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Atypical forms of isolated partial atrioventricular septal defect increase the risk of initial valve replacement and reoperation.

OBJECTIVE: We consider the short- and long-term outcomes of the repair of the isolated partial atrioventricular (AV) septal defect to determine the role played by the atypical forms on the initial AV valve replacement and on the risk of reoperation. METHODS: Two hundred and eight patients underwent an operation for this malformation between 1974 and 2001. Clinical and echocardiographic examinations were performed on all patients, the AV valve regurgitation was graded from 1 to 4 and a residual interatrial shunt was sought. Median age at the intervention was 5.8 years (3 months to 67 years). RESULTS: Median follow-up time was 7.5 years (range 0-22.6 years). The cumulative 30-day, 5- and 20-year survival rates were 96.5, 95.4 and 94.6%, respectively. AV valve replacement was associated with a high mortality (P<0.001). A reoperation was performed on 12 patients (5.7%) including six patients within less than a 30-day period, especially to repair residual AV valve regurgitation. We performed four AV valve repairs by annuloplasty and six AV valve replacements. Two patients who had initially undergone an AV valve replacement underwent a reoperation for valve thrombosis. The cumulative 30-day, 5- and 20-year rates of freedom from reoperation were 96.5, 93.6 and 83%, respectively. An atypical form was present in 24 patients (11.5%) and was a risk factor for initial AV valve replacement (P<0.001) and for reoperation (P<0.001). A complete AV block occurred in 13 patients (6.2%), all of them within a 30-day period. The AV valve replacement was a high risk factor for a complete AV block (P<0.001). At the end of our study 180 patients (96%) were in NYHA I and 8 in NYHA II. CONCLUSIONS: The morbi-mortality of the isolated partial AV septal defect is primarily perioperative and is linked with the presence of an atypical form of the lesion. This atypical form was the main reason for reoperation for AV valve regurgitation. The AV valve replacement was associated with a high mortality and with the occurrence of complete AV block. Using a standardized technique, the AV septal defect can be repaired with excellent long-term clinical and echographic results.

Adolescent↗

Palliative repair of transposition of the great arteries with criss-cross heart: ventricular septal defect and hypoplastic right (systemic) ventricle.

Four cases of palliative Mustard or Senning repair for transposition of the great arteries (TGA) with ventricular septal defect (VSD), hypoplastic right ventricle, and superior-inferior ventricular configuration are presented. The palliative Mustard procedure-a Mustard repair without VSD closure-is usually reserved for patients with pulmonary vascular obstructive disease (PVOD). In such cases, VSD closure would result in left ventricular failure. Each of our four patients had normal or only slightly elevated pulmonary resistance (1.0 to 5.2 Wood units). However, in each case a hypoplastic right ventricle precluded VSD closure. All four patients had transposition-like hemodynamics with unfavorable streaming to the great arteries, despite the fact that two patients had a levo arterial configuration. In each case, the ventricular relationship included a hypoplastic, superior right ventricle with a horizontal ventricular septum-the so-called "upstairs-downstairs" or "superior-inferior" heart. All patients had previous balloon atrial septostomy or open septectomy to improve atrial mixing. Two patients had previous pulmonary artery banding because of increased pulmonary flow. All four patients remain survivors of the palliative Mustard or Senning repair, which was performed at 10 months, 5 1/2, 12, and 16 years. In each case, there was a marked improvement of symptomatology with a decrease of hemoglobin (mean 21.1 gm/dl preoperatively to 15.3 gm/dl postoperatively) and an increase of arterial oxygen saturation (mean 78 vol % preoperatively to 93 vol % postoperatively). This is the first palliative Mustard or Senning repairs in patients with TGA, VSD, and hypoplastic right ventricle without PVOD. The procedure produces gratifying palliation for these patients.

Adolescent↗

Evaluation of intracardiac shunts with cardiac magnetic resonance.

Intracardiac shunts including atrial septal defect, ventricular septal defect, endocardial cushion defects, and surgical baffles may be identified, localized, and quantified using cardiac MRI methods. Both dark-blood and bright-blood techniques are helpful to identify anatomy. Contrast enhancement is especially useful for identifying associated vascular anomalies. Dynamic first-pass contrast agent signal-time studies may demonstrate rapid recirculation and shunting. Volumetric and phase contrast cine methods are useful to quantify flow. Pulmonary to systemic (Qp/Qs) flow ratios may be calculated noninvasively by comparing the pulmonary artery flow to the aortic flow measurement.

Coronary Circulation↗

Lung function in atrial septal defect after heart surgery.

Lung volume, indices of lung elasticity and airway patency were measured in 74 patients, 9-21 yr old (15.0 +/- 2.5 yr) with atrial septal defect (secundum type) from 2-11 (5.1 +/- 2.5) yr after successful surgical correction. Clinical condition in all patients was classified as excellent. Heart surgery was performed at the age of 4-14.8 (9.9 +/- 3.0) yr. Lung function abnormalities were found in 35 (47.3%) patients. Lung recoil pressure was significantly increased in 24 (32.4%) patients. The mean value of lung recoil pressure at 100% of total lung capacity reached 123 +/- 31% of the predicted value (P < 0.0001). Specific static compliance was within normal limits. Specific airway conductance was significantly reduced in 13 (17.6%) patients. Maximum expiratory flow at 25% of vital capacity was significantly reduced in 4 (5.4%) patients. Static lung volumes did not differ from reference values. Since various abnormalities of lung function tests (most frequently the tests suggested increased lung stiffness and obstruction of larger airways) were revealed in almost half of the studied children and adolescents after successful surgery for atrial septal defect, we propose to carry out lung function tests routinely as another criterion of health conditions in these patients.

Adolescent↗