BACKGROUND The discussion of Fontan fenestration is difficult because many institutes have different strategies over time. In our institute, we had performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy. METHODS Between August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy. Primary outcomes were Fontan-related events, including death, reoperation, catheter intervention, and postoperative complications such as arrhythmias, protein-losing enteropathy, plastic bronchitis, thrombosis, bleeding, and drainage of pleural effusion after discharge. RESULTS The median follow-up duration was 15.7 years (interquartile range (IQR): 15.3–18.4). The 1-, 2-, 3-, 5-, 10-, and 15-year occurrence of Fontan-related events was 24%, 43%, 44%, 55%, 67%, and 76%, respectively. The 1-, 2-, 3-, 5-, and 10-year occurrence of venovenous collaterals was 16%, 60%, 65%, 72%, and 81%, respectively. Ventricular end-diastolic pressure was higher in patients with venovenous collaterals (median: 5.0 mmHg, IQR: 4.0–7.0) than in those without (median: 3.5mmHg, IQR: 3.0–4.25) (p = 0.01). Multivariable Cox regression analysis showed that higher ventricular end-diastolic pressure and younger age at Fontan completion significantly increased the risk of developing venovenous collaterals, with hazard ratios of 1.22 for each (95% confidence interval: 1.052–1.41, p = 0.0085 and 0.016, respectively). CONCLUSIONS The occurrence rate of Fontan-related events was acceptable with the non-fenestrated Fontan strategy, whereby venovenous collateral development was common. The results suggest that patients with high ventricle end-diastolic pressure and young patients might benefit from fenestration.

This is the first report of total arch replacement to repair re-coarctation. A 14-year-old boy with hypoplastic left heart syndrome developed re-coarctation, severe stenosis of neck vessels, and right ventricle dysfunction after a Norwood procedure. We performed total arch replacement; the postoperative course was unremarkable. He was followed up until 18 years of age and did not need re-intervention. Using artificial blood vessels in total arch replacement is rarely indicated but can be safely achieved when required. Mismatch between patient and graft size may be an issue in the future.

A one-month-old baby boy with a complete atrioventricular septal defect underwent pulmonary artery banding. A high take-off of the left coronary artery, overlooked on the echocardiogram, was identified. It was compressed by the right pulmonary artery that was dilated owing to pulmonary artery banding. The patient developed severe heart failure, and a Lecompte maneuver was performed. The procedure helped effectively treat this congenital heart disease with a high take-off coronary artery compressed by the right pulmonary artery.

A 2-year-old girl underwent conversion to the Konno procedure by removing the Damus–Kaye–Stansel anastomosis after the neonatal Yasui procedure for an interrupted aortic arch with left ventricular outflow tract stenosis. Her postoperative course was uneventful. However, left ventricular outflow tract restenosis occurred due to narrowed ventricular septal defect and moderate neoaortic regurgitation from the old pulmonary valve. The Konno procedure was performed by removing the Damus–Kaye–Stansel anastomosis for left ventricular outflow tract restenosis and neoaortic regurgitation and performing right ventricular outflow tract reconstruction and ventricular septal defect closure. Left ventricular outflow tract restenosis was not observed.

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. 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. 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. 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.

