Repair for Congenital Mitral Valve Stenosis.

We report the techniques and long-term outcome of mitral valve (MV) repair to correct congenital mitral stenosis in children. Between 1986 and 2014, 137 children (mean age 4.1 ± 5.0, range 1 month-16.8 years) underwent repair of congenital mitral stenosis (CMS). In 48 patients, CMS is involved in Shone's anomaly. The typical congenital MS (type I) was seen in 56 patients. Hypoplastic MV (type II, n = 15) was associated with severe left ventricular outflow tract abnormalities and hypoplastic left ventricular cavity and muscle mass. Supravalvar ring (type III, n = 48) ranged from a thin membrane to a thick discrete fibrous ridge. Parachute MV (type IV, n = 10) have 2 leaflets and barely distinguishable commissures, but all chordae merged either into 1 major papillary muscle or asymmetric papillary muscles-1 dominant and the other minuscule. Hammock valve (type IV, n = 8) appeared dysplastic with shortened chordae directly inserted into the posterior left ventricular muscle mass. MV repair was performed using commissurotomy, chordal division, papillary muscle splitting and fenestration, and mitral ring resection, each applied according to the presenting morphology. During the 28-year follow-up period, 23 patients underwent repeat MV repair and 3 underwent MV replacement after failed attempts at repeat repair. At 1 and 15 years postoperatively, freedom from reoperation was 89.3 ± 5.1% and 52.8 ± 11.8%, and cumulative survival rates were 92.3 ± 4.3% and 70.3 ± 8.9, respectively. Mortality unrelated to repair accounted for 9 (20%) deaths. Long-term functional outcome of MV repair in children with CMS is satisfactory. Repeat repair or replacement may be deemed necessary during the course of follow-up.

The Double-Orifice Valve Technique to Treat Tricuspid Valve Incompetence.

BACKGROUND: A straightforward tricuspid valve (TV) repair technique was used to treat either moderate or severe functional (normal valve with dilated annulus) or for primary/organic (Ebstein's anomaly, leaflet retraction/tethering and chordal malposition/tethering, with annular dilatation) TV incompetence, and its long-term outcome assessed. METHODS: A double-orifice valve technique was employed in 91 patients (mean age 52.6 ± 23.2 years; median age 56 years; range: 0.6-82 years) with severe tricuspid regurgitation. Among the patients, three had post-transplant iatrogenic chordal rupture, five had infective endocarditis, 11 had mitral valve insufficiency, 23 had Ebstein's anomaly, and 47 had isolated severe TV incompetence. The basic principle was to reduce the distance between the coapting leaflets, wherein the most mobile leaflet could coapt to the opposite leaflet, by creating two orifices, ensuring valve competence. The TV repair was performed through a median sternotomy or right anterior thoracotomy in the fifth intercostal space under cardiopulmonary bypass. The degree and extent of creating a double-valve orifice was determined by considering the minimal body surface area (BSA)-related acceptable TV diameter. Repair was accomplished by passing pledgeted mattress sutures from the middle of the true anterior annulus to a spot on the opposite septal annulus, located approximately two-thirds of the length of the septal annulus to avoid injury to the bundle of His. The annular apposition divides the TV into a larger anterior and a smaller posterior orifices, enabling valve closure, on both sides. In adults, the diameter of the anterior valve orifice should be 23-25 mm, and the posterior orifice 15-18 mm; thus, the total valve orifice area is 5-6 cm2. In children, the total valve orifice should be a standard deviation of 1.7 mm for a BSA of <1. 0m2, and 1.5 mm for a BSA of >1.0m2. RESULTS: During a mean follow up of 8.7 ± 1.34 years (median 10 years; range: 1.5-25.9 years) there have been no reoperations for TV insufficiency or stenosis. Reoperations on three patients (mean age 42.5 ± 8.7 years) were indicated for aortic valve replacement at 14 months postoperatively (n = 1) and for assist device implantation (n = 2) who eventually underwent heart transplant at 18 and 20 months after TV repair, respectively. The cumulative 12-year survival rate was 86.9%. CONCLUSIONS: This double-orifice technique is technically a straightforward repair to abolish TV incompetence with highly satisfactory results, particularly in patients with severe annular dilatation or with leaflet and chordal tethering. In the present series, the technique provided no pitfalls (if the location of the conduction system was borne in mind), requiring only a gentle placement of sutures. It also led to no residual regurgitation or reoperation during the follow up period.

Hypothermia During Cardiopulmonary Bypass Increases Need for Inotropic Support but Does Not Impact Inflammation in Children Undergoing Surgical Ventricular Septal Defect Closure.

