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.

Simplified primum ASD closure technique in complete VSD repair: shallow suture crossing on the AV node.

The primum atrial septal defect suture line adjacent to the crux is performed carefully with various techniques to avoid atrioventricular block in repair of complete atrioventricular septal defect. We describe our technical modification to simplify the shallow suture line only into the endocardium above the atrioventricular node without conduction disturbance.

A valuable echocardiographic indicator for the optimal tightness of bilateral pulmonary artery banding.

OBJECTIVES: The optimal tightness of bilateral pulmonary artery banding (BPAB) is considered to balance not only systemic-to-pulmonary blood flow but also each pulmonary blood flow, which is still challenging. To achieve them, we adopt the end-diastolic velocity (EDV) to the peak systolic velocity (PSV) ratio at BPAB with intraoperative epicardial echocardiography. We evaluated the usefulness of the EDV to PSV ratio and the patient outcomes. METHODS: 34 patients underwent BPAB with this indicator and using a looped polytetrafluoroethylene suture. The PSV and the EDV to PSV ratio with echocardiography were measured in the intraoperative, early postoperative and late postoperative period. Lung perfusion scintigraphy was performed to quantify flow to each lung. RESULTS: There were 3 early deaths (< 30 days). Two patients required re-BPAB due to hypoxia. The intraoperative EDV to PSV ratios in the right and left were almost equal (0.50 ± 0.07 versus 0.51 ± 0.06, P = 0.73). There was no significant difference in the right and left EDV to PSV ratios throughout the postoperative course. The right PSV was smaller than the left PSV due to the Doppler angle intraoperatively (2.78 ± 0.57 versus 3.02 ± 0.50, P = 0.030). In addition, the PSV changed significantly until the late postoperative period (P < 0.001). Lung perfusion scintigraphy revealed only two patients had perfusion abnormalities. CONCLUSIONS: Our clinical outcomes are satisfactory with low early mortality and a low rate of re-BPAB. The EDV to PSV ratio can be a reliable indicator to assess flow distribution to each lung and may be a valuable adjunct to achieve balanced systemic to pulmonary flow.

Intra-Extracardiac Total Cavopulmonary Connection for Patients With Anatomical Complexity.

BACKGROUND: Currently, the extracardiac conduit total cavopulmonary connection (eTCPC) is the most widely used for Fontan modification worldwide. Nevertheless, there have been some cases that are difficult for performing eTCPC because of their anatomical complexity, such as apicocaval juxtaposition. For such cases, in 2002, we introduced the intra-extracardiac TCPC (ieTCPC). METHODS: We reviewed our 20-year single-center experience with 316 TCPC patients to compare eTCPC (n = 277) and ieTCPC (n = 39) in terms of mortality and morbidity. ieTCPC was indicated for the cases in which there was concern that the TCPC conduit would be too curved for ordinary eTCPC. RESULTS: Early death occurred in 1 patient and late death occurred in 15 patients. The actuarial survival rate in the eTCPC and the ieTCPC groups at 10 years were 95.1% and 100.0%, respectively. There was no significant difference in actuarial survival between eTCPC and ieTCPC patients. In the multivariate analysis, preoperative superior vena cava pressure and preoperative oxygen saturation were found to be the independent predictor for postoperative mortality. There was also no significant difference in actuarial rate of freedom from late-occurring complications between eTCPC and ieTCPC groups. In the multivariate analysis, dominant right ventricle and preoperative SVC pressure were independent predictors for late-occurring complications. CONCLUSIONS: The clinical outcomes in patients who undergo eTCPC and ieTCPC appear to be excellent, with low mortality and morbidity rates in the midterm. ieTCPC may be a good option for TCPC cases with anatomical complexity such as apicocaval juxtaposition and separated hepatic vein drainage.

Impact of intrapulmonary-artery septation to pulmonary vein obstruction for two-lung Fontan.

