Analysis of Achieving an "Ideal Outcome" Following Midline Unifocalization in Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

Richard D. Mainwaring, William L. Patrick, Tatiana R. Rosenblatt, Teimour Nasirov, Komal Kamra, Frank L. Hanley

Stanford University School of Medicine, Stanford, USA

Date, time and location: 2018.05.25 15:30, Press Hall, 2F

Abstract

Objective:  Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a complex form of congenital heart disease. Midline unifocalization has been developed for the surgical treatment of PA/VSD/MAPCAs. There are three measures of outcome that will determine long-term success: 1) Patients are alive, 2) Patients have achieved complete repair (i.e. VSD closure), and 3) Patients have a relatively low right ventricle to aortic pressure ratio (< 0.45). However, to date, no studies have combined these three measures of outcome to analyze the likelihood of achieving an "ideal outcome". 

Methods: This was a retrospective review of 255 patients who underwent midline unifocalization for PA/VSD/MAPCAs. The median age at unifocalization was 4.5 months. 

Results:  Two hundred thirty five (92%) patients are alive with a mean follow-up of 5.3 years.

Two hundred seventeen (85%) patients underwent a single stage complete repair, while 38 (15%) had an initial unifocalization and shunt. Twenty-four of the 38 palliated patients have subsequently had a repair. Thus, 241 (94%) patients ultimately achieved complete repair.

Of the 241 patients who have been repaired, 214 (89%) had a right ventricle to aortic peak systolic pressure ratio less than 0.45.

Combining these three measures of outcome, 77% of patients achieved an ideal outcome at one month, 73% at six months, 76% at one year, and 80% at 4 years.

Conclusions: The data demonstrate that the preponderance (80%) of patients can achieve all three measures of favorable outcome at 4 years following midline unifocalization. These results suggest that midline unifocalization is successful in achieving an ideal outcome for most patients. We would speculate that this will be a favorable portend for the future of these patients.