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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↗

[Surgical aspects of radical correction of ventricular heart septal defects].

A working scheme of indications for the surgical correction of defects of the interventricular septum (DIVS) has been developed on the basis of observation of 810 patients. An anatomo-topographic classification of DIVS on the basis of studying preparations of 109 hearts is proposed which is convenient for choosing a method of closing DIVS. The incidence of complications after operations under conditions of moderate hypothermia was shown to be not higher than after those performed under the artificial blood circulation.

Adolescent↗

Second natural history study of congenital heart defects. Ventricular septal defect: echocardiography.

BACKGROUND: Two-dimensional and Doppler echocardiography can provide structural and hemodynamic information for patients with ventricular septal defects (VSDs). Therefore, two-dimensional and Doppler echocardiographic examinations were performed on patients with VSDs returning for the Second Natural History Study of Congenital Heart Defects. METHODS AND RESULTS: Five hundred fifty-six patients with VSDs underwent two-dimensional and Doppler echocardiographic examinations. Three hundred twenty-four patients were in the medically treated group, and 232 had undergone a previous operation. The location of the VSD was classified in 235 patients. Several Doppler measurements were used to calculate pulmonary artery pressures: tricuspid regurgitation peak systolic velocity, peak systolic velocity of VSD jet, pulmonary regurgitation end-diastolic velocity, and pulmonary artery acceleration time. The two methods that were found to be reliable consisted of the tricuspid regurgitation peak systolic velocity and the pulmonary regurgitation end-diastolic velocity. These measurements were obtained in only 26% of patients at all centers. At one center in which adult and pediatric echocardiography was performed in a single laboratory, these values were obtained in 60% of patients. CONCLUSIONS: Two-dimensional and Doppler echocardiography has the ability to provide a noninvasive method of assessing morphology and hemodynamics in patients with VSDs. However, the reliability and accuracy of hemodynamic measurements are dependent on operator experience.

Blood Pressure↗