Gianluigi Perri

Transannular patching is a valid alternative for tetralogy of Fallot and complete atrioventricular septal defect repair

OBJECTIVE We report our experience with repair of tetralogy of Fallot associated with complete atrioventricular septal defect, addressing in particular the need for a pulmonary valve in the right ventricular outflow tract. METHODS Between 1992 and 2006, 33 children with tetralogy of Fallot and complete atrioventricular septal defect were admitted; 26 had Downs syndrome (79%). Thirty-two children had complete repair (18 primary, 14 staged); of the 15 who received initial palliation, 1 died before complete repair. Right ventricular outflow tract obstruction was relieved by transannular patch in 14 cases (42%), infundibular patch with preservation of the pulmonary valve in 7 (21%), and right ventricle-to-pulmonary artery conduit in 11 (33%). RESULTS There were no hospital deaths. Actuarial survival was 96% +/- 3.9% at 5 years and 85.9 +/- 1.1% at 10 years. Multivariate analysis showed that type of relief of right ventricular outflow tract obstruction did not influence survival (P = .16), nor did the choice to use a valved conduit (P = .82). Primary correction (P = .05) and lower weight at repair (P = .05) were associated with higher probability of survival. Mean follow-up was 69.3 +/- 5.9 months (range 0.2-282 months). There were 2 late deaths. Overall freedom from reoperation was 69% at 5 years and 38% at 10 years. Right ventricular outflow tract reconstruction without use of a valved conduit allowed a significantly higher freedom from reinterventions (P < .05). CONCLUSIONS Tetralogy of Fallot associated with complete atrioventricular septal defect can be corrected at low risk with favorable intermediate survival. Use of right ventricle-to-pulmonary artery conduit can be avoided in two thirds of patients with no impact on survival, possibly improving overall freedom from reintervention.

Is alternative cardiac surgery an option in adults with congenital heart disease referred for thoracic organ transplantation

OBJECTIVES We analysed the outcomes of adults with congenital heart disease (ACHD) referred for thoracic organ transplantation who underwent non-transplant cardiac surgery as an alternative management option. METHODS Adult patients with congenital heart disease assessed for heart or heart-lung transplant were identified from the departmental database. A retrospective analysis of the medical records, transplant assessment data and surgical notes was carried out. RESULTS One hundred and twenty-six patients were assessed between January 2000 and July 2011. Non-transplant cardiac surgery was performed in 14 (11%) patients. There were nine males with a median age of 37 years (range 21-42). The patients can be divided into four subgroups [left-sided lesions (n = 4), right-sided lesions (n = 3), systemic right ventricle (n = 5) and Fontan circulation (n = 2)]. Surgical procedures performed were: relief of systemic obstructive/regurgitant lesions ± endocardial fibroelastosis resection (n = 4, three pulmonary vascular resistance >6 Wood units), correction of right-sided regurgitant/stenotic lesions (n = 3), ventricular assist device for patients with a systemic right ventricle (n = 5) and re-fashioning of the Fontan pathway (n = 2). There were two early (5 and 30 days) and three late deaths (64, 232 and 374 days) with a 1-year mortality of 28%. None of the deaths occurred in patients with a two-ventricle circulation and atrio-ventricular concordance. Nine patients are alive at a median of 433 days (range 204-2456). The New York Heart Association class has improved in all survivors by at least one class at 3 and 6 months (P = 0.004 and 0.003). CONCLUSIONS Alternative cardiac surgery can be undertaken in selected patients with ACHD referred for cardiopulmonary transplantation with a low mortality in patients with two ventricles and a systemic left ventricle. Ventricular assist devices carry a significant mortality in patients with a systemic right ventricle, although this offers a valuable palliation when there are no other options. The medium and long-term results are awaited.

Mechanical circulatory support after paediatric heart transplantation

OBJECTIVES Mechanical circulatory support (MCS) may be required after orthotopic heart transplantation (OHTx) in children for the treatment of failure or rejection. We review the incidence and outcomes of post-transplant MCS in our institution. METHODS MCS was classified as early (<1 month since transplant) or late (>1 month since transplant) and the support offered was either veno-arterial extra-corporeal membrane oxygenation (VA-ECMO) or a ventricular assist device (VAD). From 2003 to the present, 100 children (<16 years) underwent OHTx. Fifteen (15%) had 17 episodes of MCS. MCS was instituted early in 10 and late in seven episodes. Two children required two episodes of support. VA-ECMO was used in 12 episodes (71%). Two children required VAD support alone (12%). In three (17%) episodes ECMO was subsequently converted from VAD. RESULTS Among 10 children with early failure, eight were successfully weaned from support with recovery of graft function. In the late failure group, three of six patients died. All but four patients underwent re-transplantation with no perioperative deaths. Overall survival to discharge was 66%. The early failure group shows a better survival rate to hospital discharge compared with the late failure group (78 vs 50%; P < 0.0001). CONCLUSIONS The incidence of post-transplant MCS for graft failure in our patients was 15%. Early graft failure has a better outcome than late failure. Re-transplantation has good mid-term outcomes in children. A stepwise approach with a multimodality MCS strategy improves survival in this group of patients.

