9 Long-term outcomes of adults with alcapa repair

Background Pulmonary artery left coronary artery anomaly (ALCAPA syndrome) is a rare congenital anomaly that can cause complications such as myocardial infarction, heart failure, death if left untreated, and it is unlikely that it will survive until becoming an adult There are only a few patients treated. . We are trying to determine the long-term outcomes of these patients in adult congenital heart disease services.

METHODS: Retrospective analysis of case records of ALCAPA patients, outpatient response reviews, surgical details, cardiac images and exercise tests

Results Eight patients (6 women) with an average age of 26.3 ± 6.2 years were identified. The average follow-up period was 1 year (range, 18 months to 12 years). All patients underwent surgical repair and one patient needed simultaneous mitral valve repair. The average age at the time of surgery was 7 years (range, 3 months to 34 years), and 3 patients (38%) underwent surgery as adults. In the last outpatient review, one patient developed symptoms of NYHA III and the rest of the patients worked well (NYHA I). The average peak oxygen consumption of the cardiopulmonary exercise test was 36 ± 2.4 ml / kg / min (33.4 to 38.8 ml / kg / min, predicted mean value 93% ± 15.9%, 78% to 112%) . All patients were sinus rhythm and no ischemia or arrhythmia was seen. Seven patients (88%) had good left ventricular function (mean EF 61%); one patient had a mild disorder (EF 50%) due to apical transmural infarct. Moderate mitral regurgitation was observed in 3 patients (38%), all patients had normal aortic root size

CONCLUSIONS: Long-term prognosis for patients with ALCAPA syndrome was described. After surgery, most patients remained asymptomatic and exercised well. Their left ventricular contraction function is good. Lifetime follow-up makes sense

Intravascular stent implantation is still a relatively new procedure, and the results have improved with other experiences. To determine which type of repair is best it requires a long-term direct randomized clinical trial. However, unless new data indicates a clear winner, individual decision making becomes very important. Men with conditions that may increase the risk of surgery may benefit from intravascular repair. On the other hand, some aneurysms are not suitable for endovascular repair. The skills and experience of medical teams and surgical teams available to patients can be balanced in this way or in other ways. If there are few statistical differences between the two options, the preference of older patients will be the guide for decision making. At present, more than 60% of all AAA restoration in the United States is done using intravascular technology.

Because AAA resembles a time bomb, doctors and patients are very nervous about them. Those who want to fix it before it breaks can understand, but things are not that simple. Even in the case of an intact and stable aneurysm, surgical repair is not a rapid modification and it is difficult to perform surgical repair. In most hospitals, the mortality rate of selective repair is 4-6%. This is a pretty dilemma, but there is another solution. The doctor is now able to determine the AAA with the highest risk of rupture. An important determinant is the size (see the figure below). As AAA gets bigger, the wall gets thinner and thinner just as the balloon becomes thinner when inflated. An important investigation shows an important role of AAA diameter