@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 = {Congenital Heart Disease},
VOLUME = {15},
YEAR = {2020},
NUMBER = {1},
PAGES = {1--11},
URL = {http://www.techscience.com/chd/v15n1/39373},
ISSN = {1747-0803},
ABSTRACT = {Background: 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. Methods:
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.
Results: 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. Conclusion: 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}
}