Berlin Heart EXCOR Paediatric Ventricular Assist Device: Does Weight Matter?

BACKGROUND: Berlin Heart EXCOR (BH) ventricular assist devices provide mechanical long-term circulatory support in children with end-stage heart failure, as a bridge to transplantation or to recovery. Most studies are from large-volume paediatric cardiac centres. AIM: The aim of this study was to analyse the experiences of three French centres and to compare these with available published data. METHOD: We performed a retrospective observational study of three paediatric cardiac intensive care units. All children supported with BH devices were included. Morbidity and mortality data were collected and risk factors analysed. RESULTS: Fifty-four (54) patients (54% male) were included. Survival rate was 73% while on a BH device. Median age at BH device implantation was 17 months (range 2-180 months). The predominant indication was dilated cardiomyopathy (61%). Bi-ventricular assist device was used in 25 (46%) cases. The total length of long-term circulatory support was 3,373 days, with a mean length per patient of 62.5 days (range 5-267 days). Thirty-two (32) patients were transplanted (59%) and seven (13%) were successfully weaned. Type and length of support did not influence morbidity. Main complications were renal dysfunction (57%), bleeding (41%), and infection (39%). In multivariate analysis, a weight <5 kg was significantly associated with higher mortality. CONCLUSIONS: The weight seems to be the most important risk factor of mortality in this precarious condition.

Mobile extracorporeal membrane oxygenation for children: single-center 10 years' experience.

OBJECTIVES: Extracorporeal membrane oxygenation has become a gold standard in treatment of severe refractory circulatory and/or pulmonary failure. Those procedures require gathering of competences and material. Therefore, they are conducted in a limited number of reference centers. Emergent need for such treatments induces either hazardous transfers or a mobile pediatric extracorporeal membrane oxygenation team able to remote implantation and transportation. The aim of this work is not to focus on pediatric extracorporeal membrane oxygenation outcomes or indications, which have been extensively discussed in the literature. This study would like to detail the implementation, safety, and feasibility, even in a middle-size pediatric cardiac surgery reference center. PATIENTS: This is a retrospective analysis of a series of patients initiated on extracorporeal membrane oxygenation in a peripheral center and transferred to a reference center. The data were collected from 10 consecutive years: from 2006 to 2016. RESULTS: A total of 57 pediatric patients with a median weight of 6.00 (3.2-14.5) kg and median age of 2.89 (0.11-37.63) months were cannulated in peripheral center and transported on extracorporeal membrane oxygenation. We did not experience any adverse event during transport. The outcomes were comparable to our literature-reported on-site extracorporeal membrane oxygenation series with 42 patients (74%) weaned from extracorporeal membrane oxygenation and a 30-day survival of 60%. Neither patient's age nor weight, indication for extracorporeal membrane oxygenation or length of transport, was statistically significant in terms of outcomes. CONCLUSION: Offsite extracorporeal membrane oxygenation implantation and ground or air transport for pediatric patients on extracorporeal membrane oxygenation appeared to be safe when performed by a dedicated and experienced team, even within a mid-size center.

Plasma ultrasensitive cardiac troponin during long-term follow-up of heart transplant recipients.

BACKGROUND: Plasma troponin is a risk factor for cardiac events in various populations. We evaluated the determinants and predictive value of plasma cardiac troponin I (cTnI) during the long-term follow-up of heart transplantation. METHODS AND RESULTS: We prospectively measured cTnI in 100 heart transplant recipients, 13.0 ± 6.0 years after transplantation, during a routine visit including echocardiography and laboratory measurements. The patients were followed for 21.3 ± 4.9 months after this troponin measurement. cTnI ≥0.006 µg/L (detection threshold) was found in 37 of these 100 patients. Plasma troponin significantly increased with the presence and severity of coronary lesions detected by means of coronary angiography and was significantly associated with age, left ventricular mass, history of post-transplantation heart failure, body mass index, and plasma creatinine. Of 37 patients with cTnI ≥0.006 µg/L, 13 had a cardiac event during follow-up compared with 2 of 63 patients with cTnI <0.006 µg/L (P < .0001). The relation between cTnI and cardiac events remained significant in multivariate analysis. CONCLUSIONS: cTnI is frequently detectable during long-term follow-up after heart transplantation and is associated with chronic graft rejection and renal failure. A detectable cTnI may help identify heart transplant recipients at high risk of cardiac events.