Central MessageCoronary artery bypass grafting (CABG) in infants includes technical challenge, but microscopic surgery with sufficient experience leads to successful CABG, with avoiding technical errors.See Commentaries on pages 444 and 446. Coronary artery bypass grafting (CABG) in infants includes technical challenge, but microscopic surgery with sufficient experience leads to successful CABG, with avoiding technical errors. See Commentaries on pages 444 and 446. With an increase in congenital heart surgeries such as arterial switch operation (ASO) or the Ross procedure, coronary artery bypass grafting (CABG) in infants has become an increasingly important option because of coronary obstruction as a result of coronary manipulation.1Mavroudis C. Backer C.L. Muster A.J. Pahl E. Sanders J.H. Zales V.R. et al.Expanding indications for pediatric coronary artery bypass.J Thorac Cardiovasc Surg. 1996; 111: 181-189Abstract Full Text Full Text PDF PubMed Scopus (83) Google Scholar,2Kitamura S. Pediatric coronary artery bypass surgery for congenital heart disease.Ann Thorac Surg. 2018; 106: 1570-1577Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar CABG in infants includes technical challenges associated with operating on small vessels. A microscope is, therefore, a promising tool to assist CABG in infants in achieving successful coronary revascularization.3Catapano J. Zuker R. Honjo O. Borschel G. Microvascular coronary artery repair and grafting in infancy and early childhood.Oper Tech Thorac Cardiovasc Surg. 2015; 20: 148-161Abstract Full Text Full Text PDF Scopus (8) Google Scholar,4Kitamura S. A new arena in cardiac surgery: pediatric coronary artery bypass surgery.Proc Jpn Acad Ser B Phys Biol Sci. 2018; 94: 1-19Crossref PubMed Scopus (11) Google Scholar This case series introduces 4 infants who underwent total microscopic CABG. The ages were 3 months in 2 cases, 4 months in 1, and 9 months in 1, weighing 3.2 to 4.8 kg (average, 4.0 kg). Informed written consents were provided from the parents of each case. For harvesting the internal mammary artery, magnifying glasses were used in cases 1, 2, and 3, and microscope was used in case 4. The anastomosis was performed with the infant under cardiac arrest, placing single interrupted sutures with 10-0 or 9-0 PROLENE (Ethicon, Somerville, NJ). The toe and heel of the graft were sutured with 9-0 PROLENE. In total, 12 to 16 sutures were placed. The surgical microscope enabled up to 20× auto focus magnification and hands-free manipulations such as magnification or minute movement of the visual field. Surgery was performed by a surgeon (K.E.) with more than 3 decades of experience in microscopic CABG. After the spontaneous beating resumed, good anastomosis was confirmed in all 4 cases using the indocyanine green fluorescence contrast function attached to the microscope (Figure 1). In all cases, postoperative angiography revealed excellent patencies of all bypasses. The average vessel diameters of 4 cases at near the anastomosis measured by the pre- or postoperative coronary angiography were 1.1 mm (ranged 0.6-1.3) in coronary artery and 1.1 mm (ranged 0.8-1.3) in internal thoracic artery. Case 1 was a previously healthy female infant. She suddenly presented dyspnea at the age of 6 months and developed heart failure requiring mechanical ventilator support. Echocardiogram showed enlarged left ventricular diastolic dimension of 38 mm and left ventricular ejection fraction (LVEF) of 40%. LVEF reduced to 21% in a couple of months. Her hemodynamic function was catecholamine dependent and had difficulty in weaning from the ventilator. Coronary angiography at 9 months revealed 99% stenosis of hypoplastic left main trunk (LMT). Then, left internal thoracic artery (LITA)-to-left anterior descending artery (LAD) bypass was performed. LVEF recovered to 46%, and she was weaned