Intermediate Atrioventricular Septal Defect Research Articles

Efficacy of atrioventricular valve regurgitation in the first trimester for the diagnosis of atrioventricular septal defect.

To investigate the efficacy of atrioventricular valve regurgitation (AVVR) for predicting atrioventricular septal defect (AVSD) in the first trimester. We performed a prospective observational study, screening for complicated congenital heart diseases and AVVR in fetuses at 11 to 13+6 weeks of gestation by advanced dynamic flow in four-chamber view and three-vessel-trachea view. 43 549 fetuses at 11 to 13+6 weeks of gestation were screened by echocardiography, of which 37 cases were diagnosed with AVSD, including complete AVSD (31 cases), intermediate AVSD (3 cases) and partial AVSD (1 cases), undiagnosed intermediate AVSD (2 cases), and misdiagnosed case (2 cases). AVVR was observed in 34 cases (34/37) in the first trimester, 59. 46% (22/37) nuchal translucency greater than 95th percentile, 29. 73% (11/37) absence of nasal bone, 32. 43% (12/37) ductus venosus A wave inversion, and 40. 54% (15/37) had tricuspid regurgitation. The sensitivity of common AVVR in predicting AVSD is better than other ultrasonic indexes. AVVR can be used as an ultrasonic indicator to predict AVSD in the first trimester, which is beneficial to detect AVSD.

Atrioventricular septal defect: An extended approach to prenatal sonographic imaging of the atrioventricular valves

AbstractAn atrioventricular septal defect (AVSD) is diagnosed with prenatal sonography using the 4‐chamber view (4CV) of the fetal heart. Prenatal 2D sonographic imaging of normal and AVSD short axis (SAX) atrioventricular (AV) valve views have not been well described. The aim is to describe the 2D sonographic 4‐chamber and SAX view of the AV valves when an AVSD is present compared to normal AV valves. The 4CV AVSD heart demonstrates no offset of AV valves due to the abnormal valve structure. Complete and intermediate AVSD has a septum primum defect and inlet ventricular septal defect (VSD). The partial AVSDs have either a septum primum defect or just an inlet VSD with an intact primum septum. The SAX view of AVSD demonstrates the common AV junction and the bridging leaflets. In ventricular diastole valves have a “figure 8” and “dumbbell” shape in partial and complete AVSD, respectively. These appearances are not seen in the normal heart in SAX as both tricuspid and mitral valve are separate valves. The SAX view can be obtained by locating a sagittal aortic arch view and then scanning slightly towards the left side of the fetus, near the base of the heart and the AV valves. An in‐depth interrogation of the abnormal fetal heart is obtained without specialised 4D capable ultrasound machines providing information to assist in counselling during the pregnancy and for postnatal surgical planning. Future research to assess the feasibility of incorporating the SAX view into routine practice and by general sonographers is suggested.

A Cardiac Arrest Case due to Left Coronary Artery Compression in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension

<h2>Abstract</h2> We report the case of an adult who had a cardiac arrest in the setting of pulmonary hypertension and a previously repaired intermediate atrioventricular septal defect, with left main coronary trunk stenosis due to dilatation of the main pulmonary artery. In patients with pulmonary hypertension exhibiting anginal symptoms, it is advisable to perform chest contrast computed tomography to confirm the pulmonary artery diameter and the presence of coronary artery compression. In addition, our case highlights the importance of early collaboration among specialists during the transition from adolescence to adulthood.

Three-dimensional transesophageal echocardiography in diagnosis of intermediate atrioventricular septal defect in the adult: case report and literature review

BackgroundIntermediate type atrioventricular septal defect is less frequent than complete or partial atrioventricular septal defect, and is rarely encountered in the elderly and the utility of three dimensional transesophageal echocardiography in the diagnosis has not been reported to date.Case presentationIn this case report, we described a rare case of an intermediate atrioventricular septal defect in an adult patient and we showed the valuable utility of real time 3D transesophageal echocardiography in the diagnosis and future surgical planning. The patient was referred to a tertiary center for an elective surgical repair. Finally, we provided a detailed review of the literature concerning the intermediate type of atrioventricular septal defect.ConclusionAlthough 2D transthoracic and transesophageal echocardiography enables diagnosis of the intermediate type atrioventricular septal defect, precise assessment of anatomy of atrioventricular septal defects and common atrioventricular valve was enabled only by real time 3D echocardiography.