OBJECTIVES: Patients with unbalanced pulmonary artery (PA) growth and decreased unilateral pulmonary circulation are considered unsuitable candidates for the Fontan procedure. Following our previous study on the utility of intrapulmonary-artery septation for patients with PA hypoplasia, we investigated its use in patients with pulmonary venous obstruction (PVO). METHODS: We recruited 42 patients who underwent intrapulmonary-artery septation for unilateral PA hypoplasia and/or PVO between 1998 and 2018 and classified them into no PVO or PVO group. We analysed overall survival, success of the Fontan procedure and data from catheterization and echocardiography. In PVO, we evaluated the functional lung area before the Fontan procedure and the relevance of this parameter to operative outcomes. RESULTS: The PVO and no-PVO group included 24 and 18 patients, respectively. One patient in the no-PVO and 6 patients in the PVO group died during follow-up; this difference was statistically significant (log-rank P = 0.040). In the no-PVO group, 15 (83%) patients achieved two-lung Fontan circulation. In the PVO group, 12 (50%) patients achieved two-lung Fontan circulation. Multivariate analysis revealed that functional lung area and shunt size [significantly larger in patients with functional lung area ≥50% in affected lung (P = 0.040)] were significant factors for successful two-lung Fontan procedure (P = 0.030). CONCLUSIONS: Intrapulmonary-artery septation may contribute to increase functional lung area after PVO release to establish two-lung Fontan circulation in patients with PVO who have unbalanced PA growth and/or decreased unilateral pulmonary circulation.

Adventitial Wrap Technique for Acute Type A Aortic Dissection.

We describe a simple and reproducible technique to achieve complete and immediate hemostasis of the distal anastomosis in total arch replacement with the frozen elephant trunk technique. The adventitia was left seamlessly 1.0 cm longer than the level of the suture line. The adventitial remnant covered most of the distal anastomosis line tightly while the distal anastomosis was completed with continuous sutures. The adventitia was thin and tough, making it suitable to cover the suture line. This technique can contribute to eliminating bleeding from the distal anastomosis independent of unreliable blood coagulability during aortic surgery for acute aortic dissection.

Arterial Switch Operation With and Without Coronary Relocation for Intramural Coronary Arteries.

BACKGROUND: The arterial switch operation (ASO) for the transposition of the great arteries (TGA) with intramural coronary arteries has been performed using several techniques to avoid coronary events. We mainly performed ASO without coronary relocation by creating an aortopulmonary fenestration (Imai technique). Coronary circulation was rerouted by covering the aortopulmonary window and coronary orifices with a nonfacing sinus flap. Long-term results have not been reported. We describe our early and late results. METHODS: Among 551 patients who underwent an ASO between 1985 and 2014, intramural coronary arteries were detected in 15 of them. Coronary arteries were managed using 2 techniques: the double-button method in 5 patients (with unroofing and trapdoor incision in 1 patient) and the Imai technique in 10 patients. RESULTS: There were 3 hospital deaths and 3 deaths after discharge, 5 of which showed coronary complications. Actual survival and freedom from coronary complications at 15 years were 70% and 67%, respectively, with the Imai technique and 40% and 20%, respectively, with the double-button method. Late coronary intervention was performed for a long intramural coronary artery stenosis in 1 patient who underwent the Imai technique. In the others, late aortography showed good patency of the aortopulmonary window and growth of the coronary pouch after the Imai technique. CONCLUSIONS: The Imai technique can be an option for coronary management in the presence of high-risk coronary anatomy, particularly distal intramural coronary artery stenosis and inseparable coronary arteries with an almost single orifice. Adequate neopulmonary artery augmentation must be performed to prevent right ventricular outflow stenosis.

Right Atrial Calcified Ball Thrombus Mimicking a Myxoma.

An 80-year-old woman was admitted to our hospital because of congestive heart failure. Transthoracic echocardiography revealed severe aortic valve stenosis, and a mobile calcified mass was attached to the interatrial septum in the right atrium. We suspected that the mass was cardiac myxoma. We urgently performed aortic valve replacement and resection of the mass. The histological diagnosis of the mass was thrombus, and her post-operative course was uneventful. Although the mechanism of right atrial thrombus formation in our case was unclear, it is important to continue anticoagulation therapy after surgery to avoid recurrence. Furthermore, strict follow-up in this patient is needed.

