Summary
Fifteen patients with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum were studied. All were operated on in the neonatal period, with valvotomy or a systemic to pulmonary arterial shunt, or both. In 12 patients, right ventricular to pulmonary arterial communication was established in the neonatal period. In three patients, only systemic to pulmonary arterial shunts were constructed. Six patients died. The median follow-up period for the surviving patients was 65 months (range, 12–87 months).
Right and left atrial and ventricular dimensions and areas, the tricuspid annular diameter, and the cross-sectional area of the aortic root were measured in cross-sectional echocardiograms from the neonatal period, at the age of 1 year, and at the latest clinical follow-up. A classification of right ventricular morphology was made, based on identification of the inlet, the trabecular, and the outlet parts.
Most of the patients had hypoplastic right ventricles at birth but at the latest follow-up, seven of nine surviving patients had right ventricles in the normal range. Right ventricular growth was better in patients who were given a right ventricular to pulmonary arterial communication in the neonatal period and those with complete right ventricular anatomy. The patients who died had severely hypoplastic right ventricles and small tricuspid valves.
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References
Bull C, de Leval M, Mercanti C, MaCartney F, Anderson R (1982) Pulmonary atresia and intact ventricular septum: A revised classification.Circulation 66:266–271.
Cobanoglu A, Metzdorff M, Pinson W, Grunkemeier G, Sunderland C, Starr A (1985) Valvotomy for pulmonary atresia with intact ventricular septum. A disciplined approach to achieve a functioning right ventricle.J Thorac Cardiovasc Surg 89:482–490.
de Leval M, Bull C, Stark J, Anderson R, Taylor J, MaCartney F (1982) Pulmonary atresia and intact ventricular septum: Surgical management based on a revised classification.Circulation 66:272–280
Dobell A, Grignon A (1977) Early and late results in pulmonary atresia.Ann Thorac Surg 24:264–274
Freedom R, Culham J, Moes C (1984) Pulmonary atresia and intact ventricular septum. In: Freedom R, Culham J, Moes C (eds)Angiocardiography of congenital heart disease 1984. Macmillan, New York, pp 231–253
Freedom R, Wilson G, Trusler G, Williams W, Rowe R (1983) Pulmonary atresia and intact ventricular septum.Scand J Thorac Cardiovasc Surg 17:1–28
Freedom R, Dische R, Rowe R (1978) The tricuspid valve in pulmonary atresia and intact ventricular septum.Arch Pathol Lab Med 102:28–29
Fricker F, Zuberbuhler J (1987) Pulmonary atresia and intact ventricular septum. In: Anderson R, MaCartney F, Shinebourne E, Tynand M (eds)Paediatric cardiology 1987. Churchill Livingstone, London, pp 711–720
Gutgesell H, Bricker T, Colvin E, Latson L, Hawkins E (1984) Atrioventricular annular diameter: two-dimensional echocardiographic-autopsy correlation.Am J Cardiol 53:1652–1655
Hanséus K, Björkhem G, Lundström NR (1988) Dimensions of cardiac chambers and great vessels by cross-sectional echocardiography in infants and children.Pediatr Cardiol 9:7–15
Joshi S, Brawn W, Mee R (1986) Pulmonary atresia with intact ventricular septum.J Thorac Cardiovasc Surg 91:192–199
King D, Smith O, Huhta J, Gutgesell H (1985) Mitral and tricuspid valve annular diameter in normal children determined by two-dimensional echocardiography.Am J Cardiol 55:787–789
Leung M, Mok CK, Hui PW (1988) Echocardiographic assessment of neonates with pulmonary atresia and intact ventricular septum.J Am Coll Cardiol 12:719–725
Lewis A, Wells W, Lindesmith G (1983) Evaluation and surgical treatment of pulmonary atresia and intact ventricular septum in infancy.Circulation 67:1318–1323
Miller G, Restifo M, Shinebourne E, Paneth M, Joseph M, Lennox S, Kerr I (1973) Pulmonary atresia with intact ventricular settum and critical pulmonary stenosis presenting in first month of life.Br Heart J 35:9–16
Moulton A, Bowman F, Edie R, Hayes C, Ellis K, Gersony W, Malm J (1979) Pulmonary atresia with intact ventricular septum.J. Thorac Cardiovasc Surg 78:527–536
Patel R, Freedom R, Moes C, Bloom K, Olley P, Williams W, Trusler G, Rowe R (1980) Right ventricular volume determinations in 18 patients with pulmonary atresia and intact ventricular septum. Analysis of factors influencing right ventricular growth.Circulation 61:428–440
Scognamiglio R, Daliento L, Razzolini R, Boffa G, Pellegrino P, Chioin R, Volta S (1986) Pulmonary atresia with intact ventricualr septum: A quantitative cineventriculographic study of the right and left ventricular function.Pediatr Cardiol 7:183–187
Silove E, de Giovanni J, Shiu M, Myint M (1983) Diagnosis of right ventricular outflow obstruction in infants by crosssectional echocardiography.Br Heart J 50:516–520
Trowitzsch E, Colan S, Sanders S (1985) Two-dimensional echocardiographic evaluation of right ventricular size and function in newborns with severe right ventricular outflow obstruction.J Am Coll Cardiol 6:388–393
Zuberbuhler J, Anderson R (1979) Morphological variations in pulmonary atresia with intact ventricular septum.Br Heart J 41:281–288
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Hanséus, K., Björkhem, G., Lundström, NR. et al. Cross-sectional echocardiographic measurements of right ventricular size and growth in patients with pulmonary atresia and intact ventricular septum. Pediatr Cardiol 12, 135–142 (1991). https://doi.org/10.1007/BF02238519
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DOI: https://doi.org/10.1007/BF02238519