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Competing Interests: Disclosures None.

Objectives: In pulmonary valve replacement (PVR) after tetralogy of Fallot (TOF) repair, the right ventricular end-diastolic and end-systolic volume index (RVEDVI and RVESVI) of cardiac magnetic resonance imaging (cMRI) are often used as indicators of the RV volume. We examined the utility of QRS duration, cardiothoracic ratio (CTR), and plasma brain natriuretic peptide (BNP) as indicators of the appropriate timing of cMRI to assess the RV volume and function before PVR., Methods: We assessed the correlation of QRS duration, CTR, and BNP with RVEDVI and RVESVI on cMRI in 26 patients after TOF repair. Fifteen underwent PVR (age, 45.2 ± 11.4 years). Twelve underwent post-PVR cMRI. The RV volume change from before to after PVR was investigated., Results: QRS duration, BNP, and CTR were positively correlated with RVEDVI and RVESVI after TOF repair. The post-PVR QRS duration was also positively correlated with post-PVR RVEDVI (p = 0.017) and RVESVI (p = 0.001). From before to after PVR, in 5 cases with QRS duration ≤ 160 ms, the QRS duration decreased from 110.4 ± 28.9 to 101.8 ± 30.5 ms (p = 0.063). Both RVEDVI and RVESVI decreased to the normal range in 4 of 5 cases. In contrast, in 7 cases with QRS duration > 160 ms, the QRS duration decreased from 183.0 ± 17.4 to 160.3 ± 23.8 ms (p = 0.013); however, RVESVI did not normalize in 6 of 7 cases., Conclusions: A prolonged QRS duration is a useful marker of RVEDVI and RVESVI enlargement after TOF repair. We recommend performing cMRI before the QRS duration reaches 160 ms due to normalization of the RV volume after PVR., (© 2022. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.)

Histologic evaluations revealed excessive accumulations of macrophages and absence of fibroblastic interstitial cells in explanted bioprosthetic valves. Comprehensive gene and protein expression analysis and histology unveiled an accumulation of fibrinogen and plasminogen, an activator of infiltrated macrophages, from degenerated valve surfaces in the interstitial spaces. These pathologies were completely reproduced in a goat model replaced with an autologous pericardium-derived aortic valve. Further preclinical animal experiments using goats demonstrated that preventing infiltration of macrophages and circulating proteins by increasing collagen density and leaflet strength is an effective treatment option., Competing Interests: This work was supported by Grants-in-Aid for Scientific Research (18K16396 to Dr Namiguchi, 16K10631, and 19H03740 to Dr Izutani, 19K17603 to Dr Hamaguchi, and 19K08585 and 16K109503 to Dr Aono) from the Ministry of Education, Culture, Sports, Science, and Technology of Japan; the Takeda Science Foundation (grants 2016 and 2020 to Dr Sakaue); the Public Trust Cardiovascular Research Foundation (grant 2017 to Dr Sakaue); the SENSHIN Medical Research Foundation (grant 2016 to Drs Aono and 2020 to Dr Sakaue); and the MSD Life Science Foundation, Public Interest Incorporated Foundation (grant 2016 to Dr Aono). Dr Nakayama is an employee of Biotube. There are no financial relationships between Dr Nakayama and the other coauthors, and experimental materials and funding used in this study were not provided by industry. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2023 The Authors.)

