Juliano Gomes Penha

Post-cardiotomy ECMO in pediatric and congenital heart surgery: impact of team training and equipment in the results.

Introduction Post-cardiotomy myocardial dysfunction requiring mechanical circulatory support occurs in about 0.5% of cases. In our environment, the use of extracorporeal membrane oxygenation has been increasing in recent years. Objective To evaluate the impact of investment in professional training and improvement of equipment in the rate of weaning from extracorporeal membrane oxygenation and survival. Methods A retrospective study. Fifty-six pediatric and/or congenital heart patients underwent post-cardiotomy extracorporeal membrane oxygenation at our institution between November 1999 and July 2014. We divided this period into two phases: phase I, 36 cases (before the structuring of the extracorporeal membrane oxygenation program) and phase II, 20 cases (after the extracorporeal membrane oxygenation program implementation) with investment in training and equipment). Were considered as primary outcomes: extracorporeal membrane oxygenation weaning and survival to hospital discharge. The results in both phases were compared using Chi-square test. To identify the impact of the different variables we used binary logistic regression analysis. Results Groups were comparable. In phase I, 9 patients (25%) were weaned from extracorporeal membrane oxygenation, but only 2 (5.5%) were discharged. In phase II, extracorporeal membrane oxygenation was used in 20 patients, weaning was possible in 17 (85%), with 9 (45%) hospital discharges (P<0.01). When the impact of several variables on discharge and weaning of extracorporeal membrane oxygenation was analyzed, we observe that phase II was an independent predictor of better results (P<0.001) and need for left cavities drainage was associated with worse survival (P=0.045). Conclusion The investment in professional training and improvement of equipment significantly increased extracorporeal membrane oxygenation results.

Use of Short-term Circulatory Support as a Bridge in Pediatric Heart Transplantation

Background Heart transplantation is considered the gold standard therapy for the advanced heart failure, but donor shortage, especially in pediatric patients, is the main limitation for this procedure, so most sick patients die while waiting for the procedure. Objective To evaluate the use of short-term circulatory support as a bridge to transplantation in end-stage cardiomyopathy. Methods Retrospective clinical study. Between January 2011 and December 2013, 40 patients with cardiomyopathy were admitted in our Pediatric Intensive Care Unit, with a mean age of 4.5 years. Twenty patients evolved during hospitalization with clinical deterioration and were classified as Intermacs 1 and 2. One patient died within 24 hours and 19 could be stabilized and were listed. They were divided into 2 groups: A, clinical support alone and B, implantation of short-term circulatory support as bridge to transplantation additionally to clinical therapy. Results We used short-term mechanical circulatory support as a bridge to transplantation in 9. In group A (n=10), eight died waiting and 2 patients (20%) were transplanted, but none was discharged. In group B (n=9), 6 patients (66.7%) were transplanted and three were discharged.The mean support time was 21,8 days (6 to 984h). The mean transplant waiting list time was 33,8 days. Renal failure and sepsis were the main complication and causeof death in group A while neurologic complications were more prevalent en group B. Conclusion Mechanical circulatory support increases survival on the pediatric heart transplantation waiting list in patients classified as Intermacs 1 and 2.

The Impact of Tacrolimus as Rescue Therapy in Children Using a Double Immunosuppressive Regimen After Heart Transplantation

BACKGROUND Organ transplant recipients with refractory rejection or intolerance to the prescribed immunosuppressant may respond to rescue therapy with tacrolimus. We sought to evaluate the clinical outcomes of children undergoing heart transplantation who required conversion from a cyclosporine-based, steroid-free therapy to a tacrolimus-based regimen. METHODS We performed a prospective, observational, cohort study of 28 children who underwent conversion from cyclosporine-based, steroid-free therapy to a tacrolimus-based therapy for refractory or late rejection or intolerance to cyclosporine. RESULTS There was complete resolution of refractory rejection episodes and adverse side effects in all patients. The incidence rate (×100) of rejection episodes before and after conversion was 7.98 and 2.11, respectively (P ≤ .0001). There was a 25% mortality rate in patients using tacrolimus after a mean period of 60 months after conversion. CONCLUSION Tacrolimus is effective as rescue therapy for refractory rejection and is a therapeutic option for pediatric patients.

Complicação pós-operatória de Fontan: fluxo pulmonar anterógrado

1. Faculty Member of FMUSP; Medical Assistant - Pediatric CardiacSurgery at the Heart Institute, Clinical Hospital at the Universityof Sao Paulo Medical School.2. Medical Assistant - Postoperative Recovery at the Heart Institute– FMUSP; Medical Assistant - Postoperative Recovery at theHeart Institute – HCFMUSP.3. Physician Assistant - Pediatric Cardiac Surgery at the HeartInstitute - FMUSP Medical Assistant - Pediatric Cardiac Surgeryat the Heart Institute – HCFMUSP.4. Full Professor of Pediatric Cardiac Surgery, University of SaoPaulo; Full Professor of Pediatric Cardiac Surgery at the Universityof Sao Paulo and Coordinator of the Pediatric Heart Transplant.

