@Article{CHD.2020.011579,
AUTHOR = {Norihisa Toh, Yasuhiro Kotani, Teiji Akagi, Yosuke Kuroko, Kenji Baba, Shin-ichi Otsuki, Shingo Kasahara, Hiroshi Ito},
TITLE = {Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum  Reaching Adulthood},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {15},
YEAR = {2020},
NUMBER = {1},
PAGES = {1--11},
URL = {http://www.techscience.com/schd/v15n1/39373},
ISSN = {3071-1738},
ABSTRACT = {<b>Background:</b> There is limited information on outcomes of adult 
patients with pulmonary atresia with intact ventricular septum (PA-IVS) due to the 
low incidence of disease and the large variation of surgical histories. <b>Methods:</b>
Among 58 patients with repaired PA-IVS, a total of 32 patients aged ≥16 years 
and who were followed at our institution between January 2003 and December 
2018 were reviewed. Surgical history, clinical outcomes, and laboratory, 
echocardiographic and electrocardiographic data were obtained by chart review. 
<b>Results:</b> Follow-up was from the age of 16 years and the median age at the latest 
follow-up was 23.7 years. Twenty-four patients had undergone biventricular repair 
(BVR), 3 had undergone one-and-a half ventricular repair (1.5VR), and 5 had 
undergone univentricular repair. Over a median follow-up period of 7.7 years 
(interquartile range: 4.1–11.0 years), 1 BVR patient died suddenly and 7 patients 
had heart failure. Arrhythmias were present in 5 patients. Ten patients underwent 
surgical re-interventions, including 4 BVR take-downs with conversion to 1.5VR 
and 3 Fontan conversions. Overall survival, heart failure-free, arrhythmia-free, and 
surgical re-intervention-free rates at 5 years and 10 years from the age of 16 years 
were 96.2% (95% confidence interval [CI], 77.2–99.4) and 96.2% (95% CI, 77.2–
99.4), 81.4% (95% CI, 62.1–92.1) and 74.6% (95%CI, 52.3–88.7), 88.7% (95% 
CI, 70.1–96.3) and 75.9% (95% CI, 51.7–90.2), and 80.7% (95% CI, 60.8–91.8) 
and 70.8% (95% CI, 49.7–85.7), respectively. <b>Conclusion:</b> Adults with PA-IVS 
have preserved long-term survival regardless of the early operative strategy, while 
they are at risk for heart failure, arrhythmia, and surgical re-intervention. Thus, 
detailed and continued follow-up is mandatory for all PA-IVS patients from 
childhood to adulthood.},
DOI = {10.32604/CHD.2020.011579}
}