Long-term outcome and cardiac function after anatomic repair of congenitally corrected transposition.

OBJECTIVES: There is limited information on long-term outcomes and trajectories of ventricular and valvular functions in patients with congenitally corrected transposition of the great arteries after anatomic repair according to the operative strategy with a median follow-up period of more than 10 years. METHODS: Twenty-nine patients who underwent anatomic repair in Okayama University Hospital between January 1994 and December 2020 were reviewed. Outcomes were compared between patients who underwent a double switch operation (DS group) and patients with an atrial switch with a Rastelli operation (Rastelli-Senning/Mustard group). RESULTS: Fifteen (52%) were in the DS group and 14 (48%) were in the Rastelli-Senning/Mustard group. The median follow-up period after anatomic repair was 12.7 (interquartile range 4.2-18.8) years. There were 3 (10%) early deaths and 3 (10%) late deaths. Survival rates for the entire cohort at 10 and 20 years were 86% and 71%, respectively, and were not different between the 2 groups. Using competing risk analysis, risks of heart failure, cardiac rhythm device implantation and atrial arrhythmia showed no significant differences between the 2 groups, whereas risk of reoperation was higher in the Rastelli-Senning/Mustard group than that in the DS group. Four patients after a DS operation and 1 patient after a Rastelli technique developed more than moderate aortic regurgitation. CONCLUSIONS: During a median follow-up period of more than 10 years, mortality rate and ventricular and valvular functions after anatomic repair were acceptable, though the incidences of late complications were relatively high, especially in the Rastelli-Senning/Mustard group.

When to intervene the pulmonary artery: Importance of anatomical assessment in the diagnosis of pulmonary artery coarctation.

OBJECTIVE: Pulmonary artery coarctation (PACoA) is a major problem that increases the frequency of intervention. However, there is little evidence regarding the prediction of PACoA development. METHODS: A retrospective chart review was performed on 42 patients who underwent modified Blalock-Taussig shunt and preoperative contrast-enhanced computed tomography. An uneven PA branching was defined as an abnormal ductus arteriosus connection to the left PA distal to the PA branching on contrast-enhanced computed tomography. RESULTS: Nineteen (45.2%) of 42 patients were diagnosed with PACoA. The median diameters of the ductus on the aorta and PA sides were 4.1 mm and 3.6 mm in the PACoA group and 3.6 mm and 2.9 mm in the non-PACoA group, respectively (P = .07 and .28, respectively). Tortuous ductus was recognized in 7 (36.8%) patients in the PACoA group and 14 (60.8%) patients in the non-PACoA group (P = .12). PACoA was associated with pulmonary atresia (16 patients [84.2%] in the PACoA group and 12 patients [52.1%] in the non-PACoA group) (P = .02). All 19 patients had uneven PA branching in the PACoA group, whereas 5 of 23 (21.7%) patients had uneven PA branching in the non-PACoA group (P < .001). CONCLUSIONS: Uneven PA branching rather than the ductus arteriosus size was strongly associated with PACoA development; therefore, morphologic assessment by contrast-enhanced computed tomography should be considered in patients with pulmonary atresia.

Strategy of delayed repair of total anomalous pulmonary venous connection in right atrial isomerism and functional single ventricle.

Objective: Repair of total anomalous pulmonary venous connection (TAPVC) in neonates with right atrial isomerism and functional single ventricle is challenging. In our novel strategy, primary draining vein stenting (DVS) was applied to patients with preoperative pulmonary vein obstruction to delay TAPVC repair. This study investigated our initial experience with a strategy of delayed TAPVC repair, incorporating DVS. Methods: Twenty-nine patients with right atrial isomerism and functional single ventricle who had a severe obstruction in the course of draining veins, who required surgical or catheter intervention in their neonatal period were retrospectively reviewed (primary DVS: n = 11; primary TAPVC repair: n = 18). Results: Patients in the primary DVS group had more mixed type TAPVC (primary DVS: n = 5, 45.5%; primary TAPVC repair: n = 2, 11.1%; P = .03) and required more systemic to pulmonary shunt surgeries during their lifetime (primary DVS: n = 9, 81.8%; primary TAPVC repair: n = 6, 33.3%; P = .047). Kaplan-Meier analysis showed that primary DVS repair was associated with improved survival compared with primary TAPVC repair (survival rates at 90 days, 1 year, 3 years and 5 years: primary DVS: 100%, 80%, 68.6%, and 54.9%; primary TAPVC repair: 55.6%, 38.9%, 38.9%, and 38.9%, respectively [P = .04]). Of the 4 patients who underwent stenting of the ductus venosus, 3 had elevated liver enzymes after surgical repair of TAPVC due to ductus venosus steal, which markedly improved after coil embolization of the stent. Conclusions: For neonates with obstructive TAPVC and functional single ventricle, our delayed TAPVC repair using primary DVS appeared to improve survival compared with the conventional strategy.

