Can Left Atrioventricular Valve Reduction Index (LAVRI) Predict the Surgical Strategy for Repair of Atrioventricular Septal Defect?

Despite improved survival, surgical treatment of atrioventricular septal defect (AVSD) remains challenging. The optimal technique for primary left atrioventricular valve (LAVV) repair and prediction of suitability for biventricular approach in unbalanced AVSD are still controversial. We evaluated the ability of our recently developed echocardiographic left atrioventricular valve reduction index (LAVRI) in predicting LAVV reoperation rate and surgical strategy for unbalanced AVSD. Retrospective echocardiographic analysis was available in 352 of 790 patients with AVSD treated in our institution and included modified atrioventricular valve index (mAVVI), ventricular cavity ratio (VCR), and right ventricle/left ventricle (RV/LV) inflow angle. LAVRI estimates LAVV area after complete cleft closure and was analyzed with regard to surgical strategy in primary LAVV repair and unbalanced AVSD. Of the entire cohort, 284/352 (80.68%) patients underwent biventricular repair and 68/352 (19.31%) patients underwent univentricular palliation. LAVV reoperation was performed in 25/284 (8.80%) patients after surgical correction of AVSD. LAVRI was significantly lower in patients requiring LAVV reoperation (1.92 cm2/m2 [IQR 1.31] vs. 2.89 cm2/m2 [IQR 1.37], p = 0.002) and significantly differed between patients receiving complete and no/partial cleft closure (2.89 cm2/m2 [IQR 1.35] vs. 2.07 cm2/m2 [IQR 1.69]; p = 0.002). Of 82 patients diagnosed with unbalanced AVSD, 14 were suitable for biventricular repair (17.07%). mAVVI, LAVRI, VCR, and RV/LV inflow angle accurately distinguished between balanced and unbalanced AVSD and predicted surgical strategy (all p < 0.001). LAVRI may predict surgical strategy in primary LAVV repair, LAVV reoperation risk, and suitability for biventricular approach in unbalanced AVSD anatomy.

Long-term outcome of perimembranous VSD closure using the Nit-Occlud® Lê VSD coil system.

OBJECTIVE: This study presents data from the admission trial to show the feasibility, safety and effectiveness of the Nit-Occlud® Lê VSD in the treatment of perimembranous ventricular septal defects with an aneurysmal configuration and a diameter up to 8 mm. BACKGROUND: The majority of ventricular septal defects (VSD) are still closed surgically, while a less invasive transcatheter treatment by closure devices is available. Device-based closure is reported to be associated with the risk of complete atrio-ventricular block, especially with double-disc devices in perimembranous defects. METHODS: In six tertiary centers in Germany and Israel, an interventional closure of a periembranous VSD was attempted in 88 patients using the Nit-Occlud® Lê VSD. RESULTS: The interventional VSD closure was performed in 85 patients. Patients had a median age of 8.0 (2-65) years and a median body weight of 26.7 (10-109) kg. A complete closure of the defects was achieved in 85.4% 2 weeks after device implantation, in 88.9% after three months and in 98.6% at the 5-year follow-up. There was no incidence of death during the study nor did any patient suffer of permanent atrio-ventricular block of higher degree. Serious adverse events, by definition, are potentially life-threatening or require surgery to correct, while major serious events require medical or transcatheter intervention to correct. The study results exhibit a serious adverse event rate of 3.5% (3/85 patients) and a major adverse event rate of 5.9% (5/85 patients). CONCLUSION: The Nit-Occlud® Lê VSD coil offers the possibility of an effective and safe approach in patients with aneurysmal perimembranous ventricular septal defects.

NT-proBNP and diastolic left ventricular function in patients with Marfan syndrome.

