Left ventricular assist device support with a centrifugal pump for 2 months in a 5-kg child.

The mid-term and long-term results of left ventricular assist device (LVAD) implantation for small children are still unsatisfactory. There have been few reports of LVAD implantation for more than a month in children weighing under 5 kg. We report the case of a 4-month-old female infant who survived for 2 months after being diagnosed with dilated cardiomyopathy (DCM) with extracorporeal centrifugal pump support. In recent years, although pumps designed for small children have been introduced and are used as a bridge to transplantation or recovery, mid-term or long-term mechanical support for small children with heart failure is still difficult. We managed to successfully provide support for a low-body-weight child with a centrifugal pump over a mid-term period. We achieved acceptable control of thrombosis, but eventually the infant died of sepsis. Autopsy revealed no prominent thrombosis in the perfusion cannula, drainage cannula, the pump, or the left ventricle. This is the first case report of LVAD support with the centrifugal pump, ROTAFLOW(®) (Maquet, Rastatt, Germany), for 2 months in a child weighing under 5 kg. Our method may potentially save severe heart failure children who need mid-term LVAD support.

Mechanism of pressure-overload right ventricular hypertrophy in infant rabbits.

Although pressure-overload right ventricular hypertrophy is a long-term risk in some congenital heart diseases such as tetralogy of Fallot, how it develops is unclear. The aim of this study was to investigate the mechanism of development of this right ventricular heart failure.Pulmonary artery banding in 10-day-old rabbits induced pressure-overload right ventricular hypertrophy as they grew. Comparisons were made with age-matched sham controls (n = 24 per group). In weekly serial echocardiography, the right ventricular contraction and diastolic function decreased from 3 weeks after surgery (P < 0.01), and the right ventricle became hypertrophic from 4 weeks after (P < 0.05). Pressure-overload increased cardiomyocyte apoptosis from 4 weeks postoperatively (TUNEL staining and Western blotting analysis, P < 0.05); and fibrosis occurred in the right ventricular cardiomyocytes at 8 weeks after operation (Masson's trichrome stain, P < 0.01). In our model, pressure-overload to the right ventricle caused the right ventricular disorder, hypertrophy, and fibrosis. Apoptosis of right ventricular cardiomyocytes was involved in progression. We have shown for the first time the mechanism whereby pressure-overload right ventricular hypertrophy develops in an infant rabbit model.

Left ventricular mechanical support with Impella provides more ventricular unloading in heart failure than extracorporeal membrane oxygenation.

The Impella microaxial-flow pump can directly unload left ventricle (LV) in cases of acute heart failure. Extracorporeal membrane oxygenation (ECMO) is widely used for circulatory support. Although the clinical effectiveness of ECMO has been demonstrated, insufficient LV loading reduction may not be advantageous for myocardial recovery. The objective was to compare ventricular loading reduction and reversibility of ventricular fibrillation (VF) with either Impella or ECMO. Six dogs were used. Extracorporeal membrane oxygenation was established by the femoral artery and right atrium. The Impella LD was inserted in LV by the ascending aorta. An acute failing heart was created by sequential coronary artery ligations. Pressure-volume (PV) relationships were acquired without a device and with ECMO or Impella. When VF occurred, direct cardioversion was performed while supported by either ECMO or Impella. The PV area, which is a measure of ventricular unloading and is correlated with myocardial oxygen consumption, decreased more with Impella than with ECMO. Successful defibrillation was achieved more effectively while under Impella support. Superior ventricular unloading with the Impella device may provide higher recovery potential to damaged hearts than ECMO and may have a significant impact not only on intensive care of patients with heart failure but also on resuscitation.

Precise evaluation of bilateral pulmonary artery banding for initial palliation in high-risk hypoplastic left heart syndrome.