We present a case for developing a millikelvin-temperature transmission electron microscope (TEM). We start by reviewing known reasons for such development, then present new possibilities that have been opened up by recent progress in superconducting quantum circuitry, and finally report on our ongoing experimental effort. Specifically, we first review possibilities to observe a quantum mechanically superposed electromagnetic field around a superconducting qubit. This is followed by a new idea on TEM observation of microwave photons in an unusual quantum state in a resonator. We then proceed to review potential applications of these phenomena, which include low dose electron microscopy beyond the standard quantum limit. Finally, anticipated engineering challenges, as well as the authors' current ongoing experimental effort towards building a millikelvin TEM are described. In addition, we provide a brief introduction to superconducting circuitry in the Appendix for the interested reader who is not familiar with the subject., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Objectives: Although primary repair in early infancy has for decades been the prevalent strategy for management of truncus arteriosus (TA), recent concerns about the levels of morbidity and mortality have led to consideration of a staged surgical approach. Our goal was to describe recent patterns of management, to characterize patients who underwent primary or staged repair and to evaluate risk factors associated with operative mortality in a contemporary multicentre cohort., Methods: In the Japanese Cardiovascular Surgery Database, we identified all cases of TA undergoing an initial surgical procedure from 2008 to 2018. Operative mortality was defined as death within 30 days of an operation or in-hospital death regardless of the length of hospital stay. The hospital volume was defined by the average volume of TA repairs per year., Results: The total number of patients undergoing initial surgery for TA was 286. Sixty-eight (24%, 68/286) underwent primary repair (primary repair group). The remaining 218 (76%, 218/286) underwent initial bilateral pulmonary artery banding as part of a planned staged approach (staged repair group). One hundred sixty-two patients out of 218 initially banded patients underwent the repair of TA during this study period. Concomitant diagnoses in the entire cohort included interrupted aortic arch repair in 36 patients and truncal valve regurgitation in 32. No centres handling an average of ≥2 truncus cases/year of the repair of TA were identified in this cohort. A total of 30% (85/286) of the cases were performed at centres that handled an average of ≥1 and <2 cases/year. The remaining 70% were at centres with <1 case/year. Overall, 37 patients (12.9%; 37/286) died. The operative mortality rates in the primary and staged repair groups were similar: that for the primary repair group was 16.2% (11/68) versus 11.9% for the staged repair group (26/218; P = 0.41). With multivariable logistic regression analysis, the factors most strongly associated with operative mortality were preoperative heart failure requiring catecholamine support (odds ratio, 4.18; 95% confidence interval 1.96-8.96) and the repeat bilateral pulmonary artery banding (odds ratio, 3.89; 95% confidence interval 1.08-14.07)., Conclusions: The staged repair of TA has emerged as the preferred option for surgical timing at most of the centres participating in the Japanese Cardiovascular Surgery Database. The management outcomes of the patients with TA were favourable, even for the patients at low-volume centres., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

A 47-year-old woman with a history of mitral valve replacement (MVR) through a median sternotomy was admitted to our hospital due to dyspnea on exertion. Echocardiography showed bioprosthetic valve dysfunction with mitral stenosis. Right heart catheter examination revealed severe pulmonary hypertension and right ventricular dysfunction. We considered that she could not tolerate the hemodynamic changes during induction of general anesthesia without any cardiopulmonary support. Therefore, the percutaneous cardiopulmonary support was started before induction of anesthesia. To avoid the risk of injury to cardiac structures, we performed redo mitral valve replacement via right mini-horacotomy in the 4th intercostal space. Severe calcification was found in the leaflets of the prosthetic valve. She was discharged home on postoperative day 42.

Background: Postoperative pericardial adhesions are considered a risk factor for redo cardiac surgery. Several large- and medium-size animal models of pericardial adhesions have been reported, but small animal models for investigating the development of anti-adhesion materials and molecular mechanisms of this condition are lacking. In this study, we aimed to establish a simple mouse model of pericardial adhesions to address this gap., Methods: We administered blood, minocycline, picibanil, and talc into the murine pericardial cavity via one-shot injection. Micro-computed tomography analyses of contrast agent-injected mice were carried out for methodological evaluation. We investigated various dosages and treatment durations for molecules identified to be inducers of pericardial adhesion. The adhesive grade was quantified by scoring the strength and volume of adhesion tissues at sacrificed time points. Histological staining with hematoxylin and eosin and Masson's trichrome, and immunostaining for F4/80 or αSMA was performed to investigate the structural features of pericardial adhesions, and pathological features of the pericardial adhesion tissue were compared with human clinical specimens., Results: Administration of talc resulted in the most extensive pericardial adhesions. Micro-computed tomography imaging data confirmed that accurate injection into the pericardial cavity was achieved. We found the optimal condition for the formation of strong pericardial adhesions to be injection of 2.5 mg/g talc for 2 weeks. Furthermore, histological analysis showed that talc administration led to an invasion of myofibroblasts and macrophages in the pericardial cavity and epicardium, consistent with pathological findings in patients with left ventricular assistive devices., Conclusions: We successfully established a simple mouse model of talc-induced pericardial adhesions, which mimics human pathology and could contribute to solving the clinical issues related to pericardial adhesions.

