A case of a large type IV aortico-left ventricular tunnel successfully repaired at 1 day after birth.

A rare case of a patient with a large type IV aortico-left ventricular tunnel who underwent successful repair at 1 day after birth was described. To the best of our knowledge, no such cases that aortic opening was over 10 mm have been reported in the English literature. Our case demonstrated no leak or significant aortic regurgitation by a two-patch repair.

[Ross-Konnno Procedure for the Patient of Intractable Subaortic Stenosis after Operation of Double Outlet Right Ventricle with Coarctation of the Aorta and Subaortic Stenosis:Report of a Case].

Recurrent left ventricular outflow obstruction (LVOTO) after intraventricular rerouting (IVR) with ventricular septal defect (VSD) enlargement and aortic arch repair for double-outlet right ventricle (DORV), non-committed VSD and coarctation of the aorta was successfully performed by a Ross-Konno procedure at 1 year 6 months as the second reoperation. Ross-Konno procedure could be an effective option in treatment of recurrent LVOTO after IVR for DORV.

Staged Biventricular Repair-Oriented Strategy in Borderline Biventricular Repair Candidates with Ventricular Septal Defect.

Although borderline biventricular repair (BVR) candidates unsuitable for primary BVR are often subjected to single-ventricle repair (SVR), some of them reach BVR by staged strategy. We evaluated our staged BVR-oriented strategy in borderline BVR candidates with ventricular septal defect (VSD) in whom a BVR/SVR decision was deferred beyond the neonatal period. Forty-two patients were treated with the approach between 1991 and 2012. They had been followed toward BVR until it was judged impossible. Outcomes, time course toward definitive repair (DR: BVR, SVR, or 1 + 1/2 ventricle repair), and hemodynamics were reviewed. A total of 57 palliative surgeries were performed before BVR or bidirectional Glenn (BDG), namely procedures to control pulmonary blood flow in 40, to improve mixing in 5, and to promote left ventricle (LV) growth in 5. LV growth was achieved in four patients. There were three cardiac deaths. Except for four awaiting patients, 19 reached BVR (50 %), 11 patients were converted to other than BVR, and 28 patients achieved DR (74 %) at the median age of 30.9 months. Cardiac cath before BVR or BDG performed at the median age of 22.5 months revealed well-preserved pulmonary vasculature with the median pulmonary artery pressure of 14 mmHg, except three patients unsuitable for SVR. In conclusion, our staged BVR-oriented strategy required longer time course and more complex palliative surgeries compared with a simple SVR strategy. Leaving open the possibility of a late crossover to an SVR pathway is mandatory when adopting staged BVR-oriented strategy in these complex patients.

Changes in Plasma Atrial and Brain Natriuretic Peptide Levels in Children Undergoing Surgical Isolated Atrial Septal Defect Closure.

Plasma atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) levels increase after cardiopulmonary bypass (CPB) in pediatric patients. However, the exact reason for the postoperative increase remains unclear. This study elucidated the perioperative changes in plasma natriuretic peptide levels in children undergoing surgical isolated atrial septal defect (ASD) closure. Between 2010 and 2012, 24 pediatric patients (median 7.1, range 2.7-15.7 years) underwent surgery for simple ASD using CPB under ventricular fibrillation (Group A, 16 patients) or under cardiac arrest (Group B, 8 patients). Natriuretic peptide levels were measured before surgery, on postoperative day 0, 1, 3, and at the first outpatient visit. The pulmonary to systemic blood flow ratio (Qp/Qs) was estimated by echocardiography using an index of right ventricle end-diastolic area. Preoperative natriuretic peptide levels positively correlated with the Qp/Qs. Plasma ANP levels peaked on postoperative day 0, and its values were higher in Group A than in Group B patients (p < 0.001). Plasma BNP levels increased significantly in both Groups on postoperative day 1, and its values were significantly greater in Group A than in Group B patients (p = 0.007). There was a weak negative correlation between the amount of postoperative increase in natriuretic peptide levels and the Qp/Qs. There was no appreciable difference in the acute postoperative clinical course and echocardiographic parameter on postoperative day 3 between Group A and B patients. In conclusion, acute postoperative natriuretic peptide levels after isolated ASD closure were multifactorial, and they might be unreliable for predicting clinical outcomes.

Repair of infracardiac total anomalous pulmonary venous connection in a 902-gram infant with asplenia syndrome after failed umbilical venous catheter intervention.

