The angulation of the septal structures impacts ventricular imbalance in atrioventricular septal defects with a common atrioventricular junction.

OBJECTIVE: Multiplanar re-formatting of full-volume three-dimensional echocardiography data sets offers new insights into the morphology of atrioventricular septal defects. We hypothesised that distortion of the alignment between the atrial and ventricular septums results in imbalanced venous return to the ventricles, with consequent proportional ventricular hypoplasia. METHODS: A single observer evaluated 31 patients, with a mean age of 52.09 months, standard deviation of 55, and with a range from 2 to 264 months, with atrioventricular septal defects, of whom 17 were boys. Ventricular imbalance, observed in nine patients, was determined by two-dimensional assessment, and confirmed at surgical inspection in selected cases when a univentricular strategy was undertaken. Offline analysis using multiplanar re-formatting was performed. A line was drawn though the length of the ventricular septum and a second line along the plane of the atrial septum, taking the angle between these two lines as the atrioventricular septal angle. We compared the angle between 22 patients with adequately sized ventricles, and those with ventricular imbalance undergoing univentricular repair. RESULTS: In the 22 patients undergoing biventricular repair, the septal angle was 0 in 14 patients; the other eight patients having angles ranging from 1 to 36, with a mean angle of 7.4°, and standard deviation of 11.1°.The mean angle in the nine patients with ventricle imbalance was 28.6°, with a standard deviation of 3.04°, and with a range from 26 to 35°. Of those undergoing univentricular repair, two patients died, with angles of 26 and 30°, respectively. CONCLUSIONS: The atrioventricular septal angle derived via multiplanar formatting gives important information regarding the degree of ventricular hypoplasia and imbalance. When this angle is above 25°, patients are likely to have ventricular imbalance requiring univentricular repair.

Multiplanar review of three-dimensional echocardiography gives new insights into the morphology of Ebstein's malformation.

INTRODUCTION: We aimed to assess the ability of the multiplanar review modality of three-dimensional echocardiography to examine the dynamic morphology and the functional characteristics of malformed tricuspid valves in patients previously identified as having Ebstein's malformation. Based on these characteristics, we attempted to differentiate Ebstein's malformation from tricuspid valvar dysplasia. METHODS: Using three-dimensional multiplanar review, analysed with either Qlab 6.0 or Tomtech Image Arena 3.0, we studied 23 patients, aged from 1 day to 70 years, previously diagnosed using cross-sectional echocardiography as having Ebstein's malformation. RESULTS: Using the features of rotational abnormality, and the orientation, of the effective tricuspid valvar orifice as diagnostic features of Ebstein's malformation, we reclassified 11 patients (48 per cent) as exhibiting tricuspid valvar dysplasia. In addition, we studied the dynamic morphology as well as the function of the tricuspid valve. Surgical treatment was undertaken on 10 patients, revealing good correlation with the findings obtained using three-dimensional multiplanar review. In those with Ebstein's malformation, we found varying degrees of rotation, with the effective valvar orifice always directed towards the right ventricular outflow tract. The opening of the orifice of dysplastic tricuspid valves, in contrast, was towards the apex of the right ventricle. The degree of delamination, and abnormalities of subcordal apparatus, were similar in the two groups. DISCUSSION: Three-dimensional multiplanar review permits accurate definition of the dynamic morphology of Ebstein's malformation, permitting clear differentiation from tricuspid valvar dysplasia.

The accuracy of three-dimensional echocardiography with multiplanar reformatting in the assessment of the aortic valve annulus prior to percutaneous balloon aortic valvuloplasty in congenital heart disease.

