Catheter Intervention for Flow Regulatory Clips on Palliative Shunts and Conduits in Patients with Congenital Heart Disease.

Catheter intervention (CI) for a Blalock-Taussig shunt (BTS) or a ventricle-to-pulmonary artery conduit (VPC) is often required after a palliative surgery for congenital heart disease. Flow regulatory clips help improve interstage mortality; their use necessitates CIs to prevent cyanosis. To study the CI outcomes in patients who underwent palliative surgery with either BTSs or VPCs with flow regulatory clips. This single-center retrospective study evaluated demographic characteristics and interventional outcomes of 49 consecutive pediatric patients who required CI for BTS (BTS group) or VPC (VPC group) between January 2008 and September 2018. Overall, 34 and 18 procedures were performed in the BTS and VPC groups, respectively. Moreover, 19/32 (59.3%) and 12/17 (70.1%) patients from the BTS and VPC groups had flow regulatory clips, respectively. All clips were unclipped successfully; one patient in each group underwent staged unclipping. A higher proportion of "clipped patients" underwent CI due to desaturation [clipped vs. non-clipped: BTS, 10/20 (50.0%) vs. 3/14 (21.4%), p = 0.092; VPC, 9/13 (69.2%) vs. 1/5 (20.0%), p = 0.060]. Most clipped patients successfully progressed to the next stage [BTS, 19/20 (95.0%); VPC, 12/13 (92.3%)]. Severe adverse events (SAEs) were more frequent in the VPC group than in the BTS group [3/13 (23.1%) vs. 0/20 (0%), p = 0.024]. Two patients developed an atrioventricular block (requiring an atropine infusion), while one died due to pulmonary overcirculation. While the indication of CI was cyanosis for a higher proportion of clipped patients, all clips were unclipped successfully. The incidence of CI-related SAEs was higher in the VPC group than in the BTS group.

Cardiac MRI for Fontan candidates after intrapulmonary-artery septation.

Intrapulmonary-artery septoplasty may be effective for establishing two-lung Fontan circulation in patients with unilateral pulmonary circulation. However, evaluation of the function of each lung by conventional modalities can be challenging in these patients due to differing sources of blood flow to the left and right lungs following intrapulmonary-artery septation. Herein, we report a case in which two-lung Fontan circulation was successfully achieved after using cardiac MRI along with conventional modalities to evaluate pulmonary circulation.

Truncal valve leaflet reconstruction with autologous pericardium in a neonate.

A boy diagnosed with persistent truncus arteriosus and severe truncal valve regurgitation had uncontrollable heart failure. Thus, truncal valve repair, ventricular septal defect closure, and right ventricular outflow tract reconstruction were performed on the second day of life. We report about a neonate with severe truncal valve regurgitation who was successfully treated with autologous pericardial truncal valve leaflet reconstruction.

Partnership Between Japan and the United States for Early Development of Pediatric Medical Devices　- Harmonization By Doing for Children.

BACKGROUND: The Harmonization By Doing (HBD) program was established in 2003 as a partnership among stakeholders of academia, industry and regulatory agencies in Japan and the United States, with a primary focus on streamlining processes of global medical device development for cardiovascular medical devices. While HBD has traditionally focused on development of devices intended to treat conditions prevalent in adults, in 2016, HBD established the "HBD-for-Children" program, which focuses on the development of pediatric devices as the development of medical devices for pediatric use lags behind that of medical devices for adults in both countries.Methods and Results:Activities of the program have included: (1) conducting a survey with industry to better understand the challenges that constrain the development of pediatric medical devices; (2) categorizing pediatric medical devices into five categories based on global availability and exploring concrete solutions for the early application and regulatory approval in both geographies; and (3) facilitating global clinical trials of pediatric medical devices in both countries. CONCLUSIONS: The establishment of the HBD-for-Children program is significant because it represents a global initiative for the introduction of pediatric medical devices for patients in a timely manner. Through the program, academia, industry and regulatory agencies can work together to facilitate innovative pediatric device development from a multi-stakeholder perspective. This activity could also encourage industry partners to pursue the development of pediatric medical devices.

