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Aortopulmonary window: factors associated with early and late success after surgical treatment
J A van Son1, F J Puga, G K Danielson
1Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905.
Insights
Surgical correction of aortopulmonary window is effective, but early intervention is crucial. High pulmonary vascular resistance increases perioperative mortality risk, necessitating careful patient selection.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Thoracic Surgery
Background:
- Aortopulmonary window is a rare congenital heart defect.
- Surgical correction is the standard treatment.
- Outcomes can be affected by associated anomalies and pulmonary vascular resistance.
Purpose of the Study:
- To evaluate the long-term outcomes of surgical correction for aortopulmonary window.
- To identify risk factors associated with perioperative mortality.
- To establish optimal timing and surgical approaches for aortopulmonary window repair.
Main Methods:
- Retrospective review of 19 patients who underwent surgical correction of aortopulmonary window between 1953 and 1990.
- Analysis of patient demographics, associated cardiac anomalies, surgical techniques (division and closure vs. patch closure), and perioperative outcomes.
- Statistical analysis of risk factors including year of operation, surgical approach, and pulmonary to systemic vascular resistance ratio (Rp/Rs).
Main Results:
- Four perioperative deaths (21%) occurred, all in patients operated on before 1962 or with high pulmonary vascular resistance (Rp ≥ 7.3 U.m2, Rp/Rs > 0.6).
- Early year of operation, division of the aortopulmonary window, and high Rp/Rs were significantly associated with perioperative death.
- Patients with low Rp (< 8 U.m2), low Rp/Rs (< 0.4), and no major associated anomalies achieved excellent long-term functional outcomes (NYHA Class I).
Conclusions:
- Early surgical correction of aortopulmonary window is recommended to prevent irreversible pulmonary vascular changes.
- Transaortic or transpulmonary patch closure may be associated with better outcomes than division and primary closure.
- Patients with high Rp/Rs require thorough assessment for operability due to increased perioperative mortality risk.
Abstract:
Between 1953 and 1990, 19 patients, who were from 7 weeks to 27 years old, underwent surgical correction of aortopulmonary window at the Mayo Clinic. Associated cardiac anomalies were present in nine patients (47%). At operation, extracorporeal circulation was used in all except one patient. In seven patients, division and primary closure were done. In four patients, the defect was closed by direct suture through a transpulmonary approach. In eight patients, the defect was closed with a patch through a transpulmonary or transaortic approach. Four deaths (21%) occurred intraoperatively or immediately postoperatively. All four patients had undergone division of the aortopulmonary window before 1962, and three of them had a pulmonary vascular resistance (Rp) that was 7.3 U.m2 or more and a ratio of Rp to systemic vascular resistance (Rp/Rs) that exceeded 0.6. One patient with an Rp of 11.8 U.m2 and an Rp/Rs of 0.72 died 16 years postoperatively. Statistical analysis of risk factors indicated that early year of operation (P = 0.022), division of the aortopulmonary window versus transaortic or transpulmonary closure (P = 0.009), and a high Rp/Rs (P = 0.021) were significantly associated with perioperative death. All patients with a preoperative Rp of 8 U.m2 or less, an Rp/Rs of less than 0.4, and no major associated cardiac anomalies were in functional class I (New York Heart Association) postoperatively. Our study confirms that infants with aortopulmonary window should undergo operation early, before irreversible pulmonary vascular changes have developed. Patients with an Rp/Rs that exceeds 0.4 should be thoroughly assessed to determine their operability.