Patch Repair for a Subepicardial Aneurysm With an Approach From Inside the Heart.

We present a case of subepicardial aneurysm that developed as a complication of acute myocardial infarction. Although the patient had stable hemodynamics, the diameter of the aneurysm tended to increase over time; thus, an operation was performed. We thought that approaching the aneurysm from outside the heart would injure the papillary muscle or distort the left ventricle and exacerbate mitral regurgitation. Therefore, we performed patch repair using bovine pericardium and approached the aneurysm from inside the heart. This is the first case report of a subepicardial aneurysm that was approached and repaired from inside the heart.

Absorbable sternal pins improve sternal closure stability within a small deviation.

OBJECTIVE: Sternum external fixation with a sternal wire is widely used. However, it was inadequate fixation in the anterior-posterior and cranial-caudal directions. Using sternal pins can improve the stability. The utility of a small deviation was important with respect to sternal pain. Here, we evaluated the efficacy of a sternal pin in a small deviation in three directions. METHODS: Polyurethane foam was used. In the wire group, models were closed using two stainless steel wires. In the wire and sternal pin group, a hydroxyapatite/poly-L-lactide acid (HA/PLLA) sternal pin was used for internal fixation in addition to wire fixation. A sternal pin was inserted inside. Both sides were fixed with the testing machine and the shear stress was directed to one side. In each group, six models were tested for three directions. The relationship between the load and displacement of the model was measured at 1-mm displacement. RESULTS: In the anterior-posterior direction, the load value was 138.8 ± 8.3 (N) in the wire and sternal pin group and 51.6 ± 9.5 (N) in the wire group (p = 0.0003). In the cranial-caudal direction, the values were 148.0 ± 12.0 (N) and 83.1 ± 26.6 (N), respectively (p < 0.0001). In the lateral direction, these values were 273.2 ± 49.3 (N) and 266.9 ± 50.6 (N), respectively (p = 0.8323). CONCLUSIONS: Additional internal fixation using an HA/PLLA sternal pin significantly improved the stability of sternotomy closure in the anterior-posterior and cranial-caudal directions within a small deviation.

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.

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.

Forty-year durability of a Smeloff-Cutter ball valve prosthesis in the mitral position.

A rare case requiring replacement of an intact Smeloff-Cutter ball prosthesis in the mitral position 40 years after implantation is presented. The Smeloff-Cutter ball valve prosthesis was designed to have two open cages. It has two potential advantages: a relatively large, effective orifice area and its self-washing effect that prevents thrombus formation. There have been only a few reports of survivors with ball valve prostheses in place for more than three decades especially in the mitral position. This is a valuable report describing the long-term durability of a Smeloff-Cutter ball valve prosthesis in the mitral position.

A refined flanged Bentall technique using Valsalva tube graft for proximal reinforcement.

Bleeding from the proximal suture line during aortic root replacement using a composite valve graft is a crucial and catastrophic problem. We present a simple flanged Bentall technique using a Valsalva tube graft to eliminate bleeding from the proximal suture line. The method is to wrap the proximal anastomosis completely by sewing the Valsalva flange to the residual aortic wall. The wrapping is facilitated by the use of part of a horizontally stretching Valsalva graft. This refined technique is effective and reproducible to prevent bleeding from the proximal suture line after the Bentall procedure.

[Aortic regurgitation due to quadricuspid aortic valve; report of a case].

A 66-year-old man was referred to our hospital for surgical treatment for aortic regurgitation. Transthoracic echocardiography revealed the diagnosis of aortic regurgitation due to quadricuspid aortic valve. During operation, we confirmed that the aortic valve consisted of 4 leaflets with almost same size (Hurwitz and Roberts classification type A). We performed valve replacement because each leaflet has degenerative change. The patient's postoperative course was uneventful and discharged the hospital without complications. Congenital quadricuspid aortic valves are rare, and patients with this disease are frequently operated because of aortic regurgitation. Presently, common operative procedure is aortic valve replacement, although in the future valvuloplasty should be investigated.