from ventilator on postoperative day (POD) 14 and discharged on POD 234. Case 2 was a boy diagnosed with transposition of the great arteries (TGA) who underwent balloon atrial septostomy immediately after birth. ASO was performed at 15 days, and he was discharged on POD 21 in good condition. He developed acute heart failure with reduced LVEF of 23% on POD 42. Urgent coronary angiography revealed 90% stenosis at the ostium of LMT. Then, a LITA-to-LAD bypass was performed at 4 months (Video 1). LVEF improved to 53%, and he was weaned from ventilator on POD 5 and discharged on POD 83. Case 3 was a boy diagnosed as having TGA immediately after birth. ASO was performed at 18 days, and he was discharged on POD 27. Acute heart failure was developed on POD 64 and coronary angiography depicted 90% stenosis of proximal LMT. At 28 days after readmission, he went into cardiogenic shock and required extracorporeal membrane oxygenation support. LITA-LAD bypass was performed the following day. He was taken off extracorporeal membrane oxygenation during surgery and was weaned from the ventilator at POD 8. LVEF at discharge (POD 43) was 70%. Case 4 underwent balloon atrial septostomy for TGA at the age of 5 days. ASO with ventricular septal defect closure was performed at 11 days. He was weaned from the mechanical ventilator at 39 days under catecholamine support. Coronary angiography at 2 months showed hypoplastic but patent left coronary artery. The left ventricle was fed by right coronary artery, which had 90% stenosis at its ostium. Right internal thoracic artery-to-right coronary artery bypass was performed at the age of 3 months (Video 2). He was weaned from ventilator the following day of the surgery and discharged from hospital on POD 81. Cases 2, 3, and 4 are now 8 years, 3 years, and 8 months old, respectively, and are growing and developing normally. Despite the excellent patency of the bypass confirmed by the angiogram (Figure 2), however, case 1 suddenly died 2 months after discharge. This case series was not required to be approved by the institutional review board of the Nagasaki University because the cases was not involved in analytical activity. Informed written consents were provided from the parents of each case. Successful coronary revascularization and improvement of cardiac function were achieved in all 4 cases with totally microscopic assisted CABG. Because of technical concerns, some institutions perform microscopic surgery in collaboration with plastic surgeons,3Catapano J. Zuker R. Honjo O. Borschel G. Microvascular coronary artery repair and grafting in infancy and early childhood.Oper Tech Thorac Cardiovasc Surg. 2015; 20: 148-161Abstract Full Text Full Text PDF Scopus (8) Google Scholar but as our result shows, with sufficient experience, microscopy-assisted infant CABG was feasible with avoidance of technical errors.4Kitamura S. A new arena in cardiac surgery: pediatric coronary artery bypass surgery.Proc Jpn Acad Ser B Phys Biol Sci. 2018; 94: 1-19Crossref PubMed Scopus (11) Google Scholar Short term, our results are satisfactory, and further follow-up is needed. We anticipate that our preferred single interrupted sutures would lead to the growth of the anastomosis orifice along to the somatic growth of the children. https://www.jtcvstechniques.org/cms/asset/c3c82863-65aa-4d78-8133-93f153d0a5fc/mmc1.mp4Loading ... Download .mp4 (103.9 MB) Help with .mp4 files Video 1Intraoperative video with pre- and postoperative coronary angiography in case 2. Video available at: https://www.jtcvs.org/article/S2666-2507(21)00580-0/fulltext.https://www.jtcvstechniques.org/cms/asset/37c865f9-e641-463e-9314-7ba58186765c/mmc2.mp4Loading ... Download .mp4 (214.56 MB) Help with .mp4 files Video 2Intraoperative video in case 4. Video available at: https://www.jtcvs.org/article/S2666-2507(21)00580-0/fulltext.

