Outcomes of Patients Undergoing the Kawashima Procedure at an Early Age: A Single Center Experience.

BACKGROUND: Previous reports have demonstrated the safety and efficacy of performing early Kawashima procedure (KP). Despite this, more recent studies have shown that the average age at the time of operation remains greater than one year of age. We report our experience with performing KP at an earlier age than previously reported. METHODS: A retrospective review was completed of patients undergoing KP at a single center (January 2000 to June 2020). Clinical outcomes were examined. RESULTS: Initial palliation was performed in 11 out of 12 patients. Age and weight at time of KP were 8.25 months (6.4-9.7) and 7.7 kg (6.5-8.6). Intensive care unit stay was 3.16 days (1-12), overall length of stay was 9.5 days (3-22). There was one unplanned reoperation, and no mortality in the cohort. Discharge oxygen saturation was 88% (80%-98%). Seven patients developed pulmonary arteriovenous malformations (PAVMs) with six proceeding to hepatic vein incorporation (HVI). Interval time to development of PAVMs was 42.3 months (16-121). Four of the 12 patients were left with antegrade pulmonary blood flow (PBF) and three (75%) remain without PAVMs. Median follow-up was 10 years (1.5-22) with 11 of 12 (91.67%) survival for the cohort. CONCLUSIONS: The KP can be done at a younger age than previously reported with adequate early and late results. Most patients will go on to develop PAVMs and require HVI but leaving patients with some antegrade PBF is likely protective but will need further investigation to show definitive benefit.

Injection Medialization in Infants with Vocal Fold Immobility Improves Dysphagia.

BACKGROUND: Unilateral vocal fold immobility (VFI) is a known cause of morbidity amongst children following congenital heart surgery. Injection medialization (IM) provides medial distraction and improves glottic closure. Limited objective data is available for the effect of IM in young children (<2 years-old) with VFI. METHODS: Retrospective case series of infants <2 who underwent IM for VFI after congenital cardiac surgery. Primary outcome was objective reduced risk of aspiration based on Dysphagia and Outcome Severity Scores (DOSS) on Video swallow study (VFSS) performed prior to and within 4 weeks following IM. Secondary analysis included perioperative complications and number of children who were able to avoid NG or G tube placement. RESULTS: 17 children <2 years of age had unilateral VFI after congenital cardiac surgery and underwent IM. The median age at time of initial cardiac surgery was 6 days (IQR 3-7). There was no intraoperative or postoperative stridor or associated complications. All 17 patients had preoperative aspiration noted on VFSS. Average swallowing outcomes on VFSS improved after IM with an increase in DOSS score (preop score 3 (IQR 2-4) to postop score 6.5 (IQR 5-7) [P = 0.001]). At 2 months following IM, of the patients who had improvement in swallowing function, 50% (n = 6) were able to feed completely orally, 25% (n = 3) were fed orally with an NG wean, and 3 (25%) had a G tube placed. CONCLUSION: Initial results suggest that IM is safe and improves early objective swallowing outcomes in children <2 years old with VFI after congenital cardiac surgery. LEVEL OF EVIDENCE: IV Laryngoscope, 2024.

Encouraging results of blood conservation in neonatal open-heart surgery.

OBJECTIVE: To report early outcomes of blood conservation in neonatal open-heart surgery. METHODS: Ninety-nine patients undergoing neonatal open-heart surgery during the implementation of a blood conservation program between May 2021 and February 2023 were reviewed. Patients either received traditional blood management (blood prime, n = 43) or received blood conservation strategies (clear prime, n = 56). Baseline characteristics and outcomes were compared between groups. RESULTS: There was no difference in body weight (median, 3.2 kg vs 3.3 kg; P = .83), age at surgery (median, 5 days vs 5 days; P = .37), distribution of The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Categories categories or duration of cardiopulmonary bypass. Patients in the clear prime group had higher preoperative hematocrit (median, 41% vs 38%; P < .01), shorter postoperative mechanical ventilation time (median, 48 hours vs 92 hours; P = .02) and postoperative intensive care unit length of stay (median, 6 days vs 9 days; P < .01) than patients in the blood prime group. Fourteen patients (25%) in the clear prime group, including 1 Norwood patient, were discharged without any transfusion. Among patients within the clear prime group, hospitalizations without blood exposure were associated with higher preoperative hematocrit (median, 43% vs 40%; P = .02), shorter postoperative mechanical ventilation times (median, 22 hours vs 66 hours; P = .01) and shorter postoperative hospital stays (median, 10 days vs 15 days; P = .02). CONCLUSIONS: Bloodless surgery is possible in a significant proportion of neonates undergoing open-heart surgery, including the Norwood operation, even in the early stages of experience. Early clinical results are favorable but long-term follow-up and continued efforts are warranted to prove safety and reproducibility.