OBJECTIVE: To review the use of three-dimensional echocardiography (3DE) with multiplanar reformatting (MPR) in children with congenital aortic stensosis undergoing percutaneous balloon aortic valvuloplasty to assess its accuracy in measuring the aortic valve annulus and any influence it may have on balloon sizing. METHODS: All percutaneous aortic balloon valvuloplasties performed from 01/01/2009 to 01/09/2011 were included in the study. All imaging performed for the procedure to determine the size of the aortic valve annulus and aid in balloon sizing was reviewed. The maximum diameter of the aortic valve annulus using two-dimensional echocardiography (2DE), 3DE with MPR, and angiography was recorded. The balloon size used in the procedure was recorded and the balloon to annulus ratio was calculated. RESULTS: A total of 27 procedures were included in the study. Age varied from 1 day to 156 months (mean age, 53 months) and weight from 2.8-58 kg (mean weight, 18.6 kg). Fourteen patients had 3DE with MPR available for analysis. The 3DE with MPR measurement (13.36 ± 5.4 mm) was not different from angiography (13.54 ± 6.4 mm; P=.803).The 2DE measurement was significantly different from angiography (11.72 ± 5 mm; P<.005). The balloon to annulus ratio based on angiographic measurements did not differ significantly between the patients with 3DE MPR and those without (0.94 ± 0.095 vs 0.91 ± 0.1; P=.468). CONCLUSION: 3DE with MPR allows a more accurate assessment of the aortic valve annulus compared to 2DE, which may reduce the tendency to undersize balloon choice. 3DE with MPR did not significantly affect our balloon choice, which was largely based on angiographic measurements.

Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension.

BACKGROUND: Complex pulmonary atresia (CPA) i.e. pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension. METHODS: A cross-sectional retrospective review of patients >16 years with CPA or Tetralogy of Fallot with MAPCAs and PA hypertension treated with sildenafil was conducted. Case notes were reviewed for baseline and follow-up (after sildenafil) characteristics. RESULTS: Five patients, 4 female, median age 28 (range 18 to 47) years, were identified. All patients experienced symptomatic improvement: 2 of 4 wheelchair bound patients responded dramatically and walked 345 and 157 m respectively in 6 min following sildenafil therapy. One of the 4 with marked PA arborization abnormalities and severe ventricular dysfunction had initial symptomatic improvement. Another patient improved from walking less 100 m to climbing 2 flights of stairs. Arterial saturations improved in 2 cases from 70 and 60% to 87 and 84% respectively, whilst arterial saturations remained static in 1 case despite embolization of a classical Blalock-Taussig shunt. One patient with PA arborization/diminished PA bed was unable to tolerate sildenafil. CONCLUSIONS: Sildenafil is well tolerated and leads to symptomatic improvement and better saturations in the majority of patients with CPA with PA hypertension when used in isolation or as an adjunct to percutaneous PA angioplasty.

Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects.

BACKGROUND: There is increasing evidence for beneficial effects of the type 5 phosphodiesterase inhibitor, sildenafil, in idiopathic pulmonary arterial hypertension (PAH). The effects of sildenafil in severe PAH associated with an atrial septal defect (ASD) have not been clearly delineated. METHODS: This extended case series reviews our experience with sildenafil treatment in three patients with severe PAH and Eisenmenger syndrome associated with an ASD. Case notes were reviewed for baseline and follow-up anatomic, clinical and haemodynamic characteristics. RESULTS: Of three patients identified, median age 44.3 years (range, 28 to 59 years), two had large secundum ASDs and the other a sinus venous defect. All patients had severe PAH and were desaturated at rest and/or during exercise. Sildenafil was started because of progressive and severe effort intolerance. All patients experienced symptomatic improvement, had higher arterial saturations (range 8-19%) and improvement in effort tolerance (88 m and 56 m improvement in 6-min walk test distance (MWTD) in two patients). Right ventricular (RV) function and Doppler derived RV systolic pressure improved in two patients. Pulmonary arterial pressures decreased in two patients who had cardiac catheterization (range 4 to 14 mm Hg). Pulmonary vascular resistance decreased from 7.58 to 3.8 Wood's units in one patient who is now awaiting surgery. Another patient developed significant pulmonary vasoreactivity (8.3 to 6.2 Wood's units with 100% oxygen) after 16 months of sildenafil therapy. CONCLUSIONS: Sildenafil not only relieves symptoms associated with severe PAH in patients with large ASDs, but also improves pulmonary arterial hemodynamics and RV function.