Clinical, echocardiographic and prognostic features of atrioventricular septal defect in adults at Ibn Rochd University hospital-Casablanca

Atrioventricular septal defect (AVSD)is a complex heart malformation affecting the lower part of atrial septum, the highest aprt of ventricular septum, mitral and tricuspid valves. It represents 3 to 7.3 % of congenital heart diseases. It is responsible for an important part of early death. The objective of this study is to precise clinical, echocardiographic and prognostic profile of AVSD in adults. Descriptive, retrospective, transversal and monocentric study conducted in a period of 10 years between December 2008 and December 2018. Were included all patients of more than 18 years presenting with an AVSD confirmed by echocardiography and followed in the congenital heart disease unit of our department. Three hundred and thirteen patients were collected. Among them, 31 were adults (9,9 %).16 patients were aged more than 30. The oldest patient had 56 years old. Sex ratio was H/F = 0,48. One patient was trisomic 21. The mean SpO2 was 91,87 ± 8,25%. Partial AVSD was mostly represented (17 cases), then complete AVSD (12 cases among which 8 type C et 4 type A of Rastelli), then intermediate AVSD (2 cases). Pulmonary arterial hypertension was present in 16 patients (51,6%) of cases, one patient was diagnosed Eisenmeger syndrome. 22,6% underwent curative surgery. Amogn +the 31 patients, 4 deaths, mortality was statistically lower than in younger patients ( P < 0,001). AVSD, particularly in its complete form, is a dangerous pathology. The diagnosis at adult age seems to have a better prognosis. Treatment is essentially surgical, the nit is important to assess the diagnosis earlier.

Long-term results after surgical repair of atrioventricular septal defect.

We analysed our 29-year experience of surgical repair of atrioventricular septal defect (AVSD) to define risk factors for mortality and reoperation. Between 1988 and 2017, 508 patients received AVSD repair in our institution; 359 patients underwent surgery for complete AVSD, 76 for intermediate AVSD and 73 for partial AVSD. The median age of the patients was 6.1 months (interquartile range 10.3 months), and the median weight was 5.6 kg (interquartile range 3.2 kg). The standard AVSD repair was performed using 2-patch technique (n = 347) and complete cleft closure (n = 496). The results were divided into 2 surgical eras (early era 1986-2004 and late era 2004-2017). Risk factors were analysed to determine the impact of patient age, weight, the presence of trisomy 21 and complex AVSD on mortality and reoperation rate. In-hospital mortality decreased from 10.2% (n = 26) in early surgical era to 1.6% (n = 4) in late surgical era (P < 0.001). Seventy-seven patients required reoperation. Freedom from reoperation was 84.4% after 25 years. The main indication for reoperation was left atrioventricular valve regurgitation (13.8%). The multivariable Cox regression analysis revealed reoperation of the left AV valve, early surgical era, patient age <3.0 months and complex AVSD to be independent risk factors for mortality. Age <3.0 months, complex AVSD and moderate/severe left AV valve regurgitation at discharge predicted reoperation. AVSD repair can be performed with low mortality and reoperation rate. Age <3 months, complex AVSD and moderate/severe regurgitation of the left AV valve at discharge were predictors for reoperation. Reoperation of the left AV valve was the strongest risk factor for mortality.

Cardioplegic or Fibrillatory Arrest for Correction of Partial and Intermediate Atrioventricular Septal Defects?

Cardioplegic or Fibrillatory Arrest for Correction of Partial and Intermediate Atrioventricular Septal Defects?

Disturbed Intracardiac Flow Organization After Atrioventricular Septal Defect Correction as Assessed With 4D Flow Magnetic Resonance Imaging and Quantitative Particle Tracing.

Four-dimensional (3 spatial directions and time) velocity-encoded flow magnetic resonance imaging with quantitative particle tracing analysis allows assessment of left ventricular (LV) blood flow organization. Corrected atrioventricular septal defect (AVSD) patients have an abnormal left atrioventricular valve shape. We aimed to analyze flow organization in corrected AVSD patients and healthy controls. A total of 32 patients (age, 25 ± 14 years), 21 after partial AVSD correction and 11 after complete/intermediate AVSD correction, and 30 healthy volunteers (26 ± 12 years) underwent whole-heart four-dimensional velocity-encoded flow magnetic resonance imaging. Particle tracing in the 16-segment LV cavity model was used to quantitatively evaluate blood flow organization discriminating multiple components. Patients showed a smaller percentage of direct flow compared with controls (30% ± 9% vs 44% ± 11%; P < 0.001). In patients, more inflow was observed in the basal inferior segment (22% ± 11% vs controls, 17% ± 5%; P = 0.005), with less direct but more retained inflow (ie, part of inflow that is not ejected from LV in subsequent systole). In patients, more inflow reached the midventricular level (68% ± 13% vs controls, 58% ± 9%; P < 0.001), most notably as retained inflow in the lateral segments. Subsequently, in patients, more (mostly retained) inflow reached the apex (23% ± 13% vs 14% ± 7%; P < 0.001), which correlated with early peak filling velocity (r = 0.637, P < 0.001). Patients with a corrected complete or intermediate AVSD presented with less direct flow (24% ± 8% vs 33% ± 8%; P = 0.003) and more apical inflow (30% ± 14% vs 18% ± 12%; P = 0.014) compared with a corrected partial AVSD. Multicomponent particle tracing combined with 16-segment analysis quantitatively demonstrated altered LV flow organization after AVSD correction, with less direct and more retained inflow in apical and lateral LV cavity segments, which may contribute to decreased cardiac pumping efficiency.

Utility of three-dimensional transthoracic echocardiography in diagnosis of intermediate atrioventricular septal defect: report of an adult case.

