What is the role of apical ventriculotomy in children and young adults with hypertrophic cardiomyopathy?

Background: The transapical approach has been utilized in adult HCM patients with either mid-ventricular obstruction or a small LV cavity; however, there are little data on its use in children. Methods: We retrospectively reviewed all patients (age < 21 years) with HCM who underwent a transapical myectomy from January 2002 to December 2016. Indication for surgery was mid-ventricular obstruction in 19/23 (83%) and small LV cavity in 4 (17%). Preoperative symptoms included: dyspnea (96%), chest pain (65%), presyncope (61%), and syncope (35%). The mean age at the time of operation was 14 +/- 4.0 years (range, 4-20). Results: Overall, 23 patients (12 males) underwent transapical myectomy. A concomitant transaortic approach was performed in 16/19 (84%) with obstruction. The intraventricular gradient decreased from 71 mm Hg (IQR 44-92 mm Hg) preoperatively to 18 mm Hg (IQR 8-34 mm Hg, P<.0001) after myectomy. In patients with a small LV cavity, the mean left ventricular end diastolic dimension (LVEDD) increased from 40 +/- 3 mm to 46 +/- 3 mm (P=.05) after myectomy. There were no early deaths. Postoperative morbidity included complete heart block in 3 patients, 2 of which required pacemakers. Median follow up was 3.5 years (IQR 1.6-5.6). Symptoms improved in 95% of patients; the number of patients in NYHA class 3 or 4 heart failure decreased from 10/23 (43%) preoperatively to 3/23 (13%) postoperatively (P<.0001). Overall survival at 5 years postsurgery was 100%. Transplant-free survival was 91% and 87% at 1 and 5 years, respectively. Conclusion: In children with HCM, transapical myectomy is an effective adjunct to a transaortic approach to abolish midventricular obstruction and it effectively increases LV stroke volume in patients with small LV cavities and nonobstructive HCM. It may be beneficial for these patients with significant symptoms and who have failed medical therapy as a treatment alternative to cardiac transplantation.