Minimizing the systemic inflammatory response caused by cardiopulmonary bypass is a major concern. It has been suggested that the perfusion temperature affects the inflammatory response. The aim of this prospective study was to compare the effects of moderate hypothermia (32°C) and normothermia (36°C) during cardiopulmonary bypass on markers of the inflammatory response and clinical outcomes (time on ventilator) after surgical closure of ventricular septal defects. During surgical closure of ventricular septal defects under cardiopulmonary bypass, 20 children (median age 4.9 months, range 2.3-38 months; median weight 7.2 kg, range 5.2-11.7 kg) were randomized to a perfusion temperature of either 32°C (Group 1, n = 10) or 36°C (Group 2, n = 10). The clinical data and blood samples were collected before cardiopulmonary bypass, directly after aortic cross-clamp release, and 4 and 24 h after weaning from cardiopulmonary bypass. Time on ventilation as primary outcome did not differ between the two groups. Other clinical outcome parameters like fluid balance or length of stay in the intensive care were also similar in the two groups. Compared with Group 2, Group 1 needed significantly higher and longer inotropic support (P < 0.001). In Group 1, two infants had junctional ectopic tachycardia, and another had a pulmonary hypertensive crisis. Perfusion temperature did not influence cytokine release, organ injury, or coagulation. Cardiopulmonary bypass temperature does not influence time on ventilation or inflammatory marker release. However, in the present study, with a small patient cohort, patients operated under hypothermic bypass needed higher and longer inotropic support. The use of hypothermic cardiopulmonary bypass in infants and children should be approached with care.

Pediatric heart allocation and transplantation in Eurotransplant.

Pediatric heart allocation in Eurotransplant (ET) has evolved over the past decades to better serve patients and improve utilization. Pediatric heart transplants (HT) account for 6% of the annual transplant volume in ET. Death rates on the pediatric heart transplant waiting list have decreased over the years, from 25% in 1997 to 18% in 2011. Within the first year after listing, 32% of all infants (<12 months), 20% of all children aged 1-10 years, and 15% of all children aged 11-15 years died without having received a heart transplant. Survival after transplantation improved over the years, and in almost a decade, the 1-year survival went from 83% to 89%, and the 3-year rates increased from 81% to 85%. Improved medical management of heart failure patients and the availability of mechanical support for children have significantly improved the prospects for children on the heart transplant waiting list.

Creation of a tricuspid valve regurgitation model from tricuspid annular dilatation using the cardioport video-assisted imaging system.

BACKGROUND AND AIM OF THE STUDY: Experimental models of tricuspid valve regurgitation (TR) are used to study novel annuloplasty techniques (including prosthetic rings), and they can also serve as physiologic models to investigate TR pathophysiology. The study aim was to develop an appropriate simple and reproducible experimental model of TR from annular dilatation. METHODS: Acute TR was successfully created through multiple small 3- to 5-mm incisions in the annulus using a custom-made optical port with an instrument shaft (the Cardioport) that accepts a standard endoscopic imaging system. The Cardioport was inserted, via a thoracotomy, through the right atrium of seven Yorkshire pigs, and directed towards the tricuspid valve annulus to create the annular incisions. Tricuspid valve anatomy and function were evaluated using 2D and 3D echocardiography. The presence and severity of TR, annulus diameter, and changes in heart rate and atrial pressures after making the annular incisions were documented. To monitor tricuspid annular dilatation and the progression of TR, follow up echocardiography and color Doppler examinations were performed at two and eight weeks postoperatively. RESULTS: The acute onset of TR was well tolerated, and there were no deaths or significant morbidity associated with the procedure. The annular diameter was increased from a preoperative mean of 23.1 +/- 1.7 mm, to 32.2 +/- 2.5 mm at two weeks postoperatively, and to 37.3 +/- 3.6 mm at eight weeks postoperatively. Overall, the TR progressed from mild (grade I) to severe (grade III) in all of the animals. CONCLUSION: This novel porcine model represents a relatively simple and a reproducible surgical technique for the creation of annular dilatation and TR, and may also serve as a chronic model of the latter condition.

Fate of the aortic valve following the arterial switch operation.

