Long-term follow-up and outcomes of discrete subaortic stenosis resection in children.

BACKGROUND: Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution. MATERIALS AND METHODS: We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency. RESULTS: The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25-13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1-13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively (P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67-16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1-9.1 years) after the initial repair. Risk factors for reoperation were age <6 years at initial repair. Eighteen (66.66%, 18/27) patients had AI preoperatively and progression of AI occurred in 70.37% (19/27). This included 4 (22.22%, 4/18) patients who had worsening of their preoperative AI. Short valve-to-membrane distance was found to be prognostically unfavorable. One (3.7%, 1/27) patient had an iatrogenic ventricular septal defect, and 2 (7.4%, 2/27) patients had complete AV block following membrane resection. CONCLUSIONS: Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life.

Normal reference ranges for aortic diameters in preterm infants.

OBJECTIVE: To establish normal reference ranges and Z-scores for aortic diameters in preterm infants according to the body surface area and assess their correlation with body weight, body surface area, and gestational age. PATIENTS AND METHODS: In a prospective study, 268 preterm infants who fulfilled the criteria for inclusion were examined. Echocardiograms were performed to measure the ascending aorta, transverse aorta, and aortic isthmus diameters on 0 days to 6 days of life and at weekly intervals until the babies reached 36 weeks. Body surface area was divided into 13 groups from 0.07 m2 to 0.19 m2. RESULTS: The mean gestational age was 29.8 [± 2.38 standard deviation (SD)] weeks, ranging from 24 weeks to 35 weeks. The mean body weight was 1479 (± 413 SD) g, ranging from 588 g to 3380 g, and the mean body surface area was 0.13 m2, ranging from 0.07 m2 to 0.19 m2. All the aortic diameters correlated well with both body weight and body surface area. Reference ranges with the mean ±â€¯SD, range, and Z-scores were calculated for aortic diameters according to the body surface area. A significant gradual increase was observed in ascending aorta, transverse aorta, and aortic isthmus diameters with increasing body surface area. Overall, a progressive and significant increase in ascending aorta, transverse aorta, and aortic isthmus diameters was observed during the first 9 weeks of life. CONCLUSION: The ascending aorta, transverse aorta, and aortic isthmus diameters exhibited a significant correlation with the body surface area and body weight. This study provides reference data with Z-scores that can be used as a normal reference tool for the ascending aorta, transverse aorta, and aortic isthmus diameters for preterm infants based on the body surface area.