A current approach to heart failure in Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is a genetic, progressive neuromuscular condition that is marked by the long-term muscle deterioration with significant implications of pulmonary and cardiac dysfunction. As such, end-stage heart failure (HF) in DMD is increasingly becoming the main cause of death in this population. The early detection of cardiomyopathy is often challenging, due to a long subclinical phase of ventricular dysfunction and difficulties in assessment of cardiovascular symptomatology in these patients who usually loose ambulation during the early adolescence. However, an early diagnosis of cardiovascular disease in patients with DMD is decisive since it allows a timely initiation of cardioprotective therapies that can mitigate HF symptoms and delay detrimental heart muscle remodelling. Echocardiography and ECG are standardly used for screening and detection of cardiovascular abnormalities in these patients, although these tools are not always adequate to detect an early, clinically asymptomatic phases of disease progression. In this regard, cardiovascular magnetic resonance (CMR) with late gadolinium enhancement is emerging as a promising method for the detection of early cardiac involvement in patients with DMD. The early detection of cardiac dysfunction allows the therapeutic institution of various classes of drugs such as corticosteroids, beta-blockers, ACE inhibitors, antimineralocorticoid diuretics and novel pharmacological and surgical solutions in the multimodal and multidisciplinary care for this group of patients. This review will focus on these challenges and available options for HF in patients with DMD.

Successful Pediatric Orthotopic Heart Transplantation After Three Runs of Mechanical Circulatory Support

Mechanical circulatory support has become a well-established therapy to bridge patients with intractable heart failure to either heart transplantation or recovery. We report our experience of a child with a diagnosis of unexplained familial dilated cardiomyopathy, who presented with cyclical episodes of severe life-threatening heart failure treated with 3 different runs of MCS between August 2008 and May 2011.

Surgical repair of an unusual type of supra-cardiac total anomalous pulmonary venous connection to the superior vena cava.

Abstract  Anomalies of the pulmonary venous drainage vary widely in their anatomic spectrum and clinical presentation. We describe an unusual case of supra‐cardiac total anomalous pulmonary venous connection (TAPVC), where the pulmonary veins drained directly in the posterior aspect of proximal right superior vena cava (SVC) through separate ostia. The veins were re‐routed with a patch to the left atrium via the secundum atrial septal defect (ASD). The continuity between distal SVC and right atrium was re‐established by re‐implanting the SVC to the right atrial appendage (Warden Procedure). (J Card Surg 2012;27:384‐386)

Mechanical Circulatory Support for Single Ventricle Failure

Mechanical circulatory support (MCS) for failing single ventricle (SV) physiology is a complex and challenging problem, which has not yet been satisfactorily addressed. Advancements in surgical strategies and techniques along with intensive care management have substantially improved the outcomes of neonatal palliation for SV physiology, particularly for hypoplastic left heart syndrome (HLHS). This is associated with a steady increase in the number of SV patients who are susceptible to develop heart failure (HF) and would potentially require MCS at a certain stage in their palliation. We have reviewed the literature regarding the reported modalities of MCS use in the management of SV patients. This includes analysis of various devices and strategies used for failing circulation at distinct stages of the SV pathway: after neonatal palliation, after the superior cavo-pulmonary connection (SCPC), and after total cavo-pulmonary connection (TCPC).

Comment on: ‘Implantation of a left ventricular assist device to provide long term support for end-stage Duchenne muscular dystrophy-associated cardiomyopathy’ by Stoller et al.: Letter to the Editor