Ductus venosus stenting via a transumbilical approach for pulmonary venous obstruction in infracardiac total anomalous pulmonary venous connection has been described. In a 902-gram infant who was diagnosed with asplenia syndrome and infracardiac total anomalous pulmonary venous connection, ductus venosus stenting was attempted by a transumbilical approach. However, ductus venosus stenting was discontinued due to bleeding from the portal vein. The bleeding subsided in time spontaneously, and total anomalous pulmonary venous connection repair with pulmonary artery banding was performed on 21 days after birth. To our knowledge, this is the first report that describes total anomalous pulmonary venous connection repair in a neonate under 1000 g body weight. Learning objective: Ductus venosus stenting is an effective palliative option, especially in the presence of high surgical risk, such as heterotaxy syndrome and a low birth weight. However, ductus venosus stenting should carefully be evaluated by assessment of anatomical configuration of umbilical vein and ductus venosus. If ductus venosus stenting is anatomically difficult, primary surgical repair may be an option even in an extremely low birth weight infant.

Long-term results of one-and-a-half ventricle repair with open azygos vein.

BACKGROUND: The one-and-a-half ventricle repair (1.5VR) is an option for definitive surgery of cardiac defects with hypoplastic right ventricle (RV). The 1.5VR with open azygos vein was reported to provide a theoretical advantage of decompressing the supra vena cava (SVC) or the right atrium in patients with SVC hypertension or severe RV dysfunction. The aim of this study is to review and evaluate our experience with this procedure in the long-term period. METHODS: Medical records of the patient undergoing 1.5VR with open azygos vein between January 2000 and December 2019 were reviewed retrospective. Pre- and postoperative echocardiography and cardiac catheterization data were also analyzed. RESULTS: 4 patients underwent the 1.5VR with open azygos vein. The median age at time of surgery was 2.2 years, and the median weight was 9.8 kg. The median tricuspid valve (TV) Z-score was - 3.5, the median right ventricular end-diastolic volume (RVEDV) was 54.0% of normal. There were no operative death and early adverse events. Median follow-up time was 17.1 years. There were no late death and re-operation during the follow-up. The postoperative TV diameter and RVEDV were larger than preoperative data in 3 of 4 patients. CONCLUSIONS: The 1.5VR with open azygos vein is a good surgical option for congenital heart disease with hypoplastic RV. The TV and RVEDV may grow and biventricular conversion may be possible.

Repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) by sinus pouch technique.

A 5-month-old girl, weighing 5.3 kg, diagnosed ALCAPA underwent emergency surgery. The left coronary artery (LCA) originated from the posterior pulmonary artery (PA), and the left main trunk (LMT) was very short (1.5 mm), with moderate level of mitral valve regurgitation (MR). The distance from the origin to the pulmonary valve (Pv) was also short. A free extension conduit was created using adjacent sinus Valsalva flaps and implanted in the ascending aorta to avoid distortion of the coronary artery and the Pv.

Atrioventricular groove patch plasty in an infant with pulmonary stenosis after arterial switch operation: a case report.

A 9-month-old infant developed pulmonary stenosis (PS) after an arterial switch operation for transposition of the great arteries, accompanied by a Shaher Type 4 coronary anatomy. As the right coronary artery (RCA) ran across the anterior side of the right ventricle (RV), atrioventricular (AV) groove patch plasty was performed to relieve PS. The distance between the RCA and tricuspid valve was confirmed by preoperative-computed tomography. The AV groove was carefully incised, ensuring the position of the tricuspid valve, and maintaining a distance of 3 mm from the tricuspid annulus to avoid approaching the RCA. While suturing the monocuspid valve patch, only the endocardial side of the RV was sutured, and RCA injury was prevented. Thus, especially in patients < 1 year of age, careful incision of the AV groove and suturing only the endocardial side is important to avoid injuring the RCA in AV groove patch plasty.

Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report.

BACKGROUND: Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare. CASE PRESENTATION: We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7-0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction. CONCLUSION: We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

Neurodevelopment in 1-year-old Japanese infants after congenital heart surgery.