OBJECTIVES: In patients with high-risk hypoplastic left heart syndrome (HLHS), the Norwood operation (NW) in the neonatal period still results in high mortality compared with other cardiac surgery. Bilateral pulmonary artery banding (bPAB), a very effective initial procedure for HLHS, for which the specific evaluation is as yet unsatisfactory, was performed, and we report our findings in the present study. METHODS: We have performed bPAB since 2006. A total of 17 patients with HLHS or a variant underwent bPAB before the NW. Echocardiography was performed between bPAB and the NW, and the flow acceleration just after bPAB and before NW was evaluated. Before the NW, a catheter examination was also performed. RESULTS: bPAB was performed at 6.6 ± 0.6 days of age, and the NW at 130 ± 88 days. The patients' mean body weight (BW) was 2.5 ± 0.4 kg at bPAB and 4.0 ± 1.1 kg at the NW. The length of the tape for bPAB was 9.9 ± 0.6 mm in the right pulmonary artery (RPA) and 9.4 ± 0.6 mm in the left (LPA) because the RPA was usually wider than the LPA. The tape width was 2 mm in all cases. The catheter examination was performed at 95 ± 85 days after bPAB. The arterial oxygen saturation (SaO2) was 71% ± 8.6%. Multivariate regression analysis revealed that SaO2 was estimated well using 4 factors: the banding size of the RPA, BW at bPAB, BW at NW, and BW in the period between bPAB and catheter examination (R² = 0.79). Echocardiography just after bPAB showed that the blood flow at the bPAB had accelerated to 3.0 ± 0.8 m/s in the RPA and 3.3 ± 0.8 m/s in the LPA (P = .004). The estimated pressure gradient was 39.2 ± 17.6 mm Hg in the RPA and 46.1 ± 23.0 mm Hg in the LPA (P = .006). The blood flow at bPAB was accelerated to 3.7 ± 0.7 m/s in the RPA and 4.0 ± 0.6 m/s in the LPA before NW (P = .013). The estimated pressure gradient was 62.6 ± 27.6 mm Hg in the RPA and 56.1 ± 19.6 mm Hg in the LPA before NW (P = .014). The catheter examination revealed mean wedge pressures of 18.0 ± 7.2 mm Hg for the RPA and 16.2 ± 4.3 mm Hg for the LPA. The operative mortality rate was 0%. One patient required a repeat operation to adjust the bPAB, and prolonged pleural effusion was observed in 1 case. CONCLUSIONS: The postoperative SaO2 after bPAB correlated closely with the banding size and BW at bPAB, NW and during the period after bPAB. Because the mean PA pressure before NW was low enough for single ventricular circulation, the bPAB in this study was an effective option for high-risk patients undergoing HLHS or a variant. We believe the bPAB sizes used were suitable and were determined as follows: BW plus 7 mm for the LPA and BW plus 7.5 mm for the RPA.

Development of left ventricular diastolic dysfunction with preservation of ejection fraction during progression of infant right ventricular hypertrophy.

BACKGROUND: Progressive left ventricular (LV) dysfunction can be a major late complication in patients with chronic right ventricular pressure overload (eg, tetralogy of Fallot). Therefore, we examined LV function (serial echocardiography and ex vivo Langendorff) and histology in a model of infant pressure-load right ventricular hypertrophy (RVH). METHODS AND RESULTS: Ten-day-old rabbits (n=6 per time point, total n=48) that underwent pulmonary artery banding were euthanized at 2 to 8 weeks after pulmonary artery banding, and comparisons were made with age-matched sham controls. LV performance (myocardial performance index) decreased during the progression of RVH, although the LV ejection fraction was maintained. In addition, RVH caused significant septal displacement, reduced septal contractility, and decreased LV end-systolic and end-diastolic dimensions, resulting in LV diastolic dysfunction with the appearance of preserved ejection fraction. Significant septal and LV free-wall apoptosis (myocyte-specific TUNEL and activated caspase-3), fibrosis (Masson trichrome stain), and reduced capillary density (CD31 immunostaining) occurred in the pulmonary artery banding group after 6 to 8 weeks (all P<0.05). CONCLUSIONS: This is the first study showing that pressure overload of the right ventricular resulting in RVH causes LV diastolic dysfunction while preserving ejection fraction through mechanical and molecular effects on the septum and LV myocardium. In particular, the development of RVH is associated with septal and LV apoptosis and reduced LV capillary density.

Beating-heart patch closure of muscular ventricular septal defects under real-time three-dimensional echocardiographic guidance: a preclinical study.

OBJECTIVES: Safe and effective device closure of ventricular septal defects remains a challenge. We have developed a transcardiac approach to close ventricular septal defects using a patch delivery and fixation system that can be secured under real-time three-dimensional echocardiographic guidance. METHODS: In Yorkshire pigs (n = 8) a coring device was introduced into the left ventricle through a purse-string suture placed on the left ventricular apex, and a muscular ventricular septal defect was created. The patch deployment device containing a 20-mm polyester patch was advanced toward the ventricular septal defect through another purse-string suture on the left ventricular apex, and the patch was deployed under real-time three-dimensional echocardiographic guidance. The anchor delivery device was then introduced into the left ventricle through the first purse-string suture. Nitinol anchors to attach the patch around the ventricular septal defect were deployed under real-time three-dimensional echocardiographic guidance. After patch attachment, residual shunts were sought by means of two-dimensional and three-dimensional color Doppler echocardiography. The heart was then excised, and the septum with the patch was inspected. RESULTS: A ventricular septal defect was created in the midventricular (n = 4), anterior (n = 2), and apical (n = 2) septum. The mean size was 9.8 mm (8.2-12.0 mm), as determined by means of two-dimensional color Doppler scanning. The ventricular septal defects were completely closed in 7 animals. In one a 2.4-mm residual shunt was identified. No anatomic structures were compromised. CONCLUSIONS: Beating-heart perventricular muscular ventricular septal defect closure without cardiopulmonary bypass can be successfully achieved by using a catheter-based patch delivery and fixation system under real-time three-dimensional echocardiographic guidance. This approach might be a better alternative to cardiac surgery or transcatheter device closure.

