Elsevier

The Annals of Thoracic Surgery

Volume 98, Issue 5, November 2014, Pages 1680-1686
The Annals of Thoracic Surgery

Original article
Congenital heart surgery
More Than 25 Years of Experience in Managing Pulmonary Atresia With Intact Ventricular Septum

https://doi.org/10.1016/j.athoracsur.2014.05.085Get rights and content

Background

Management of pulmonary atresia with intact ventricular septum (PAIVS) is challenging and depends on the individual patient's morphologic characteristics. We evaluated outcomes of 25 years of experience in morphologically driven management of PAIVS.

Methods

Between January 1985 and December 2011, 60 patients were treated for PAIVS. Patients were retrospectively categorized into mild (n = 27), moderate (n = 18), and severe (n = 15) right ventricular (RV) hypoplasia subgroups based on morphologic characteristics at first presentation. Tricuspid valve (TV) annulus z score increases were analyzed in patients who underwent biventricular repair and in patients who underwent 1.5-ventricular repair. Median follow-up time was 9.8 years (range, 0–25 years). One patient was lost to follow-up.

Results

Fifty-nine (98.3%) patients underwent operation. There were 7 early postprocedure deaths and 1 late death. Estimated overall survival was 86.5% at 10 years after initial admission for the total group, and 96.3%, 77.8%, and 79.4% for the subgroups, respectively. In the respective subgroups, biventricular repair was achieved in 24 (88.9%), 3 (16.7%), and 0 patients. In the patients who underwent biventricular repair, there was a significant increase of the TV annulus z score. At latest follow-up, mean New York Heart Association (NYHA) class was 1.3 for the total group and 1.1, 1.2, and 1.5 for the patients who underwent biventricular, 1.5-ventricular, and univentricular repair, respectively.

Conclusions

Our individualized management approach to PAIVS showed good early and long-term results. Early decision on the management goal, determined by individual morphologic characteristics at presentation, is of paramount importance. In specific patients, however, taking into account RV development at a later point in time may improve outcome.

Section snippets

Patients and Data Collection

This descriptive retrospective study was approved by the ethics committee of our institution. We identified all patients diagnosed with or treated for PAIVS in our institution between January 1, 1985 and December 31, 2011. A total of 60 patients were included in this study. For each patient, available medical records, echocardiograms, angiograms, and operative reports were reviewed. Patients’ clinical characteristics at first presentation are shown in Table 1.

Morphologic Subgroups

Patients were categorized into 1 of

Results

Follow-up data were complete for 59 of 60 patients. One patient returned to his country of origin after definitive repair was achieved. For the total study population, median follow-up time from the date of first hospital admission was 9.8 years (range, 2 days–25.9 years). For surviving patients with complete follow-up, it was 11.2 years (range, 9 months–25.9 years).

Comment

For the total series of 60 patients, estimated overall survival at 1, 5, and 15 years after initial admission was 88.3%, 86.5%, and 86.5%, respectively. This is congruent with other studies that reported most patients with PAIVS die within the first years of life 2, 3, 7, 10. All deaths except 1 in our series were early postprocedure deaths. The last death occurred in the year 2000, suggesting improved surgical or perioperative treatment, or both. Variable survival rates have been reported in

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