Calcification of bioprosthetic valves (BVs) implanted in aortic position can result in gradual deterioration and necessitate aortic valve replacement. The molecular mechanism of calcium deposition on BV leaflets has been investigated, but remains to be fully elucidated. The present study aimed to identify explanted bioprosthetic valve (eBV)-specific proteins using a proteomics approach and to unveil their biochemical and histological involvements in calcium deposition on BV leaflets. Calcification, fibrosis, and glycosylation of the valves were histologically assessed using Von Kossa, Masson's Trichrome and Alcian Blue staining, as well as immunostaining. Protein expression in the explanted biological valves was analysed using proteomics and western blotting. In a histological evaluation, αSMA-positive myofibroblasts were not observed in eBV, whereas severe fibrosis occurred around calcified areas. SDS-PAGE revealed three major bands with considerably increased intensity in BV leaflets that were identified as plasminogen and fibrinogen gamma chain (100 kDa), and fibrinogen beta chain (50 and 37 kDa) by mass analysis. Immunohistochemistry showed that fibrinogen β-chain was distributed throughout the valve tissue. On the contrary, plasminogen was strongly stained in CD68-positive macrophages, as evidenced by immunofluorescence. The results suggest that two important blood coagulation-related proteins, plasminogen and fibrinogen, might affect the progression of BV degeneration., Competing Interests: Competing interestsThe authors declare no competing or financial interests., (© 2018. Published by The Company of Biologists Ltd.)

Background: Congenital long QT syndrome (LQTS) can cause ventricular arrhythmic events with syncope and sudden death resulting from malignant torsades de pointes (TdP) followed by ventricular fibrillations (VFs). However, the syndrome is often overlooked prior to the development of arrhythmic events in patients with congenital heart diseases demonstrating right bundle branch block on electrocardiogram (ECG). We present a case of an adult patient with congenital heart disease who developed VFs postoperatively, potentially due to his mutation in a LQTS related gene, which was not identified on preoperative assessment due to incomplete evaluation of his family history., Case Presentation: A 64-year-old man was diagnosed as having multiple atrial septal defects. He presented with no symptoms of heart failure. His preoperative ECG showed complete right bundle branch block (CRBBB) with a corrected QT interval time of 478 ms. He underwent open-heart surgery to close the defects through median sternotomy access. Three hours after the operation, he developed multiple events of TdP and VFs in the intensive care unit. Cardiopulmonary resuscitation and multiple cardioversions were attempted for his repetitive TdP and VFs. He eventually reverted to sinus rhythm, and intravenous beta-blocker was administered to maintain the sinus rhythm. After this event, his family history was reviewed, and it was confirmed that his daughter and grandson had a medical history of arrhythmia. A genetic test confirmed that he had a missense mutation in CACNA1C, p.K1580 T, which is the cause for type 8., Conclusions: This case highlights the importance of paying attention to other ECG findings in patients with CRBBB, which can mask prolonged QT intervals.

Aortic coarctation rarely occurs after an arterial switch operation for D-transposition of the great arteries with intact ventricular septum. We report the case of a neonate patient in whom aortic coarctation developed 28 days after an uncomplicated arterial switch operation. Preoperatively, the aorta was noted to have an irregular shape, but there was no pressure gradient across the lesion. The patient underwent successful reoperation to correct the coarctation. We hope that our report raises awareness of a rare early complication after arterial switch operation with intact ventricular septum, and the need to carefully monitor the aortic isthmus in patients who have aortic irregularities, even in the absence of a pressure gradient.

Collateral vascular arteries from the descending aorta to the pulmonary arteries are uncommon after arterial switch operation. Here, we report the case of a baby girl treated with coil embolization for abnormal blood flow from the descending aorta to the pulmonary arteries after arterial switch operation. A baby girl weighing 1324 g was delivered at 32 weeks 4 days of gestation, and she had D-transposition of the great arteries and a ventricular septal defect. She underwent nitrogen inhalation to reduce pulmonary blood flow before arterial switch operation. After the operation, she presented with left heart failure due to the presence of abnormal blood flow from the descending aorta to the pulmonary arteries, and she was successfully treated with coil embolization. After the treatment, her condition improved dramatically, and she was discharged without any complications.