Fate of equine pericardial roll conduit for Rastelli operation during long-term follow-up.

BACKGROUND: Right ventricular outflow tract obstruction is a frequent condition after Rastelli operation. Although several modifications have been reported elsewhere, ideal conduit has not been developed yet during long-term follow-up. We reviewed our experiences over 15-year long-term follow-up with patients who underwent Rastelli operation using house-made equine pericardial roll graft. METHODS: Since June 1981, 16 patients underwent Rastelli operation with the pericardial roll graft. Median follow-up time was 15.6 years (7.3-26.8 years). RESULTS: Twelve out of 16 patients using pericardial roll graft with (n = 6) or without (n = 6) cusps underwent 13 reoperations during the follow-up period. Median time from first Rastelli to re-do operation was 8.4 years with median time to reoperation of 8 years. Major indication for reoperation was conduit obstruction (n = 10), but not conduit regurgitation. Conduit problem includes kinking and compression of the graft. Reoperation procedures include 7 Danielson procedures, 2 patch augmentations, 1 homograft replacement, 1 pericardial roll graft, 1 expanded polytetrafluoroethylene tube graft replacement, and 1 patch closure for pulmonary artery aneurysm. Balloon angioplasty was not effective for pericardial roll conduit stenosis. CONCLUSION: We conclude that house-made equine pericardial roll graft was durable for certain time period, but conduit change may be inevitable. Because of excellent handling and wide application, further modification may be warranted.

Surgical outcome of heterotaxy syndrome in a single institution.

Heterotaxy syndrome encompasses a wide range of anatomical variants including poor pulmonary vascular development. Surgical outcome remains poor in this difficult subgroup. Between April 1996 and November 2004, 27 patients with visceral heterotaxy were enrolled in this study. The median age at presentation was 25 days. There were 11 patients with asplenia and 16 with polysplenia. There were 5 deaths (4 in patients with asplenia, 1 in a patient with polysplenia). All patients with asplenia were scheduled for single-ventricle repair, and 5 completed a modified Fontan procedure. Of the 16 patients with polysplenia, 8 completed 2-ventricle repair and 6 had a modified Fontan. In polysplenia, actuarial survival was 93.8% at 4 months, and remained constant thereafter. In asplenia, survival was 81.8% at 1 month, and 53.0% at 1 year. A significant correlation was found between nonconfluent pulmonary arteries and mortality. The medium-term result in polysplenia was satisfactory, but not in asplenia. Nonconfluent pulmonary arteries carry a high risk of mortality, and a strategy to create pulmonary vascular confluence and satisfactory pulmonary blood flow is mandatory.

Retrograde cerebral perfusion with intermittent pressure augmentation provides adequate neuroprotection: diffusion- and perfusion-weighted magnetic resonance imaging study in an experimental canine model.

