicated. We included 276 patients (73% male, mean age 59.9 ± 9.9 years). Paroxysmal and persistent AF were present in 178 (64%) and 98 (36%) patients, respectively. Mean left ventricular ejection fraction and indexed LA volume in 3-D (LAVI) were 52 ± 12% and 42 ± 13 mL/m2 , respectively. AF recurrence was observed in 110 (40%) patients after a single procedure. Median (interquartile range) time to AF recurrence was 123 (92; 236) days. In multivariable Cox regression models, the only predictors for AF recurrence were the minimal, maximal, and indexed 3-D LA volumes, P = 0.024, P = 0.016, and P = 0.014, respectively. Quartile specific analysis of 3-D LAVI showed an HR of 1.885 (95%CI 1.066-3.334; P for trend = 0.015) for the highest compared to the lowest quartile. CONCLUSION: Our results show the important role of LA volume for the long-term freedom from arrhythmia after AF ablation. These data also highlight the potential of 3-D echocardiography in this context and may facilitate patient selection for AF ablation.

Determinants of Left Atrial Volume in Patients with Atrial Fibrillation.

INTRODUCTION: Left atrial (LA) enlargement is an important risk factor for incident stroke and a key determinant for the success of rhythm control strategies in patients with atrial fibrillation (AF). However, factors associated with LA volume in AF patients remain poorly understood. METHODS: Patients with paroxysmal or persistent AF were enrolled in this study. Real time 3-D echocardiography was performed in all participants and analyzed offline in a standardized manner. We performed stepwise backward linear regression analyses using a broad set of clinical parameters to determine independent correlates for 3-D LA volume. RESULTS: We included 210 patients (70.9% male, mean age 61±11years). Paroxysmal and persistent AF were present in 95 (45%) and 115 (55%) patients, respectively. Overall, 115 (55%) had hypertension, 11 (5%) had diabetes, and 18 (9%) had ischemic heart disease. Mean indexed LA volume was 36±12ml/m2. In multivariable models, significant associations were found for female sex (ß coefficient -10.51 (95% confidence interval (CI) -17.85;-3.16), p = 0.0053), undergoing cardioversion (ß 11.95 (CI 5.15; 18.74), p = 0.0006), diabetes (ß 14.23 (CI 2.36; 26.10), p = 0.019), body surface area (BSA) (ß 34.21 (CI 19.30; 49.12), p<0.0001), glomerular filtration rate (ß -0.21 (CI -0.36; -0.06), p = 0.0064) and plasma levels of NT-pro brain natriuretic peptide (NT-proBNP) (ß 6.79 (CI 4.05; 9.52), p<0.0001), but not age (p = 0.59) or hypertension (p = 0.42). Our final model explained 52% of the LA volume variability. CONCLUSIONS: In patients with AF, the most important correlates with LA volume are sex, BSA, diabetes, renal function and NT-proBNP, but not age or hypertension. These results may help to refine rhythm control strategies in AF patients.

Fluoroscopy-Free Pulmonary Vein Isolation in Patients with Atrial Fibrillation and a Patent Foramen Ovale Using Solely an Electroanatomic Mapping System.

INTRODUCTION: The advent of electroanatomical mapping (EAM) systems for pulmonary vein isolation (PVI) has dramatically decreased radiation exposure. However, the need for some fluoroscopy remains for obtaining left atrial (LA) access. The aim was to test the feasibility of fluoroscopy-free PVI in patients with atrial fibrillation (AF) and a patent foramen ovale (PFO) guided solely by an EAM system. METHODS: Consecutive patients with AF undergoing PVI and documented PFO were studied. An EAM-guided approach without fluoroscopy and ultrasound was used. After completing the map of the right atrium, the superior vena cava and the coronary sinus, a catheter pull-down to the PFO was performed allowing LA access. The map of the LA and subsequent PVI was also performed without fluoroscopy. RESULTS: 30 patients [age 61±12 years, 73% male, ejection fraction 0.64 (0.53-0.65), LA size in parasternal long axis 38±7 mm] undergoing PVI were included. The time required for right atrial mapping including transseptal crossing was 9±4 minutes. Total procedure time was 127±37 minutes. Fluoroscopy-free PVI was feasible in 26/30 (87%) patients. In four patients, fluoroscopy was needed to access (n = 3) or to re-access (n = 1) the LA. In these four patients, total fluoroscopy time was 5±3 min and the DAP was 14.9±13.4 Gy*cm2. Single-procedure success rate was 80% (24/30) after a median follow-up of 12 months. CONCLUSION: In patients with a documented PFO, completely fluoroscopy-free PVI is feasible in the vast majority of cases.

