Aortic Valve Repair in Pediatric Patients: 30 Years Single Center Experience.

BACKGROUND: Valve repair is the procedure of choice for congenital aortic valve disease. With increasing experience, the surgical armamentarium broadened from simple commissurotomy to more complex techniques. We report our 30-year experience with pediatric aortic valve repair. METHODS: A retrospective chart review of all patients aged less than 18 years who underwent aortic valve repair from May 1985 to April 2020 was conducted. Mortality was cross-checked with the national health insurance database (96% complete mortality follow-up in April 2020). Primary study endpoints were survival and incidence of reoperations. RESULTS: From May 1985 until April 2020, 126 patients underwent aortic valve repair at a median age of 1.8 years (interquartile range, 0.2-10). Early mortality was 5.6% (7 of 126). All early deaths occurred in neonates with critical aortic stenosis undergoing commissurotomy. No early deaths were observed after 2002. Kaplan-Meier estimated survival was 90.8% (95% CI, 84.0-94.8) at 10 years, 86.9% (95% CI, 78.7-92.2) at 20 years, and 83.5% (95% CI, 71.7-90.6) at 30 years. The cumulative incidence of aortic valve replacement was 37% (95% CI, 27.7-46.3) at 10 years, 62.2% (95% CI, 50.1-72.1) at 20 years, and 67.4% (51.2-79.2) at 30 years. Nine patients had undergone re-repair of the aortic valve. The majority of valve replacements were Ross procedures. CONCLUSIONS: Our results support a repair-first strategy for patients with congenital heart disease and underline that aortic valve reconstruction can be a successful long-term solution. Longevity did not differ between aortic valve commissurotomy and complex aortic valve reconstruction.

Long-term outcomes after surgical repair of subvalvular aortic stenosis in pediatric patients.

Objectives: Subvalvular aortic stenosis (SAS) can occur as discrete or tunnel-like obstruction of the left ventricular outflow tract and as progressive disease often leads to aortic valve regurgitation. We report our 30-year single-center experience after surgical repair of SAS. Methods: A retrospective chart review of all patients aged < 18 years, who underwent surgical repair of SAS from May 1985 to April 2020, was conducted. Mortality was cross-checked with the national health insurance database (93.8% complete mortality follow-up in April 2020). Survival and competing risks analysis were used to analyze the primary endpoints survival and incidence of reoperations. Results: From May 1985 until April 2020 103 patients (median age 5.5 years) underwent surgical repair of SAS. Survival was 90.8% at 10 years and 88.7% at 20 and 30 years. Age < 1 year at time of surgery, Shone's complex, mitral stenosis and concomitant mitral valve surgery were associated with mortality. The cumulative incidence of reoperation for SAS was 21.6% at 10 years, 28.2% at 20 and 30 years. The incidence of reoperation for SAS did not differ between the myectomy, membrane resection and combined myectomy and membrane resection groups. The cumulative incidence of reoperation on the aortic valve was 13.5% at 20 years. Conclusion: Recurrence rate of SAS is not to be neglected, though surgical repair of subaortic stenosis has good long-term results. Patients who needed a combined membrane resection and septal myectomy are not more prone to recurrence than patients who underwent solitaire myectomy or membrane resection.

First-in-man use of the EXCOR Venous Cannula for combined cavopulmonary and systemic ventricular support in Fontan circulation failure.

BACKGROUND AND AIM: The Berlin Heart EXCOR system has been developed for mechanical circulatory support (MCS) of pediatric patients with terminal heart failure. A recently introduced iteration of the system (EXCOR Venous Cannula, Berlin Heart GmbH, Berlin, Germany) is dedicated to support patients with univentricular physiologies by facilitating implantation of the EXCOR device into the Fontan pathway. CASE PRESENTATION: We report the worldwide first successful implantation of the EXCOR Venous Cannula in a biventricular support concept for a 12-year-old boy (140 cm, 42.7 kg, body surface area 1.29 m2, Pedimacs Level 2) with severe systemic ventricle dysfunction and failing Fontan circulation. Surgery comprised of standard Berlin Heart EXCOR implantation to support the failing ventricle (12 mm apex / staged 12/9 mm arterial cannula / 50 ml ventricle). Cannulation for subpulmonary EXCOR support was achieved by performing a total cavopulmonary connection takedown with subsequent anastomosis of a staged 12/9 mm outflow cannula to the pulmonary artery and implantation of a 14/18 mm EXCOR Venous Cannula as subpulmonary inflow graft, which was connected to the superior vena cava and Fontan tunnel using GORE-TEX grafts. In the postoperative course, cardiac output and central venous pressures rapidly improved with hepatic and renal functions restoring to age- and condition-specific norm values. CONCLUSION: The Berlin Heart EXCOR Venous Cannula is the first system for standardized mechanical support of Fontan circulatory failure. In our patient, subpulmonary support restoring a biventricular circulation combined with systemic MCS normalized hemodynamics and reversed end-organ dysfunction.