Lessons Learned From a Critical Analysis of the Fontan Operation Over Three Decades in a Single Institution

Background: The Fontan operation (FO) has evolved and many centers have demonstrated improved results relative to those from earlier eras. We report a single-institution experience over three decades, describing the outcomes and evaluating risk factors. Methods: Successive patients undergoing primary FO were divided into era I (1984-1994), era II (1995-2004), and era III (2005-2014). Clinical and operative notes were reviewed for demographic, anatomic, and procedure details. End points included early and late mortality and a composite of death, heart transplantation (HTX), or Fontan takedown. Results: A total of 420 patients underwent 18 atriopulmonary connections, 82 lateral tunnels (LT), and 320 extracardiac conduit (EC) Fontan procedures. Forty-six (11%) patients died; early and late mortality were 7.9% and 3.1%, respectively. Eight (1.9%) patients underwent HTX, 11 (2.6%) underwent Fontan conversion to EC, and 1 (0.2%) takedown of EC to bidirectional Glenn shunt. Prevalence of concomitant valve surgery (P < .001) and pulmonary artery reconstruction (P < .001) differed over the eras. Preoperative valve regurgitation was associated with likelihood of early mortality (odds ratio [OR] = 3.5, P = .002). Embolic events (OR = 1.9, P = .047), preoperative valve regurgitation (OR = 2.3, P = .029), diagnosis of unbalanced atrioventricular canal defect (OR = 1.14, P = .03), and concomitant valve replacement (OR = 6.9, P = .001) during the FO were associated with increased risk of the composite end point (death, HTX, or takedown). Conclusion: Technical modifications did not result in improved results across eras, due in part to more liberal indications for surgery in the recent years. Valve regurgitation, unbalanced atrioventricular canal, embolic events, or concomitant valve replacement were associated with FO failure.

Palliative Senning in the Treatment of Congenital Heart Disease with Severe Pulmonary Hypertension

Background Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level. Objective Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure. Methods Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included. Results After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced. Conclusion The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis.

Heart Transplantation in Pediatric Population and in Adults With Congenital Heart Disease: Long-term Follow-up, Critical Clinical Analysis, and Perspective for the Future

BACKGROUND Heart transplantation is a treatment option for children as well as for adults with congenital heart disease. OBJECTIVE To report the experience of a tertiary center with heart transplant program in pediatric population and in adults with congenital heart disease. PATIENTS AND METHODS The study consisted of the evaluation of pediatric as well as adult patients undergoing heart transplantation for congenital heart disease. We evaluated the following indication and complications such as renal dialysis, graft vascular disease, tumors and survival. RESULTS From October 1992 to November 2013, 134 patients had transplantation, and there were 139 transplantations and 5 retransplantations. The immunosuppression regimen is based on calcineurin inhibitors and cytostatic drugs. The type of heart disease indicated for transplantation was cardiomyopathies in 70% and congenital heart disease in 30%. Of these 134 patients, 85 patients were alive. Actuarial survival is 77.4%, 69.6%, 59.3% at 1, 5, and 10 years after transplantation. Three patients underwent renal transplantation, 1 patient is in renal dialysis, and 8.2% of patients had post-transplant lymphoproliferative disease. Two patients had retransplantation for graft vascular disease; 1 of them required a simultaneous kidney transplant and died 30 days after the procedure and 1 patient is clinically well 2 years after retransplantation. CONCLUSION Heart transplantation in children and in adults with congenital heart disease is a promising therapeutic option and enables long-term survival for these patients.

Introducing extracorporeal membrane oxygenation (ECMO) for healthcare professionals: the importance of a basic theoretical course

A multidisciplinary team is essential for a high quality ECMO service. However, few health professionals have any knowledge on ECMO and, in addition, there are discrepancies between the level of expertise among these professionals. Thus, we evaluated the impact of a basic theoretical ECMO course on the knowledge gain of different healthcare professionals.

Rational Use of Mechanical Circulatory Support as a Bridge to Pediatric and Congenital Heart Transplantation

Introduction Donor shortage and organ allocation is the main problem in pediatric heart transplant. Mechanical circulatory support is known to increase waiting list survival, but it is not routinely used in pediatric programs in Latin America. Methods All patients listed for heart transplant and supported by a mechanical circulatory support between January 2012 and March 2016 were included in this retrospective single-center study. The endpoints were mechanical circulatory support time, complications, heart transplant survival and discharge from the hospital. Results Twenty-nine patients from our waiting list were assessed. Twelve (45%) patients were initially supported by extracorporeal membrane oxygenation (ECMO) and a centrifugal pump was implanted in 17 (55%) patients. Five patients initially supported by ECMO were bridged to another device. One was bridged to a centrifugal pump and four were bridged to Berlin Heart Excor®. Among the 29 supported patients, 18 (62%) managed to have a heart transplant. Thirty-day survival period after heart transplant was 56% (10 patients). Median support duration was 12 days (interquartile range [IQR] 4 - 26 days) per run and the waiting time for heart transplant was 9.5 days (IQR 2.5-25 days). Acute kidney injury was identified as a mortality predictor (OR=22.6 [CI=1.04-494.6]; P=0.04). Conclusion Mechanical circulatory support was able to bridge most INTERMACS 1 and 2 pediatric patients to transplant with an acceptable complication rate. Acute renal failure increased mortality after mechanical circulatory support in our experience.

20-year experience with pediatric heart transplant in a developing country

Results The mean age at transplantation was 5.9 years (min=12 days, max=18 years). The preoperative diagnosis was cardiomyopathy in 79% and congenital heart disease in 21%. The surgical technique for all cases was orthotopic transplantation, having been used the biatrial method until 1996 and, since then, the bicaval method. There were four retransplantation, three due to hyperacute rejection and one due to graft vasculopathy. Survival was 84% during hospital stay, 81% in 1 year, 72% in 5 years, 62% in 10 years and 56% in 15 years. Among the postransplant comorbidities, recurrent lung infection was present in 70%, hypertension in 30%, graft vasculopathy in 25% and lymphoproliferative disease in 10%. During follow-up, one patient underwent renal transplantation 16 years after heart transplantation.