The impact of surgical repair on left ventricular outflow tract in atrioventricular septal defect with common atrioventricular valve orifice.

Objective: Although a narrow left ventricular outflow tract in atrioventricular septal defect is related to its intrinsic morphology, the contribution from the repair technique remains to be quantified. Methods: A total of 108 patients with an atrioventricular septal defect with a common atrioventricular valve orifice were divided into 2 groups: 2-patch (N = 67) and modified 1-patch (N = 41) repair. The left ventricular outflow tract morphometric was analyzed by quantifying the degree of disproportion between subaortic and aortic annular dimensions (disproportionate morphometrics ratio was defined as ≤ 0.9). Z-scores (median, interquartile range) were further analyzed in a subset of 80 patients with immediate preoperative and postoperative echocardiography. A total of 44 subjects with ventricular septal defects served as controls. Results: Before repair, 13 patients (12%) with an atrioventricular septal defect had disproportionate morphometrics (vs 6 [14%] ventricular septal defect P = .79), but the subaortic Z-score (-0.53, -1.07 to 0.06) was lower than the ventricular septal defect (0.07, -0.57 to 1.17; P < .001). After repair, both 2-patch (8 [12%] preoperatively vs 25 [37%] postoperatively; P = .001) and modified 1-patch (5 [12%] vs 21 [51%], P < .001) procedures showed a greater degree of disproportionate morphometrics. Both 2-patch (postoperatively -0.73, -1.56 to 0.08 vs preoperatively -0.43, -0.98 to 0.28; P = .011) and modified 1-patch (-1.42, -2.63 to -0.78 vs -0.70, -1.18 to -0.25; P = .001) procedures also demonstrated lower subaortic Z-scores postrepair. The postrepair subaortic Z-scores were lower in the modified 1-patch group (-1.42 [-2.63 to -0.78]) compared with the 2-patch group (-0.73 [-1.56 to 0.08]; P = .004). Low postrepair subaortic Z-scores (<-2) were observed in 12 patients (41%) in the modified 1-patch group and 6 patients (12%) in the 2-patch group (P = .004). Conclusions: Surgical correction resulted in greater disproportionate morphometrics seen immediately postrepair. The impact on the left ventricular outflow tract was observed in all repair techniques, with a greater burden seen after modified 1-patch repair. Video Abstract: This morphometric study in AVSD with common atrio-ventricular valve orifice confirmed further derangements of LV outflow tract morphometrics immediately after surgical repair.

The role of abnormal subaortic morphometry as a substrate for left ventricular outflow tract obstruction following atrioventricular septal defect repair.

OBJECTIVES: Although left ventricular outflow tract (LVOT) obstruction is a recognized risk after atrioventricular (AV) septal defect (AVSD) repair, quantitative assessments to define the substrate of the obstruction are lacking. METHODS: Morphometric analyses were based on measurements from early 2-dimensional echocardiographic scans (within 3 months postoperatively) for 117 patients (82 CAVVO = common AV valve; 35 SAVVO = separate AV valve orifices), which were compared to 50 age/weight matched controls (atrial septal defect/ventricular septal defect). Late echocardiographic analyses were performed in 57 patients with AVSD (follow-up range, 1.2-10.7 years). RESULTS: Adequate z scores (above -2.5) were observed in 109 (93%) patients with AVSD at the aortic annulus and in 89 (76%) with AVSD in the subaortic area. Compared to the control group, patients with AVSD had lower median z scores at the aortic annulus (-0.64 vs 0.60; P < 0.001) and the subaortic areas (-1.48 vs 0.59; P < 0.001), disproportionate subaortic/aortic annulus ratio <1.00 (67% vs 22%; P < 0.001), narrower annuloaortic-septal angle (94.0 vs 104.0; P < 0.001) and annuloaortic left AV valve angle (78.0 vs 90.0; P < 0.001). Compared to patients with CAVVO, those with SAVVO had narrower annuloaortic-septal angles (P = 0.022) that persisted at late analysis, with lower subaortic/aortic annular ratios (P = 0.039). In patients with CAVVO, lower early postoperative subaortic z scores were found following modified single-patch repairs (median -2.12 vs -1.02 in two-patch repairs; P = 0.004). A total of 6/117 (5%) patients (4 CAVVO, 5% and 2 SAVVO, 6%) required reoperations for LVOT obstruction (mean 6.9 years postoperatively), with no difference in morphology or types of operations. CONCLUSIONS: Despite having adequate z scores, patients with AVSD demonstrated abnormal LVOT morphometrics early postoperatively. Besides intrinsic morphology, repair techniques may have an impact on postoperative LVOT morphometrics and requires further evaluation.