Stenting right ventricular outflow in an infant with tetralogy of Fallot and well-developed pulmonary arteries.

A right ventricular outflow tract stent was implanted in a 1-month-old (3.5 kg) baby boy with tetralogy of Fallot and worsening cyanosis to relieve infundibular and pulmonary valve stenosis in the setting of well-developed pulmonary artery branches. This management allowed a symmetric and optimal growth of the pulmonary artery branches, with right and left pulmonary arteries measuring 7-mm (z-score, +1.3 SD) and 7.3-mm (z-score, +1.7 SD), respectively, 3 months after stent implantation. No signs of pulmonary overcirculation developed, and complete surgical repair was performed at the age of 6 months.

Severe aortic valve regurgitation after percutaneous ventricular septal defect closure.

Percutaneous closure of ventricular septal defect is now commonly used. We report a rare case, and especially a rare image of aortic leaflet damage caused by percutaneous ventricular septal device.

Thoracic computed tomography in absent pulmonary valve syndrome management.

A neonate with absent pulmonary valve syndrome (APVS) and features of tetralogy of Fallot presented respiratory distress. The prolonged need for mechanical ventilation was an indication for surgical repair. Preoperative thoracic CT imaging showed nearly complete closure of the distal trachea, compressed by the aneurysmal dilatation of the pulmonary arteries, and its reopening after ventilator pressure was increased. Management of APVS may require high-pressure ventilation to prevent bronchial collapse due to compression by the pulmonary arteries. In this context, thoracic CT imaging is very useful for adjusting ventilation support so that surgical repair can be performed in optimal conditions.

Cryopreserved homograft in the Ross procedure: outcomes and prognostic factors.

BACKGROUND AND AIM OF THE STUDY: The study aim was to determine the dynamics and risk factors related to cryopreserved homografts implanted to reconstruct the right ventricular outflow tract (RVOT) during the Ross procedure. METHODS: A retrospective study, conducted between 1993 and 2009, included 107 consecutive patients who underwent a Ross procedure with implantation of a homograft (102 pulmonary, five aortic). The median patient age at implant was 18.8 years (range: 2 months - 67 years). The main cardiac defects were congenital aortic stenosis (n = 44), congenital aortic regurgitation (n = 20), and combined (n = 23) and acquired (n = 20) aortic regurgitation. The study endpoints were homograft stenosis (peak gradient > or = 20 mmHg), regurgitation (grade > or = moderate), homograft dysfunction (stenosis > or = 50 mmHg or regurgitation > or = moderate), homograft explant, and failure (explant or balloon dilation). The mean follow up was 5.7 +/- 0.4 years. The statistical analysis was conducted using univariate and multivariate Cox regression tests. RESULTS: The overall patient survival was 97% at 18 years. Homograft stenosis occurred in 54% of patients, regurgitation in 18%, dysfunction in 27%, explant in 5%, and failure in 6%. Freedom from homograft dysfunction was 75% and 55% at five and 10 years, respectively, while freedom from homograft failure was 99% and 84% at five and 10 years, respectively. The main multivariate risk factors for dysfunction were: homograft diameter < 22 mm (OR: 11, p = 0.019), aortic homograft (OR: 18, p = 0.019) and preoperative right ventricle/left ventricle pressure ratio > 0.4 (OR: 5, p = 0.018). Univariate risk factors for failure were: homograft diameter < 22 mm (p = 0.006), donor age < 30 years (p = 0.03), cold ischemia time < 2 days (p = 0.04), and decontamination time < 12 h (p = 0.01). CONCLUSION: In the Ross procedure, the cryopreserved homograft represents an excellent means to reconstruct the RVOT, with a good long-term longevity. Almost 85% of patients did not require any surgery or percutaneous intervention at 10 years after implantation. The homograft long-term durability depends on both homograft-related and patient-related factors. Pulmonary homografts, with a diameter > 22 mm, a donor age > 30 years, and a cold ischemia time > 2 days are to be preferred.

Anomalous left coronary artery from the pulmonary artery: modified extra-anatomic reimplantation.