Objective: To evaluate the relationship between preoperative severity of coronary stenosis occurring with fractional flow reserve (FFR), and the intraoperative bypass graft flow pattern., Methods: In all, 72 patients were enrolled in this retrospective study. The FFR value of the left anterior descending artery was evaluated, and data on "in situ" bypass grafting from the internal thoracic artery to the left anterior descending artery were assessed. Patients were divided into 3 groups according to preoperative FFR values (Group S: FFR < 0.70; group M: 0.70 ≤ FFR < 0.75; and group N: FFR ≥ 0.75)., Results: In groups S, M, and N, respectively, mean graft flow was 24.7 ± 10.6 mL/minute, 19.2 ± 14.0 mL/minute, and 16.0 ± 9.7 mL mL/minute; pulsatility index was 2.35 ± 0.6, 3.02 ± 1.1, and 5.51 ± 8.20; and number of patients with systolic reverse flow was 3 (6.8%), 5 (35.7%), and 4 (28.6%). Significant differences were observed in graft flow (P = .009), pulsatility index (P = .038), and proportion of systolic reverse flow (P = .023) among the 3 groups. In all patients, graft patency was confirmed with intraoperative fluorescence imaging; postoperative graft patency was confirmed with multislice computed tomography or coronary angiography in 69 patients (follow-up interval: 213 days). Early graft failure occurred in 1 patient., Conclusions: As coronary stenosis severity increased, graft flow increased, pulsatility index decreased, and proportion of patients with systolic reverse flow increased. In mild coronary artery stenosis, the chance of flow competition between the native coronary artery and the bypass graft increased., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Although the outcomes of total arch replacement have been improving, the prevention of cerebral infarction is still an important consideration in aortic arch surgery. Herein, we reviewed our experience with total arch replacement using antegrade selective cerebral perfusion under right axillary artery perfusion. Between January 2002 and March 2013, total arch replacement was performed for 125 patients including 31 patients with acute aortic dissection. An 8 mm polyester grafts was sutured to the axillary artery through the right subclavicular incision and was connected to cardiopulmonary bypass circuit. Antegrade selective cerebral perfusion under hypothermic circulatory arrest was initiated using right axillary artery perfusion. Postoperative cerebral infarction occurred in 5.6% of patients. Hospital mortality rate was 3.2%. The 5-year rate of freedom from aortic event was 83%. The 5-year survival rate was 75%. We consider that right axillary artery perfusion is advantageous for preventing cerebral infarction in total arch replacement.

We report a successful staged repair for a quite rare combination of truncus arteriosus (TA), Van Praagh type A4, and abnormal origin of the left coronary artery (CA). Furthermore, the case was complicated by a variant of the chromosomal anomaly in cat-cry syndrome. The presence of interruption of the aortic arch (IAA) and abnormal CA origin has been previously reported to increase mortality. To decrease the risk of bronchomalacia in infants, bilateral pulmonary artery banding (PAB) was performed as the first stage procedure for adjusting the pulmonary flow. Staged repair is a useful strategy for infants with complex TA.

Embolization of collateral veins is often treated with rigid coils (Gianturco and interlocking detachable coils type). However, when dealing with tortuous and dilated collateral veins, there is a high risk for technical failure and coil migration due to inflexibility of the coils. To safely and successfully solve this problem, Guglielmi detachable coils (GDC) can be used for embolization. Their flexibility allows for easy navigation in tortuous veins, low risk of unintended coil release or coil migration, and safe deployment. A 12-year-old girl with a single ventricle had severe cyanosis and a low exercise tolerance 5 years after Fontan procedure. The symptoms were caused by a tortuous and dilated collateral from the left phrenic vein into the left pulmonary vein, forming a right-to-left shunt. The collateral, which had a large diameter and high flow, and therefore a high risk of coil migration, was successfully embolized with 8 GDC. There were no complications such as coil migration or cerebral infarction. Transcatheter embolization increased her systemic oxygen saturation from 81%-84% to 94%-95%, and increased her ability to exercise. The embolization procedure using flexible GDC was low risk compared with other rigid coil embolization techniques when performing embolization of tortuous and dilated collateral veins.