OBJECTIVE: We evaluated the long-term outcome of aortic valve after arterial switch operation (ASO). METHODS: A retrospective review of 324 hospital survivors after ASO was performed. Echocardiographic findings and incidence and progression of aortic regurgitation (AR) were investigated. Endpoints of the study were the first documented occurrence of moderate or severe AR or need for aortic valve replacement (AVR). RESULTS: Upon discharge from hospital 5.2% of the patients showed mild AR, progressing to 11.4% after 1 year. At a mean follow-up time of 14.4 ± 0.54 years, AR was absent in 307 (94.7%), trivial in six (1.8%), mild in nine (2.7%), and moderate-to-severe in two (0.6%) patients. There is a progression of AR with time after ASO (p < 0.001). A total of two patients reached the combined endpoint, both of whom underwent AVR at a mean time of 10.82 years after ASO. Freedom from aortic valve reoperation was 98.7 ± 0.9% at 5 and 10 years and 94.7 ± 4% at 15 years. Univariate analysis identified the following risk factors: ventricular septal defect (VSD) (p = 0.005), prior pulmonary artery banding (p < 0.003), and postoperative incidence of mild AR (p < 0.0001). CONCLUSION: AR is not rare after ASO, but it is stable without progressive intensity; new AR developed in 5.2% after 10 years, and is present only in 2.7% after a median follow-up 14.4 ± 0.54 years. If trivial AR is excluded, it is present only in 0.9%. New AR can develop even up to 15 years. However, severe AR and need for AVR are rare.

Retrosternal compression seven years after surgical correction of partial anomalous pulmonary venous connection: Scimitar syndrome.

Seven years after surgical correction of Scimitar syndrome, a 19-year-old patient complained of progressive dyspnea and tachycardia. Transthoracic echocardiography revealed a mass compressing the right ventricle. Magnetic resonance tomogram showed its exact retrosternal location and nature. A computed tomography-guided drainage decompressed the mass. Because of cystic wall persistence, a surgical extirpation of the whole cystic cavity was deemed necessary.

Staged surgical palliation in hypoplastic left heart syndrome and its variants.

BACKGROUND: Surgical options for infants with hypoplastic left heart syndrome (HLHS) and/or its variants are cardiac transplantation or the heart-preserving staged palliation with Norwood operation,followed by a two-staged Fontan procedure. We describe our 17-year experience with staged palliation of HLHS and/or its variants. METHODS: Between December 1989 and December 2006, 64 patients with HLHS and/or its variants underwent a Norwood procedure (mean age/weight, 11.8+/-2.5 days/3.4 kg). Forty-four patients had classical HLHS. Twenty-eight percent had associated congenital cardiac, structural, and genetic anomalies. Subsequently, 25 patients underwent a bidirectional Glenn procedure (stage II) and 11 patients a modified Fontan procedure (stage III). Others await stage II and/or stage III. The follow-up was 143.2 patient-years. RESULTS: Including the learning curve, overall early mortality from 1989 to 1999 after the Norwood procedure was 39.06%. This decreased tremendously for the last seven years, and reduced to 12.8% in 2000 to 2003 until 0% in 2004 to 2006 (p < 0.005). The causes of mortality were sepsis, capillary leak,or heart failure. Three patients died between stages II and III. One patient underwent heart transplantation after the second stage because of heart failure. Among 34 Norwood survivors, four are slightly tachypneic from a mild pulmonary hyperperfusion; one presents symptoms of minimal brain disease. CONCLUSION: This report identified an outcome improvement after staged palliation of HLHS, attributed to an increase in experience and expertise gained over time. Lower operative weight, ascending aortic size, prolonged duration of cardiopulmonary bypass, and hypothermic circulatory arrest were identified to significantly influence early mortality after the Norwood procedure.

Biventricular repair in children with complete atrioventricular septal defect and a small left ventricle.

OBJECTIVE: Biventricular repair of complete atrioventricular septal defect (CAVSD) with small left ventricle aims to restore the normal loading conditions of the left ventricle. This report retrospectively evaluates the outcome of biventricular repair in 19 children with CAVSD and a small left ventricle. METHODS: Our computer database was searched for all patients with CAVSD operated on between January 1988 and December 2005. Patients who underwent biventricular repair of CAVSD were considered for investigation if they had a preoperative left ventricle-to-right ventricle long axis ratio (LAR) of <1.1 as determined by cardiac catheterization. RESULTS: There were 259 patients who underwent surgical correction of complete atrioventricular septal defect. Nineteen (10.3%) of 184 patients who underwent biventricular repair had small left ventricle based on LAR measurements. These children had no other associated congenital heart anomalies and had no previous surgery. Nine of these children had associated Trisomy 21. There were 10 (52.6%) males and 9 (47.4%) females, with age of 1-210 (122+/-67) days and weight of 2.5-9.0 (5.26+/-1.7) kg at surgery. Mean LAR was 0.76+/-0.14. Two patients (10.5%) died on the 8th and 11th postoperative day, respectively. Both had very small left ventricle (LAR of 0.45 and 0.60, respectively) and received ECMO support for postoperative low output syndrome and intractable pulmonary hypertension. A patient with an LAR of 0.62 who had ECMO support for postoperative myocardial failure underwent successful heart transplantation on the 21st postoperative day. The long-term survivors (89.5%) with LAR>0.65 had an uncomplicated postoperative course, had undergone regular follow-up (65+/-36 months) with echocardiographic assessment of the left ventricle, and had good left ventricular function. There was no early reoperation for residual ventricular septal defect, left AV valve regurgitation, or left ventricular outflow tract obstruction. Late reoperation was performed in three patients (17.4%) who underwent mitral valve repair for significant regurgitation in the 18th, 59th, and 87th month postoperatively. CONCLUSIONS: Biventricular repair of CAVSD with small left ventricle in infants and children whose LAR is >0.65, although not without risks, improve patients' functional and clinical status even in long-term follow-up. Particular caution should be taken in patients with LAR of <0.65, since these are patients who may not be amenable to biventricular repair, but for whom univentricular palliation may be more suitable.