A Ruptured Coronary Artery Aneurysm Secondary to Kawasaki Disease.

Coronary artery aneurysm occurs in 0.3%-0.8% of patients with Kawasaki disease, and cases of rupture are extremely rare. Only 2 cases have been reported in which the patients survived. We report a case of ruptured coronary artery aneurysm that was treated with coronary artery bypass grafting and extracorporeal membrane oxygenation. (Level of Difficulty: Advanced.).

Papillary-Ventricular Complex Tugging for Ischemic/Functional Mitral Regurgitation.

Surgical restoration of subvalvular geometry is very important to prevent recurrence of ischemic/functional mitral regurgitation. We evaluated an approach to the radical repair of left ventricular (LV) remodeling for three different cases with responsible coronary lesions. Leaflet tethering was corrected by tugging of the papillary-ventricular complex, which consists of the base of papillary muscles and posterior LV wall. The main lesion of the postinfarction scar was concomitantly excluded. Restoration of LV remodeling diminished mitral regurgitation with minimal leaflet tethering and improved systolic LV function. This technique may be an aggressive and encouraged approach for patients with ischemic/functional mitral regurgitation.

Staged Repair of Tetralogy of Fallot: A Strategy for Optimizing Clinical and Functional Outcomes.

BACKGROUND: This study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot on pulmonary valve annulus growth, the rate of valve-sparing repair at the time of intracardiac repair, and long-term functional outcomes. METHODS: This retrospective study involved 330 patients with tetralogy of Fallot who underwent intracardiac repair between 1991 and 2019 and included 57 patients (17%) who underwent BTS. The mean follow-up period was 15.0 ± 7.3 years. We compared the data of patients who underwent BTS and patients who did not undergo BTS before intracardiac repair. RESULTS: The median age before BTS was 71 days (range, 28 to 199) and the median body weight was 4.3 kg (range, 3.3 to 6.8 kg). There were no inhospital or interstage deaths after BTS. The pulmonary valve annulus Z scores of patients with BTS revealed significant growth after BTS (from -4.2 ± 1.8 to -3.0 ± 1.7, P < .001). Valve-sparing repair was eventually performed in 207 patients (63%), including 26 (46%) who underwent staged repair. The overall freedom from pulmonary regurgitation-related reintervention was 99.7%, 99.1%, and 95.8% at 1, 5, and 20 years, respectively. CONCLUSIONS: A staged surgical strategy incorporating BTS as the first palliation for symptomatic patients resulted in no mortality. Blalock-Taussig shunt may have contributed to the avoidance of primary transannular patch repair and facilitated pulmonary valve annulus growth; therefore, approximately half of the symptomatic neonates and infants were recruited for valve-sparing repair. Staged repair may have led to functionally reliable delayed transannular patch repair, thereby resulting in fewer surgical reinterventions.

Impact of pulmonary artery coarctation on pulmonary artery growth and definitive repair following modified Blalock-Taussig shunt.

OBJECTIVE: Pulmonary artery coarctation may pose a risk for pulmonary stenosis and subsequent failure to achieve definitive repair. We sought to assess the impact of pulmonary artery coarctation on pulmonary artery growth. METHODS: A retrospective chart review was performed in 130 patients, including 37 single ventricles with a modified Blalock-Taussig shunt as first palliation. Pulmonary artery coarctation was defined as discrete stenosis of the pulmonary artery, with a diameter of less than 3 mm and with the ductus arteriosus connected. Preoperative echocardiography showed pulmonary artery coarctation in 29 patients (22%). Concomitant pulmonary artery plasty was performed in 14 patients with discrete stenosis having a diameter of less than 2 mm. RESULTS: Pre-modified Blalock-Taussig shunt left pulmonary artery z-scores were lower in patients with pulmonary artery coarctation than in those without (-4.0 [-5.8, -2.1] vs -1.7 [-2.6, -0.8], P < .001), and this remained the same even after modified Blalock-Taussig shunt (-2.5 [-5.1, -0.5] vs -0.5 [-2.4, 0.8], P = .010). Concomitant pulmonary artery plasty did not result in catch-up growth of the left pulmonary artery (post-modified Blalock-Taussig shunt left pulmonary artery z-score in patients with pulmonary artery plasty: -3.0 (-6.5, -2.0) versus those without: -1.8 (-3.3, -0.3), P = .279). Definitive repair/Fontan completion was achieved in 111 patients (85%), and this was not affected by the presence of pulmonary artery coarctation. CONCLUSIONS: Pulmonary artery coarctation affected disproportionate pulmonary artery growth throughout the staged repair, but did not result in failure of definitive repair/Fontan completion. Pulmonary artery plasty during the neonatal period did not contribute to catch-up growth of the left pulmonary artery; therefore, surgical indications and timing should be carefully considered.