A 75-year-old man with gastric cancer underwent preoperative cardiac examination by echocardiography, and an unruptured extracardiac aneurysm was detected in the right sinus of Valsalva. Coronary angiography by multidetector computed tomography demonstrated a single left coronary artery. Patch closure of the orifice of the aneurysm of the right sinus of Valsalva was successfully performed for this extremely rare combination., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Operative stress of cardiovascular surgery can alter the blood levels of various physiologically active substances (e.g., cytokines, growth factors), and thus potentially affect cancer cell proliferation. How the combination of changes in blood levels of these substances affects cancer cells has not been adequately addressed. We investigated the stimulatory capacity on cancer cells of serum from patients after cardiovascular surgery, using a novel in vitro assay method., Methods: The subjects were 22 patients undergoing cardiovascular surgery, consisting of 11 off-pump and 11 on-pump procedures. Blood was sampled from each subject immediately before surgery, immediately after surgery, and after transfer to the intensive care unit. Human lung cancer cells were exposed to the serum of each blood sample from each patient, and an MTT assay was conducted to evaluate cell proliferation., Results: Serum samples of all patients showed an inhibitory effect for lung cancer cell proliferation. This inhibitory effect was lower in postoperative serum compared with serum samples before surgery. As a result, lung cancer cell proliferation was better with postoperative serum samples than preoperative serum samples. The proliferation rate after surgery, when it was compared with preoperative serum, was significantly higher in patients with on-pump procedures than in patients with off-pump procedures., Conclusion: The results of this study suggest that the operative stress of cardiovascular surgery induces changes in serum to make it less inhibitory for the cancer cell proliferation. This phenomenon is greater in patients with extracorporeal circulation.

Anomalous subaortic left brachiocephalic vein (ASLBV) is a rare systemic venous anomaly. We review our experience with patients associated with ASLBV who underwent cardiac surgery at three institutions. From 1989 to 2009, the medical records of surgically treated patients with ASLBV were analyzed; the incidence of ASLBV, clinical characteristics, and associated anatomical findings were assessed. Fifteen patients had ASLBV. All ASLBVs coursed left lateral to the aortic arch, passed under the ascending aorta anterior to the central pulmonary artery, and joined the right brachiocephalic vein. Fourteen patients had congenital heart disease (CHD), and the remaining patient did not have cardiac anomalies. Its incidence was 0.57% (14 of 2,449) in patients with CHD and only 0.02% (1 of 4,805) in patients without CHD. In patients with CHD, 73.3% (11 of 15) of the patients had conotruncal cardiac anomalies such as tetralogy of Fallot, ventricular septal defect with pulmonary atresia, truncus arteriosus, and interruption of the aortic arch. Eight patients had aortic arch anomalies, including right aortic arch and cervical aortic arch. The deletion of chromosomal 22q11.2 was confirmed in two patients, and one patient was diagnosed with DiGeorge syndrome. ASLBV was clinically silent even without any surgical intervention. ASLBV is a very rare anomaly and is highly associated with conotruncal cardiac anomalies and aortic arch anomalies, including right aortic arch and cervical aortic arch. Preoperative diagnosis is important when any surgical interventions are intended, especially, in patients with conotruncal cardiac anomalies.

Background: Hyperacute xenograft rejection (HXR) is characterized by complement activation and intravascular thrombosis. The pathogenesis of HXR is attributed to antibodies binding to α-Gal-epitopes on the endothelial cells (EC) of the xenograft, activating complement and thrombin-mediated coagulation mechanisms. Our aim was to evaluate the influence of thrombin inhibition upon HXR and tissue integrity in an ex-vivo working heart model., Material/methods: Eighteen isolated porcine hearts were perfused with human whole blood in a working heart model. The blood was treated with heparin (n=9) in group G-I and with heparin and additionally recombinant hirudin (0.012 mg/ml bolus, afterwards 4.5 µg/ml/h continuously) in group G-II (n=9). The experiments were terminated at end of cardiac output. Histological analysis was performed after the experiments., Results: Working heart time of G-II was significantly longer (712.0±37.8 vs. 125.0±31.4 min, p<0.01). Heart weight increase in G-II was lower (0.05±0.01 vs. 0.30±0.06%/min, p<0.01). Stroke work index and specific coronary flow improved significantly in G-II after 120 minutes. Histological analysis revealed increased tissue damage and thrombosis phenomena in G-I. Moreover, immunohistochemistry showed increased C3 and C5b-C9 upon EC of G-I., Conclusions: Direct thrombin inhibition with Hirudin could be a successful strategy in primate xenotransplantation experiments to prevent tissue damage thus improving the graft survival.

The Ionescu-Shiley pericardial valve (Shiley, Inc, Irvine, CA, USA) is a first generation bioprosthesis made from bovine pericardium. Despite its excellent hemodynamic performance, use of this prosthesis ceased because it had an unacceptably high rate of early structural deteriorations, especially in the era of the standard profile valve. We experienced a rare case of very long durability of an Ionescu-Shiley standard profile (ISSP) bioprosthesis.