Role of paediatric assist device in bridge to transplant.

BACKGROUND: While heart transplantation has gained recognition as the gold standard therapy for advanced heart failure, the scarcity of donor organs has become an important concern. The evolution of surgical alternatives such as ventricular assist devices (VADs), allow for recovery of the myocardium and ensure patient survival until heart transplantation becomes possible. This report elaborates the role of VADs as a bridge to heart transplantation in infants and children (≤18 years old) with end-stage heart failure. METHODS: A retrospective review of the medical records of 201 heart transplant recipients between May 1986 and September 2014 identified 78 children [38.8%; mean age 7.2 (7.8±6.0) years old; IQR: 2.6-11.8 years] with advanced heart failure who were supported with a VAD [left VAD (LVAD) =21; biventricular VAD (BVAD) =57] as a bridge to heart transplantation. Fourteen (17.9%) patients were less than 1 year old; 15 (19.2%) children had a cardiac arrest and underwent cardiopulmonary resuscitation, with 7 of these patients also requiring extracorporeal membrane oxygenation (ECMO) support prior to implantation of a VAD. The aetiology of heart failure was primarily cardiomyopathy (dilative, restrictive from endocardial fibrosis, idiopathic or toxic-induced), reported in 56 (71.8%) patients. The VADs employed were primarily Berlin Heart EXCOR® (n=63), HeartWare (n=13), Berlin Heart INCOR® (n=1), and Toyobo (n=1). RESULTS: Mean duration of VAD support was 59 (133.37±191.57) days (range, 1-945 days; IQR: 23-133 days) before a donor heart became available. The primary complication encountered while patients were being bridged to transplant was mediastinal bleeding (7.8%). The main indication for pump exchanges was thrombus formation in the valves. There was no incidence of technical failure of the blood pump or driving system components. Skin infections around the cannulae occurred in 2.5%. Adverse neurological symptoms (thromboembolism 11.1%, cerebral haemorrhage 3.6%) that occurred did not have any permanent neurological sequelae that could be detected on clinical examination in this study. Mean duration of follow-up was 9.4 (10.3±7.6) years (IQR: 3.74-15.14 years). Cumulative survival rates of patients bridged to transplantation with VAD were 93.6%±2.8%, 84.6%±4.1%, 79.1%±4.7%, 63.8%±6.2%, 61.6%±7.1%, and 52.1%±9.3% at 30 days, 1, 5, 10, 15 and 20 years, respectively. There was no statistically significant difference (P=0.79) in survival rates of patients bridged to heart transplantation with VAD compared to those who underwent primary heart transplantation. Post-transplant survival rates stratified according to the type of VAD implanted and number of ventricles supported were not statistically different (P=0.93 and 0.73, respectively). In addition, post-transplant survival rates were not significantly different when age, gender and diagnosis were adjusted for. Furthermore, no statistically significant difference was found when post-transplant survival rates of children who had episodes of rejection were compared to those who did not have episodes of rejection. CONCLUSIONS: The results in this series demonstrate that VADs satisfactorily support paediatric patients with advanced heart failure from a variety of aetiologies until heart transplantation. The data further suggests that patients bridged with VADs have comparable long-term post-transplant survival as those undergoing primary heart transplantation.

Bridge to recovery in children on ventricular assist devices-protocol, predictors of recovery, and long-term follow-up.