Aortic valve replacement in pediatric patients: 30 years single center experience.

BACKGROUND: The choice of aortic valve replacement needs to be decided in an interdisciplinary approach and together with the patients and their families regarding the need for re-operation and risks accompanying anticoagulation. We report long-term outcomes after different AVR options. METHODS: A chart review of patients aged < 18 years at time of surgery, who had undergone AVR from May 1985 until April 2020 was conducted. Contraindications for Ross procedure, which is performed since 1991 at the center were reviewed in the observed non-Ross AVR cohort. The study endpoints were compared between the mechanical AVR and the biological AVR cohort. RESULTS: From May 1985 to April 2020 fifty-five patients received sixty AVRs: 33 mechanical AVRs and 27 biological AVRs. In over half of the fifty-three AVRs performed after 1991 (58.5%; 31/53) a contraindication for Ross procedure was present. Early mortality was 5% (3/60). All early deaths occurred in patients aged < 1 year at time of surgery. Two late deaths occurred and survival was 94.5% ± 3.1% at 10 years and 86.4% ± 6.2% at 30 years. Freedom from aortic valve re-operation was higher (p < 0.001) in the mechanical AVR than in the biological AVR cohort with 95.2% ± 4.6% and 33.6% ± 13.4% freedom from re-operation at 10 years respectively. CONCLUSIONS: Re-operation was less frequent in the mechanical AVR cohort than in the biological AVR cohort. For mechanical AVR, the risk for thromboembolic and bleeding events was considerable with a composite linearized event rate per valve-year of 3.2%.

Long-term outcomes after the paediatric Ross and Ross-Konno procedures.

OBJECTIVES: The Ross procedure is an attractive option for the management of aortic valve disease in paediatric patients. We reviewed our experience with the paediatric Ross procedure to determine survival and freedom from reoperation in the third decade after surgery. METHODS: We reviewed the data of 124 paediatric patients [71% male, median age at time of surgery 11.1 years (interquartile range 6-14.8 years); 63.7% bicuspid aortic valve], who underwent the Ross procedure at 2 tertiary centres from April 1991 to April 2020. The Ross-Konno procedures were performed on 14 (11.3%) patients. Deaths were cross-checked with the national health insurance database, and survival status was available for 96.8% of the patients. The median follow-up time was 12.1 years (interquartile range 3-18 years). RESULTS: There were 3 early and 6 late deaths. All early deaths occurred in patients aged <1 year at the time of surgery. The 25-year survival was 90.3%. Actuarial freedom from reoperation (linearized rates in parentheses) was as follows: Autograft reoperation was 90.8% (0.48%/patient-year) and right ventricular outflow tract (RVOT) reoperation was 67% (2.07%/patient year) at 25 years. The univariable Cox-proportional hazard analysis revealed younger age at time of surgery (P < 0.001), smaller implanted valve size (P < 0.001) and the use of a xenograft rather than a homograft (P < 0.001) as predictors of RVOT reoperation. At multivariable Cox-proportional hazard analysis, only age was an independent risk factor for RVOT reoperation (P = 0.041). CONCLUSIONS: The Ross and the Ross-Konno procedures are associated with good outcomes in paediatric patients. Reoperation of the RVOT is frequent and associated with younger age.

Reversal of pulmonary hypertension in paediatric patients with restrictive cardiomyopathy.

Left ventricular assist devices can reverse pulmonary hypertension in cardiac transplant candidates with heart failure with a reduced ejection fraction. Whether a similar approach is applicable in restrictive cardiomyopathy is uncertain. We report the successful implantation of a Medtronic HVAD left ventricular assist device in a bridge-to-candidacy concept in 2 paediatric patients with restrictive cardiomyopathy.

Surgical stented transcatheter valve-in-valve implantation in atrioventricular position in children.

The Melody valve (Medtronic, Minneapolis, MN, USA) is a stented bovine jugular vein graft that was primarily approved for transcatheter implantation in a pulmonary valve position. The prosthetic valve can also be implanted in an atrioventricular position in infants and young children, and in these cases it must be modified appropriately.  In this tutorial we demonstrate the surgical preparation of a stented transcatheter Melody valve for implantation in the atrioventricular position. Additionally, we present a safe and effective method for surgical valve-in-valve implantation in a 3-year-old patient with hypoplastic left heart syndrome.

Implantation of a decellularized aortic homograft in a child.

The implantation of a decellularized aortic homograft in children and young adults has been shown to be a good alternative to existing surgical approaches. Lower risk of calcification and the potential of growth render a homograft a promising valve substitute. The child presented in this video tutorial is a 10-year-old boy diagnosed with congenital aortic stenosis which was treated by balloon valvuloplasty early in life. Current echocardiographic findings show severe aortic regurgitation and stenosis. The tutorial provides detailed insight into how to implant a decellularized aortic homograft as a total root replacement.