Between August 1999 and December 2007, 72 consecutive patients with single ventricle physiology underwent a modified Fontan procedure after a bidirectional Glenn shunt using an extracardiac polytetrafluoroethylene conduit without fenestration. Nitric oxide gas inhalation was commenced just after cardiopulmonary bypass together with intravenous phosphodiesterase III inhibitor administration. After oral intake was started, pulmonary vascular dilators such as beraprost, sildenafil, bosentan were given orally according to amount of chest drainage and patient's condition. After discharge, oxygen therapy at home was continued for three months. No hospital death occurred after surgery. All patients were followed by our institute and follow-up period was 44.2+/-26 (36-106.8) months. One late death occurred during this follow-up period after re-operation. Cardiac catheterization after the Fontan completion showed transpulmonary gradient of 5.9+/-2.4 mmHg, systemic output of 3.4+/-2.1 l/min m2. Arterial oxygen saturation (SaO(2)) at the latest outpatient visit was 94.4+/-3.8%. According to our clinical experience with two-staged total cavopulmonary connection using an extracardiac conduit without fenestration, fenestration in the Fontan circuit is not necessary when performing the Fontan completion. Two-staged extracardiac total cavopulmonary connection without fenestration can be satisfactorily completed with the aid of pulmonary vasodilation therapy.

We report an extremely rare case of pulmonary artery dissection caused by an abrupt rupture of an aortic arch aneurysm into the pulmonary artery. An asymptomatic 80-year-old man was admitted to our hospital for elective surgical repair of aortic arch aneurysm. After admission, sudden onset of hoarseness and dyspnea developed. Echocardiography demonstrated an intimal flap in the pulmonary artery and abnormal shunt flow from aortic arch aneurysm into the pulmonary artery. At surgery, the pulmonary artery dissection involved the main pulmonary artery and both major branches. Total arch replacement and pulmonary artery reconstruction were successfully performed.

The feature of perioperative care for congenital heart surgery in newborn and infant is that the change of environment surrounding the baby should be considered. Especially the baby with anomaly of pulmonary artery, pulmonary vein, relation of the great vessels or patent ductus arteriosus (PDA) dependent heart defects is in a stable condition in the womb. Cardiopulmonary system changes immediately after birth, and symptoms of congenital heart disease will appear. In this part, we describe the pre- and post-operative care in newborn and infant with congenital heart defects.

The symptomatic newborn infant with Ebstein's anomaly is in critical condition and is difficult to treat successfully. Furthermore, supraventricular tachyarrhythmia in patients with Ebstein's anomaly may determine the early and late results. We report a successfully treated case of extracardiac total cavopulmonary connection with a right-sided maze procedure after a modified Starnes' operation during the neonatal period.

A median sternotomy would be very difficult for Cantrell syndrome with severe ectopia cordis. For Cantrell syndrome and tricuspid atresia after left modified Blalock-Taussig shunt with severe ectopia cordis, defect in the middle and inferior portion of the sternum, and the closing of ventriculo-peritoneal shunt, we performed extracardiac total cavopulmonary connection through a right lateral thoracotomy after establishing right modified Blalock-Taussig shunt and performing coil embolization of left modified Blalock-Taussig shunt by cardiologists.

A one-month-old girl with a diagnosis of situs solitus, d-transposition of the great arteries, coarctation of the aorta, and right aortic arch underwent a successful rapid two-stage repair. The combined anatomy of coarctation and right aortic arch is relatively rare, and even rarer in combination with transposition of the great arteries. We report the first successful surgical intervention to correct these anomalies.

A 2.8 kg-female newborn diagnosed as hypoplastic left heart syndrome was transferred to our hospital after resuscitation against systemic circulatory failure due to closure of the ductus arteriosus. Her systemic circulation was stabilized after alprostadil (lipo-PGE1) and inotorpic agent infusion along with mechanical ventilation support. On admission, signs of a pulmonary high-flow and low systemic perfusion were evident. Impaired coagulation aspect was gradually noted. Because a surgical palliation with cardiopulmonary bypass was considered to be at high-risk, a bilateral pulmonary artery banding was indicated. Through a median sternotomy, the right and left pulmonary arteries were individually banded. The patient was continued on the same ventilation strategy. Cardiac, hepatic and renal dysfunctions were improved over next few days and the patient weaned from ventilatory support. She underwent combined Norwood stage I and II repair at 4 months of age with weight of 4.8 kg. Postoperative course has been uneventful and the patient is now followed up in preparation for Fontan operation.

