Severe Ebstein's anomaly can benefit from a small ventricular septal defect: two cases.

Ebstein's anomaly is a rare congenital heart defect. Associated lesions are uncommon, and the mortality rates can be as high as 54% during the first month of life. Two cases of severe Ebstein's anomaly with ventricular septal defect are described. It is speculated that this rare association, allowing adequate forward pulmonary blood flow in the neonate, permitted the reported patients to survive the neonatal period, which is the most life-threatening time. The authors propose that the presence of a small ventricular septal defect can be beneficial for such patients, averting the need for surgery during early infancy when the risk is highest.

Minimally invasive or interventional repair of atrial septal defects in children: experience in 171 cases and comparison with conventional strategies.

OBJECTIVES: The goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children. BACKGROUND: Concern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization. METHODS: A retrospective analysis was performed on the patients treated from June 1996 to December 1998. RESULTS: One hundred seventy-one children (median age 5.8 years, median weight 22.1 kg) underwent 52 device implants, 72 minimally invasive surgical operations and 50 conventional sternotomy operations. There were no deaths and no residual left to right shunt in any of the groups. The overall complication rate causing delayed discharge was 12.6% for minimally invasive surgery, 12.0% for midline sternotomy and 3.8% for transcatheter device closure (p < 0.01). The mean hospital stay was 2.8 +/- 1.0 days, 6.5 +/- 2.1 days and 2.1 +/- 0.5 days (p < 0.01); the skin-to-skin time was 196 +/- 43 min, 163 +/- 46 min and 118 +/- 58 min, respectively (p < 0.001). Extracorporeal circulation time was 49.9 +/- 10.1 min in the minithoracotomy group versus 37.2 +/- 13.8 min in the sternotomy group (p < 0.01) but without differences in aortic cross-clamping time. Sternotomy was the most expensive procedure (15,000 EUR +/- 1,050 EUR vs. 12,250 EUR +/- 472 EUR for minithoracotomy and 13,000 EUR +/- 300 EUR for percutaneous devices). CONCLUSIONS: While equally effective compared with sternotomy, the cosmetic and financial appeal of the percutaneous and minimally invasive approaches must be weighed against their greater exposure to technical pitfalls. Adequate training is needed if a strategy of surgical or percutaneous minimally invasive closure of ASD in children is planned in place of conventional surgery.

Comparison of three different atrial septal defect occlusion devices.

Three different devices were used to close secundum-type atrial septal defects in 28 patients. The "Amplatzer" is associated with an easier and shorter procedure than are the "Sideris Buttoned Device" and the Microvena "Angel Wings" devices.

Pitfalls in echocardiographic-based repair of aortic coarctation.

The knowledge of echocardiographic pitfalls, which may impair the correct indication for surgery of aortic coarctation, is of great importance to pediatric cardiologists. We believe that only the suspicion of coronary artery anomalies is a clear indication to perform a cardiac catherization.

Echocardiographically guided repair of tetralogy of Fallot.

Although 2-dimensional, Doppler, color flow echocardiography is accepted as a safe diagnostic method to guide the surgical treatment of certain heart defects, cardiac catheterization remains mandatory for patients with tetralogy of Fallot. Based on the excellent diagnostic correlation between echocardiography and angiocardiography, a policy of echo-guided primary repair of uncomplicated, selected cases of tetralogy of Fallot was introduced at Ospedale Bambino Gesù. In the last 5 years, of 139 patients who had surgery for tetralogy of Fallot, 105 underwent primary repair. In 56 patients (53.3%), surgery was guided only by echocardiography (group I). In the remaining 49 patients (46.7%) (group II), unclear imaging of cardiovascular anatomy (n = 23), or echo-suspected associated malformations (n = 26) prompted cardiac catheterization. The 2 groups did not differ in age and weight at surgery. Intraoperative examination did not show diagnostic errors in patients of group I; cardiac catheterization and surgery confirmed the suspected associated anomalies in 16 of 26 patients of group II (38.4% false-positive). Echocardiography showed an overall sensitivity of 100% and specificity of 85% for detection of associated malformations. In conclusion, echocardiography proved to be adequate for selection of patients with uncomplicated forms of tetralogy of Fallot for primary repair.

Staged Fontan operation for complex cardiac anomalies with subaortic obstruction.