OBJECTIVE: Diffusion- and perfusion-weighted magnetic resonance imaging can identify ischemic brain injury in the hyperacute stage. For neuroprotection during thoracic aortic surgery, we developed a novel retrograde cerebral perfusion with intermittent pressure augmentation. The purpose of this study was to assess the efficiency of this novel method for neuroprotection in real time by using diffusion- and perfusion-weighted magnetic resonance imaging. METHODS: Sixteen beagle dogs were randomly divided into 4 groups: the antegrade selective cerebral perfusion group (n = 4; antegrade selective cerebral perfusion at a flow rate of 10 mL x kg(-1) x min(-1)); the intermittent retrograde cerebral perfusion group (n = 4; retrograde cerebral perfusion at a baseline pressure of 15 mm Hg with intermittent pressure augmentation to 45 mm Hg every 30 seconds); the conventional retrograde cerebral perfusion group (n = 4; conventional retrograde cerebral perfusion at a fixed pressure of 25 mm Hg); and the circulatory arrest group (n = 4; only circulatory arrest). Diffusion- and perfusion-weighted magnetic resonance images were acquired during each session of cerebral perfusion. Regions of interest were defined, and the apparent diffusion coefficient and relative regional cerebral blood volume were calculated in these regions of interest. Finally, the brain was evaluated for its histopathologic damage score. RESULTS: The best apparent diffusion coefficient values were observed in the intermittent retrograde cerebral perfusion group in all the regions of interest, although the relative regional cerebral blood volume values were mostly lower than those in the antegrade selective cerebral perfusion group. The total Histopathologic Damage Score (0, normal; 32, worst) in the intermittent retrograde cerebral perfusion group (8.0 +/- 0.6) was significantly lower than that in the conventional retrograde cerebral perfusion (17.5 +/- 1.7; P < .01) and circulatory arrest (25 +/- 1.0; P < 0.01) groups and was equivalent to that in the antegrade selective cerebral perfusion group (7.8 +/- 0.8; P = .9). CONCLUSION: Intermittent retrograde cerebral perfusion provides adequate neuroprotection by allowing high apparent diffusion coefficient values to be maintained.

Intermittent pressure augmentation during retrograde cerebral perfusion under moderate hypothermia provides adequate neuroprotection: an experimental study.

OBJECTIVE: For cerebral protection during aortic surgery, we introduced a novel retrograde cerebral perfusion method with intermittent pressure augmentation. We then assessed whether this novel method provides benefits similar to those provided by antegrade selective cerebral perfusion. METHODS: Eighteen dogs were randomly divided into 3 groups: the RCP-INT group, intermittent-retrograde cerebral perfusion at 15 mm Hg with intermittent pressure augmentation to 45 mm Hg (n = 6); the ASCP group, antegrade selective cerebral perfusion at a flow rate of 10 mL x kg(-1) x min(-1) (n = 6); and the sham group, no circulatory arrest (n = 6). Cooling (26 degrees C) with cardiopulmonary bypass and 60 minutes of circulatory arrest were performed in the RCP-INT and ASCP groups. The levels of tau protein in the cerebrospinal fluid and the diameters of the retinal vessels were measured. The neurologic deficit scores and the histopathologic damage scores of the brains were determined. RESULTS: The total postoperative tau protein levels (calculated as the area under the curve) did not differ significantly between the RCP-INT and ASCP groups (203 +/- 87 pg x mL(-1) x h vs 154 +/- 69 pg x mL(-1) x h, P = .95). The retinal vessels were effectively dilated at an augmented pressure of 45 mm Hg in the RCP-INT group. The total neurologic deficit score (0 = normal, 500 = brain death) and histopathologic damage score (0 = normal, 40 = worst) were not significantly different between the RCP-INT and ASCP groups (neurologic deficit score: 75 +/- 21 vs 70 +/- 21, P = .98; histopathologic damage score: 13.5 +/- 1.5 vs 14.2 +/- 1.3, P = .84). CONCLUSIONS: Intermittent augmented pressure dilated the cerebral vessels, allowing adequate blood supply without injuring the brain. This retrograde cerebral perfusion method provides adequate neuroprotection during moderate hypothermia.

Erythropoietin protects the central nervous system during prolonged hypothermic circulatory arrest: an experimental study in a canine model.

OBJECTIVE: Current data suggest that erythropoietin protects the brain and the spinal cord from ischemic and traumatic injury. In this study, we determined whether erythropoietin protects the central nervous system during prolonged hypothermic circulatory arrest in an experimental canine model. METHODS: Ten adult beagle dogs were randomly and intravenously injected with either 5000 U/kg recombinant human erythropoietin or normal saline. Each dog was then subjected to a cardiopulmonary bypass and 120 minutes of deep hypothermic circulatory arrest (18 degrees C). The level of tau proteins in the cerebrospinal fluid, a modified marker of neurologic deficit in dogs, and the histopathologic characteristics of the brains and spinal cords were then examined. RESULTS: The level of tau proteins was significantly lower in the erythropoietin-treated group than in the untreated group at 6 hours (20 +/- 12 vs 144 +/- 54 pg/mL; P = .036) and 12 hours (64 +/- 35 vs 478 +/- 103 pg/mL; P = .01) after the operation. The total Neurologic Deficit Score was 59 +/- 31 (0, normal; 500, brain death) in the erythropoietin-treated group, compared with 376 +/- 30 in the untreated group (P = .0117). Histopathologic examination revealed that ischemic neuronal changes and apoptosis in the hippocampus CA1 were significantly lower in the erythropoietin-treated group (P < .01 and P = .028, respectively). CONCLUSIONS: This study showed that erythropoietin protected the central nervous system during prolonged hypothermic circulatory arrest, partly by preventing both necrosis (ischemic neuronal changes) and apoptosis.

