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Two-dimensional echocardiographic detection of pulmonary venous channel stenosis after Senning's operation.

Stenosis of the common pulmonary venous (PV) channel after Senning's operation for complete transposition of the great arteries is a serious problem, and a method for noninvasive diagnosis is highly desirable. Therefore, our purpose was to devise a method to diagnose PV channel stenosis noninvasively. The newly created common PV channels were measured and the short-axis views of the left ventricle were observed by means of two-dimensional echocardiography in 16 patients who had undergone Senning's procedure. In seven patients we used the original procedure and in nine patients we used a modified procedure with a pedicled pericardial patch. The shape of the left ventricle became round abruptly when the width of the PV channel decreased below a critical value (a range of 6 to 9 mm). Three patients with a relatively round-shaped left ventricle and a narrow PV channel (below 9 mm) were all confirmed to have PV channel stenosis at reoperation. All of the patients with a wide PV channel (above 10 mm) and a flat-shaped left ventricle were in good condition both clinically and as determined from the catheterization data. After the operation, patients who had tricuspid regurgitation, pulmonary vascular obstruction, or residual left ventricular outflow tract stenosis had a relatively round-shaped left ventricle even though the PV channel was wide enough (above 10 mm). Measurement of the PV channel confirmed the effects of these conditions. Correct diagnosis of PV channel stenosis can therefore be made by observing the shape of the left ventricle and by measuring the PV channel.

Child↗

Cardiopulmonary rehabilitation in a patient with Noonan syndrome.

Noonan syndrome, an autosomal dominant disease occurring with an incidence of 1 in 1,000 to 1 in 2,500 live births, is characterized by its particular cardiovascular abnormalities, including pulmonic valve stenosis, pulmonary artery stenosis, and, more rarely, septal defects and coarctation of the aorta. The case of a 20-year-old man admitted for inpatient cardiopulmonary rehabilitation after pulmonic valve repair, left pulmonary artery angioplasty, and pectus excavatum repair is presented. His endurance was markedly decreased, thus limiting his ability to perform activities of daily living and reducing his exercise tolerance. With participation in a comprehensive cardiopulmonary rehabilitation program, he experienced marked improvement with independence in his activities of daily living and an increase in his metabolic equivalent levels from to 2.8 to 5.4. After inpatient rehabilitation, he underwent left pulmonary stent placement before being discharged home. Subsequent outpatient cardiopulmonary rehabilitation has continued to improve significantly his overall exercise tolerance. Given that Noonan syndrome is viewed as the most common syndrome associated with congenital heart disease after Down syndrome, physiatrists must be familiar with its presentation, its associated abnormalities, and the treatment approach to optimize the patient's cardiopulmonary, musculoskeletal, and psychological status.

Adult↗

[Modified Fontan's operation].

Nine-year-old female patient presented with cianosis since she was born, fatique and dyspnea when sucking. The diagnosis was univentricular heart with left ventricular morphology, transposition of the great arteries, moderate pulmonary valve stenosis and atrial septal defect. Submitted to surgical correction with superior vena cava-right pulmonary artery anastomosis, inferior vena cava anastomosis using lateral tunnel, with cardiopulmonary bypass. After surgical correction, the clinical and laboratorial (echocardiogram and cardiac catheterization) evaluation showed Fontan operation with good surgical results. Total cavopulmonary connection was proposed as a modification of the Fontan procedure that might have greater benefits than previous proposed techniques. The results demonstrate that this modification provides excellent early definitive treatment, increasing hemodynamic profile, with low morbidity and mortality, for a variety of complex congenital heart lesions.

Child↗