BACKGROUND: The purpose of this study was to determine the prevalence of persistent neurodevelopmental sequelae in 1-year-old infants after open heart surgery for congenital heart disease, using the Bayley Scales of Infant Development second edition (BSID-II). A secondary objective was to confirm the applicability and usefulness of the BSID-II in Japanese infants. METHODS: Thirty-nine infants who underwent repair of a ventricular septal defect before 6 months of age and 108 normal Japanese infants at 1 year of age were assessed using the BSID-II. RESULTS: In normal infants, scores on the Mental Development Index and the Psychomotor Development Index components of the BSID-II ranged from borderline retardation to very superior following a normal distribution similar to those obtained for US controls. No problems were encountered, either in translation or in following the instructions when the BSID-II was used to evaluate the 1-year-old Japanese infants. On the other hand, the mean scores on the Mental Development Index and the Psychomotor Development Index were significantly lower in Japanese patients than in normal Japanese infants, particularly for gross motor development (P < 0.001). Linear regression analysis showed that a longer intensive care unit stay was associated with impaired cognitive development at 1 year of age (P= 0.03). CONCLUSIONS: Neurodevelopmental functions in 1-year-old infants with congenital heart disease were lower than those in normal infants, especially for gross motor function using the BSID-II. One risk factor that correlated with neurodevelopmental sequelae was the length of intensive care unit stay.

Novel technique of repairing right partial anomalous pulmonary venous connection with intact atrial septum using in situ interatrial septum as a flap in a 68-year-old-woman: a case report.

BACKGROUND: Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown. CASE PRESENTATION: This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic. CONCLUSIONS: The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.

Norwood Operation With Anterior Translocation of Pulmonary Artery.

Even in the current era of improved clinical outcome of Norwood operation, postoperative pulmonary artery stenosis, recoarctation of the aorta, ventricular dysfunction, and atrioventricular valve regurgitation remain serious clinical concerns. We present 2 cases of successful total cavopulmonary connection after Norwood operation using the right ventricle-to-pulmonary artery shunt with translocation of the pulmonary bifurcation anterior to the reconstructed neoaortic arch. This modification provided a better surgical access to an obstructive pulmonary artery at staged single ventricle strategy and a space for the growth of the pulmonary arteries.

Activation of cardiac progenitor cells through paracrine effects of mesenchymal stem cells.

Mesenchymal stem cells (MSC) transplantation has been proved to be promising strategy to treat the failing heart. The effect of MSC transplantation is thought to be mediated mainly in a paracrine manner. Recent reports have suggested that cardiac progenitor cells (CPC) reside in the heart. In this study, we investigated whether MSC had paracrine effects on CPC in vitro. CPC were isolated from the neonatal rat heart using an explant method. MSC were isolated from the adult rat bone marrow. MSC-derived conditioned medium promoted proliferation of CPC and inhibited apoptosis of CPC induced by hypoxia and serum starvation. Chemotaxis chamber assay demonstrated that MSC-derived conditioned medium enhanced migration of CPC. Furthermore, MSC-derived conditioned medium upregulated expression of cardiomyocyte-related genes in CPC such as beta-myosin heavy chain (beta-MHC) and atrial natriuretic peptide (ANP). In conclusion, MSC-derived conditioned medium had protective effects on CPC and enhanced their migration and differentiation.

Preoperative small pulmonary artery did not affect the midterm results of Fontan operation.

OBJECTIVE: It was well discussed, in the primary Fontan era, that small pulmonary arterial size could affect the results immediately after the Fontan procedure. The objective of the present study is to determine whether this feature remains as a risk factor in the era of the staged Fontan completion and poses functional impediments in the longer terms. METHODS: Between June 1991 and November 2004, the staged Fontan completion was carried out subsequent to the bidirectional Glenn procedure in consecutive 57 patients with a preoperative pulmonary artery index less than 250 mm2/m2 (Group-S; minimum index 104 mm2/m2). Clinical data were reviewed retrospectively. As background and reference information, similar data were collected in another consecutive 64 patients with larger pulmonary arteries who underwent the staged Fontan completion during the same period (Group-L; maximum index 697 mm2/m2). RESULTS: No patients died after the Fontan procedure in Group-S, while six early deaths in Group-L. No takedown of the Fontan circulation was carried out in either group. The latest catheterizations, at 2.8+/-2.7 years postoperatively, showed a pulmonary artery index significantly lower than the preoperative index (Group-S: 198+/-37-176+/-49 mm2/m2; P=0.0082, Group-L: 360+/-94-266+/-89 mm2/m2; P<0.0001). Hemodynamics in Group-S during the intermediate term were identical with those in Group-L in mean pulmonary arterial pressure (10+/-2 in Group-S and 10+/-3 mmHg in Group-L), mean atrial pressure for the systemic chambers (5+/-2 and 6+/-3 mmHg, respectively), mean transpulmonary gradient (5+/-2 and 4+/-2 mmHg, respectively), cardiac index (3.0+/-0.7 and 3.0+/-0.6l/min/m2, respectively), and arterial oxygen saturation (93+/-3% and 94+/-2%, respectively). Similarly, brain natriuretic peptides concentration in the serum (19.4+/-15.6 in Group-S and 28.3+/-37.2 pg/ml in Group-L) and peak oxygen consumption on exercise testing (24.8+/-4.5 and 24.0+/-6.3 ml/kg/min, respectively) were not inferior in Group-S to those in Group-L. CONCLUSIONS: The outcome after the Fontan completion, including functional ones in the intermediate term, was acceptable in patients having a preoperative PA index smaller than 250 mm2/m2. Pulmonary artery index decreased still further postoperatively, but did not obviously militate against functional efficacy of the Fontan circulation.