We present the case of a 54-year-old male with an intermediate atrioventricular septal defect (AVSD). He was referred to our institute for treatment of shortness of breath. Two-dimensional transthoracic echocardiography demonstrated an ostium primum defect of the atrial septum, a common atrioventricular valve with mild regurgitations, and an interventricular communication, which are the features of complete AVSD. However, three-dimensional transthoracic echocardiography (3D-TTE) could clearly visualize the tricuspid pouch (i.e., persistence of the tissue in the endocardial cushion), a feature of partial AVSD. 3D-TTE appears to be a useful modality for diagnosis of intermediate AVSD.

Real-Time 3-Dimensional Transesophageal Echocardiography of the Atrioventricular Septal Defect

Being able to view the morphological details of the heart malformation in any patient with the diagnosis of atrioventricular septal defect (AVSD) is invaluable in surgical planning, especially in the current era of modified 1-patch versus 2-patch techniques. Real-time 3-dimensional transesophageal echocardiography (RT 3D TEE) is a recently developed technique that allows real-time high-quality images akin to viewing the heart specimen. We present the principal pathomorphological features of AVSD as visualized by RT 3D TEE. The hallmark of AVSD is a common atrioventricular junction guarded by a common atrioventricular valve.1 In the normal heart, when seen from atrial perspective, the aortic valve is wedged between the mitral and tricuspid valve. In AVSD, because of the common AV junction, the aortic valve is in an “unwedged” position (Figure 1). The valve consists of 5 leaflets: the superior and inferior bridging leaflets, each of which overrides the ventricular septum, a left mural leaflet, a right anterior, and a right inferior leaflet (Figure 2). Whether the bridging leaflets are joined together distinguishes the complete form from the partial form. In partial AVSD (also termed “ostium primum ASD”), a tongue of leaflet tissue connects the bridging leaflets and the undersides of these leaflets …

More Than 30 Years' Experience With Surgical Correction of Atrioventricular Septal Defects

Outcome of surgical correction of atrioventricular septal defects (AVSD) still varies despite enhanced results. We reviewed our 30-year experience with AVSD repair and identified risk factors for mortality and reoperation. Between 1975 and 2006, 312 patients underwent surgery for complete AVSD (n = 209; 67.0%), partial AVSD (n = 76; 24.4%), or intermediate AVSD (n = 27; 8.6%). Mean age was 2.4 ± 3.9 years; 142 patients (45.5%) were younger than 6 months. Follow-up was 99.0% complete. There were 26 in-hospital deaths (8.3%) and 6 late deaths (2.1% of 283). Estimated overall survival for the total study population was 91.3%, 90.6%, and 88.6% at 1, 5, and 15 years, respectively. In the multivariable logistic regression analysis, surgical era 1975 to 1995 (p < 0.001) and younger age (p = 0.004) were found to be independent risk factors for early mortality, whereas preoperative AV valve insufficiency showed a tendency toward statistical significance (p = 0.052). Of the hospital survivors, 43 patients required a late reoperation. Estimated freedom from late reoperation was 96.4%, 89.3%, and 81.8% at 1, 5, and 15 years, respectively. Multivariable Cox regression analysis showed associated cardiovascular anomalies (p < 0.001), left AV valve dysplasia (p < 0.001), and absence of cleft closure (p = 0.003) to be independent risk factors for late reoperation. AVSD repair can be accomplished with good long-term results. Early surgical era, associated cardiovascular anomalies, left AV valve dysplasia, and absence of cleft closure negatively influence survival and risk of reoperation.

Surgical management of atrio ventricular septal defects with normal caryotype.

Atrio ventricular septal defects (AVSD) with normal caryotype represent in average 25% of AVSD. They constitute a more complex group of patients characterized by frequent left sided heart obstructive lesions, raising the problem of the appropriate indications between biventricular and univentricular procedures. Sixty-nine consecutive patients, who had AVSD with normal caryotype underwent surgery. According to the anatomical complexity there were 22 intermediate AVSD, 36 complete AVSD and 11 complex AVSD. Associated lesions were present in 68% of the patients including left sided heart anomalies in 57%. According to the size of the left ventricle (LV) evaluated on the LV/RV end diastolic diameter ratio calculated at 2D echocardiogram: right ventricular (RV) dominance was found in 29%; with border line LV in 13 patients and truly hypoplastic LV in 7 patients. Biventricular repair was always favored in case of border line LV and precluded when the LV/RV ratio was less than 0.33. There were 57 biventricular repairs with 10 years actuarial survival of 70 +/- 8% and respectively, 100% in the complex AVSD, 86% in the intermediate AVSD and 51% in the complete AVSD (P = 0.006). The risk factors for biventricular repair were the association to a subaortic stenosis (P = 0.01) and the severity of the mitral valve lesions (P = 0.03) that led to 38% reoperation. There were 12 univentricular palliation with 10 years survival of 66 +/- 14%. The risk factor for univentricular palliation was the association to a severe pre-operative mitral regurgitation (P = 0.005). Biventricular repair should be precluded in patients presenting with subaortic stenosis. Severe mitral valve anomalies lead to elevated mortality and morbidity with frequent reoperations. Univentricular repair might have larger indications and cardiac transplantation might be considered in patients with truly hypoplastic LV presenting with severe pre-operative AV valve regurgitation.