OBJECTIVES
The purpose of this study was to review the outcomes of surgical treatment in patients with aorto-left ventricular tunnel and to investigate what kind of patient cohort is more likely to have adverse events.
METHODS
Twenty-one patients with a median age of 6.58 [interquartile range (IQR) 4.17–24.50] years who received surgical treatment of aorto-left ventricular tunnel from March 2002 to December 2019 were reviewed. The median follow-up time was 64.50 (IQR 25.15–120.50) months. Clinical characteristics, surgical methods and follow-up outcomes were summarized in separate groups of patients with or without preoperative aortic valve (AoV) issues. Composite adverse events were defined as death or requirement of reoperation. Time-related analysis of freedom from death and requirement of reoperation was performed with the Kaplan–Meier method.
RESULTS
The average tunnel size was 8.68 (standard deviation: 3.62) mm. The most common and the most important associated lesions were AoV lesions. Tunnels in 20 patients were closed with direct sutures or a patch. For 1 patient with an irreparable AoV, the tunnel was cut open simultaneously with aortic valve replacement and aortic root plasty. In the group of patients with preoperative AoV issues, 4 patients received aortic valve replacement with mechanical prosthetic valves and 6 patients received aortic valvuloplasty repair. The follow-up outcomes differed significantly between groups (the incidence of death was 15.38% and the incidence of requiring a reoperation was 46.15% in patients with preoperative AoV issues). In the group without preoperative AoV issues, there were no deaths and no reoperations (Fisher’s exact test; P = 0.018). The probability of freedom from death and of the requirement for reoperation between the 2 groups was not significantly different (log-rank, P = 0.09). Overall, the estimated probability of freedom from death and requirement of reoperation was 77.30% (standard error: 10.20%) [95% confidence interval (CI): 49.53–91.00] at 5 years, 67.64% (standard error: 12.70%) (95% CI: 36.71–85.84) at 10 years.
CONCLUSIONS
Patients with aorto-left ventricular tunnel with preoperative AoV issues are more prone to die or to require a reoperation. In contrast, patients without preoperative AoV issues can be free from death or reoperation for a longer period of time. Patients with preoperative AoV issues need much stricter postoperative long-term echocardiographic follow-up.
Conjoined twins (CT) are rare with possible serious malformations in which soft tissue, bone, or some organs are joined in utero. The extent of cardiac fusion and intracardiac anatomy of CT determine the viability, natural history, and outcome of potential surgical intervention. Early prenatal diagnosis and assessment may provide a window of opportunity to counsel the family for their informed decision on the pregnancy and to plan for prenatal and perinatal care. In this report, we describe a case of thoracopagus twins diagnosed by fetal echocardiography at 23-week gestation. The 2 hearts fused at the atrial and ventricular levels. The outcome and review of literature on fetal echocardiographic characteristics of this malformation are discussed.
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