Exercise-Induced Ischemic ST-Segment Elevation in Anomalous Origin of the Right Coronary Artery From the Left Sinus of Valsalva With an Intramural Course and Blocked Coronary Bypass.

Sudden cardiac events in young athletes are a major concern in the field of sports cardiology. Although coronary artery anomalies remain a major cause of cardiac events in young athletes, only a few cases have been diagnosed prior to critical events. Here, we present the case of a previously asymptomatic young male runner who experienced sudden cardiac arrest at the end of a marathon. The patient immediately received cardiopulmonary resuscitation from a bystander and was transported to an emergency hospital. As his electrocardiogram showed ventricular fibrillation, he was treated with electric shock, and his rhythm was successfully converted to a normal sinus rhythm. Following successful resuscitation, the patient was diagnosed with an anomalous origin of the right coronary artery from the left sinus of Valsalva with an intramural course. The patient underwent coronary artery bypass using the right internal thoracic artery. Fifteen years later, the coronary bypass was found to be blocked, but the patient was asymptomatic. However, an exercise electrocardiogram revealed ST-segment elevation in the inferior leads. The patient then underwent an unroofing procedure. He has remained asymptomatic without complications for two years after the second surgery.

Does the size of pulmonary artery impact on recoarctation of the aorta after the Norwood procedure without patch?

OBJECTIVES: This study aimed to evaluate whether recoarctation of the aorta (reCoA) after the Norwood procedure for hypoplastic left heart syndrome correlates with pre- and postoperative anatomic factors. METHODS: This retrospective study included 48 patients who underwent Norwood procedure with right ventricle-to-pulmonary artery conduit between 2009 and 2017. Anatomical factors such as preoperative length, diameter of the main pulmonary artery (MPA), and postoperative neoaortic arch angle stratified by arch reconstruction technique were analysed using the receiver operating characteristic analysis. RESULTS: Eleven patients needed surgical intervention for reCoA at stage 2. Out of the 30 patients who underwent direct anastomosis during arch reconstruction, 7 developed reCoA. Seven patients received the full patch augmentation (patch augmentation for both lesser and greater curvatures) and were all spared from reCoA. Among the patients who had direct anastomosis, the preoperative MPA length was correlated with the postoperative arch angle (P = 0.021) and was associated with the occurrence of reCoA (P = 0.002) and the best cutoff value for MPA length was 10 mm. The postoperative arch angle was also correlated with the incidence of reCoA (P < 0.001) and was larger in patients who underwent the full patch augmentation than in patients who had direct anastomosis (126° vs 112°, P = 0.005) despite comparable MPA length. CONCLUSIONS: ReCoA after the Norwood procedure correlates with MPA length when a direct anastomosis was used. Direct anastomosis can be considered in patients with a longer preoperative MPA. In other cases, the full patch augmentation should be considered for obtaining a large and smooth neoaortic arch.

Risk factors and outcomes of pediatric extracorporeal membrane oxygenation.