Circumflex retroesophageal aorta mimicking aortic interruption: a rare cause of aortic obstruction in a neonate.

Critical obstruction of the aorta presenting in the neonate typically occurs with aortic interruption or coarctation, following ductus arteriosus closure. Circumflex retroesophageal aortic arch is a rare aortic arch anomaly in which a right aortic arch continues as a retroesophageal component and descends on the left of the spine. Obstruction within the arch can occur, usually identified incidentally in older children. We report a neonate with circumflex aorta with ventricular septal defect in which there was hypoplasia of the retroesophageal segment. The patient presented with cardiovascular collapse following duct closure mimicking aortic interruption. The case is presented, together with the surgical repair.

Surgical management of double-chambered right ventricle in adults.

Double-chambered right ventricle is a congenital anomaly in which the right ventricle is divided into 2 portions by anomalous muscle bundles. These cases often present in children, but rarely in adults. We discuss 2 cases of double-chambered right ventricle, in patients aged 42 and 35 years. When cases remain asymptomatic until adulthood, they can present with unusual symptoms that lead to incorrect diagnosis. Our cases represent 2 very different manifestations of double-chambered right ventricle, which differ in presentation, in the site of abnormal obstructive muscle bundles, and in the presence of associated lesions. Both of our patients underwent successful surgical resection of the obstruction. One patient also underwent closure of a ventricular septal defect. We also review the literature on the various mechanisms that have been proposed to account for the complex morphology of the abnormal muscle bundles.

Myocardial revascularization using on-pump beating heart among patients with left ventricular dysfunction.

OBJECTIVES: On-pump beating heart technique for myocardial revascularization has been used successfully among both low and high risk patients. Its application among low ejection fraction patients is limited. The aim of our study is to evaluate this technique among patients with low ejection fraction and to compare results with off-pump bypass technique. METHODS: This retrospective study includes 137 patients with ejection fraction below 0.35 who underwent isolated coronary artery bypass surgery. 39 patients underwent myocardial revascularization using on-pump beating heart (ONCAB/BH), while 98 patients had off-pump beating heart (OPCAB). Different preoperative, operative and postoperative variables were evaluated among both groups. RESULTS: Patients profiles and risk factors were similar among both groups, except for the number of patients undergoing redo CABG which was significantly higher among ONCAB/BH (13% vs 3%; p = 0.025). Ejection fraction (EF) varied from 10-34%. The mean EF for patients who underwent ONCAB/BH was 28 ± 6 in comparison to 26 ± 5 for OPCAB patients (P = 0.093). Predicted risk for surgery according to EuroSCORE was similar among both groups (P = 0.443). The number of grafts performed per patient was significantly more among patients who underwent ONCAB/BH (2.2 ± 0.7 Vs 1.7 ± 0.7; P = 0.002). Completeness of revascularization was significantly greater in the ONCAB/BH patients (72% Vs 46%, P = 0.015). The incidence of hospital mortality and combined major morbidity was more among ONCAB/BH in comparison to OPCAB, but the difference was not significant. However, the incidence of blood loss, ventricular arrhythmias, inotropic support, ICU, hospital stay and blood transfusion were significantly greater among patients who underwent ONCAB/BH. CONCLUSIONS: On-pump beating heart technique can be used in myocardial revascularization among patients with left ventricular dysfunction. The technique was found to be associated with better myocardial revascularization when compared with OPCAB technique. However, the incidence of morbidity and mortality was more than OPCAB.