We read with great interest the case reported by Stoller and colleagues, regarding the implantation of a centrifugal continuous left ventricular assist device (LVAD) as a long-term support in a patient with Duchenne muscular dystrophy (DMD) presented with cardiogenic shock. The authors addressed an important issue: despite increased awareness among clinicians, heart failure (HF) is increasingly becoming the leading cause of death in DMD patients. Unfortunately, none of the available therapeutic options address the progressive deterioration of ventricular performance. Moreover, DMD has traditionally been considered as a relative contraindication for cardiac transplantation. A possible treatment for end-stage HF in these patients is the use of LVAD as a destination therapy (DT). Nevertheless, caution has to be exercised since the current International Society for Heart & Lung Transplantation (ISHLT) indication, conceived for chronic old patients, excludes patients with important disability. Therefore, the frail status in DMD patients is an important point to be addressed and discussed with every single patient, especially when defining the strategy in accordance with the family’s and caregivers’ expectations. Our group reported in 2012 the first two cases of successful LVAD implantation as DT in adolescents with DMD, using the Jarvik 2000 (Jarvik Heart, Inc, New York), a thumb-sized axial flow impeller pump. Since that time, we have treated seven DMD patients. All patients survived to hospital discharge: this represents the most extensive cohort of patients available. At a median follow-up time of 21.7 months (range, 3–45 months), there have been three deaths: one patient died of a lung infection after 45 months, one died of tracheal bleeding after 29 months, and one died of cerebral haemorrhage after 14 months. Remarkably, a patient survived at a follow-up of 1825 days: to the best of our knowledge, this is the longest follow-up available for a patient with an LVAD affected by DMD. Our current data are consistent with those presented by Stoller et al. and also by other groups, confirming the feasibility of using LVAD as DT in DMD subjects with HF. A multidisciplinary approach with careful evaluation of frailty and co-morbidities is crucial to assess the proper selection of DMD patients. Actually, a shared decision process is needed to obtain a collaborative contact with patient, parents, and caregivers, making this strategy successful. We strongly believe that DMD patients deserve LVAD as DT, and so we encourage other groups to consider this therapeutic strategy and share it with patients, family, and all caregivers as a part of palliative strategy.

Continuous-Flow Pumps in Infants, Jarvik Infant System, and Destination Therapy in Pediatrics

The need for long-term mechanical support in the pediatric population has been recognized for many years. Annually, about 300–500 pediatric heart transplants are performed worldwide, but many more could be done if donors were available. The US National Institutes of Health (NIH) estimates that in the USA there are ~1800 infant deaths from congenital heart defects annually and several hundred additional pediatric patients under 5 years old who develop heart failure. Left ventricular assist devices (LVADs) in pediatrics are frequently implanted as a bridge to transplantation (BTT) and rarely for recovery or as destination therapy (DT). The number of pediatric patients suffering from end-stage heart failure is continuously increasing, and assisting smallest ones in a long-term strategy remains an unsolved problem. This is mainly because today only one-labeled VAD is available for neonates and infants, namely, the Berlin Heart EXCOR (BHE) that is a paracorporeal pulsatile pneumatic-driven system. This has substantially limited the system’s portability which has been a barrier to hospital discharge. BHE is a second-generation device, and neurological complications vary between 25 and 30% with a survival rate approaching 80% [1–4].

Concomitant pulsatile and continuous flow VAD in biventricular and univentricular physiology: a comparison study with a numerical model

Introduction To develop and test a lumped parameter model to simulate and compare the effects of the simultaneous use of continuous flow (CF) and pulsatile flow (PF) ventricular assist devices (VADs) to assist biventricular circulation vs. single ventricle circulation in pediatrics. Methods Baseline data of 5 patients with biventricular circulation eligible for LVAD and of 5 patients with Fontan physiology were retrospectively collected and used to simulate patient baselines. Then, for each patient the following simulations were performed: (a) CF VAD to assist the left ventricle (single ventricle) + a PF VAD to assist the right ventricle (cavo-pulmonary connection) (LCF + RPF); (b) PF VAD to assist the left ventricle (single ventricle) + a CF VAD to assist the right ventricle (cavo-pulmonary connection) (RCF + LPF) Results In biventricular circulation, the following results were found: cardiac output (17% RCF + LPF, 21% LCF + RPF), artero-ventricular coupling (-36% for the left ventricle and -21.6% for the right ventricle), pulsatility index (+6.4% RCF + LPF, p = 0.02; -8.5% LCF + RPF, p = 0.00009). Right (left) atrial pressure and right (left) ventricular volumes are decreased by the RCF + LPF (by RPF + LCF). Pulmonary arterial pressure decreases in the LCF + RPF configuration. In Fontan physiology: cardiac output (LCF + RPF 35% vs. 8% in RCF + LPF), ventricular preload (+4% RCF + LPF, -10% LCF + RPF), Fontan conduit pressure (-5% RCF + LPF, +7% LCF + RPF), artero-ventricular coupling (-14% RCF + LPF vs. -41% LCF + RPF) and pulsatility (+13% RCF + LPF, - 8% LCF + RPF). Conclusions A numerical model supports clinicians in defining and innovating the VAD implantation strategy to maximize the hemodynamic benefits. Results suggest that the hemodynamic benefits are maximized by the LCF + RPF configuration.