Prenatal Diagnosis of Single Ventricle Physiology Impacts on Cardiac Morbidity and Mortality.

We sought to evaluate the impact of prenatal diagnosis on morbidity and mortality in single ventricle (SV) lesions. All consecutive patients with pre- or postnatally diagnosed SV physiology admitted to our centre between January 2001 and June 2013 were reviewed. Primary endpoints included survival until 30 days after bidirectional cavopulmonary connection (BCPC) without transplant or BCPC takedown. Prenatal diagnosis was performed in 160 of 259 cases (62%). After excluding all cases with termination of pregnancy, intrauterine demise or treated with comfort care, a total of 180 neonates were admitted to our centre for treatment, including 87 with a prenatal and 93 with a postnatal diagnosis. Both groups showed similar distribution regarding diagnosis, dominant ventricle and risk factors such as restrictive foramen or some form of atrial isomerism. A larger proportion of postnatally diagnosed children presented at admission with elevated lactate > 10 mmol/l (p = 0.02), a higher dose of prostaglandin (p = 0.0013) and need for mechanical ventilation (p < 0.0001). Critical lesions such as hypoplastic left heart syndrome were an important determinant for morbidity and mortality. Thirty-days survival after BCPC was better in patients with prenatal diagnosis (p = 0.025). Prenatal diagnosis is associated with higher survival in neonates with SV physiology.

Cardiac transplantation in a neonate-First case in Switzerland and European overview.

Twenty-four percent of pediatric heart transplantations (pHTx) are carried out in infants. Neonatal heart transplantation is both rarely performed and challenging. We report on a newborn baby girl suffering from cardiac failure due to a huge tumor (24×52 mm) within the free wall of the left ventricle (LV) and subtotal obstruction of the main left bronchus. Following a surgical tumor resection, a Berlin Heart EXCOR left ventricular assist device was implanted as the bridge to the transplantation. In spite of an organ donor/recipient mismatch of >200%, both heart transplantation and the postoperative course were successful. In addition to this case report, the authors also present data from a survey on performed infant and neonatal transplantations in Western Europe. As neonatal heart transplantation is a rare event in Europe, the authors think it is of crucial importance to share this limited experience. We discuss an alternative strategy-namely, palliative surgical correction using the Fontan pathway. The challenges of donor/recipient weight mismatch and the possibilities of overcoming infant donor organ shortage as a postoperative immunosuppressive regimen are discussed as well.

Psychosocial impact on families with an infant with a hypoplastic left heart syndrome during and after the interstage monitoring period - a prospective mixed-method study.

AIMS AND OBJECTIVES: To investigate parents' experiences, coping ability and quality of life while monitoring their sick child with hypoplastic left heart syndrome at home. BACKGROUND: Interstage home monitoring for children with hypoplastic left heart syndrome reduces interstage mortality between Norwood stages I and II. Little is known about the psychosocial impact of interstage home monitoring. DESIGN: Prospective mixed-method study. METHODS: This study assessed the psychosocial impact on parents during interstage home monitoring. This contains for quantitative assessment the Short Form Health Survey questionnaire and the Impact of Family Scale administered one and five weeks following discharge before and after stage II. For qualitative assessment, semi-structured interviews focussing on the postdischarge coping strategies were conducted twice, five weeks after hospital discharge before and after stage II. RESULTS: Ten infants (eight males) with hypoplastic left heart syndrome (n = 7) or other types of univentricular heart malformations (n = 3), and their parents (nine mother/father two-parent households, one single mother) were included. There were no interstage deaths. Mental Health Composite Summary scores were low in both parents (mothers: 40·45 ± 9·07; fathers: 40·58 ± 9·69) and lowest for the item 'vitality' (mothers: 37·0 ± 19·46; fathers: 43·12 ± 25·9) before and after stage II. Impact of Family Scale values showed higher daily and social burdens for mothers. 'Becoming a family' was the most important task as coping strategy to equilibrate the fragile emotional balance. The parents judged interstage home monitoring as a protective intervention. CONCLUSIONS: Although psychosocial burden before and after stage II remains high, becoming a family is an essential experience for parents and confirms their parenthood. RELEVANCE TO CLINICAL PRACTICE: Healthcare professionals must be aware of parents' needs during this vulnerable interstage period and to provide psychosocial and nursing support.

Interventional Closure of Secundum Type Atrial Septal Defects in Infants Less Than 10 Kilograms: Indications and Procedural Outcome.

AIMS: This study set out to assess indications, feasibility, complications, and clinical outcome of percutaneous transcatheter device closure of atrial septal defects (ASDs) in infants with a bodyweight below 10 kg. METHODS AND RESULTS: Retrospective single center chart and echocardiography review study from 8/2005-12/2013. Twenty-eight children with ASD (13 female) with a median age of 1.15 years (0.2-2.8) and a median weight of 7.2 kg (4.5-9.9) were analyzed. Indications for early ASD closure were failure to thrive (n = 15, 54%), bronchopulmonary dysplasia (BPD) with supplemental oxygen dependency (n = 7, 25%), and genetic syndromes with suspected pulmonary hypertension (n = 12, 43%). Device implantation was successful in all patients without any periprocedural mortality or major complication. Clinical outcome after a median follow-up period of 2.1 years (0.25-7.3) revealed no residual shunt and a significant decrease of right ventricular volume load. Patients with pulmonary hypertension experienced a significant reduction of pulmonary artery/RV pressure. Patients also showed decreased supplemental oxygen dependency and less cardiac medications, but no significant "catch-up growth" in those with failure to thrive. CONCLUSION: Interventional ASD closure in children weighing less than 10 kg can be performed without any additional major risks and shows a favorable outcome, especially in selected patients with significant non cardiac co-morbidities.

