Surgical strategies for the management of end‐stage heart failure in infants and children: A 15‐year experience with a patient‐tailored approach

Abstract

End‐stage heart failure (ESHF) in pediatric age is an ongoing challenge. Heart transplantation is the final option, but its long‐term outcomes are still suboptimal in children. An alternative patient‐tailored surgical protocol to manage ESHF in children is described. Retrospective, single‐center analysis of pediatric patients admitted to our institution between April 2004 and February 2021 for ESHF. Our current protocol is as follows: (a) Patients <1 year with isolated left ventricular dysfunction due to dilated cardiomyopathy underwent pulmonary artery banding (PAB). (b) Patients <10 years and <20 kg, who did not meet previous criteria were managed with Berlin Heart EXCOR. (c) Patients >10 years or >20 kg, underwent placement of intracorporeal Heartware. Primary outcomes were survival, transplant incidence, and postoperative adverse events. A total of 24 patients (mean age 5.3 ± 5.9 years) underwent 26 procedures: PAB in 6 patients, Berlin Heart in 11, and Heartware in 7. Two patients shifted from PAB to Berlin Heart. Overall survival at 1‐year follow‐up and 5‐year follow‐up was 78.7% (95%CI = 62%‐95.4%) and 74.1% (95%CI = 56.1%‐92.1%), respectively. Berlin Heart was adopted in higher‐risk settings showing inferior outcomes, whereas a PAB enabled 67% of patients to avoid transplantation, with no mortality. An integrated, patient‐tailored surgical strategy, comprehensive of PAB and different types of ventricular assist devices, can provide satisfactory medium‐term results for bridging to transplant or recovery. The early postoperative period is critical and requires strict clinical vigilance. Selected infants can benefit from PAB that has demonstrated to be a safe bridge to recovery.

Pulmonary Artery Banding for Dilated Cardiomyopathy in Children: Returning to the Bench from Bedside

“…In this view, it is not surprising that a supposed regenerative strategy (PAB) that relies on the residual repair potential of the native heart and organism would be less beneficial when performed “too late”. Moreover, LV noncompaction, which we found as a possible contributing factor to PAB failure in our experience [ 31 ], was unusually frequent in the US cohort (5/14 patients, 39% vs. 8/61 cases, 13% in the world network report [ 29 ]), revealing the presence of a structural detrimental substrate, traditionally associated with relevant irreversible myocardial fibrosis [ 35 ], in a consistent quote of patients.…”

“…After fifteen years from the first case report of the successful application of PAB in a 2-month-old baby with DCM [ 25 ], less than 20 centers worldwide have published their clinical results [ 28 , 29 , 30 , 31 ]. From these findings, what we have learned is that PAB has a concrete potential to improve LV function in 30% to 80% of treated patients, when RV function is preserved.…”

“…Since 2015, we have embarked on a surgical protocol alternative to HTX to treat pediatric end-stage HF [ 31 ], including PAB. So far, we have treated with PAB a total of eight patients affected by DCM (four males, 50%) at a median age of 8 (5–10) months.…”

“…So far, we have treated with PAB a total of eight patients affected by DCM (four males, 50%) at a median age of 8 (5–10) months. In two cases (25%), the PAB strategy failed, and both patients required MCS with the Berlin Heart, followed by a successful HTX [ 31 ]. In one case (with a diagnosis of LV noncompaction and a known heterozygous mutation of the TPM1 gene and the ABCC9 gene), the PAB strategy was initially successful, allowing home discharge, but 3 months after PAB, the patient developed severe RV failure after acute pneumonia.…”

“…In our experience, a possible cause of PAB failure is the age of patients [ 31 ]. Schranz et al suggested a 6-year-old age limit for PAB in DCM [ 55 ].…”