Introduction and objectives: To identify anatomical and functional characteristics associated with survival in adult patients with an absent atrioventricular connection and to highlight the diagnostic importance of echocardiography.
Methods: The clinical histories and echocardiographic and hemodynamic test results of 24 patients were recorded.
Results: Some 87.5% of patients were in New York Heart Association (NYHA) functional class I/II. In 92%, the ECG demonstrated sinus rhythm and left ventricular dilatation. Chest X-ray showed grade-II cardiomegaly in 83%. Situs solitus and an absent right atrioventricular connection were found on echocardiography in 92%. The ventriculoarterial connection was most frequently concordant (in 71%). All patients had an atrial septal defect, 21 had a ventricular septal defect and 21 had decreased pulmonary flow. The ejection fraction of the main ventricle in the whole patient group was 55% +/- 10%; 52% +/- 12% in those who did not undergo surgery and 58% +/- 8% in those who did (P=NS). Factors associated with poor survival were an ostium secundum atrial septal defect, hemoglobin <16 g/dL and a main ventricle ejection fraction <50%. Of the 54% of patients who underwent surgery, 85% are alive and the majority are in NYHA functional class I/II. Among those who did not, 82% are alive and 73% are in NYHA functional class I/II.
Conclusions: The presence of a wide atrial septal defect, a normal hemoglobin level and a normal main ventricle ejection fraction were associated with the survival of these patients into adulthood. Echocardiography can provide clinicians and surgeons with information that is valuable for selecting treatment and monitoring follow-up.