Single stage biventricular repair of hypoplastic right ventricle with straddling tricuspid valve.

We present the case of a five-year-old child with an inlet ventricular septal defect, subpulmonic stenosis, hypoplastic right ventricle, and straddling tricuspid valve who received a successful one-stage biventricular repair with right ventricular rehabilitation, right ventricular outflow tract augmentation, papillary muscle transposition, ventricular septal defect closure, and fenestrated atrial septation. This report outlines the surgical decision making and operative technique.

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

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.

Biventricular Conversion in the Borderline Hypoplastic Heart.

PURPOSE OF REVIEW: The development of biventricular repair and conversion pathways for patients with borderline hypoplastic heart disease represents an area of recent inquiry and innovation. This review summarizes emerging techniques and novel treatment algorithms for borderline hypoplastic heart disease with a focus on surgical advances within the last 10 years. RECENT FINDINGS: Many patients with borderline hypoplastic heart disease are amenable to primary biventricular repair, or biventricular conversion following single-ventricle palliation coupled with ventricular rehabilitation strategies. New insights into the potential for growth and recovery of borderline ventricles have been uncovered. However, questions remain regarding optimal patient selection and the long-term outcomes of select patient groups treated with single-ventricle palliation versus biventricular repair/conversion or transplantation. Efforts to direct a greater proportion of borderline hypoplastic heart patients towards a biventricular circulation are accelerating and represent important avenues for progress and future research in the field of congenital heart disease.

Small and borderline left ventricular outflow tract - a perplexing maladie.

The left ventricular outflow tract (LVOT) comprises of the subvalvular area, the aortic valve, and the supravalvular region. Obstructive lesion of LVOT is a spectrum with varying levels and degree of obstruction with or without associated hypoplasia of the left ventricle. Decision-making in small and borderline LVOT can be challenging. Imaging modalities such as echocardiography and magnetic resonance imaging and scores based on imaging aid in the decision making in truly borderline cases. Newer treatment strategies like staged left ventricular rehabilitation and hybrid procedure have come to the fore in the past decade or so. Although these do not address small LVOT per se, they delay the decision-making to a more appropriate age. The goal of management in these cases is to achieve a biventricular repair whenever feasible. Several surgical techniques could be employed to achieve this goal. However, it is important to be cognizant of the fact that an overzealous approach to achieve a biventricular repair might be counterproductive. A univentricular palliation could be a safer alternative; especially considering the possibility of a future transplant candidacy.

Staged Left Ventricular Recruitment and Biventricular Conversion in Hypoplastic Left Heart Syndrome.

We describe a relatively long left ventricular recruitment pathway consisting of early and serial aortic valvuloplasties and multiple endocardial fibroelastosis resections resulting in successful biventricular conversion of hypoplastic left heart syndrome.