The anomalous origin of the left coronary artery from the pulmonary artery in the position of a non-facing coronary sinus is extremely rare. The anatomical position of the ectopic ostia will determine which is the appropriate operative approach to create a dual-coronary supply. This report describes a technique of modified extra-anatomical rerouting using autologous pericardium patch and pulmonary artery flap to create a neo-composite coronary trunk passing anterior to the right ventricular outflow tract.

Antenatal Diagnosis of a Partial Atrioventricular Canal with Ebstein's Anomaly.

The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein's anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. Only 22 cases are described in the literature. This patient's antenatal diagnosis of both Ebstein's anomaly and partial AVCD was made at 25 weeks of gestation. The delivery was organized in a tertiary center. The initial neonatal course was difficult but with adequate treatment, a rapid improvement allowed for a gap of almost 2 years before a complete surgical repair including a cone tricuspid plasty. To our knowledge, this is the first case of antenatal diagnosis, with carefully tailored delivery, neonatal care and subsequent follow-up before indication for successful surgery.

Ross and Ross-Konno procedures in infants, children and adolescents: a 13-year experience.

BACKGROUND AND AIM OF THE STUDY: Due to hemodynamic performance and potential for growth of the pulmonary autograft, the Ross operation is considered to be the surgery of choice for irreparable aortic valve disease in pediatric patients. The study aim was to analyze the long-term clinical and echocardiographic results of the Ross operation. METHODS: Between February 1993 and July 2006, 55 consecutive patients (mean age 10.0 +/- 6.2 years; range: 3 months to 18 years) underwent eithera Ross operation (n=46) or a Ross-Konno procedure (n=9). The underlying left ventricular outflow tract pathology was mainly congenital (n=47). Among patients, 23 (42%) had undergone a previous aortic valve procedure. Concomitant procedures were performed in 16 patients (29%). The Ross operation was performed as a root replacement in all cases; the mean cross-clamp time was 132 min (range: 100-188 min). The autograft diameter was indexed to the body surface area and compared to normal values. The mean follow up was 5.5 +/- 3.8 years, and was 100% complete. RESULTS: There was one early death (2%) and two late deaths (4%). The actuarial patient survival was 93% at 10 years. None of the patients developed moderate or severe autograft regurgitation. All measured maximal root diameters were above the 90th percentile of normal aortic diameter, without correlation to autograft regurgitation. Five patients (9%) had a mean homograft gradient > or = 40 mmHg, and two (4%) were reoperated on. The freedom from reoperation for homograft degeneration was 91% at 10 years. CONCLUSION: Autograft regurgitation after the Ross and Ross-Konno procedures is uncommon, and the risk of homograft degeneration appears low. Autograft dilatation is common but does not correlate with autograft regurgitation. When considering long-term freedom from autograft and homograft degeneration, the results of the present study confirm the Ross operation as the surgery of choice for irreparable aortic valve disease in infants, children and young adults.

Medical and surgical perspectives of cardiac hypertrophy in Costello syndrome.

We describe our experience with 2 patients having Costello syndrome, aged 11 and 36 months, who suffered systolic anterior motion of the aortic leaflet of the mitral valve and obstructive cardiac hypertrophy requiring surgery, comparing their cardiac characteristics to those described previously. We conclude that the heterogeneous nature of the cardiac hypertrophy in this syndrome can be considered and managed as the sum of a diffuse hypertrophy accessible to beta-blockade, and an asymmetric hypertrophy accessible to surgical myomectomy.

Anatomic Repair of a Left Coronary Artery Main Stem Atresia.

Atresia of the main stem of the left coronary artery is the least observed congenital coronary anomaly; most patients tend to receive a coronary artery bypass graft, although some anatomical corrections have been described. A 17-year-old female patient with left coronary artery main stem atresia underwent a coronary trunk construction with an autologous pericardial patch in our department. At a 3-year follow-up, the patient was asymptomatic, with a normal cardiac stress test. The coronary computed tomography showed no stenosis between the aorta and coronary bifurcation. Long-term patency has yet to be determined.

Should transcatheter closure of atrial septal defects with inferior-posterior deficient rim still be attempted?