[Joint Cardiovascular Surgeries of Two Hospitals in Shizuoka for Adult Congenital Heart Disease (ACHD) Patients].

BACKGROUND: As for the medical management including surgery for the patients with adult congenital heart disease(ACHD), it may be difficult for most hospitals other than that have both adult/congenital cardiologists/cardiovascular surgeons. Between Shizuoka Prefectural General Hospital and Mt. Fuji Shizuoka Children's Hospital, medical stuff and information have been shared for these 4 years. And joint cardiovascular surgeries have started since 2015 autumn at Shizuoka Prefectural General Hospital. PURPOSE: The contents and the results of these joint operations were evaluated. PATIENTS AND METHODS: Thirteen joint operations were performed and median age at operation was 55 years old( male 3, female 10). The original diagnosis was tetralogy of Fallot/pulmonary atresia with ventricular septal defect 6, ventricular septal defect( VSD)±pulmonary stenosis 4, atrioventicular septal defect/two chamber right ventricle/Ebstein's anomaly 1 ( each). The procedures were pulmonary valve replacement/right ventricle out flow tract reconstruction 7, mitral valve plasty/tricuspid annuloplasty 4, Bentall 2, VSD closure 2 etc.(included multiple choices). RESULTS: There was no early mortality. One late mortality was occurred 17 months after the surgery due to acute myeloid leukemia. General conditions in other patients have been feasible and most of them were followed in Shizuoka Prefectural General Hospital. CONCLUSIONS: The results of the joint operations were feasible in the present study. Our joint project may become more important in the future.

Long-Term Results of Bilateral Pulmonary Artery Banding Versus Primary Norwood Procedure.

Bilateral pulmonary artery banding (PAB) has emerged to be an attractive option as an initial procedure for the treatment of hypoplastic left heart syndrome (HLHS), and some centers report excellent survival. However, its usage is variable among institutions and its true efficacy is unknown due to reporting biases. We aimed to describe the results of bilateral PAB use, preoperative risk factors, and long-term outcomes compared with primary Norwood procedure, using a national database. Infants who underwent bilateral PAB or Norwood procedure as an initial palliation for HLHS between January 2008 and December 2012 listed in the Japan Congenital Cardiovascular Surgery Database (JCCVSD) were included. The total number of patients diagnosed with HLHS was 334. Bilateral PABs were performed for 256 patients and primary Norwood procedures for 78 patients, as an initial procedure. Actuarial 5-year survival was 59.0%. The primary Norwood procedure group had better 5-year survival [75.5%; 95% confidence interval (CI) 63.2-84.1] than the bilateral PAB group (75.5 vs. 54.0%, log-rank p < 0.001). However, the bilateral PAB group had more significant risk factors. When the risk-adjusted outcomes were evaluated, there was no significant difference between the primary Norwood group and the bilateral PAB group (76.4 vs. 78.1%, log-rank p = 0.87) in higher volume institutions. The primary Norwood group had better 5-year survival than the bilateral PAB group, but preoperative risk was higher in the bilateral PAB group. Because outcomes are comparable when performed at higher HLHS volume institutions, proper patient selection is important in achieving good long-term result.

[Perioperative Care for Patients with Hypoplastic Left Heart Syndrome].

Hypoplastic Left Heart Syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. In normal risk cohort, its surgical outcome has been improved dramatically since Dr. William Norwood reported the 1st successful case more than 30 years ago. The introduction of routine bilateral pulmonary artery banding with subsequent Norwood operation during early infantile( we call it as "rapid 2 stage Norwood operation") has brought an earlier hemodynamic stabilization after Norwood operation. However, the outcome of high risk patients, who are complicated with highly restrictive inter-atrial communication or who have significant tricuspid valve regurgitation from the early stage, is unsatisfactory even in current era. The 1st hospitalization with Norwood procedure is the beginning of staged palliations toward good Fontan circulation in the future. Very careful perioperative management is necessary to achieve survival discharge without complications.