Transapical Transcatheter Aortic Valve Implantation Using the JenaValve: A One-Year Follow-up.

OBJECTIVE: Since the first transcatheter aortic valve implantation (TAVI) in 2002, TAVI technique has gained an increasing popularity especially in high-risk patients. In this study, we present the first echocardiographic midterm outcome with the second-generation transapical JenaValve TAVI system (JenaValve Technology GmbH, Munich, Germany) in patients with aortic stenosis (AS). METHODS: Between November 2011 and November 2012, a total of 28 patients received transapical TAVI using the JenaValve. Primary endpoint was a combined efficacy endpoint after 1 year, which included all-cause mortality after more than 30 days, failure of current therapy for AS requiring hospitalization for symptoms of valve-related cardiac decompensation or prosthetic heart valve dysfunction. Moreover, we analyzed secondary endpoints after 3 and 12 months including cardiovascular mortality; major stroke; and life-threatening, disabling, or major bleeding. Mean echocardiographic follow-up was 471.35 ± 102.72 days. RESULTS: Mean age was 80.43 ± 6.03 years and EuroSCORE II was 8.80 ± 7.21%. Successful implantation was accomplished in 100% (n = 28). Median transvalvular aortic mean pressure gradient was 44.5 mm Hg (interquartile range [IQR]: 34.5; 55.5) preoperatively, 12 mm Hg (IQR: 9; 16) postoperatively, and 11 mm Hg (IQR: 8; 16) after 1 year. After 12 months, no paravalvular leakage was seen in 52.38% of the patients and grade 1 paravalvular leakage was seen in 47.62% of the patients. There was no grade 2 or 3 leakage detected. Stroke, valve thrombosis or dislocation, myocardial infarction, or bleeding was also not observed. However, criteria for the combined efficacy endpoint after 1 year were met in five patients (17.86%). Thirty-day mortality was 14.29% (n = 4) and all-cause mortality after 1 year was 21.43% (n = 6). CONCLUSION: The JenaValve transapical TAVI system is a safe and feasible procedure with low peri- and postoperative complications and convincing midterm performance of the prosthesis.

Assessment of left atrial functional parameters using a novel dedicated analysis tool for real-time three-dimensional echocardiography: validation in comparison to magnetic resonance imaging.

Real-time three-dimensional echocardiography (RT3DE) is superior to two-dimensional echocardiography in assessing left atrial (LA) parameters, but to date algorithms developed for the left ventricle were applied due to a lack of dedicated LA software. In addition, no data are available on RT3DE assessment of active atrial contraction. The aim of this study was to validate a novel RT3DE analysis tool specifically dedicated to evaluate the LA. Cardiac magnetic resonance imaging (MRI) served as standard of reference. Fifty-five patients scheduled for pulmonary vein isolation underwent cardiac MRI and RT3DE. On ultrasound image datasets, a dynamic polyhedron model of the LA was generated from which LA maximum and minimum volumes (LAmax and LAmin), passive atrial emptying fraction (LAEF), and active atrial ejection fraction (LAEFtrue) were derived and compared to values obtained from cardiac MRI. High intraclass correlations between RT3DE and MRI were found for LAmax (r = 0.94, p < 0.001), LAmin (r = 0.95, p < 0.001), LAEF (r = 0.92, p < 0.001), and LAEFtrue (r = 0.87, p < 0.001). Similarly, Bland-Altman analysis revealed narrow limits of agreement for LAmax (-28.6 to 14.1 ml), LAmin (-26.8 to 12.4 ml), LAEF (-11.2 to 14.9 %), and LAEFtrue (-10.6 to 6.8 %). LAmax, LAmin and LAEFtrue were measured significantly (p < 0.05) lower by RT3DE (111 ± 38 ml vs. 118 ± 39 ml, 73 ± 38 ml vs. 80 ± 41 ml, and 23 ± 14 % vs. 27 ± 14 %, respectively). Interobserver and intraobserver RT3DE measurements correlated closely. RT3DE using a novel dedicated software tool is valid, accurate and reproducible for assessing LA dimensional and functional parameters. This study corroborates previous reports and extends its validity to the assessment of active LA contraction.