We report a case of transposition of the great arteries （ TGA ） with severe pulmonary hypertension from the right to left shunt in the right modified Blalock-Taussig shunt. The patient was diagnosed with TGA with a small ventricular septal defect, restrictive patent foramen ovale, and patent ductus arteriosus. Balloon atrial septostomy was performed, and an arterial switch operation （ ASO ） was planned. However, ASO was delayed during the neonatal period due to cerebral bleeding. Moreover, left outflow tract obstruction was noted ; hence, the surgical strategy was shifted to an atrial switch operation or Rastelli type operation. The patient was palliated at the age of 5 months with a right 4-mm Gore-Tex modified Blalock-Taussig shunt. After this procedure, he was followed up at the outpatient clinic with good saturation level. However, at 9 months, he revisited the hospital due to septic shock. His SpO 2 was 60% in the upper right limbs and 40% in the upper left and lower limbs. Nitric oxide inhalation and 100% oxygen were administered to improve pulmonary hypertension and subsequent differential cyanosis. Pulmonary hypertension decreased from over-systemic to 70% of the systemic arterial pressure. The Senning procedure with a fenestration in an atrial baffle was successfully performed at the age of 1 year. Jpn. J. : 240 - 243 （ 2021 ）

Yuki Nakayama1), Yusuke Iwata1), Toshihide Nishimori1), Takashi Kuwahara2), Naoki Kuwabara2), Hiroko Goto2), Kentaro Omoya2), Tetsuya Yamamoto2), Atsushi Terazawa2), Tatsuhiko Masue3), Sadahiro Kato4), and Takamasa Takeuchi1) 1) Department of Pediatric Cardiac Surgery, Gifu Prefectural General Medical Center, Gifu, Japan 2) Department of Pediatric Cardiology, Gifu Prefectural General Medical Center, Gifu, Japan 3) Department of Anesthesiology, Gifu Prefectural General Medical Center, Gifu, Japan 4) Department of Pediatric Surgery, Gifu Prefectural General Medical Center, Gifu, Japan

Background: For children with congenital heart disease (CHD) resulting from trisomy 18, we do not perform intracardiac repair; instead, we opt for palliative surgery when their parents wish to proceed with surgical intervention. Methods: From hospital medical records, we retrospectively identified 17 patients whose attending physicians had consulted our department between January 2010 and September 2016. We classified patients into two groups: those who required surgical intervention for CHD to promote transition to the home environment and who underwent surgery with parental consent (Group I: 5 patients), and those who did not require surgical intervention for CHD and did not undergo surgery (Group N: 12 patients). We compared the ease of transition to life in the patient’s own home between Group I and Group N. Results: The median survival of the patients was 427 days in Group I and 173.5 days in Group N (p=0.0534). The median number of days spent in the patients’ own homes was 647 days in Group I and 72 days in Group N (p=0.0495). Conclusion: Palliative surgery for CHD in patients with trisomy 18 extended the period of time that patients and their families could spend together in the home environment following hospital discharge.

A 1-month-old girl, diagnosed with a common atrioventricular canal, moderate atrioventricular valvular regurgitation, and pulmonary hypertension, underwent pulmonary artery banding. Postoperatively, methicillin-resistant Staphylococcus aureus wound infection was treated with antibiotics. One month later, emergency surgery was performed for oozing rupture of an infected pulmonary aneurysm. The pulmonary aneurysm was completely resected, the banding tape was removed, and pulmonary angioplasty was performed to create pulmonary stenosis without using foreign material. Methicillin-resistant Staphylococcus aureus was cultured from the resected tissues and banding tape. The patient was discharged after antibiotic administration. Correction was performed at 1 year of age, and she remains well.

Biatrial drainage of the right superior vena cava resulting from a sinus venosus defect is a rare congenital malformation. It can result in severe desaturation although a sinus venosus defect usually presents as a left-to-right shunt. A male baby was noted to have cyanosis while nursing and was referred to us for medical treatment on his second day of life. Echocardiography showed that most of the blood flowing through the superior vena cava drained into the left atrium. He underwent successful surgery to correct this defect at the age of 2 years.

Atypical hemolytic uremic syndrome (aHUS) can be distinguished from typical or Shiga-like toxin-induced HUS. The clinical outcome is unfavorable; up to 50% of affected patients progress to end-stage renal failure and 25% die during the acute phase. Multiple conditions have been associated with aHUS, including infections, drugs, autoimmune conditions, transplantation, pregnancy, and metabolic conditions. aHUS in the nontransplant postsurgical period, however, is rare. An 8-month-old boy underwent surgical repair of tetralogy of Fallot. Neurological disturbances, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia developed 25 days later, and aHUS was diagnosed. Further evaluation revealed that his complement factor H (CFH) level was normal and that anti-FH antibodies were not detected in his plasma. Sequencing of his CFH, complement factor I, membrane cofactor protein, complement factor B, and thrombomodulin genes was normal. His ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin-1 repeats 13) activity was also normal. However, he had a potentially causative mutation (R425C) in complement component C3. Restriction fragment length polymorphism analysis revealed that his father and aunt also had this mutation; however, they had no symptoms of aHUS. We herein report a case of aHUS that developed after cardiovascular surgery and was caused by a complement C3 mutation.