Pulmonary haemorrhage due to an aortopulmonary collateral artery after arterial switch.

A neonate with transposition of the great arteries and intact ventricular septum presented without pulmonary over-circulation, and subsequently developed pulmonary haemorrhage after corrective surgery. Postoperative CT revealed an aortopulmonary collateral artery arising from the descending aorta, and we performed successful embolisation on postoperative day 9. Aggressive imaging modalities such as angiography and/or CT imaging with contrast can detect unexpected extra-pulmonary blood supply and guide further management.

[Current Surgical Treatment for Patients with Hypoplastic Left Heart Syndrome, from the Viewpoint of Emergent Operation].

Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. Since the 1st successful surgical repair was reported by Norwood et al. more than 30 years ago, some modifications in surgical maneuver [e. g. introduction of right ventricule (RV)-pulmonary artery (PA) shunt] and improvement of perioperative management have brought better outcome for this patient cohort. Recent years our institute have changed the treatment strategy from primary Norwood operation during neonatal period to bilateral PA banding and subsequent Norwood operation during early infantile (we call it as "rapid 2 stage Norwood operation"). Indeed, the introduction of this new strategy has lead to earlier hemodynamic stabilization after Norwood operation. Although these operations are performed electively for most of HLHS patients, emergent operations are necessary if they have restrictive inter-atrial communication with sever pulmonary congestion, or if their patent ductus arteriosus (PDA) s tend to close in spite of prostagrandine infusion, or if pulmonary over circulation develops so rapidly. Recently more than half of HLHS patients are diagonosed in fetal. The advances in fetal diagnosis allow us to find the patients earlier and to assess the severity of the disease. Most of HLHS patients who required emergent operation just after their birth are diagnosed in fetal. However, their surgical outcome is unsatisfactory so far. Treatment for these HLHS patients has become the next challenge.

The effect of the conduit size on middle-term outcomes in patients with extracardiac total cavopulmonary connection.

OBJECTIVES: The 18- and 16-mm conduits in extracardiac total cavopulmonary connection (eTCPC) were reported to be optimal based on energy loss and flow stagnation at the relatively early phase. However, because the artificial conduit lacks growth potential, we have recently encountered some cases in which the conduit needs to be changed several years after eTCPC. These cases prompted us to reconsider the surgical strategy for eTCPC. METHODS: We reviewed our 20-year single-centre experience with eTCPC patients (n = 256) to compare the 18-mm conduit (n = 195) and 16-mm conduit (n = 61) in terms of mortality and morbidity. RESULTS: The 16-mm conduit was used significantly more frequently in patients whose main chamber was right ventricle (P < 0.001). There was also a significant difference in preoperative inferior vena cava pressure (P = 0.008). There was a significant difference in the actuarial rate of freedom from late-occurring complications, including mortality, between the 2 groups (P = 0.003). There was a significant difference in the actuarial rate of reoperation-free survival (P = 0.042); however, there was no significant difference in resurgical intervention for the conduit (P = 0.333). In multivariate analysis, preoperative inferior vena cava pressure was an independent predictor for late-occurring complications (hazard ratio 1.19; P = 0.026). Conduit size (18 or 16 mm) itself was not an independent predictive factor for late-occurring complications (P = 0.690). CONCLUSIONS: The mid-term clinical outcomes in patients who underwent eTCPC were excellent with low mortality. Preoperative inferior vena cava pressure was the only predictive risk factor for postoperative morbidity, and the 16 mm conduit was not predictive thereof.

Investigation of real-world heparin resistance and anticoagulation management prior to cardiopulmonary bypass: report from a nationwide survey by the Japanese Association for Thoracic Surgery heparin resistance working group.