BACKGROUND: The majority of children supported with ventricular assist devices (VADs) are bridged to heart transplantation. Although bridge to recovery has been reported, low recovery patient numbers has precluded systematic analysis. The aim of this study was to delineate recovery rates and predictors of recovery and to report on long-term follow-up after VAD explantation in children. METHODS: Children bridged to recovery at our institution from January 1990 to May 2016 were compared with a non-recovery cohort. Clinical and echocardiographic data before and at pump stoppages and after VAD explantation were analyzed. Kaplan‒Meier estimates of event-free survival, defined as freedom from death or transplantation after VAD removal, were determined. RESULTS: One hundred forty-nine children (median age 5.8 years) were identified. Of these, 65.2% had cardiomyopathy, 9.4% had myocarditis, and 24.8% had congenital heart disease. The overall recovery rate was 14.2%, and was 7.1% in patients with dilated cardiomyopathy. Predictors of recovery were age <2 years (recovery rate 27.8%, odds ratio [OR] 5.64, 95% confidence interval [CI] 2.0 to 16.6) and diagnosis of myocarditis (rate 57.1%; OR 17.56, 95% CI 4.6 to 67.4). After a median follow-up of 10.8 years, 15 patients (83.3%) were in Functional Class I and 3 (16.7%) in were in Class II. Mean left ventricular ejection fraction was 53% (range 28% to 64%). Ten- and 15-year event-free survival rates were both 84.1 ± 8.4%. CONCLUSIONS: Children <2 years of age and those diagnosed with myocarditis have the highest probability of recovery. Long-term survival after weaning from the VAD was better than after heart transplantation, as demonstrated in the excellent long-term stability of ejection fraction and functional class.

Existing issues and valid concerns in continuous-flow ventricular assist devices.

INTRODUCTION: Through concerted endeavours in the fields of surgery, medicine, and biomedical engineering, patronized by government and industry, ventricular assist devices have become an established surgical treatment to support the failing heart, which could easily and simply surpass the frequency of heart transplantation. Be it as it may, complications and adverse events have unfolded, and these facilitated the way to newer biomedically-engineered pump designs in search of the perfect support for a failing heart. Areas covered: This review focuses on the evolution of the continous-flow ventricular assist device as an accepted mode of treatment in advanced heart failure, as a bridge to myocardial recovery, as a bridge to heart transplantation and as a permanent therapy, with its attendant complications and long-term outcome. Expert commentary: Continuous flow devices offer improved durability, less surgical trauma due to their smaller size, high-energy efficiency, and lower thrombogenicity, regardless of administration of single or dual anti-thrombotic therapy. These benefits translate into better survival, lower frequency of adverse events, improved quality of life and higher functional capacity of patients. Through ceaseless innovations and technology, newer pumps have been developed; however, none has been proven to be superior over the others.

Long-term outcome of simultaneous septal myectomy and anterior mitral leaflet retention plasty in hypertrophic obstructive cardiomyopathy: the Berlin experience.

BACKGROUND: Various surgical strategies designed to relieve left ventricular outflow tract obstruction (LVOTO) and correct mitral regurgitation (MR) in hypertrophic obstructive cardiomyopathy (HOCM) have evolved, yet reports on the long-term outcomes of each technique are scarce. We provide an update on over 20 years' experience at our institution in the standardized surgical treatment of HOCM. METHODS: Between April 1986 and April 2014, 320 cases of endomyocardial resection and 305 septal myectomies were performed at our institution. Out of this sample, 57 patients (mean age 38±2.5 years, median 16.2, range 3 months-79.8 years) underwent surgery for HOCM involving septal myectomy and anterior leaflet retention plasty (ALRP), intended to obviate the systolic anterior motion (SAM) phenomenon. The preoperative mean LVOT pressure gradient was 98.98±26.2 (median 90, range 60-160) mmHg with moderate-severe MR. Standard transaortic septal myectomy was performed by resecting long blocks of septal myocardium, continued apically beyond the point of the mitral-septal contact. Through a left atriotomy, the segment of anterior mitral leaflet (AML) closest to the trigones was sutured to the corresponding posterior annulus on both sides. Cardiopulmonary bypass was resumed for repeat septal myectomy if the LVOT pressure gradient was greater than 20 mmHg. RESULTS: Following surgical correction, the mean LVOT pressure gradient was significantly decreased to 12.3±2.7 (median 14, 18-25) mmHg (P<0.001). Septal thickness was reduced from a preoperative mean of 28.2±3.4 (median 30, 25-34) to 10.5±1.1 (12, 15-23) mm (P<0.001). During a mean follow-up of 17.5±1.3 years (median 12, range 1-23.2 years), MR was trivial in 87% and SAM was non-existent in all, outcomes that were maintained at the latest follow-up. Two patients underwent mitral valve (MV) replacement 1 and 5 years after ALRP for recurrent MR. Two patients eventually underwent heart transplantation for end-stage heart failure, 2 and 11 years later, respectively. Twenty-year freedom from repeat MV intervention and cumulative survival rate was 92.9% and 91.2%, respectively. CONCLUSIONS: Long-term follow up of HOCM patients who underwent simultaneous septal myectomy and ALRP showed sustained absence of SAM, attenuation of MI, absence of residual LVOT obstruction and sustained improvement in hemodynamic and functional status.