BACKGROUND: Transcatheter closure for atrial septal defect (ASD) with inferior-posterior rim deficiency has been scarcely reported with proper identification of the indications and limits. We aimed to assess the safety and feasibility of transcatheter closure of ASDs with deficient rims, paying particular attention to cases with inferior-posterior rim deficiency. METHODS: From January 2008 to January 2013, 241 patients underwent transcatheter ASD closure, including 50 cases (20.7%) with deficient rims, other than the anterior-superior one. Eighteen patients (12 females) presented inferior-posterior rim deficiency. Their median age was 8 (1.4-85) years and their median weight was 24 [9-97] kg. Transcatheter closure was performed in all cases under transesophageal echocardiography (TEE) guidance in children and intracardiac echocardiography (ICE) guidance in adults. RESULTS: Out of 18 patients with inferior-posterior rim deficiency, only 8 underwent successful immediate transcatheter closure. Four cases failed to be closed. Major complications occurred in 6 patients, including 4 device embolizations, 1 pericardial effusion and 1 complete atrioventricular block that resolved after surgical removal of the device. During a median follow up of 54±13 months, a residual right-to-left shunt was documented in 2 more cases, requiring surgery in one case because of cyanosis. Transcatheter closure was successfully performed in the rest of the 223 patients, including in the 32 cases with deficient rims other than inferior-posterior. CONCLUSIONS: Transcatheter closure of ASDs with inferior-posterior rim deficiency cannot be recommended.

Mitral annuloplasty in dilated cardiomyopathy: eighteen-year follow up in a pediatric case.

Dilated cardiomyopathy is often complicated by severe mitral regurgitation (MR) caused by dilation of the annulus. Heart transplantation remains the ultimate treatment when heart failure fails to respond to medical treatment. Mitral annuloplasty for severe MR in dilated or ischemic cardiomyopathy may delay or replace the need for heart transplantation. The case is reported of a six-year-old child with dilated cardiomyopathy, severe MR and high pulmonary arterial pressure. Following beneficial mitral annuloplasty in 1989, the patient is in excellent functional condition eighteen years later.

Hybrid Melody Valve Implantation in Mitral Position in a Child: Usefulness of a 3-Dimensional Printed Model for Preprocedural Planning.

We present the case of a 4-month-old child with atrioventricular canal associated with severe left atrioventricular valve dysfunction who previously underwent 3 surgical valve reconstructions without significant improvement. A Hybrid Melody valve (Medtronic, Minneapolis, MN) insertion was planned. Because of the low weight, the risk of left ventricular outflow tract obstruction was significant and therefore evaluated preprocedurally using a cardiac computed tomography-derived 3-dimensional printed model. In vitro tests showed good anchorage of the valve without subaortic obstruction and the procedure was then achieved with an excellent clinical result.

[Extracorporeal life support and heart-lung transplant in children]. / Assistance circulatoire et transplantation d'organes thoraciques chez l'enfant.

Extracorporeal life support and heart and/or lung transplant are the last resort in children with end-stage cardiac and/or pulmonary failure and short-term life threaten. Currently, circulatory support is used as a bridge to recovery or as a bridge to transplant but not as a destination therapy. The Excor Berlin Heart is the long-lasting external pneumatic ventricular assist system that is currently available from infancy to adulthood. Long-term prognosis after pediatric cardiac and/or pulmonary transplant is conditioned by the occurrence of graft failure, coronary disease of the cardiac graft, viral infections and bronchiolitis obliterans of the pulmonary graft, the incidence of which increase with time. The scarcity of grafts and the risk of acute rejection due to lack of compliance with immunosuppressive treatment require the transplant specialized teams to choose the best candidates according to psychosocial and biological criteria. The next expected developments concern mainly long-term ventricular assistance with systems that allow for greater autonomy and a return to the child's home.

Secondary subaortic stenosis in heart defects without any initial subaortic obstruction: a multifactorial postoperative event.