Ultrasound Screening After Cardiac Surgery Shows Vocal Fold Impairment and Predicts Aspiration.

INTRODUCTION: Vocal fold motion impairment (VFMI) is a known consequence after high-risk cardiac surgery. We implemented a universal laryngeal ultrasound (LUS) screening protocol for VFMI after the Norwood and aortic arch surgery. We hypothesized that LUS would accurately identify VFMI and predict postoperative aspiration. METHODS: We implemented a screening algorithm with LUS for patients undergoing high-risk cardiac surgery at a tertiary care pediatric hospital. Positively screened patients underwent flexible nasolaryngoscopy (FNL). Patients with an abnormal FNL underwent a video-fluoroscopic swallow study (VFSS). Patient demographics, length of stay, and swallowing outcomes were assessed. Two-tailed chi square and Wilcoxon rank sum tests were used to assess for differences. RESULTS: Sixty-seven patients underwent either Norwood or arch reconstruction over a 16-month period and underwent universal LUS. The average birth weight was 3.24 kg (SD 0.57). Of the 67 patients, VFMI was identified by LUS and 100% confirmed on FNL in 58.21% (n = 39/67) of patients. Aspiration and penetration on VFSS were higher in the group with VFMI as compared with those without VFMI (53.8% vs. 21.4%, p = 0.008). There was no difference in length of stay between patients who did not have a diagnosis of VFMI and those found to have VFMI (41.0 days vs 45.3 days p = 0.73). CONCLUSIONS: Universal LUS screening for patients following high-risk cardiac surgery may lead to earlier identification of postoperative VFMI and aspiration. Recognition of VFMI through this universal screening program could lead to earlier interventions and possibly improved swallowing outcomes. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:1939-1944, 2024.

Melody Valve in Aortic Position to Facilitate Mechanical Circulatory Support as a Bridge to Cardiac Transplantation.

We report a patient with congenital heart disease and end stage heart failure after interventions for congenital heart disease resulting in aortic regurgitation that could not be treated with mechanical support to bridge to transplantation. We used a Melody valve (Medtronic Inc, Minneapolis, MN) in aortic position to achieve aortic competence to allow use of a left ventricular assist device to bridge the patient successfully to cardiac transplantation.

Cerebral Thromboembolism From Ligated Blalock-Taussig Shunt in an Adult Patient.

A 26-year-old woman underwent tetralogy of Fallot repair and classic Blalock-Taussig shunt (BTS) ligation. On postoperative day 4 she developed severe left-sided hemiplegia due to cerebral infarction. Thrombi in the BTS stump, right brachiocephalic artery, and its branches were removed surgically and the BTS stump was resected. The patient gradually recovered from hemiplegia and is leading a normal life with moderate left hemiparesis and is in good functional status 4 years after the event. An aggressive approach in treating this complication was crucial to prevent further thrombosis and neurologic deficit progression in this patient. We recommend reducing the size of the BTS stump during definitive repair.

Prenatal diagnosis of left pulmonary artery-to-pulmonary vein fistula and its successful surgical repair in a neonate.

Pulmonary arteriovenous fistula is a rare disease. To the best of our knowledge, prenatal diagnosis of a fistula between the left pulmonary artery and the left pulmonary vein has not been described in the medical literature. We report a case of the prenatal diagnosis of a left pulmonary artery-to-pulmonary vein fistula, followed by successful neonatal surgical repair.

Side-to-side aorto-GoreTex central shunt warrants central shunt patency and pulmonary arteries growth.