Extra-anatomical bypass in complex and recurrent aortic coarctation and hypoplastic arch.

OBJECTIVES: Our goal was to report the selection schemes, technical variations and long-term outcome of extra-anatomical bypass to correct complex, recurrent aortic coarctation and hypoplastic aortic arch. METHODS: Between 1989 and 2012, 53 patients (mean age 13.2 ± 4.3, median 11.6, range 9-23 years) with complex aortic coarctation (n = 33; long-segment hypoplastic aortic arch in 15), recurrent coarctation (n = 20; anastomosic pseudoaneurysm in 10), underwent correction using extra-anatomical bypass, either with (n = 18: femoral bypass = 13, left heart bypass = 5) or without (n = 35) extracorporeal circulation via a left lateral thoracotomy (n= 48) and combined median sternotomy and median laparotomy (n = 5). The decision to use extracorporeal circulation was based on the anatomical location of the coarctation, the length of the hypoplasia and a history of previous repair. Preoperatively, mean systolic blood pressure was 130 ± 30 mmHg at rest and 180 ± 40 mmHg during exercise, with a mean pressure gradient of 80 ± 11.6 (range 40-120) mmHg. RESULTS: Various extra-anatomical bypass strategies included left subclavian artery to descending aorta (n = 38), ascending aorta to left subclavian artery (n = 3), ascending aorta to descending aorta (n = 4), aortic arch to descending aorta (n = 3) and ascending aorta to abdominal aorta (n = 5). Graft size (median 18, range 10-26, mm) was chosen according to the diameter of the vessel proximal and distal to the planned graft. No operative deaths, paraplegia or abdominal malperfusion occurred. The mean reduction in systolic blood pressure was 60 ± 25 mmHg without pressure gradients. During a mean follow-up of 18.3 ± 3.7 years, there were no reoperations, graft complications or pseudoaneurysm formation on anastomotic sites. Seven (11.6%) patients are on antihypertensive medications. No patient presented with claudication nor did anyone experience orthostatic problems from the steal phenomenon. CONCLUSIONS: Extra-anatomical bypass is safe, an effective technique, and achieves satisfactory long-term results.

Repair of Parachute and Hammock Valve in Infants and Children: Early and Late Outcomes.

Parachute and hammock valves in children remain one of the most challenging congenital malformations to correct. We report our institutional experience with valve-preserving repair techniques and the early and late surgical outcomes in parachute and hammock valves in infants and children. From January 1990-June 2014, 20 infants and children with parachute (n = 12, median age = 2.5 years, range: 2 months-13 years) and hammock (n = 8, median age = 7 months, range: 1 month-14.9 years) valves underwent mitral valve (MV) repair. Children with parachute valves have predominant stenosis, whereas those with hammock valves often have predominant insufficiency. Intraoperative findings included fused and shortened chordae with single papillary muscles in children with parachute valves. MV repair was performed using annuloplasty, commissurotomy, leaflet incision toward the body of the papillary muscles, and split toward its base. Children with hammock valves have dysplastic and shortened chordae, absence of papillary muscles with fused and thickened commissures. MV repair consisted of carving off a suitably thick part of the left ventricular wall carrying the rudimentary chordae. The degree and extent of incision and commissurotomy is determined by the minimal age-related acceptable MV diameter to avoid mitral stenosis. During a median duration of follow-up of 9.6 years (range: 6.4-21.4 years), cumulative survival rate and freedom from reoperation in parachute valves were 43.7 ± 1.6% and 53.0 ± 1.8%, respectively. In hammock valves, during a median duration of follow-up of 6.7 years (range: 2.7-19.4 years), cumulative survival rate and freedom from reoperation was 72.9 ± 1.6% and 30.0 ± 1.7%, respectively. Age less than 1 year proved to be a high-risk factor for reoperation and mortality (P < 0.005). In conclusion, children with parachute and hammock valves, repeat MV repair may be necessary during the course of follow-up. Infants have a greater risk for reoperation and mortality.