BACKGROUND/OBJECTIVE: Secondary subaortic stenosis (SSS) can occur after surgery for various congenital heart defects with or without initial left ventricular outflow tract obstruction (LVOTO). The objective of this study was to highlight the anatomical lesions and surgical procedures associated with the development of SSS after surgery on defects without initial LVOTO. METHODS: A retrospective study of 4710 patients was performed (1984-2005). The criterion for inclusion was a fixed subaortic obstruction requiring surgery, after an open- or closed-heart operation. The criterion for exclusion was an LVOTO at the time of the first operation. RESULTS: Twenty-eight patients were studied. The mean age at initial surgery was 32 months (4 days-47 years; median: 2 months). SSS occurred after three main types of surgery: repair of coarctation of the aorta, repair of AVSD and LV-aorta rerouting for double outlet right ventricle or transposition of great arteries. The mean delay of occurrence was 4.4 years (2 months-19 years). Frequently associated initial anatomical conditions were coarctation of the aorta (40%), lesions of the mitral valve (32%), bicuspid aortic valve (21%) and left superior vena cava (LSVC) (14%). Preoperative anatomical lesions of the LVOT were present in 93% of the cases. After the initial operation, only one patient had a mean echo-Doppler pressure gradient across the LVOT>20 mmHg. SSS was most frequently a subaortic membrane (n=23). The mean pressure gradient across SSS at the time of reoperation was 47+/-29 mmHg. Five patients developed a second SSS after 7.4 years (mean). One patient developed a third SSS. No patient died. When compared with patients without SSS, significant risk factors for SSS were low age at surgery (32 vs 74.9 months, p<10(-4)), pre-existing coarctation of the aorta (40 vs 10%, p<10(-4)), bicuspid aortic valve (21 vs 6%, p=0.002) and LSVC (14 vs 4%, p=0.02). CONCLUSIONS: SSS development is multifactorial, depending on initial anatomical lesions and initial surgery. Low age at initial surgery, coarctation of the aorta, bicuspid aortic valve and LSVC significantly increase the risk of SSS. These elements warrant long-term follow-up for early detection of SSS.

Transposition of the great arteries: Rationale for tailored preoperative management.

As preoperative morbi-mortality remains significant, care of newborns with transposition of the great arteries is still challenging. In this review of the literature, we discuss the different treatments that could improve the patient's condition into the preoperative period. Instead of a standardized management, we advocate personalized care of these neonates. Considering the deleterious effects of hypoxia, special attention is given to the use of non-invasive technologies to assess oxygenation of the tissues. As a prolonged preoperative time with low cerebral oxygenation is associated with cerebral injuries, distinguishing neonates who should undergo early surgery from those who could wait longer is crucial and requires full expertise in the management of neonatal congenital heart disease. Finally, to treat these newborns as soon as possible, we support a planned delivery policy for foetuses with transposition of the great arteries.

Early to mid-term results after total cavopulmonary connection performed in the second decade of life.

OBJECTIVES: Total cavopulmonary connection (TCPC) performed in the second decade of life has rarely been studied. Thus, we investigated (bicentric study) early and late morbidity and mortality following completion of TCPC in these patients. METHODS: From January 1999 to June 2014, 63 patients (14.5 ± 2.9 years) underwent TCPC (extracardiac conduit). Palliation before completion was an isolated bidirectional cavopulmonary shunt (BCPS) in 3 patients or BCPS associated with additional pulmonary blood flow (APBF) that was either antegrade (Group 1) in 38 (63%) or retrograde (Group 2) in 22 (37%). Preoperative and perioperative data were reviewed retrospectively. RESULTS: Mean pulmonary arterial and ventricular end-diastolic pressures were 12.2 and 9.2 mmHg, respectively. Mean Nakata index was 279 ± 123 and 228 ± 87 mm 2 /m 2 in Groups 1 and 2, respectively ( P = 0.01). Aortic cross-clamping was performed in 22 from Group 1 and 8 from Group 2 ( P = 0.04). Mean follow-up was 4.57 years [0.8-15]. Nine patients had prolonged stays in the intensive care unit (>6 days). There were 1 early and 2 late deaths (non-cardiac related). Actuarial survival was 96% at 4 years. At last follow-up, single-ventricle function remained normal or improved in all patients (Group 1) compared to 82% in Group 2 ( P = 0.02). New York Heart Association (NYHA) class had improved in both groups: 47 patients were NYHA class II and 16 class III preoperatively vs 50 class I and 10 class II postoperatively ( P < 0.001). CONCLUSIONS: Single-ventricle palliation with BCPS and APBF allowed completion of TCPC in the second decade of life, with encouraging mid-term results. However, BCPS with retrograde APBF was associated with single-ventricle dysfunction: thus, this technique needs to be used cautiously as long-lasting palliation.