Successful heart transplantation after prolonged cardiac arrest and extracorporeal life support in organ donor-a case report.

BACKGROUND: Although heart transplantation is a successful therapy for patients suffering from end-stage heart failure, the therapeutic is limited by the lack of organs. Donor cardiac arrest is a classic hindrance to heart retrieval as it raises issues on post-transplant outcomes. CASE PRESENTATION: The present case reports a successful heart transplantation after prolonged donor cardiac arrest (total lowflow time of 95 minutes) due to anaphylactic shock necessitating extracorporeal life support. We further provide an overview of the current evidence and outcomes of heart transplantation in cases of donor cardiac arrest. CONCLUSION: Providing that donor and recipient criteria are respected, donor cardiac arrest does not seem to be an adverse predictor in heart transplantation.

Pediatric heart transplantation.

Pediatric heart transplantation (pHTx) represents a small (14%) but very important and particular part in the field of cardiac transplantation. This treatment has lifelong impact on children. To achieve the best short and especially long-term survival with adequate quality of life, which is of crucial importance for this young patient population, one has to realize and understand the differences with adult HTx. Indication for transplantation, waitlist management including ABO incompatible (ABOi) transplantation and immunosuppression differ. Although young transplant recipients are ultimately likely to be considered for re-transplantation. One has to distinguish between myopathy and complex congenital heart disease (CHD). The differences in anatomy and physiology make the surgical procedure much more complex and create unique challenges. These recipients need a well-organized and educated team with pediatric cardiologists and intensivists, including a high skilled surgeon, which is dedicated to pHTx. Therefore, these types of transplants are best concentrated in specialized centers to achieve promising outcome.

VAD as bridge to recovery in anthracycline-induced cardiomyopathy and HHV6 myocarditis.

This report describes an 8-year-old child with acute anthracycline-induced cardiomyopathy triggered by human herpesvirus 6 and the subsequent implantation of an intracorporeal continuous-flow left ventricular assist device (LVAD) and the process to discharge the child from the hospital. After barely 3 months on mechanical support, the device was explanted after thorough examination. Experiences regarding LVAD removal are limited, and no guidelines for echocardiographic and hemodynamic criteria for LVAD removal in children have been published thus far. We present our institutional algorithm for device selection, surveillance in an ambulatory setting, and testing for myocardial recovery, as well as our criteria for LVAD explantation in children.

Clinical course and interstage monitoring after the Norwood and hybrid procedures for hypoplastic left heart syndrome.

Infants with hypoplastic left heart syndrome (HLHS) are at risk for interstage morbidity and mortality, especially between the first and second surgical stages after the Norwood and hybrid procedures. This study compared the morbidity and mortality of patients treated by either the Norwood or the hybrid procedure for HLHS between the first and second stages who were undergoing interstage monitoring. Between October 2008 and December 2011, 26 infants (14 boys) with HLHS (n = 16) and other univentricular heart malformations with aortic arch anomaly (n = 10) were scheduled for interstage monitoring after Norwood I (n = 12) and hybrid (n = 14) procedures. Three infants (11.5 %) died after first-stage palliation (one hybrid patient and two Norwood patients), and three infants (11.5 %) died after second-stage palliation (two hybrid patients and one Norwood patient) (p = 0.83), all after early second-stage surgery (<90 days). The Norwood I and hybrid procedures did not differ in terms of overall mortality (23 %) (three hybrid and three Norwood patients; p = 1.00). Seven infants (26.9 %) could not be discharged from the hospital due to hemodynamic instability and were referred for early second-stage surgery (<90 days). After the first stage, the invasive reevaluation rate before discharge was high (53.8 %), with cardiac catheterizations for 8 of 14 patients after the hybrid procedure and for 6 of 12 patients after the Norwood procedure (p = 0.69). A total of 11 reinterventions were performed (eight by catheter and three by surgery). Of the eight catheter reinterventions, five were performed for hybrid patients (p = 0.22). For 14 infants, 89 days (range 10-177 days) of interstage monitoring were scheduled. One infant (3.9 %) died during the interstage monitoring. The findings showed a breach of the physiologic criteria for interstage monitoring in seven infants (50 %) after 10 days (range 4-68 days) (five hybrid and two Norwood patients), leading to rehospitalization and catheterization for six patients (four hybrid and two Norwood patients), requiring interventions for two patients (patent arterial duct stent dilation, and atrial septal defect stenting, all for hybrid patients). Overall, three of the seven patients with red flag events of interstage monitoring were candidates for early second-stage surgery. In conclusion, morbidity among infants treated for HLHS remains high, either before or after hospital discharge, emphasizing the need of interstage monitoring programs. Despite retrograde aortic flow in infants with HLHS after the hybrid procedure, the mortality rate was comparable between the two groups. Mortality occurs after early second-stage surgery (<90 days).

Acute chemotherapy-induced cardiomyopathy treated with intracorporeal left ventricular assist device in an 8-year-old child.

Evolution of ventricular assist devices (VADs) leading to miniaturization has made intracorporeal implantation in children feasible. Ventricular assist device therapy for anthracycline-induced cardiomyopathy (CMP) in adults has been reported. We report the case of an 8-year-old child (body surface area 0.97 m) presenting with anthracycline-induced CMP being successfully treated with an intracorporeal left ventricular assist device (LVAD) as a bridge to candidacy/recovery. We present our institutional algorithm, which advises intracorporeal LVAD implantation for long-term ventricular assist, in children with a body surface area >0.6 m. Advantages are better mobilization and the possibility to discharge home, leading to enhanced quality of life.