BACKGROUND: Congenital heart disease (CHD) is the most common neonatal and pediatric cardiac indication for extracorporeal membrane oxygenation (ECMO). Risk factors of survival and neurologic complication were different in many centers. We sought to evaluate survival and neurological outcome after ECMO in patients with CHD. METHODS: We retrospectively reviewed the medical records of 37 patients (<16 years old) who received ECMO. Indications for ECMO were failure to wean from cardiopulmonary bypass in 18 patients, extracorporeal cardiopulmonary resuscitation (ECPR) in 13 patients, and others in 6 patients. The median cardiopulmonary resuscitation (CPR) duration in ECPR patients was 48 min (interquartile range: 38-53 min). Neurological outcomes were evaluated using the Pediatric Cerebral Performance Category (PCPC) scale one year after hospital discharge. RESULTS: The median ECMO duration was 160 (91-286) h. Twenty-nine patients (78%) were successfully weaned off ECMO. Overall survival to hospital discharge was 59%. Risk factors of mortality were as follows: ECMO duration >1 week and urine output <1 mL/kg/h in the first 24 h after ECMO induction by multivariable analysis. Of the 22 survivors, 15 (68%) patients had a favorable outcome (PCPC ≤2). Risk factors for unfavorable outcomes (PCPC ≥3) included ECPR as indication and CPR of longer than 40 min. CONCLUSIONS: Longer ECMO duration and lower urine output were associated with increased mortality. Neurologic outcomes were not satisfactory when CPR was required for a longer period before ECMO establishment.

Successful Handmade Monobloc Aortomitral Valve Replacement for Extensive Aortic Root Abscess.

Aortic root abscess is one of the most catastrophic complications of aortic valve endocarditis. Complete débridement is recommended regardless of the kind of infected lesion. A 37-year-old man with extensive aortic root abscess due to prosthetic aortic valve endocarditis was surgically treated. The main lesion was the aortomitral continuity extending to the commissure between the left and right coronary cusps. After débridement, the aortic annulus underneath the left coronary artery was reconstructed using a handmade aortomitral monobloc valve without aortic annuloplasty. This valve was required for the extensive root abscess of the left and noncoronary sinus to achieve complete débridement.

Endocardial Hemangioma of the Right Ventricle: Complete Excision With Right Ventricular Restoration.

Primary cardiac tumor can arise from any location in the right and left cardiac chamber. Complete excision is generally recommended because of uncertainty regarding malignancy; however it is important to minimize the resultant functional deterioration after surgery. We report a case of endocardial hemangioma (4 × 3 × 3 cm) on the free wall of the right ventricle, located between the anterior and posterior papillary muscles. We describe details of the procedure to preserve the right ventricular volume and competence of the tricuspid valve.

Anatomic Risk Factors for Reintervention After Arterial Switch Operation for Taussig-Bing Anomaly.

BACKGROUND: This study aimed to determine the factors related to reintervention, especially for pulmonary stenosis (PS), in patients with Taussig-Bing anomaly (TBA) after the arterial switch operation. METHODS: This retrospective study included 34 patients with TBA who underwent the arterial switch operation between 1993 and 2018. Preoperative anatomic and physiologic differences and long-term outcomes were determined using a case-matched control with transposition of the great arteries with ventricular septal defect and TBA with an anterior and rightward aorta. RESULTS: At arterial switch operation, median age was 43 days (range, 16 to 102) and median body weight was 3.6 kg (range, 2.8 to 3.8 kg). Aortic arch obstruction and coronary anomalies were present in 64% and 41% of patients, respectively. The hospital mortality rate was 11%, including one cardiac death, and the late mortality rate was 2.9%. Furthermore, 41% patients underwent 26 reinterventions for PS. Patients undergoing PS-related reintervention had a significantly larger native pulmonary artery to aortic annulus size ratio than patients not receiving reintervention (1.69 vs 1.41, P = .02). This ratio was the only predictor of PS-related reintervention; it was significantly higher in the TBA group than in the transposition of great arteries/ventricular septal defect group. The PS-related reintervention was required more in the TBA group than in the transposition of great arteries/ventricular septal defect group. CONCLUSIONS: Regardless of complex coronary anatomy and associated anomalies, early and late survival were acceptable. Postoperative PS was strongly associated with having a larger native pulmonary valve, suggesting that an optimal surgical reconstruction was required for achieving an appropriate aortopulmonary anatomic relationship during the arterial switch operation.

Norwood procedure with right ventricle to pulmonary artery conduit: a single-centre 20-year experience.

