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.

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.

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.

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.

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.

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.

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.

[Mediastinal Bronchial Artery Aneurysm Resected together with the Descending Aorta under the Partial Extracorporeal Circulation;Report of a Case].

Bronchial artery aneurysm(BAA) is quite rare, but its rupture is often lethal. Once it is found, treatments should be aggressively considered. A 67-year-old woman was diagnosed to have a 26 mm mediastinal BAA on computed tomography (CT) which was performed for screening. CT revealed a very short inflow vessel of the BAA and arteriovenous fistula at the outflow. Considering these features of the aneurysm, endovascular interventions deemed difficult and surgery was carried out. Because of the fragility, the aneurysm was resected together with the descending aorta and the graft replacement was performed under partial extracorporeal circulation. The patient has no untoward event for 1 year postoperatively. Although most recent reports advocate endovascular interventions, we think surgical treatment is a variable option in selected patients. Careful evaluation for each BAA case would be essential to determine the treatment strategy.

[Hybrid Treatment for Aortic Arch Aneurysm Concomitant with Right Subclavian Artery Aneurysm].

A 78-year-old man was hospitalized for aortic arch aneurysm concomitant with right subclavian artery aneurysm. Maximum diameter of each aneurysm was 65 mm and 40 mm, respectively. Both aneurysms clearly needed to be treated. However, simultaneous surgery of total arch replacement (TAR) and right subclavian artery grafting carries both technical difficulty of surgical exposure and considerable risk of bilateral recurrent nerve palsy. Thus, to avoid these serious problems, we chose hybrid treatment. TAR was performed as the 1st procedure, followed by stent graft placement to right subclavian artery aneurysm. At the 1st procedure, an 8 mm graft was anastomosed to right common carotid artery in end to side fashion. This was used for cerebral perfusion, and after that, another end of this graft was anastomosed to a branch of quadrant graft which was anastomosed to brachiocephalic artery. Then, right common carotid artery was ligated at proximal portion to create a proximal landing zone. As the 2nd procedure, excluder leg was deployed via right axillary artery without difficulty. He was discharged with uneventful postoperative course.

Spinal cord protection during thoracoabdominal aortic replacement: spinal cord perfusion maintenance.

OBJECTIVES: Spinal cord protection during thoracoabdominal aortic surgery is challenging for surgeons. We performed thoracoabdominal replacement using a strategy for maintaining spinal cord perfusion pressure. Here, we report our experience with this procedure and the surgical outcomes. METHODS: Between January 2000 and December 2014, 130 patients [male: 91 (74.6%), female: 39 (25.4%); mean age: 66.6 ± 12.8 years] underwent thoracoabdominal replacement using cardiopulmonary bypass at Hiroshima Shimin Hospital, Japan. The surgical outcomes of these patients were analysed. RESULTS: The in-hospital mortality rate of all patients was 2.5%. The incidence of postoperative paraplegia was 3.8%. Aortic event-free survival rates at 1, 3 and 5 years were 98.2%, 93.9% and 80.7%, respectively. CONCLUSIONS: The present study suggests that our strategy for maintaining spinal cord perfusion pressure provides acceptable outcomes.

Outlet strut fracture and leaflet escape of Bjork-Shiley convexo-concave valve.

Prosthetic valve fracture is a serious complication and may arise in patient post-valve replacement. We experienced an outlet strut fracture and leaflet escape of a Bjork-Shiley convexo-concave valve. We performed an emergency redo mitral valve replacement and successfully retrieved the fractured strut and escaped leaflet from superficial femoral artery and the abdominal aorta. The patient showed an uneventful postoperative recovery.

Outcomes of Anterolateral Thoracotomy With or Without Partial Sternotomy for Kommerell Diverticulum.

BACKGROUND: Kommerell diverticulum is a rare aortic arch anomaly. The indications for operative intervention and surgical strategy are still controversial. The standard surgical procedure at our institution is total aortic arch plus descending aortic replacement using anterolateral thoracotomy with partial sternotomy. The aberrant subclavian artery is reconstructed anatomically or extraanatomically. METHODS: From 2002 to 2014, 6 patients (1 woman), aged 55 to 78 years, underwent graft replacement through an anterolateral thoracotomy or anterolateral thoracotomy with a partial sternotomy approach for Kommerell diverticulum. All patients underwent graft replacement of the descending aorta or total aortic arch plus descending aorta in addition to aberrant subclavian artery reconstruction. RESULTS: No hospital deaths or major complications occurred. Recurrent nerve injury developed at discharge in 1 patient. All patients were well during the follow-up period (range, 11 to 116 months). CONCLUSIONS: Kommerell diverticulum and its associated symptoms were surgically treated with acceptable outcomes.

Operative results of the anterolateral thoracotomy with partial sternotomy approach for chronic-type B aortic dissection involving the aortic arch.