Use of a Transvenous Endocardial Lead as a Shock Cord Placed Subcutaneously in an Infant with Congenital Long QT Syndrome Hiroko Goto1, 3), Naoki Kuwabara1), Kentaro Omoya1), Atsushi Terasawa1), Ai Kojima2), Yusuke Iwata2), Takamasa Takeuchi2), Takashi Kuwahara1) 1)Department of Pediatric Cardiology, 2)Pediatric Cardiac Surgery, 3)Heart Rhythm Management, Gifu Prefectural General Medical Center, Gifu, Japan

This report describes a technique for repairing an aortic stenosis in an elderly patient with a small aortic annulus and a narrow left ventricular outflow tract. Preoperative echocardiography in an 80-year-old woman showed severe aortic stenosis with a narrow outflow tract: the aortic valve area was 0.48 cm(2), the aortic annular diameter was 14 mm, and the left ventricular outflow tract diameter was 14 mm. The Konno procedure was used to enlarge both the small aortic annulus and the left ventricular outflow tract, and a 19-mm Carpentier-Edwards bioprosthetic valve was implanted. The patient's postoperative course was uneventful. The left ventricular mass decreased from a preoperative value of 236 g to 96 g, 3 years after surgery. Only a few reports have described the use of the Konno operation in adult patients. In the present case, the Konno operation was demonstrated to be a good option for aortic stenosis accompanied by a small aortic annulus and a narrow left ventricular outflow tract, even in an elderly patient.

We report a case of successful staged repair of tricuspid atresia with dysplasia of the right ventricular myocardium and absence of the pulmonary valve. The patient underwent the modified Blalock-Taussig shunt operation at 1 month the bidirectional Glenn procedure at 11 months, and the total cavopulmonary connection procedure using extracardiac graft at 2 years. It is important to prevent the left ventricular outflow tract obstruction (LVOTO) caused by the non-functioning right ventricle when the Fontan completion. We were safely able to disconnect the pulmonary trunk from the right ventricle using the transesophageal echocardiography to evaluate the LVOTO during operation.

Background. In conventional conduit operations, longevity has been essentially limited by the inevitable need for conduit replacement. This study was undertaken to compare long-term results of the use of equine pericardial conduits, autologous pericardial conduits, and direct anastomosis repair.Methods. Between 1982 and 2001, 366 patients underwent primary establishment of right ventricle-pulmonary artery continuity at our institution. The mean age at the time of operation was 6.2 years (range, 4 days to 28 years) and mean weight was 17.2 kg (range, 1.6 to 61 kg). Three different repair techniques were used for connection: hand-made valved equine pericardial conduits (n = 179), autologous pericardial conduits (n = 71), and direct anastomosis without a conduit (n = 116). Mean follow-up period for early survivors was 8.6 years in the equine group, 6.1 years in the direct anastomosis group, and 5.1 years in the autologous pericardium group.Results. Direct anastomosis repair (p = 0.0002) was associated with significantly better freedom from late events (conduit replacement or late death) than equine pericardial conduits. The hazard ratio was less with the autologous pericardium conduit than with the equine pericardium, but the difference was not statistically significant (p = 0.2122). Younger age at operation, and postoperative pressure ratio from right to left ventricle were also predictors of conduit longevity.Conclusions. To decrease the probability of late events, direct anastomosis is an encouraging technique compared with traditional equine pericardium extracardiac conduit repair. An autologous pericardial conduit, because of its benefits, would be an alternative when direct anastomosis is not suitable. (C) 2004 by The Society of Thoracic Surgeons.

We report the case of a severely symptomatic neonate with Ebstein's anomaly. A modified Starnes operation was performed, but insufficient drainage of venous blood returning through thebesian veins caused overdistention of the right ventricle and severe left ventricular dysfunction. Urgent reestablishment of right ventricular-right atrial communication successfully resolved these problems.

We present a case of mitral annular aneurysm as a very rare complication developing soon after the surgical relief of subaortic stenosis. The cause of the aneurysm was considered to be the disruption of aorto-mitral intervalvular fibrosa at the initial operation. The surgical repair was successfully performed without compromising either aortic or mitral valvular functions. The preoperative transesophageal echocardiography was useful in delineating the precise anatomic features of this rare complication.