We performed concomitant pulmonary artery angioplasty and the Blalock-Taussig shunt under median sternotomy and cardiopulmonary bypass at the neonatal stage in Fontan candidates with pulmonary coarctation to obtain optimum pulmonary circulation. This surgical strategy realized appropriate early growth of the pulmonary artery necessary for the Fontan operation.

Between January 1980 and December 1994, seventeen premature infants weighing less than 2500 g underwent surgical management for the isolated patent ductus arteriosus (PDA). Indometacine therapy for closure of PDA was not effective for all these patients. In terms of the body weight at birth, they were divided into two groups; Group I (G-I) consisted of ten patients with birth weight less than 1000 g and Group II (G-II) of seven patients weighing more than 1000 g. The age at operation was 22.1 +/- 15.4 days in the G-I and 14.3 +/- 11.4 days in the G-II. The ductus was ligated in all patients but one of the G-II, in whom it was divided. There were five (50%) hospital deaths in the G-I and none in the G-II. The causes of death in the G-I were related to necrotizing enterocolitis (NEC) in two and infant respiratory distress syndrome (IRDS), acute renal failure, and broncho-esophagial fistula in each. The age at operation tended to be older in nonsurvivors rather than in survivors in the G-I (28.0 +/- 16.8 days vs. 16.2 +/- 14.0 days, respectively, but the difference did not reach significance). Before surgery, all patients in the G-I required mechanical ventilator support and nine of them were associated with IRDS. In contrast, only two patients in the G-II needed mechanical ventilation preoperatively. The postoperative period of intubation was also significantly longer in the G-I than in the G-II (51.6 +/- 35.2 days vs. 2.2 +/- 1.5 days, respectively, p < 0.05). In conclusions, the surgery for PDA can be safely performed even in small premature babies weighing less than 1000 g. When medical therapy for PDA is not effective in the premature patients, the surgical management should be considered as early as possible before their conditions become worse due to such critical complications as NEC, IRDS, and renal failure.

We report a two-year-old girl with asplenia, [A, L, L] DORV, pulmonary atresia, common AV valve, PDA, and TAPVC, who successfully underwent total cavo pulmonary connection (TCPC). Deep cyanosis was pointed out since birth. Cardiac catheterization performed on the sixth day after birth revealed a diminutive pulmonary artery tree of which PA index was 41 mm2/m2. Left modified Blalock-Taussig shunt was created at 27 days of age. The PA index increased to 282 mm2/mm2, but disparity in diameter between the left and the right pulmonary artery was yielded by PDA subsidence. Therefore additional contralateral B-T shunt was made at one year of age. Follow-up cardiac catheterization at 28 months of age showed well developed pulmonary artery; PA index of 460 mm2/m2, right pulmonary resistance (Rp) of 3.49 units, left Rp of 2.33 units, and estimated total Rp was 1.39. According to study, bidirectional Glenn procedure or TCPC was indicated. Considering necessity of urgent repair of common pulmonary vein obstruction, regurgitation of the common atrio-ventricular valve and pulmonary artery stenosis, TCPC was performed with concomitant repair of the associated lesions. Severe butterfly-figure stenosis of the central PA was augmented by anastomosing both the left SVC and the left-sided atrium. In conclusion, diminutive pulmonary artery could be adequately grown by phase-in Blalock-Taussig shunts. Pulmonary blood flow scintigraphy was thought to be useful for estimation of pulmonary resistance in such cases with different pulmonary resistance between right and left PA.

Primary cardiac angiosarcoma occurs rarely, and surgical resection is often required to relieve symptoms. A 66-year-old man whose presenting symptoms were palpitations and general fatigue is presented. Echocardiography revealed a large tumour occupying most of the right atrium. When superior vena caval (SVC) syndrome developed, surgical resection of the tumour was attempted. The tumour was found to have invaded the inflow of SVC, left atrium, right ventricle and ascending aorta. The bulk of the tumour was resected, requiring reconstruction of the right atrium and caval inflows. Histopathological diagnosis of the tumour was primary angiosarcoma. In the postoperative period, symptoms of SVC syndrome recurred, which were relieved temporarily by balloon angioplasty. After one month, recurrence of symptoms was again managed by catheter dilation. On this occasion a metallic stent was deployed, which prevented further symptoms of SVC syndrome during the eight months before the patient died with generalized metastases.