Paediatric mechanical circulatory support with Berlin Heart EXCOR: development and outcome of a 23-year experience.

This paper reviews the development and establishment of the Berlin Heart EXCOR® (BHE®) as a paediatric mechanical circulatory support and reports our entire experience with regard to indications, timing of implantation and explantation and outcome. The Berlin group reported the first successful paediatric bridge to transplantation using a pulsatile pneumatic paracorporeal biventricular assist device, the BHE®, in 1990 in an 8-year-old boy with end-stage heart failure and coarctation of the aorta. This experience prompted them to develop miniaturized pump systems for children through the company Berlin Heart Mediproduct GmbH. The development and production of BHE® to support paediatric patients with heart failure then began. Between 1990 and 2013, the BHE® has been implanted in 122 patients (median age 8.64 years, range 3 days to 17 years) with heart failure, who were inotrope-dependent or switched from extracorporeal membrane oxygenation support or had postcardiotomy low-output syndrome. Thirty-five patients were <1 year old (median 125 days). The aetiology of heart failure included cardiomyopathy in 56 (median age 9.14 years), fulminant myocarditis in 17 (median age 8.2 years), end-stage congenital heart disease in 18 (median age 6.4 years), postcardiotomy heart failure (after correction of congenital heart disease) in 28 (median age 9.6 years) and transplant graft failure in 3 (median age 12.5 years). The overall median duration of implantation was 63.6 (range 1-841) days. Fifty-six children eventually underwent orthotopic heart transplantation. Eighteen patients had myocardial recovery and were weaned successfully. They had entirely normal cardiac function after a range of 4-10 years after surgery. At the time of this report, five patients were still on support, with a duration of 354-369 days. Forty-three patients died on the system from loss of peripheral circulatory resistance, multiorgan damage, sepsis or haemorrhagic or thrombotic complications. Re-exploration because of bleeding was necessary in 22 patients. Pump exchange because of thrombus formation in the valves was necessary 35 times. With the introduction of a modified anticoagulation regimen in 2000, the pump exchange rate has decreased. The BHE® can reliably support the circulation at any age for long periods with good results. It is now an established treatment for children with heart failure of any aetiology.

Cardiomyopathy in Marfan syndrome.

OBJECTIVES: This report aims to evaluate the existence of primary and secondary cardiomyopathy in patients with Marfan syndrome (MFS) who underwent surgical management for primary cardiovascular sequelae of this genetic disorder. Likewise, we aim to determine whether the myocardium in MFS is susceptible to ischaemia independent of myocardial protection used during surgery. METHODS: Between April 1986 and May 2012, 421 patients with MFS were surgically treated for cardiovascular manifestations. Among them, 47 (mean age: 39.45 ± 12.64, median: 36, range: 19-66, years) eventually were surgically treated for cardiomyopathy. They were grouped into A: patients who subsequently developed ischaemic cardiomyopathy and eventually underwent coronary revascularization for coronary artery disease (n = 11); B: patients who subsequently developed end-stage cardiomyopathy for which a mechanical circulatory support device was implanted to support the failing heart (n = 13) and C: patients who subsequently developed end-stage cardiomyopathy (n = 23), among whom 21 underwent primary heart transplantation, while 2 patients are still waiting for donor hearts. RESULTS: Retrospective analysis of the medical records of 47 patients revealed the following: In Group A, 3 (27.2%) patients had already existing ischaemic cardiomyopathy before the first various cardiovascular surgeries, while ischaemic cardiomyopathy in the other 8 (72.7%) developed postoperatively. The interval between previous surgery and development of cardiomyopathy was a mean of 8.0 ± 07 years. In Group B, 5 (38.4%) had existing primary cardiomyopathy prior to surgery, while 8 (61.5%) developed end-stage cardiomyopathy postoperatively. The interval between previous surgery and development of cardiomyopathy was a mean of 9.0 ± 4 months. In Group C, 5 (21.7%) had been diagnosed with cardiomyopathy prior to the cardiovascular surgery, while 18 (78.2%) developed end-stage cardiomyopathy postoperatively. The mean interval between previous surgery and development of cardiomyopathy was 3 ± 0.9 years. At a mean follow-up of 9.4 ± 1.37 years, the overall survival rate is 51.8%. Categorized based on the surgical treatment done for cardiomyopathy, survival rates of 54.5% (the mean follow-up of 9.35 ± 1.8 years), 40.1% (mean follow-up of 7.01 ± 2.8 years) and 70% (mean follow-up of 10.5 ± 2.0 years) were seen in Groups A, B and C, respectively. There is no significant difference in survival rates (P = 0.56) among groups. Likewise, the type of myocardial protection and duration of ischaemic times were not significant (P > 0.78) to the development of cardiomyopathy. CONCLUSIONS: Our finding supports the existence of cardiomyopathy in a subset of patients with MFS. Marfan cardiomyopathy appears to be independent of the type of myocardial protection and duration of ischaemia.

