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.

Interventricular septal hematoma following repair of a ventricular septal defect.

An interventricular septal hematoma is a rare complication after patch closure of a ventricular septal defect (VSD). We describe three cases of interventricular septal hematomas following patch VSD and discuss their management.

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.

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.

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.

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.

Takedown of Yasui procedure due to closed ventricular septal defect.

A two and a half year old girl who had undergone the Yasui procedure as a neonate for ventricular septal defect, subaortic stenosis, and interrupted aortic arch underwent follow-up catheterization 2 years postoperatively. It showed that the neo-left ventricular tract reconstructed by Damus-Kaye-Stansel anastomosis had occluded due to closure of the ventricular septal defect and residual subaortic stenosis at the original left ventricular outflow tract. The patient therefore underwent takedown of the Yasui procedure.

Staged approach for congenitally corrected transposition of the great arteries associated with dextrocardia and nonconfluent pulmonary artery.

We completed double switch operation (Senning plus Rastelli procedure; SLL) after staged palliative operations for SLL, dextrocardia, congenitally corrected transposition of the great arteries ventricular septal defect, atrial septal defect, and nonconfluent pulmonary artery. The patient had undergone three preparatory operations including central pulmonary artery reconstruction with autologous pericardium and morphologic right ventricle-pulmonary artery shunt and then double switch operation was performed. During the double switch operation, the previous conduit which was established as right ventricle-pulmonary artery shunt in the third palliation was replaced by a larger conduit. This conduit was used as Rastelli external conduit to avoid coronary artery injury. When systemic venous chamber was reconstructed, suture line pouch technique was used, in which dog ears were made at the sites of superior and inferior vena cava orifices, respectively, to prevent systemic venous return obstruction. Pulmonary venous chamber was reconstructed without any augmentation, which reduced the possibility of postoperative arrhythmia. Systemic and pulmonary venous pathway obstructions were not observed after the operation. Postoperative arrhythmia was not detected.

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.

Risk factor of interim failure and early detection of the high-risk patients with functional single ventricle after Blalock-Taussing shunt.

PURPOSE: The purpose of this study was to identify the risk factor of interim failure and to consider the method for detecting high-risk patients after Blalock-Taussing shunt. METHODS: Between 1990 and 2004, 64 patients with functional single ventricle and low pulmonary flow underwent Blalock-Taussing shunt. We evaluated risk factors of interim failure by Logistic regression. Furthermore, assessments by catheterization and echocardiography were compared between Group Completion (Group C; 36 patients who had completed the Fontan operation or were Fontan candidates) and Group Failure (Group F; 20 patients who died before the Fontan operation or dropped out from Fontan candidate). RESULTS: In Group C, the survival at 10 years was 93%. The risk factor of interim failure was a systemic ventricular end-diastolic volume (SVEDV) of more than 250% of normal after shunt. The ventricular volume by echocardiography in Group C had been maintained between 190% and 200% of normal. By contrast, the ventricle in Group F had been significantly dilated after postshunt short term. CONCLUSIONS: The risk factor of interim failure was postshunt ventricular dilation, and the dilation was identified after postshunt short term. We recommend volume study within two to three months after shunt, and bidirectional cavopulmonary anastomosis should be immediately indicated to patients with ventricular dilation.

Creation of an intraatrial tunnel to produce a total cavo-pulmonary connection in a patient with dominant right ventricle, totally anomalous pulmonary venous connection, and pulmonary atresia with non-confluent pulmonary arteries.

The surgical strategy for patients having a functionally single ventricle associated with totally anomalous pulmonary venous connection and pulmonary atresia with non-confluent pulmonary artery has yet to be agreed. We created an intraatrial tunnel to produce a total cavo-pulmonary connection in such a patient, also creating a confluence for the pulmonary arteries. By minimizing the use of the GoreTex patch, the patient was able to discontinue the use of warfarin.

[Arterial switch operation: late results and a future perspective].

Intermediate results of the arterial switch operation for variety of cardiac anomaly with the transposed great arteries were studied to elucidate late results and problems. Seventy-five survivors of the arterial switch operation at our institute between 1991 and January 2007 were studied retrospectively. Mean follow-up time was 7.5 years. There were 2 late deaths, 3 reoperations, and 9 cases of catheter intervention, resulting in actuarial survival of 97%, and event-free rate of 83% at 16 years. Seventy patients (93%) were medication free and had no restriction on physical activities. Moderate pulmonary stenosis in 2 patients and mild pulmonary stenosis in 14 were observed. Moderate aortic regurgitation in 2 and mild aortic regurgitation in 20 were observed. Risk factors for right ventricular outflow obstruction were a diagnosis of double outlet right ventricle (DORV), association with aortic arch obstruction, and prior pulmonary artery banding (PAB). Risk factors for branch pulmonary artery stenosis (PS) were not clear; however, scar formation with use of xenograft patch and in-fold formation by redundant autologous pericardial patch for closure of the defects in the old aortic root seemed to be related with the stenosis. Risk factors for aortic regurgitation were age at operation and prior PAB. Early primary arterial switch operation may resolve these problems.

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.

Biventricular repair of double-outlet right ventricle and closing ventricular septal defect.

We report an infant with double-outlet right ventricle and progressive left ventricular failure caused by a closing apical muscular ventricular septal defect detected during follow-up, who achieved successful biventricular repair after surgical opening and enlargement of a closed perimembranous ventricular septal defect.

Lack of opiate effects on cat C polymodal nociceptive fibers.

The direct application of preservative-free morphine sulfate (1.5%, 1 ml, 19.8 mumol) or fentanyl (0.06%, 1 ml, 1.07 mumol) on the superficial radial or saphenous nerve of cats did not alter the response of single C polymodal nociceptive fibers (PMNs) to noxious radiant heat stimulation of their peripheral receptive fields. Intravenous administration of fentanyl (100 or 200 micrograms/kg, 0.179 or 0.358 mumol/kg) also showed a similar lack of effect on the radiant heat evoked responses of single PMNs. Slight changes in the mean latencies following drug administration were recognized, which were not statistically significant. The use of morphine (1.5%, 1 ml, 19.8 mumol) with preservatives (chlorbutanol 0.5% and sodium bisulfite less than 0.1%) caused conduction block of PMNs within 6-15 min. Subsequent washout of the drug resulted in the return of the unitary discharges within 8 min. Lidocaine (0.25 and 0.5%, 10.7 mumol and 21.4 mumol) caused conduction block within 5-18 min. These data support the classically held concept that opiates, in clinically useful concentrations, do not alter peripheral nerve function.