AIMS: Subclinical diastolic dysfuntion in patients with preclinical heart failure with preserved ejection fraction (HFpEF) has been demonstrated in patients with Marfan syndrome (MFS). We investigated the relationship between diastolic dysfunction and NT-proBNP levels in patients with MFS. METHODS AND RESULTS: NT-proBNP, C-reactive protein (CRP) and diastolic function were assessed in 217 patients with MFS (31 ± 16 y, 110 f. and in 339 patients referred for suspected MFS in whom the diagnosis was ruled out according to the Ghent nosology (30 ± 15 y, 154 f). Assessment of cardiovascular remodeling, diastolic function in echocardiography, and NT-proBNP was analyzed with univariate analysis and multi-parameter analysis of covariance (MANCOVA). NT-proBNP was 70.6 ± 74.8 pg/ml in patients with Marfan syndrome and 58.4 ± 100.3 pg/ml in controls (p = 0.002, Kolmogorov-Smirnov). There were significant intergroup differences regarding end-diastolic left ventricular volume (p < 0.001), and aortic diameter (p < 0.001). The ratio of early diastolic mitral flow velocity (E) to early relaxation velocity in tissue Doppler (e'), E/e' (p < 0.001) was significantly higher in patients with Marfan syndrome than in controls, whereas e' (p < 0.001) and the ratio of E to inflow velocity during atrial contraction (A), E/A (p = 0.012) was significantly lower. Besides age and gender, diagnosis of MFS, diastolic function (e' and E/e'), Z-Score of aortic diameter, and left ventricular size were identified as significant independent parameters with impact on NT-proBNP levels. CONCLUSIONS: MFS patients presenting with normal ejection fraction show disturbed diastolic function and higher NT-proBNP levels, which is partly explained by aortic Z-score. Assessment of diastolic function and NT-proBNP levels may therefore detect early abnormalities and guide surveillance and prevention management of patients with MFS.

4D-flow MRI of double aortic arch in a 14-year-old patient.

Double aortic arch is a rare congenital anomaly. It is usually diagnosed and surgically corrected at an early age due to symptoms as dyspnea and dysphagia caused by an obstruction of trachea and/or esophagus in the vascular ring. We present the case of an asymptomatic 14-year-old patient with complete double aortic arch as demonstrated by CMR. Blood flow in the right and left aortic arch was visualized and quantified by 4D-flow MRI.

Analysis of arrhythmias after correction of partial anomalous pulmonary venous connection.

BACKGROUND: Several methods for surgical repair of partial anomalous pulmonary venous connection have been described. Sinus node dysfunction is known as a cause of morbidity after surgical repair. In this retrospective study, we attempted to determine the incidence of arrhythmias after use of two different techniques for repair of partial anomalous pulmonary venous connection. METHODS: Between 1988 and 2006, 119 patients (61 male, 58 female; aged 5 months to 66 years) with anomalous drainage of the pulmonary vein into the superior vena cava or the right atrium were analyzed. All patients had sinus rhythm before operation. In 64 patients (group 1), rerouting of the pulmonary veins was accomplished through a right atriotomy; and in 54 patients (group 2), the atriotomy incision was extended into the superior vena cava through the cavoatrial junction. RESULTS: There were no perioperative deaths. New-onset nodal rhythm and atrial dysrhythmias developed significantly more often in patients with extended incision through the cavoatrial junction (group 1, 26.5%, versus group 2, 54.5%; p < 0.004). At discharge, the rate of dysrhythmias was 14% in group 1 and 32.7% in group 2 (p < 0.01). The hospital stay was longer in group 2. At 1-year follow-up of 58 patients, the rate of arrhythmias was 6.25% in group 1 versus 18.1% in group 2. CONCLUSIONS: Extended incision through the cavoatrial junction increases atrial dysrhythmias, especially early postoperatively, but the incidence of such sinus node dysfunction decreased with time.

Huge pulmonary arteriovenous fistula: diagnosis and treatment and an unusual complication of embolization.

We report the case of a 42-year-old woman with Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia) and a huge pulmonary arteriovenous fistula that involved the entire right middle lobe. She had a history of dyspnea and intermittent atrial flutter for 6 months. A chest radiograph showed a discrete shadowing of the middle part of the right lung. Arteriovenous fistula was suspected based on the echocardiographic finding of immediate bubble detection in the left atrium on introducing echocardiographic contrast medium into the venous system. Atrial shunt was excluded. Angiography revealed a huge pulmonary arteriovenous fistula in the entire middle lobe of the right lung. A trial of interventional embolization was performed, but the size of the fistula made it impossible to achieve complete closure of all segmental fistulas. Furthermore, the patient complained of unusual, severe chest pain after implantation of the first coil, so that the coil had to be removed. Therefore the patient underwent surgical resection of the entire right middle lobe and the upper part of the right lower lobe. Anatomy was clearly delineated and all connecting vessels were suture-closed, but both lungs showed diffuse microscopic superficial pulmonary arteriovenous fistulas, which were too small to be detected by angiography and which were also partly closed. The postoperative course was uneventful: the patient recovered completely, she no longer had shortness of breath, and blood gas analysis showed normal P(aO)(2). It is not clear whether the patient's improved physical performance will last, because the development of diffuse microscopic arteriovenous fistulas bilaterally in the lungs is not predictable. Therefore close follow-up is necessary and in the case of recurrence (ie, enlargement of the existing small fistulas), early interventional embolization should be performed.