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J Thorac Cardiovasc Surg 2003;126:711-717
© 2003 The American Association for Thoracic Surgery

Surgery for congenital heart disease

Rapid progression of midventricular obstruction in adults with double-chambered right ventricle

José María Oliver, MDa,*, Ana Garrido, MDa, Ana González, MDa, Fernando Benito, MDa, Marta Mateos, MDa, Angel Aroca, MDa, Ernesto Sanz, MDa

a Adult Congenital Heart Disease Unit, Hospital Universitario La Paz, Madrid, Spain

Received for publication May 29, 2002; revisions received August 1, 2002; revisions received September 5, 2002; accepted for publication September 17, 2002.

* Address for reprints: Dr José María Oliver, Unidad Médico-Quirúrgica de Cardiología, Hospital Universitario La Paz, La Castellana 261, Madrid 28046, Spain
pepeoliver{at}jet.es

OBJECTIVE: The purpose of this study was to determine the rate of progression of midventricular obstruction in adolescents and adults with double-chambered right ventricle.

METHODS: Clinical and echocardiographic findings in 45 patients (mean age 26 ± 6 years, range 15-44) diagnosed with double-chambered right ventricle were retrospectively analyzed. Twenty patients underwent surgical repair before the age of 15 years. The relationship between Doppler midventricular pressure gradient and patient age was analyzed in 25 patients without previous repair. Sequential change in midventricular obstruction was determined for patients with 2 or more Doppler echocardiographic examinations performed within at least a 2-year interval.

RESULTS: Right midventricular pressure gradient in nonrepaired patients was 70 ± 38 mm Hg (range 25-150). A significant relationship between midventricular obstruction and patient age (r = 0.64, P < .001) was found. Midventricular pressure gradient at initial evaluation was 32 ± 27 mm Hg in 16 patients < 25 years and 73 ± 45 mm Hg in 9 patients >= 25 years (P < .03). After the initial study, 5 patients underwent surgical repair and 13 patients without repair were followed up for a period of 6.1 ± 2.7 years (range 2-9), in which midventricular pressure gradient increased from 32 ± 26 mm Hg to 67 ± 35 mm Hg (P < .001). The slope of the change in midventricular pressure gradient was 6.2 ± 3 mm Hg per year of follow-up. Seven more patients underwent surgical repair during follow-up due to progression of the obstruction. There was no mortality nor residual midventricular obstruction in surgically repaired patients.

CONCLUSIONS: Mild right midventricular obstruction shows a fast rate of progression in adolescents and young adults. Thus, close clinical and echocardiographic follow-up is advised, and surgical repair should be considered if significant progression of obstruction is detected.




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