Repair of Atrioventricular Septal Defect and Transposition in Left Isomerism.

We report the successful primary anatomic correction of an atrioventricular septal defect with transposition of the great arteries and pulmonary stenosis in a 4-year-old girl with left isomerism by common atrioventricular valve division, Mustard-type atrial septation, and a Rastelli operation with ventricular septal defect enlargement. To the best of our knowledge, this is the first report of total correction for this combination of cardiac anomalies.

Effects of pH management during deep hypothermic bypass on cerebral microcirculation: alpha-stat versus pH-stat.

OBJECTIVE: There is controversy regarding the optimal pH strategy during deep hypothermic bypass in children. We directly visualized the effects of the pH-stat and alpha-stat strategy on cerebral microcirculation (including leukocyte/endothelial cell interactions) in a piglet model using intravital fluorescence microscopy. METHODS: Two groups of 5 piglets (mean weight 9.6+/-1.3 kg) with a cranial window over parietal cerebral cortex underwent 10-minute normothermic bypass, 40-minute cooling on cardiopulmonary bypass ([CPB] Hct 30%, 100 mL/kg/min), 60-minute circulatory arrest at 15 degrees C, and 40-minute rewarming with alpha-stat (group alpha) or pH-stat (group pH). Plasma was labeled with fluorescein-ITC-dextran for assessment of microvascular diameter. Circulating leukocytes were labeled and observed in postcapillary venules for adhesion before and up to 120 minutes after CPB. Cerebral tissue oxygenation was evaluated by quantification of NADH autofluorescence, which increases during ischemia. RESULTS: At the end of normothermic bypass diameter of cerebrocortical microvessels increased to 116+/-9% (alpha) versus 119+/-10% (pH) of pre-CPB baseline values. During cooling microvascular diameter decreased in group alpha and significantly increased in group pH (89+/-11% (alpha) versus 132+/-13% (pH) at the end of cooling; P<0.001). During the first 10 minutes of rewarming, the cerebral microvascular diameter was significantly larger when the pH stat strategy was used. Tissue oxygenation at the end of cooling was significantly greater in the pH-stat group (P=0.008). On reperfusion, the pH-stat strategy resulted in significantly more rapid return of tissue oxygenation toward baseline although at the end of rewarming the metabolic recovery was complete in both groups. The whole body lactate during early rewarming was significantly less with the pH stat strategy. There was no significant difference between the groups regarding the number of adherent leukocytes throughout the time course of the experiment. CONCLUSIONS: pH-stat management increases tissue oxygenation during deep hypothermic bypass and after circulatory arrest. Leukocyte/endothelial cell interactions during hypothermic bypass are mild with both alpha-stat and pH-stat.

Tissue oxygenation index is a useful monitor of histologic and neurologic outcome after cardiopulmonary bypass in piglets.

OBJECTIVE: Tissue oxygenation index is a novel monitoring indicator derived by near-infrared spectroscopy. We hypothesized that tissue oxygenation index could predict a minimum safe flow rate for specific bypass conditions. METHODS: Thirty-six piglets (age, 43 +/- 5 days; weight, 9.0 +/- 1.1 kg) underwent cardiopulmonary bypass with cerebral near-infrared spectroscopy (NIRO-300; Hamamatsu Photonics K.K., Hamamatsu City, Japan). Animals were cooled for 40 minutes to 15 degrees C, 25 degrees C, or 34 degrees C (pH-stat, hematocrit value of 20% or 30%, and pump flow of 100 mL . kg -1 . min -1), followed by low-flow perfusion (10, 25, or 50 mL . kg -1 . min -1) for 2 hours. Neurologic and behavioral evaluations were determined for 4 days. The brain was then fixed for histologic assessment. Tissue oxygenation index was defined as the average signal during low-flow bypass. RESULTS: Animals with an average tissue oxygenation index of less than 55% showed cerebral injury, whereas animals with an index of greater than 55% showed minimal or no evidence of injury. Correlations were found between average tissue oxygenation index and histologic score (Spearman rho = -0.65, P < .001) and neurologic deficit score (Pearson r = -0.50, P = .002) on the first postoperative day. Temperature (P < .001), flow rate (P < .001), and hematocrit value (P = .002) were multivariable predictors of tissue oxygenation index, as determined by means of multivariable analysis of variance. CONCLUSION: Tissue oxygenation index is a useful monitor for defining the minimum safe flow rate during cardiopulmonary bypass. An index value of less than 55% is a strong predictor of neurologic injury.