Ventricular hypertrophy is a recognized risk factor for the Fontan operation in cases of complex cardiac anomalies with unrestricted pulmonary blood flow and subaortic obstruction. Between 1986 and 1991 we have treated 23 such patients with a new type of palliation combining a main pulmonary artery-ascending aorta anastomosis with a bidirectional cavopulmonary anastomosis. Their ages averaged 57 +/- 36 months (7 to 155 months). Nine patients had (S,L,L) single ventricule with transposition and the other 14 had more complex cardiac anomalies unsuitable for biventricular repair. Subaortic obstruction was defined by a hemodynamic criterion (n = 6) or by a morphologic criterion (n = 17). There were five hospital deaths (21.7%): All three patients older than 7 years of age died (p = not significant). Follow-up averaged 28 +/- 21 months (range 1 to 58 months). Among the 18 hospital survivors, the proportions surviving 1 and 5 years after the operation were 78% and 63%, respectively. Control cardiac catheterization in 11 patients showed no or trivial subaortic gradient, a mean cavopulmonary pressure of 10 +/- 3 mm Hg, and a mean arterial oxygen saturation of 83%. Nine patients underwent secondary Fontan repair in our institution 21 +/- 4 months after palliation, without deaths. Another patient died elsewhere, 3.7 years after palliation, as a result of hemorrhage at sternal reentry during attempted Fontan operation (overall mortality at repair, 10%). The other eight patients are awaiting Fontan operation. This staged approach reduces both pressure and volume ventricular load and provides adequate oxygenation before the Fontan operation. In our experience, it resulted in reduced mortality at definitive repair.

Abnormal intraventricular flow patterns in left ventricular dysfunction determined by color Doppler study.

We examined the relation between left ventricular (LV) flow dynamics measured by color Doppler, and either global or regional LV function in 19 normal subjects (group 1), in 55 patients with old myocardial infarction (MI) (29 without [group 2] and 26 with LV aneurysm [group 3]), and in 16 with idiopathic dilated cardiomyopathy (group 4). We calculated by M-mode color Doppler a flow persistence index (FPI) (duration of flow directed in systole toward the apex/LV ejection time). Contrast echocardiography was performed as a control method in 14 patients of the four groups. In normal subjects, rapid systolic inversion of flow toward the aorta was evident (FPI: 0.11 +/- 0.16). In all but one patient in group 2, a similar LV flow pattern was observed, but FPI was greater (0.32 +/- 0.26). In groups 3 and 4, a paradoxical antegrade LV flow pattern was evident during the entire period of systole (FPI: 1.13 +/- 0.42 and 1.28 +/- 0.36, respectively). LV flow patterns were reproduced in echo-contrast studies. FPI was related to LV end-diastolic volume (r = 0.77), end-systolic volume (r = 0.82), and ejection fraction (r = -0.84). However, when data were analyzed separately in the different groups, these correlations were significant only in groups 2 and 3. Paradoxical flow pattern is not peculiar to regional LV dysfunction; it also occurs in global LV dysfunction. This LV flow abnormality may develop after MI even in the absence of severe LV dyssynergy or dilation, and is quantitatively related to the degree of LV dysfunction.

Systolic ejection murmurs in the elderly: aortic valve and carotid arteries echo-Doppler findings.

UNLABELLED: Two-dimensional echographic and color Doppler studies of the heart and carotid arteries (CA) were performed in 45 patients greater than sixty-five years old without aortic stenosis, 23 with (Group 1) and 22 without (group 2) precordial ejection systolic murmur (SM). Aortic cusps thickening was found in 11 Group 1 (48%) and 2 Group 2 (9%) patients (p less than 0.001). Aortic root and aortic arch size were similar in the two groups. Maximum aortic flow velocity was significantly greater in Group 1 (200 60 cm/sec) than in Group 2 (120 20 cm/sec) (p less than 0.001). Left ventricular outflow systolic maximum velocity was similar in the two groups. A bilateral neck murmur was heard in 10/23 Group 1 patients (43%); in this group, patients with cervical SM had a greater maximum aortic flow velocity than those without cervical SM (230 + 60 cm/sec vs 172 + 32 cm/sec, p less than 0.001). In Group 1, 3 patients had a cervical SM louder on one neck side; only in these 3 patients were ipsilateral obstructive CA plaques found. A unilateral neck SM was heard in 4/22 Group 2 patients (18%); in these 4, ipsilateral obstructive CA were found. CONCLUSIONS: (1) in the elderly, precordial ejection SM is related to mild increase in maximum aortic flow velocity and thickening of aortic cusps; (2) in patients with precordial SM radiated to both neck sides, maximum aortic flow velocity tends to be more markedly increased; (3) in patients with precordial SM, a cervical SM louder on one neck side should suggest coexistent ipsilateral CA stenosis.