OBJECTIVE: Heparin resistance is often encountered during cardiopulmonary bypass. Heparin dose and activated clotting time target values for the initiation of cardiopulmonary bypass are not yet universally standardized; further no consensus exists on the management of heparin resistance. This study aimed to investigate the current real-world practice on heparin management and anticoagulant treatment for heparin resistance in Japan. METHODS: A questionnaire survey was conducted at medical institutions nationwide with which The Japanese Society of Extra-Corporeal Technology in Medicine members are affiliated, targeting surgical cases with cardiopulmonary bypass performed from January 2019 through December 2019. RESULTS: Among 69% (230/332) of the participating institutions, the criterion for heparin resistance was defined as "the target activated clotting time value not reached even with an additional dose of heparin administration". Cases of heparin resistance were reported in 89.8% (202/225) of the responded institutions. Of note, 75% (106/141) of the responded institutions reported heparin resistance associated with antithrombin activity ≥ 80%. Antithrombin concentrate was used in 38.4% (238/619 responses) or third dose of heparin in 37.8% (234/619 responses) for advanced heparin resistance treatment. Antithrombin concentrate was found to be effective in resolving heparin resistance in patients having normal, as well as lower antithrombin activity. CONCLUSION: Heparin resistance has occurred in many cardiovascular centers, even among patients with normal antithrombin activities. Interestingly, the administration of antithrombin concentrate resolved heparin resistance, regardless of the baseline antithrombin activity value.

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.

Mitral and tricuspid valve repair using interannular bridging in children.

OBJECTIVES: In children, it is difficult to control mitral valve (MV) or tricuspid valve (TV) regurgitation with conventional procedures alone because complex factors hamper easy improvement of valve coaptation. We investigated interannular bridging in children with MV/TV regurgitation. METHODS: The subjects were 9 patients who underwent interannular bridging to control TV or MV regurgitation between January 2014 and December 2021. We analysed reintervention for the valve, progression of stenosis/regurgitation and valve growth. RESULTS: The TV and MV groups included 4 and 5 patients, respectively. At operation, the median age was 5.8 (1.4-14) years in TV and 3.6 (0.3-7.0) years in MV. The median weight was 13.0 (8.4-41.2) kg in TV and 11.0 (4.8-18.3) kg in MV. The median follow-up periods were 78 (11-94) months for TV and 30 (4-34) months for MV. None of the patients in either group underwent reintervention. Moderate or greater regurgitation recurred in 1 TV patient but subsequently improved to mild regurgitation. Valve stenosis (mean diastolic pressure gradient >10 mmHg) was not detected. The median valve diameter (Z-score) ranged from -1.17 (-3.7 to 0.85) at discharge to -0.59 (-1.2 to 2.01) at the latest follow-up in TV patients. In MV patients, valve diameter changed from 1.14 (-1.68 to 1.46) to 0.72 (-0.23 to 1.36). After bridging, the coaptation height was maintained at the same value over time. CONCLUSIONS: Interannular bridging could be a useful approach for complicated TV/MV regurgitation in children.

Effect of procedural volume on the outcomes of congenital heart surgery in Japan.