OBJECTIVES: The aim of this study was to evaluate the long-term outcomes of the Norwood procedure with right ventricle-pulmonary artery (RV-PA) conduit for hypoplastic left heart complex. METHODS: A retrospective observational study was performed in 136 patients with hypoplastic left heart complex who underwent a Norwood procedure with RV-PA conduit between 1998 and 2017. The probabilities of survival, reintervention and Fontan completion were analysed. RESULTS: Stage 1 survival was 91.9% (125/136). Reintervention for PA stenosis was needed for 22% and 30% at stages 2 and 3, respectively, while 15% underwent reintervention for aortic arch recoarctation. Among 106 bidirectional Glenn survivors, 93 (68% of the total number of patients) had a Fontan completion, while 4 were not considered to be Fontan candidates. Risk factors for overall mortality included weighing <2.5 kg at the time of the Norwood procedure, intact atrium septum, total anomalous pulmonary vein connection and more than mild atrioventricular regurgitation at the time of the Norwood procedure. Overall survival was 80.9%, 72.3% and 62.8% at 1, 5 and 20 years, respectively. CONCLUSIONS: Probabilities of survival and Fontan completion were acceptable under the current surgical strategy incorporating RV-PA Norwood procedure as the first palliation. Incorporating a strategy to maintain PA growth and ventricular function through the staged repair is of prime importance. Further studies are necessary to observe changes in atrioventricular regurgitation as well as in right ventricular function, in patients who require atrioventricular valve interventions during the staged Fontan completion.

Clinical impact of transcatheter atrial septal defect closure on new onset atrial fibrillation in adult patients: Comparison with surgical closure.

BACKGROUND: Previous studies demonstrated that the incidence of atrial fibrillation (AF) was significantly increased when patients aged ≥40 years had surgical atrial septal defect (ASD) closure (sASD). However, limited information is available on such findings in transcatheter ASD closure (tASD). The purpose of this study was to investigate the incidence of newly developed AF after tASD in patients aged ≥40 years in whom preoperative AF or atrial flutter (AFL) had not been detected and to compare with the incidence after sASD. METHODS: The medical records of patients aged ≥40 years without a history of AF or AFL who underwent tASD (n = 281) or sASD (n = 24) were reviewed. Patients who had catheter ablation before the ASD closure were excluded. Patients with a patent foramen ovale were also excluded. The incidence of newly developed AF after ASD closure and the risk factors for that were evaluated statistically. RESULTS: Eleven patients had newly developed AF (5 in tASD and 6 in sASD) postoperatively including 6 persistent AF (3 each after tASD and sASD). The cumulative incidence of newly developed AF was 0.7% in tASD and 16.7% in sASD, and 2.7% and 20.8% at 1 and 5 years, respectively (p < 0.001). ASD diameter ≥30 mm and sASD were potential risk factors for newly developed AF after ASD closure and postoperative persistent AF. CONCLUSIONS: In patients aged ≥40 years without a history of AF or AFL, the incidence of newly developed AF after tASD closure was lower than that after sASD. A large ASD more than 30 mm diameter was a potential risk factor for development of AF even if it is closed by transcatheter procedure. Further long-term evaluation after tASD is required to clarify preventive benefit for new onset AF in adult ASD population.

The impact of pulmonary artery banding and cardiac resynchronization therapy for the adult patient with congenitally corrected transposition of the great arteries.

It has been controversial how to manage severe dysfunction of the systemic right ventricle and severe tricuspid regurgitation for adult patients with congenitally corrected transposition of the great arteries. We reported a 43-year-old man with these disorders, who received pulmonary artery banding and cardiac resynchronization therapy without cardiopulmonary bypass. The degree of the tricuspid regurgitation was improved to mild-moderate and systemic right ventricular ejection fraction was improved from 28 to 45% after surgery. Cardiopulmonary exercise testing showed that the predictive value of anaerobic threshold and peak oxygen uptake improved from 65 to 99% and 59 to 92%, respectively. In conclusion, pulmonary artery banding and cardiac resynchronization therapy can be the first choice of surgery for severe tricuspid regurgitation with severely impaired systemic right ventricular function. After improving systemic right ventricular dysfunction tricuspid surgery could be the next choice as a surgical intervention in the future.

[Subdural Hematoma after Open Heart Surgery for Infective Endocarditis Presenting with Pre-operative Intracranial Hemorrhage Infarction;Report of a Case].

A 47-year-old woman with a history of radiation enteritis and implantation of a central venous port was admitted to our intensive care unit(ICU) suffering from high fever. She was diagnosed with active infective endocarditis due to catheter-related blood stream infection. Although echocardiography showed a large vegetation on the mitral valve, surgical therapy was postponed for 5 weeks because of intracranial hemorrhage infarction. On the 3rd day after mitral valve repair, she developed consciousness disturbance and computed tomography(CT) revealed acute subdural hematoma of the posterior cranial fossa. Fortunately, she fully recovered from the neurological complication without surgical intervention.