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PubMed1981

Hemodynamic abnormalities in response to supine exercise in patients after operative correction of tetrad of Fallot after early childhood.

The exercise hemodynamic values in two groups of patients with repaired tetrad of Fallot (eight patients with some residual pulmonary insufficiency and seven patients without insufficiency) were compared with values in seven patients with trivial pulmonary stenosis who had not been operated on. The patients with tetrad of Fallot underwent surgery after age 8 years and all had a good hemodynamic repair (no shunts and a right ventricular systolic pressure at rest of less than 60 mm Hg). Exercise increased the right ventricular outflow tract gradient by the same magnitude in all three groups of patients. However, both surgically treated groups experienced impaired cardiac pump function on supine exercise (that is, a lower than anticipated cardiac index for the amount of oxygen consumed and a significant decrease in stroke index). Exercise also caused both groups with repair to have a decrease in stroke index and a concomitant increase in right ventricular end-diastolic and pulmonary wedge pressures; in contrast, the patients with pulmonary arterial stenosis had an increase in stroke index and a concomitant decrease in right ventricular end-diastolic and pulmonary wedge pressures. These findings indicate that an impaired cardiac response to supine exercise can occur in patients in whom intracardiac repair of tetrad of Fallot was performed after early childhood, even though they have had a good hemodynamic repair. In addition, the impaired cardiac response to supine exercise in these patients was probably due largely to an altered myocardial compliance rather than to either residual pulmonary stenosis or pulmonary insufficiency.

Adolescent↗
PubMed1992

[Biventricular repair for right isomerism associated with transposition of great arteries and common atrioventricular canal].

A 5-years old boy, whose thoracic situs was right isomerism, was diagnosed as transposition of great arteries, common atrioventricular canal (intermediate type), severe tricuspid valve regurgitation, pulmonary valve stenosis, bilateral superior vena cava and left sided inferior vena cava. The preoperative RVEDV was 95% and LVEDV 160%, and LV/RV pressure ratio was 0.54. Atrial rerouting using pericardial patch, replacement of tricuspid valve and pulmonary valvotomy were performed. Postoperative course was uneventful. This was a rare case of right isomerism who received biventricular repair.

Child, Preschool↗
PubMed1993

Is there an anatomic basis for subvalvular right ventricular outflow tract obstruction after an arterial switch repair for complete transposition? A morphometric study and review.

The study was initiated by reports on right ventricular outflow tract obstruction in complete transposition of the great arteries after an arterial switch repair. We investigated 39 heart specimens with native, unoperated transposition of the great arteries. Of these, 14 hearts had a ventricular septal defect; 25 had an intact ventricular septum. In each heart specimen the narrowest site of the subaortic outflow tract was measured and compared with the circumference of the aortic orifice. Obstruction was considered to be present if the outflow tract circumference was less than that of the aortic orifice. In addition, the diameter of the ascending aorta immediately above the level of the valve orifices was measured and compared with that of the pulmonary trunk. An obstruction was present in the subaortic right ventricular outflow tract of two hearts (5.1%): one of the obstructions, in a neonatal heart with intact ventricular septum, was caused by a prominent supraventricular crest and anterior trabeculations; the other obstruction was an additional extensive muscular hypertrophy, in the heart of a 13-year-old patient with a similar anatomy, and a septal defect. A mismatch between the diameters of the ascending aorta and the pulmonary trunk was present in 15 of 32 hearts measured. Our observations and a review of the literature confirm that subvalvular right ventricular outflow tract obstruction in hearts with native transposition of the great arteries is infrequent. Nevertheless, the anatomic characteristics of the right ventricular outflow tract are such that the tract is intrinsically narrow and muscular hypertrophy may easily lead to obstruction. After an arterial switch operation, subvalvular obstruction could be caused by dynamic processes analogous to those observed after relief of isolated pulmonary valve stenosis. Anatomic subvalvular obstruction could be due to either an obstruction that was not identified before operation or (a purely speculative hypothesis) subtle degrees of mismatch in size between the proximal aorta and the pulmonary trunk, which may be considered irrelevant at time of operation but may also set into pace a process of ongoing adaptive infundibular hypertrophy.

Academic Medical Centers↗
PubMed1993

Echocardiographic evaluation of isolated pulmonary valve disease in adolescents and adults.

Congenital pulmonary valve disease is often not discovered until adolescence or adulthood. Transthoracic two-dimensional echocardiography can provide detailed information regarding right ventricular outflow anatomy, although images are often less satisfactory than those obtained in infants and children. The more recent addition of biplanar transesophageal echocardiography has enhanced our ability to image the right ventricular outflow tract, pulmonary valve, and pulmonary artery noninvasively. Pulsed and continuous-wave Doppler estimates of subvalvular and transvalvular gradients have proved to be accurate. Doppler color flow mapping has proved useful in determining the location and direction of stenotic and regurgitant flow. With no accepted standard for comparison, quantification of regurgitation remains problematic. In many cases, echocardiography has replaced catheterization and angiography in the evaluation and long-term follow-up of congenital pulmonary valve disease before and after intervention.

Adolescent↗
PubMed1989

[Transluminal angioplasty in total anomalous drainage of obstructed pulmonary veins. A case report].

Transluminal balloon angioplasty has been successfully used in the treatment of various congenital heart diseases, specially in pulmonary valve stenosis. Dilation angioplasty has also been used in venous obstructions in children, but the results have been contradictory. This report describes the case of a three-month-old female infant with obstructed total anomalous pulmonary venous return into the right superior vena cava. Percutaneous angioplasty was performed with two 4 mm diameter balloon catheters (Simpson Ultra Low Profile), with success. Right ventricular systolic pressure decreased from 68 to 49 mmHg and mean pulmonary vein pressure decreased from 41 to 17 mmHg. There was significant clinical improvement. Although it would be an unique experience in our Department, authors suggest that transluminal balloon angioplasty could be an initial therapeutic alternative in infants with obstructive total anomalous pulmonary venous return, a high-risk group for total surgical repair.

Angioplasty, Balloon↗