Dynamic assessment of right ventricular volumes and function by real-time three-dimensional echocardiography: a comparison study with magnetic resonance imaging in 100 adult patients.

BACKGROUND: The aim of this study was to validate a novel real-time three-dimensional echocardiographic (RT3DE) analysis tool for the determination of right ventricular volumes and function in unselected adult patients. METHODS: A total of 100 consecutive adult patients with normal or pathologic right ventricles were enrolled in the study. A dynamic polyhedron model of the right ventricle was generated using dedicated RT3DE software. Volumes and ejection fractions were determined and compared with results obtained on magnetic resonance imaging (MRI) in 88 patients with adequate acquisitions. RESULTS: End-diastolic, end-systolic, and stroke volumes were slightly lower on RT3DE imaging than on MRI (124.0 +/- 34.4 vs 134.2 +/- 39.2 mL, P < .001; 65.2 +/- 23.5 vs 69.7 +/- 25.5 mL, P = .02; and 58.8 +/- 18.4 vs 64.5 +/- 24.1 mL, P < .01, respectively), while no significant difference was observed for ejection fraction (47.8 +/- 8.5% vs 48.2 +/- 10.8%, P = .57). Correlation coefficients on Bland-Altman analysis were r = 0.84 (mean difference, 10.2 mL; 95% confidence interval [CI], -31.3 to 51.7 mL) for end-diastolic volume, r = 0.83 (mean difference, 4.5 mL; 95% CI, -23.8 to 32.9 mL) for end-systolic volume, r = 0.77 (mean difference, 5.7 mL; 95% CI, -24.6 to 36.0 mL) for stroke volume, and r = 0.72 (mean difference, 0.4%; 95% CI, -14.2% to 15.1%) for ejection fraction. CONCLUSION: Right ventricular volumes and ejection fractions as assessed using RT3DE imaging compare well with MRI measurements. RT3DE imaging may become a time-saving and cost-saving alternative to MRI for the quantitative assessment of right ventricular size and function.

Diffusion-weighted imaging in stroke attributable to patent foramen ovale: significance of concomitant atrial septum aneurysm.

BACKGROUND AND PURPOSE: Patent foramen ovale (PFO) is an established cause of stroke in young patients without other determined etiologies (ie, cryptogenic stroke). The additional presence of atrial septum aneurysm (ASA) possibly increases stroke risk, but it remains undetermined which factors best predict thromboembolism in patients with PFO. Diffusion-weighted imaging (DWI) may help to distinguish the characteristics of cerebral embolism associated with different features of the interatrial septum in PFO stroke. METHODS: In a stroke databank-based cohort study, DWI and transthoracic/transesophageal echocardiography findings were assessed in 48 consecutive patients with cryptogenic ischemic stroke associated with PFO. The number, size, and distribution of acute ischemic lesions on DWI were correlated with PFO size, degree of interatrial right-to-left shunt (RLS), and the presence of ASA. RESULTS: Patients with PFO plus ASA combined more often had multiple acute DWI lesions (16 of 30, 53%) than those with PFO alone (3 of 18, 17%; P=0.01). This association remained significant after correction for PFO size, degree of RLS, and vascular risk factors in a logistic-regression analysis (P=0.04). No significant associations between DWI lesion characteristics and PFO size or degree of RLS were found. CONCLUSIONS: The presence of concomitant ASA is independently associated with multiple cerebral ischemic lesions in PFO stroke, which may indicate an increased embolic risk.

Inferior sinus venosus defect associated with incomplete cor triatriatum dexter and patent foramen ovale.

Sinus venosus atrial septal defect (SVD) is a rare cardiac abnormality in adults. Particularly, the inferior type is difficult to depict by transthoracic echocardiography because of its infero-posterior location to the fossa ovalis. We describe the case of a 33-year-old woman whose chest X-ray taken during bronchopneumonia revealed a cardiomegaly. Further echocardiographic investigations showed an underlying inferior SVD, an incomplete cor triatriatum dexter and a large patent foramen ovale (PFO). The diagnosis was confirmed by cardiac magnetic resonance imaging (CMR) and during surgical repair.