Improved outcome with composite graft versus homograft root replacement for children with aortic root aneurysms.

OBJECTIVE: Review of surgical repair of aortic root aneurysms using composite graft or homograft in children. METHODS: A consecutive series of 34 children (mean age 10.8+/-5.4 years) who underwent elective aortic root replacement using composite graft or homograft from 1987 to 2003 (mean follow-up 5.7+/-3.7 years). RESULTS: Preoperatively, the aortic annulus and aortic root average z-scores were 4.1+/-2.2 and 9.4+/-4.7, respectively. Composite graft root replacement was performed in 22 patients, and cryopreserved aortic homograft root replacement in 12 patients. There was one perioperative death in the homograft group due to sudden cardiovascular collapse. There was one late death in the composite graft group due to acute aortic dissection, and two late deaths in the homograft root replacement group, one at 7 months postoperatively due to coronary artery thrombosis and one due to severe chronic myocardial dysfunction 5 years postoperatively. One patient who initially had a homograft died due to mechanical valve thrombosis following reoperative composite graft replacement. Five patients had reoperations at a median of 7.1 years after initial surgery. One patient in the composite graft group underwent arch replacement. There were no graft related reoperations after composite graft root replacement, but 4 patients in the homograft group had reoperative composite graft replacement. Predictors of reoperation included age at surgery, lower weight, and longer ICU time (P<0.05). CONCLUSIONS: In children with aortic root aneurysms, reoperation is more common after homograft root replacement than composite graft replacement. Composite graft root replacement provides more stable repair of the aortic root.

Long-term outcome of right ventricular outflow tract reconstruction using a handmade tri-leaflet conduit.

OBJECTIVE: Since 1985, we have implanted handmade tri-leaflet conduits made of heterologous pericardium or expanded polytetrafluoroethylene (ePTFE), as an alternative to homograft for right ventricular outflow tract reconstruction. This report assesses the long-term outcome of these prostheses. METHODS: From 1985 to 2003, 216 handmade tri-leaflet conduits were implanted in 191 patients. Forty-two patients had previous conduit repairs. The mean age at operation was 8.1+/-7.7 years (range: 15 days-44 years). The underlying diagnoses were pulmonary atresia with ventricular septal defect in 83 patients, atrioventricular discordance in 36, transposition of the great arteries in 26, double outlet right ventricle in 14, and truncus arteriosus in 17. Whole heterologous pericardial tri-leaflet conduits were implanted in 169 patients, in the early series (porcine: 85; equine: 58; bovine: 26); bovine pericardial conduits containing ePTFE leaflets were implanted in 26 patients since 1996; whole ePTFE tri-leaflet rolls were employed in the most recent 21 patients. The conduit size was 21.1+/-3.1mm (range: 12-27 mm), 147.4+/-21.4% (range: 82.6-202.6%) of the anticipated diameter of the pulmonary valve. Follow-up was complete. RESULTS: There were 28 early deaths and 24 late deaths. The indication for conduit replacement was a peak instantaneous pressure gradient of greater than 50 mmHg. Sixty-five conduits required reoperation for conduit obstruction at 8.6+/-3.3 years after implantation. The freedom from reoperation at 5, 10, and 15 years was 93.9+/-1.9, 61.4+/-4.5, and 35.5+/-5.6%, respectively. Patients with smaller conduit size and young age at operation were predisposed to reoperation. None of the 47 ePTFE tri-leaflet conduits developed significant obstruction. The freedom from important pulmonary valve regurgitation (PR) as assessed by echocardiography was 68.3+/-3.7% at 5 years, 33.0+/-4.5% at 10 years, and 21.6+/-4.9% at 15 years. No patient required reoperation due to PR or right ventricular dysfunction. CONCLUSIONS: Handmade tri-leaflet conduits provide a reliable alternative for RVOT reconstruction in children, yielding as good a long-term outcome as do homografts. Longer follow-up is needed to determine how well ePTFE leaflets will fare.