OBJECTIVES: The present study developed a new risk model for congenital heart surgery in Japan and determined the relationship between hospital procedural volume and mortality using the developed model. METHODS: We analyzed 47,164 operations performed between 2013 and 2018 registered in the Japan Cardiovascular Surgery Database-Congenital and created a new risk model to predict the 90-day/in-hospital mortality using the Japanese congenital heart surgery mortality categories and patient characteristics. The observed/expected ratios of mortality were compared among 4 groups based on annual hospital procedural volume (group A [5539 procedures performed in 90 hospitals]: ≤50, group B [9322 procedures in 24 hospitals]: 51-100, group C [13,331 procedures in 21 hospitals]: 101-150, group D [18,972 procedures in 15 hospitals]: ≥151). RESULTS: The overall mortality rate was 2.64%. The new risk model using the surgical mortality category, age-weight categories, urgency, and preoperative mechanical ventilation and inotropic use achieved a c-index of 0.81. The observed/expected ratios based on the new risk model were 1.37 (95% confidence interval, 1.18-1.58), 1.21 (1.08-1.33), 1.04 (0.94-1.14), and 0.78 (0.71-0.86) in groups A, B, C, and D, respectively. In the per-procedure analysis, the observed/expected ratios of the Rastelli, coarctation complex repair, and arterial switch procedures in group A were all more than 3.0. CONCLUSIONS: The risk-adjusted mortality rate for low-volume hospitals was high for not only high-risk but also medium-risk procedures. Although the overall mortality rate for congenital heart surgeries is low in Japan, the observed volume-mortality relationship suggests potential for improvement in surgical outcomes.

[Breast deformities caused by the operation for congenital heart disease].

Breast deformities are seen as one of the late postoperative complications in thoracotomy, but there are very few reports in the literature. We investigated causes and treatments in 5 patients who have consulted to our department after operations for congenital heart disease between April 1989 and March 2011. The injured breast bud in the cardiac operation resulted in hypoplastic deformities, and deformities became apparent during puberty with breast growth. These patients required release of the scar contracture to lead to normal breast development, and also have to been treated chest deformity. After stopping at breast growth in adolescence, some augmentations were necessary if bilateral asymmetry had been remained. Breast deformities are very important issue in the viewpoints of patients' quality of life (QOL), though not relating directly to vital prognosis. We'd like to introduce these complications and some choices about breast reconstruction to thoracic surgeons.

Open-sleeve technique: A new approach for aortic valve leaflet reconstruction in small children.

A boy was diagnosed with a unicuspid aortic valve with severe stenosis at birth. Percutaneous balloon aortic valvuloplasty was performed four times; however, he had severe heart and growth failure. Thus, aortic valve repair was performed at age 2 years and 6 months. He weighed 6.6 kg. The aortic valve annulus diameter was 9.8 mm (z value = 0.43). We report on a small toddler with congenital aortic stenosis who was successfully treated with autologous pericardial aortic valve leaflet reconstruction using the open-sleeve technique.

Primary central pulmonary artery plasty for right atrial isomerism with pulmonary coarctation.

OBJECTIVE: Patients with unbalanced pulmonary artery growth resulting from pulmonary coarctation are considered unsuitable candidates for the Fontan procedure. Particularly, patients with right isomerism pose a challenge. We aimed to investigate the use of primary central pulmonary artery plasty at initial palliation in patients with right isomerism. METHODS: We recruited 34 right isomerism patients with pulmonary atresia and pulmonary coarctation who underwent modified Blalock-Taussig shunt with or without primary central pulmonary artery plasty between 1998 and 2014. We classified them into the primary central pulmonary artery plasty (group P) and no primary central pulmonary artery plasty (group N) groups. We retrospectively analyzed reintervention for pulmonary artery after initial palliation, difference in size between the left and right pulmonary arteries, overall survival, success of the Fontan procedure. RESULTS: The group P and group N included 25 and 9 patients, respectively. Five (20%) and six (67%) patients in group P and group N, respectively, required reintervention for pulmonary artery after initial palliation (p = 0.017). No patient underwent reintervention for the pulmonary artery before bidirectional cavopulmonary shunt in group P. There was a significant difference in the bilateral pulmonary artery size balance between the groups before bidirectional cavopulmonary shunt (p = 0.041). The two-lung Fontan procedure was successful in 14 (56%) and 1 (11%) patient in group P and group N. CONCLUSION: Primary central pulmonary artery plasty may contribute toward improving the balance in the size of the PA and preclude the need for reintervention for PA.

The future of Asian Cardiovascular Annals: Goals and quality.

The positional statement of the Asian Cardiovascular and Thoracic Annals is presented.