Objectives: There are various treatment strategies for chronic-type B aortic dissection involving the aortic arch. Our aim was to review our surgical experience in the anterolateral thoracotomy with the partial sternotomy approach for chronic-type B aortic dissection involving the aortic arch. Methods: From January 2000 to October 2015, 39 patients underwent the single-stage open surgery for chronic-type B aortic dissection involving the aortic arch using the anterolateral thoracotomy with partial sternotomy approach. Results: Among the 39 patients, 32 were men (82.1%; mean age at surgery, 61.3 ± 11.9 years), with a mean dissecting aortic aneurysm diameter of 50.21 ± 12.20 mm; 28 patients (71.8%) had patent false lumens of the descending aorta. The median interval from dissection occurrence until surgery was 34.05 ± 52.34 months. Twenty-one patients underwent descending aortic replacement plus total aortic arch replacement and 18 underwent descending aortic replacement (plus partial aortic arch replacement). Overall in-hospital mortality and postoperative stroke rates were 5.1% (2 patients) and 10.3% (4 patients), respectively. Survival rates at 1, 3 and 5 years were 94.7%, 94.7% and 90.2%, respectively. Aortic event-free rates at 1, 3 and 5 years were 90.9%, 90.9% and 80.2%, respectively. Conclusions: The anterolateral thoracotomy with partial sternotomy approach is a useful surgical procedure with acceptable outcomes for chronic-type B aortic dissection cases involving the aortic arch, when aortic remodelling using thoracic endovascular aortic repair cannot be performed.

[Closure of Ascending Aortic Entry by Debranching Endovascular Management in an Elderly Patient with Acute Stanford Type A Dissection].

An 85-year-old woman was hospitalized by emergency for an acute Stanford type A aortic dissection. Computed tomography showed a primary entry on the ascending aorta and pericardial effusion. Although her hemodynamics was unstable due to cardiac shock, her family wished no open surgery considering her age and frailty. A couple of days later, her condition became stabilized with antihypertensive therapy. Since the dissection was limited within the ascending aorta, closure of the entry with the stentgraft was considered appropriate and much less invasive as compared with an open surgery. After obtaining informed consent with her family, thoracic endovascular aortic repair was performed with Gore C-TAG with 2-debranch. The procedure was completed without complications and the entry closure was confirmed by aortography. Her postoperative course was uneventful. Her physical activity restored to the preoperative level and she was discharged.

Left Ventricular Myxoma Occluding the Suprarenal Abdominal Aorta in an Infant.

Myxoma is the most common primary cardiac tumor in adults; however, it is extremely rare in infants. Acute occlusion of the abdominal aorta by a cardiac myxoma is also rare. We report the case of an infant with acute occlusion of the suprarenal abdominal aorta by a left ventricular myxoma. The patient underwent successful catheter embolectomy of the abdominal aorta and surgical resection of the cardiac myxoma. This is a very rare case report of the combination of infantile left ventricular myxoma and acute occlusion of the abdominal aorta.

Intracoronary autologous cardiac progenitor cell transfer in patients with hypoplastic left heart syndrome: the TICAP prospective phase 1 controlled trial.

RATIONALE: Hypoplastic left heart syndrome (HLHS) remains a lethal congenital cardiac defect. Recent studies have suggested that intracoronary administration of autologous cardiosphere-derived cells (CDCs) may improve ventricular function. OBJECTIVE: The aim of this study was to test whether intracoronary delivery of CDCs is feasible and safe in patients with hypoplastic left heart syndrome. METHODS AND RESULTS: Between January 5, 2011, and January 16, 2012, 14 patients (1.8±1.5 years) were prospectively assigned to receive intracoronary infusion of autologous CDCs 33.4±8.1 days after staged procedures (n=7), followed by 7 controls with standard palliation alone. The primary end point was to assess the safety, and the secondary end point included the preliminary efficacy to verify the right ventricular ejection fraction improvements between baseline and 3 months. Manufacturing and intracoronary delivery of CDCs were feasible, and no serious adverse events were reported within the 18-month follow-up. Patients treated with CDCs showed right ventricular ejection fraction improvement from baseline to 3-month follow-up (46.9%±4.6% to 52.1%±2.4%; P=0.008). Compared with controls at 18 months, cardiac MRI analysis of CDC-treated patients showed a higher right ventricular ejection fraction (31.5%±6.8% versus 40.4%±7.6%; P=0.049), improved somatic growth (P=0.0005), reduced heart failure status (P=0.003), and lower incidence of coil occlusion for collaterals (P=0.007). CONCLUSIONS: Intracoronary infusion of autologous CDCs seems to be feasible and safe in children with hypoplastic left heart syndrome after staged surgery. Large phase 2 trials are warranted to examine the potential effects of cardiac function improvements and the long-term benefits of clinical outcomes. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01273857.

The effect of pulmonary root translocation on the left ventricular outflow tract.

We report the effect of pulmonary root translocation on the left ventricular outflow tract. A double switch operation with pulmonary root translocation was performed in a 6-year-old boy whose diagnosis was dextrocardia, congenitally corrected transposition of the great arteries, ventricular septal defect, and pulmonary stenosis. Postoperative magnetic resonance imaging showed more natural left ventricular outflow than preoperatively (19 mm vs 22 mm in length between the top of the interventricular septum and the aortic valve). This technique does not require coronary transfer and enables preservation of the aortic root structure. The long-term results, including left ventricular outflow tract morphology, should be evaluated.