Rotary Blood Pumps as Long-Term Mechanical Circulatory Support: A Review of a 15-Year Berlin Experience.

This article reports our 15-year single-center experience with rotary blood pumps (RBPs) as long-term mechanical circulatory support (MCS) with emphasis on outcomes. For more than 15-year period, we have used various RBPs as bridge to transplantation or to myocardial recovery. Our group performed the first human implantation worldwide of RBCs, the MicroMed DeBakey ventricular assist device in November 1998 in a patient with end-stage heart failure who was supported for 47 days until his death. Based on this initial experience, we recognized the feasibility of providing long-term support and since then it has been our primary armamentarium in treating patients with heart failure. Between 1987 and September 2013, we have implanted 2208 ventricular assist devices ranging from pulsatile to continuous-flow systems, as short-term, long-term, or permanent support in patients with end-stage heart failure. In total, 1009 RBPs were implanted on 908 patients, and their outcomes are reported here. We have shared some milestones in MCS including the first implantation of Jarvik 2000 on the oldest patient (81-year old) in 2008 and the first worldwide implantation of a biventricular HeartWare. Over time, implantation techniques, anticoagulation, and postoperative care have been modified and individualized. A relevant aspect of our experience has been the incidence of pump thrombosis. This is particularly frustrating because the problem has occurred in the setting of full anticoagulation and antiplatelet therapy, guided by strict anticoagulation monitoring. It has become clear to us that the devices are still not perfect. Technical pump failures such as cable breaks also occur, prompting urgent pump exchange, and infection. A 15-year cumulative mortality rate is 46.9%. This report emphasizes that MCS with RBPs has evolved into a routine treatment in heart failure and is a highly feasible option for permanent therapy particularly for elderly patients.

Primary Cardiac Tumors in Infants and Children: Surgical Strategy and Long-Term Outcome.

BACKGROUND: Primary cardiac tumors in infants and children are extremely rare; hence, there is very little literature available, and most knowledge is based on collections of case reports. This report is a comprehensive review of our 26-year experience with primary cardiac tumors in children with emphasis on surgical indications, strategies, and long-term outcome. METHODS: Between 1986 and 2012, 47 children (mean age 5.9 ± 2.4 months; range, 1 day to 17 years) underwent either subtotal or total resection of cardiac tumors (rhabdomyoma, 13; fibroma, 12; teratoma, 9; myxoma, 8; hemangioma, 2; rhabdomyosarcoma, 1; non-Hodgkin's lymphoma, 1; lymphangioma, 1). The majority were diagnosed by echocardiography (n = 33). Clinical patterns were varied: 40 had an atypical heart murmur and 6 were asymptomatic. Outflow tract obstruction of more than 30 mm Hg was present in 11 children. Three patients had abnormal coronary arteries secondary to pressure from tumor bulk. Indications of resection were hemodynamic/respiratory compromise, severe arrhythmia, and a significant embolization risk. Strategy of resection varied according to location and hemodynamic status without damage to adjacent structures. RESULTS: Morbidity included bleeding in a patient and a transient low output state in another. A 5-month-old infant with left ventricular fibroma underwent left ventricular assist device implantation secondary to failure from weaning off cardiopulmonary bypass, and she eventually underwent heart transplantation 17 days later. Early mortality (n = 2, 4.2%) included a 5-month-old infant who underwent complete resection of rhabdomyoma located in the left ventricle, with concomitant pulmonary valve replacement; unfortunately, he underwent left ventricular assist device implantation for postoperative heart failure and died on the 13th postoperative day. An 8-month-old child with 3 cm × 4 cm fibroma obstructing the right ventricular outflow tract compressing the right coronary artery died of severe right-side heart failure on the 13th postoperative day. One late death (2.1%) occurred; a 16-year-old with non-Hodgkin's lymphoma died 7 months after the surgery. Mean duration of follow-up is 11.6 ± 3.5 years. All survivors (93.4%) are well, free of tumor-related symptoms and tumor recurrence/progression, even when resection was incomplete. CONCLUSIONS: This study illustrates that although primary cardiac tumors in infants and children have a wide and unusual spectrum of clinical presentation, an individualized approach to tumor resection allows restoration of an adequate hemodynamic function and satisfactory long-term, tumor-free outcome.