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.

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.

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.

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.

[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.

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.

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.

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.

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.

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.

Adipose-derived stem cell sheet transplantation therapy in a porcine model of chronic heart failure.

Adipose-derived stem cells (ASCs) are a promising resource for cell transplantation therapy for damaged heart tissue. Cell death in the graft early after transplantation represents the main cause of unsatisfactory therapeutic efficacy, but tissue-engineered cell sheets grown in temperature-responsive cell culture dishes may enable improved engraftment of transplanted cells. We investigated the therapeutic potential of this method in chronic myocardial ischemia in swine. We created a porcine model of chronic heart failure by implanting an ameroid constrictor around the main trunk of the left anterior descending artery, just distal to the circumflex branch. Simultaneously, ASCs were obtained from a piece of subcutaneous adipose tissue and expanded to form ASC sheets using temperature-responsive dishes. Four weeks after ameroid constrictor placement, triple-layered ASC sheets were transplanted onto the area of the ischemic myocardium (sheet group, n = 7). Controls (n = 7) received no sheet. Just before and 4 weeks after transplantation, left ventriculography (LVG) and coronary angiography (CAG) were performed. LVG revealed a significant improvement in the left ventricular ejection fraction of the sheet group compared with controls (47.6 ± 2.9% vs 41.4 ± 2.8%, P < 0.05). Furthermore, development of collateral vessels was only detected in the sheet group with right CAG. Histologic analysis demonstrated that engrafted ASC sheets grew to form a thickened layer that included newly formed vessels. ASC sheet transplantation therapy is an intriguing therapeutic method for ischemic heart failure.

Successful biventricular repair of double-outlet right ventricle with transposition of the great arteries, pulmonary stenosis, and straddling mitral valve.

A 9-month-old boy with polysplenia, double-outlet right ventricle (DORV), transposition of the great arteries (TGA), a large ventricular septal defect (VSD), straddling mitral valve, pulmonary stenosis (PS), and a normal-sized pulmonary annulus underwent ventricular septation at the right side of the straddling mitral chord as well as an en bloc truncal switch procedure to minimize reduction of right ventricular (RV) volume from the intraventricular conduit. Echocardiography 5 months postoperatively showed laminar flow without obstruction through both ventricular outflow tracts, normal ventricular function, trivial aortic regurgitation, and mild pulmonary regurgitation (PR). This procedure is a good option for biventricular repair in the presence of a straddling mitral valve.

Case of congenital aneurysm of sinus of Valsalva with common arterial trunk.

We describe an unusual case of a patient with common arterial trunk and interrupted aortic arch, complicated by congenital aneurysm of the sinus of Valsalva, who underwent successful repair. To our knowledge, no such case has been reported in the English literature. The combination of common arterial trunk and congenital aneurysm of the sinus of Valsalva may yield insights into the morphogenesis of congenital aneurysm of the sinus of Valsalva.

Severe cyanosis after Kawashima operation resulting from congenital portosystemic shunt.

We describe an unusual case of a patient with polysplenia, inferior vena cava (IVC) interruption, and unrecognized congenital portosystemic venous connection (CPVC) in whom severe cyanosis developed in the acute postoperative stage after a Kawashima operation and required urgent surgical division of the CPVC. It has been stressed recently that CPVCs and other abdominal venous abnormalities have been underdiagnosed in patients with polysplenia and IVC interruption, and we think that a routine evaluation of abdominal venous connections should be mandatory before considering the Kawashima operation for these patients.

Long-term results of total cavopulmonary connection with low ejection fraction.

PURPOSE: The aim of this study was to investigate the surgical outcomes and change in cardiac performance after total cavopulmonary connection (TCPC) in patients with reduced ventricular ejection preoperatively. METHODS: Among the 195 patients undergoing TCPC between 1990 and 2001 were 52 who had a preoperative ejection fraction of ≤50% (30%-50%, mean 44%). A dominant ventricle was of the morphologically right type in 81% of patients. RESULTS: A total of 9 of the 52 patients died early. Two patients died in the intermediate term. Postoperative exercise testing showed maximum oxygen uptake of 26.4 ± 5.8 ml/kg/min (anaerobic threshold 18.3 ± 3.2 ml/kg/ min). The cardiac index was 3.1 ± 0.9 l/min/m(2) at 1 year after TCPC, with no fundamental change at 5 years (3.1 ± 0.8 l/min/m(2)). The end-diastolic volume of the dominant ventricle was 130% ± 74% of the anticipated normal value at 1 year and 93% ± 27% at 5 years after TCPC, with ejection fractions of 48% ± 13% and 49% ± 9%, respectively. When these parameters were plotted for the individual patients, the ejection fraction increased during the first postoperative year, with the percent end-diastolic volume decreasing in 31 survivors; the trend appeared atypical in the remaining 12 survivors. Even in these patients, however, the parameters eventually changed toward favorable circumstances 5 years after TCPC. CONCLUSION: Change in ventricular function was not necessarily pessimistic after TCPC in patients with reduced ventricular contraction preoperatively.

Ventricular performance in long-term survivors after Fontan operation.

BACKGROUND: Ventricular function and arrhythmia in patients with Fontan circulation in long-term follow-up are still unknown. METHODS: We retrospectively reviewed 48 patients who survived and were followed up for more than 15 years, among 110 patients who underwent Fontan operation in our institute from 1979 to 1992. Atriopulmonary connection was performed in 26 patients and total cavopulmonary connection in 22. The patients were categorized into right ventricle, left ventricle, and biventricle groups. Follow-up cardiac catheterization and exercise test were performed routinely every 5 years post surgery. Median age at Fontan operation was 5 years. RESULTS: Mean follow-up was 18.5 years. Cardiac index in the total cavopulmonary connection group was higher than in the atriopulmonary connection group at 10 and 15 years post surgery (p<0.05). Ejection fraction in the left-ventricle group was higher than in the right-ventricle group. End-diastolic volume at 5, 10, and 15 years was significantly lower than at 1 year (p<0.05). End-diastolic pressure at 10 years was significantly higher than at 1 and 5 years (p<0.05). Beyond 15 years, 6 patients developed ventricular tachycardia. The only significant risk factors for the onset of ventricular tachycardia in a multivariate analysis were age at Fontan operation and absolute age (p<0.05). CONCLUSIONS: Long-term follow-up of patients demonstrated that postoperative ventricular systolic performance seemed to become steady. Ventricular tachycardia was detected 15 years post surgery, especially in older patients with older age at Fontan operation, possibly revealing a risk factor in the long-term postoperative period, thereby meriting further consideration.