BACKGROUND: Central shunts may be associated with a high rate of thrombosis and pulmonary artery distortion. METHODS: Between January 2000 and April 2010, 68 consecutive patients underwent side-to-side aorto-Gore-Tex central shunts (W.L. Gore & Associates, Flagstaff, AZ). Median age at surgery was 31 days (8 to 122). Cardiac morphologies were tetralogy of Fallot (33), pulmonary atresia with collateral dependent lung circulation (20), and other (15). Shunt sizes ranged from 3 to 6 mm. The procedure was performed on cardiopulmonary bypass in 43 patients (63%). RESULTS: Six patients died during hospital stay (9%) of low output syndrome (3), sepsis (2), and stroke (1). Only one shunt needed early redo. Follow-up was 100% complete. After a median follow-up of 236 days (116 to 340), there were 7 late deaths related to sudden death (3), sepsis (2), reoperation (1), and lack of growth of pulmonary arteries with a patent shunt (1). Repair was completed in 42 patients and still pending in 12. Only one patient needed a late redo shunt (221 days). A larger shunt was performed in 5 patients after a median of 139 days (130 to 258). In the 45 patients who had serial assessment of pulmonary arteries sizes, Nakata index increased from 83 ± 62 mm(2)/m(2) to 153 ± 83 mm(2)/m(2) over a median period of 227 days (146 to 330), with equal growth observed in both pulmonary arteries (p < 0.001). CONCLUSIONS: Central shunts can be performed in neonates and children with minimal risk of shunt occlusion. Side-to-side aorto-Gore-Tex anastomosis seems to reliably warrant shunt patency and harmonious growth of pulmonary arteries.

Outcomes of second-run extracorporeal life support in children: a single-institution experience.

BACKGROUND: After weaning from mechanical circulatory support with extracorporeal membrane oxygenation or ventricular assist devices, patients may recurrently deteriorate and require reinstitution of support. Potential benefits of this desperate strategy are not well documented. METHODS: We reviewed the hospital records of all patients in whom second-run mechanical circulatory support was instituted from May 1988 to August 2010. RESULTS: Second-run support was instigated in 26 (4.6%) of 567 patients who underwent short-term mechanical circulatory support. Underlying pathologies requiring support were cardiac in 20 patients (76.9%) and noncardiac in 6 patients (23.1%).The majority of patients were younger than 1 year old (73.1%, n=19). Fifteen patients (57.7%) survived second-run support, but only 7 patients (26.9%) survived to discharge from the hospital. After a median follow-up of 42.5 months (range, 16 to 66 months), 4 patients (15.4%) were alive, but 3 of them had various degrees of developmental delay. CONCLUSIONS: Selection of patients who can benefit from second-run support is a complex process with unpredictable results. Survival after second-run mechanical circulatory support in children is worse compared with single-run patients. Long-term prospects for survivors are so grim that this strategy should probably not be recommended.

Surgical repair of truncus arteriosus associated with interrupted aortic arch: long-term outcomes.

BACKGROUND: Truncus arteriosus (TA) with interrupted aortic arch (IAA) is a rare combination of complex anomalies. We report long-term follow-up after one-stage repair of TA associated with IAA. METHODS: From April 1985 to August 2007, 16 patients with TA associated with IAA underwent one-stage repair. RESULTS: There were 2 (12.5%) early deaths and no late deaths. Follow-up was 92.9% complete (13 of 14 patients), with 1 patient lost to follow-up. Median duration of follow-up was 18.2 years (range, 2.1 to 21.9 years). Functional status in all patients was good. Thirteen patients underwent 25 surgical reoperations and 5 interventional procedures (3 aortic arch balloon angioplasties and 2 pulmonary artery balloon angioplasties). Overall freedom from any reoperation was 69.2% at 1 month, 54.5% at 3 years, 30% at 5 years, 11.1% at 10 years, and 0% at 15 years after the initial operation. Freedom from aortic reoperation was 76.9% at 1 month, 72.7% at 3 years, 70% at 5 years, 66.7% at 10 years, and 57.1% at 15 years; while freedom from right ventricular-to-pulmonary artery conduit replacement was 84.6% at 1 month, 63.6% at 3 years, 40% at 5 years, 11.1% at 10 years, and 0% at 15 years. Finally, freedom from truncal valve reoperation was 100% at 5 years, 88.9% at 10 years, and 85.7% at 15 years. CONCLUSIONS: One-stage repair of TA-IAA can be undertaken with good long-term results. Despite a significant reoperation rate, patients' functional status remains good at the long-term follow-up.