Prevention of postoperative pericardial adhesions with a novel regenerative collagen sheet.

BACKGROUND: Postoperative pericardial adhesions make a repeat sternotomy time-consuming and dangerous. The purpose of this study was to evaluate the efficacy of a new collagen pericardial substitute for preventing postoperative pericardial adhesions. METHODS: Our absorbable substitute consists of three layers: a middle layer of aterocollagen between two layers of sodium hyaluronic acid and aterocollagen. In experiment 1 in this study, the patch, made of 9,000 filaments of aterocollagen fibers, (group 1; n = 5) was compared with a patch made of 6,000 filaments (group 2; n = 7), an expanded polytetrafluoroethylene sheet (group 3; n = 6), and a control group (group 4; n = 4). Subsequently, in experiment 2, the patch was examined at 4 weeks (n = 5), 12 weeks (n = 5), and 24 weeks (n = 4) after the operation by light microscopy and scanning electron microscopy. RESULTS: The area of adhesion in group 1 was significantly less as compared with that in the other three groups, and the coronary vessels were clearly identifiable; on the other hand, all the animals in the control group showed moderate to severe adhesions, and the coronary vessels were completely obscured. In experiment 2, formation of a membranous tissue resembling the native pericardial membrane was observed in all animals, and the thickness of this membrane showed a marked increase by 24 weeks after the operation. Light microscopy and scanning electron microscopy also showed the formation of a mesothelium-like lining. CONCLUSIONS: The new absorbable and regenerative collagen patch seemed to be biocompatible, and its use was associated with minimal adhesion formation and preserved coronary anatomy.

A novel protocol of retrograde cerebral perfusion with intermittent pressure augmentation for brain protection.

OBJECTIVES: We examined a novel protocol of retrograde cerebral perfusion with intermittent pressure augmentation to improve the clinical usefulness of this procedure, in a canine model, because a high retrograde cerebral perfusion pressure may be required to open cerebral vessels. METHODS: Eighteen dogs (25.2 +/- 4.1 kg) were randomly divided into the following 3 groups: circulatory arrest group (circulatory arrest alone), conventional-retrograde cerebral perfusion group (conventional retrograde cerebral perfusion at 25 mm Hg), and intermittent-retrograde cerebral perfusion group (retrograde cerebral perfusion at 15 mm Hg with intermittent pressure augmentation to 45 mm Hg). The animals were cooled down to 26 degrees C under cardiopulmonary bypass and underwent 60 minutes of circulatory arrest with or without retrograde cerebral perfusion in accordance with the protocol described. They were weaned from cardiopulmonary bypass after rewarming and observed for 12 hours after the procedures. The retinal vessels were observed as a means of noninvasive direct visualization of the cerebral vascular system. The level of Tau proteins in the cerebrospinal fluid was measured as a marker of neuronal damage. RESULTS: While the retinal vessels were fully distended with blood (100%) at a retrograde cerebral perfusion pressure of 45 mm Hg in the intermittent-retrograde cerebral perfusion group, full distension of the retinal vessels was not observed in the conventional-retrograde cerebral perfusion group (67%). The level of Tau proteins, measured 12 hours after the operation, was lower in the intermittent-retrograde cerebral perfusion group (247 +/- 70 pg/mL) than in the circulatory arrest group (1313 +/- 463 pg/mL; P < .05) or the conventional-retrograde cerebral perfusion group (1449 +/- 693 pg/mL; P < .05). Histopathologic examination revealed that the most effective brain protection was obtained in the intermittent-retrograde cerebral perfusion group (P < .05). CONCLUSIONS: Intermittent-retrograde cerebral perfusion effectively opens up cerebral vessels to allow adequate blood supply to the brain, thereby minimizing brain damage. This novel method may protect the cerebral system effectively from ischemia during circulatory arrest.

Total cavopulmonary connection in a bedridden patient with Sotos syndrome.

Sotos syndrome is an overgrowth disorder of unknown etiology associated with a high incidence of congenital heart defects. Of 60 patients with Sotos syndrome treated in our hospital, 6 had congenital heart defects. We describe a case of successful total cavopulmonary connection at 30 months of age in a patient having pulmonary atresia with intact ventricular septum and a patent ductus arteriosus who had walking disability. The postoperative course was uneventful.