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Clinical Outcomes of Patients with Pulmonary Agenesis
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  • Shouji Fukuoka,
  • Hazumu Nagata,
  • Kenichiro Yamamura,
  • Daisuke Toyomura,
  • Yusaku Nagatomo,
  • Yoshimi Eguchi,
  • Kiyoshi Uike,
  • Yuichiro Hirata,
  • Hirosuke Inoue,
  • Masayuki Ochiai,
  • Shouichi Ohga
Shouji Fukuoka
Kyushu Daigaku

Corresponding Author:[email protected]

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Hazumu Nagata
Kyushu Daigaku
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Kenichiro Yamamura
Kyushu Daigaku
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Daisuke Toyomura
Kyushu Daigaku
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Yusaku Nagatomo
Kyushu Daigaku
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Yoshimi Eguchi
Kyushu Daigaku
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Kiyoshi Uike
Kyushu Daigaku
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Yuichiro Hirata
Kyushu Daigaku
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Hirosuke Inoue
Kyushu Daigaku
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Masayuki Ochiai
Kyushu Daigaku
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Shouichi Ohga
Kyushu Daigaku
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Introduction: Pulmonary agenesis is a complete absence of the pulmonary parenchyma, bronchi beyond the bifurcation, and pulmonary vasculature unilaterally or bilaterally. Because of the rare occurrence, its pathophysiology and outcome remain elusive. We evaluate the clinical features and risk factors for mortality due to pulmonary agenesis. Methods: Two patients we experienced are presented as index cases. All reported cases of pulmonary agenesis were collected from online and publication databases between 1955 and 2020. We assessed the impact of comorbidity and intervention on the survival outcome. Results: We identified 230 patients—138 (60%) with right-sided, and 14 (6%) with bilateral agenesis—among 164 articles and our cases. There were 93 (40%) cardiovascular, 70 (30%) skeletal, and 48 (21%) gastrointestinal anomalies; 47 (20%) tracheal stenoses; and 33 (14%) genitourinary anomalies. Fifty-two (23%) patients had isolated pulmonary agenesis. The 2-year overall survival (OS) rate was 66% and there was no subsequent death until 13 years of age. The right agenesis was significantly associated with a lower 2-year OS rate (58% vs. 78%, p=0.019) or more frequent tracheal stenosis (28% vs. 12%, p=0.006) than left-sided disease. A multivariable analysis indicated that tracheal stenosis (hazard ratio [HR] 2.4, 95% confidence interval [CI] 1.5-4.1, p=0.004) and gastrointestinal anomaly (HR 1.9, 95% CI 1.1-3.3, p=0.018) were prognostic factors for mortality. Conclusions: The poor prognostic factors were tracheal stenosis, right agenesis and gastrointestinal anomaly. Tracheal and gastrointestinal controls are targeted at birth and in infancy for the improved survival of unilateral pulmonary agenesis.
02 Aug 2021Submitted to Pediatric Pulmonology
02 Aug 2021Submission Checks Completed
02 Aug 2021Assigned to Editor
25 Aug 2021Reviewer(s) Assigned
26 Sep 2021Review(s) Completed, Editorial Evaluation Pending
09 Oct 2021Editorial Decision: Revise Major
03 Jan 20221st Revision Received
04 Jan 2022Submission Checks Completed
04 Jan 2022Assigned to Editor
04 Jan 2022Reviewer(s) Assigned
07 Feb 2022Review(s) Completed, Editorial Evaluation Pending
07 Feb 2022Editorial Decision: Revise Minor
04 Mar 20222nd Revision Received
07 Mar 2022Submission Checks Completed
07 Mar 2022Assigned to Editor
07 Mar 2022Reviewer(s) Assigned
20 Mar 2022Review(s) Completed, Editorial Evaluation Pending
17 Apr 2022Editorial Decision: Revise Minor
05 May 20223rd Revision Received
05 May 2022Submission Checks Completed
05 May 2022Assigned to Editor
05 May 2022Reviewer(s) Assigned
23 May 2022Review(s) Completed, Editorial Evaluation Pending
11 Jun 2022Editorial Decision: Revise Minor
17 Jun 20224th Revision Received
05 Jul 2022Assigned to Editor
05 Jul 2022Submission Checks Completed
05 Jul 2022Reviewer(s) Assigned
16 Jul 2022Review(s) Completed, Editorial Evaluation Pending
29 Jul 2022Editorial Decision: Revise Minor
01 Aug 20225th Revision Received
02 Aug 2022Assigned to Editor
02 Aug 2022Submission Checks Completed
02 Aug 2022Reviewer(s) Assigned
15 Aug 2022Review(s) Completed, Editorial Evaluation Pending
28 Aug 2022Editorial Decision: Accept